Chapter 16: Multiple Sclerosis

Multiple sclerosis (MS) is an autoimmune disease characterized by inflammation, selective demyelination, and gliosis. It causes both acute and chronic symptoms and can result in significant disability and impaired quality of life. MS affects approximately 400,000 persons in the United States; worldwide MS affects approximately 2.1 million people.¹ It was first defined by Dr. Jean Charcot in 1868 by its clinical and pathological characteristics: paralysis and the cardinal symptoms of intention tremor, scanning speech, and nystagmus, later termed Charcot's triad. Using autopsy studies he identified areas of hardened plaques and termed the disease sclerosis in plaques.²

The onset of MS typically occurs between ages 20 and 40 years. MS is rare in children, as is the onset of symptoms in adults older than age 50 years. The disease is more common in woman than in men by a ratio of 2:1 to 3:1. Although the incidence and prevalence of MS overall have increased over the last 5 decades, this increase appears to be mostly related to an increased prevalence in women.³ There are also ethnic differences. MS affects predominantly white populations; African Americans demonstrate approximately half the risk of acquiring the disease. Low rates are also reported in Asians and Native Americans.¹

Epidemiological studies have established a geographical pattern of MS prevalence with areas of high, medium, and low frequency. High-frequency areas include the temperate zones of the northern United States, the Scandinavian countries, northern Europe, southern Canada, New Zealand, and southern Australia. Areas of medium frequency closer to the equator include the southern United States and Europe and the rest of Australia, and low-frequency tropical areas include Asia, Africa, and South America. Migration studies indicate that the geographical risk associated with an individual's birthplace is retained if emigration occurs after age 15 years. Individuals migrating before this age assume the risk of their new location.^4,5

The risk of MS is increased in persons with an affected family member. The risk is 3.0% for a sibling, 5.0% for a fraternal co-twin, and rises to 25.0% for an identical co-twin. Genetic studies have revealed many interacting alleles that may contribute to MS susceptibility with mutations in the human leukocyte antigen major histocompatibility complex (MHC) gene most strongly correlated. It appears that although individuals do not inherit the disease, they may inherit a genetic susceptibility to immune system dysfunction.¹

When persons with a genetic susceptibility are exposed to a viral agent, the immune system responds with activated myelin-reactive lymphocytes, a concept known as molecular mimicry. Implicated viruses in this process under investigation include the Epstein-Barr virus, measles, canine distemper, human herpesvirus-6, and Chlamydia pneumoniae, though none have been definitely proven to trigger MS. The viruses may be retained in the body, resulting in a self-perpetuating autoimmune process. Risk of MS may also be increased with vitamin D deficiency and smoking.¹

In patients with MS, the immune response triggers activation of immune cells (e.g., T cells, CD4+ helper T cells, B cells) that cross the blood–brain barrier. In turn, these cells activate autoantigens, producing autoimmune cytotoxic effects within the central nervous system (CNS) (this process can be viewed as a form of "friendly fire"). Phagocytic activity of macrophages may also contribute to demyelination.¹ Myelin serves as an insulator, speeding up the conduction along nerve fibers from one node of Ranvier to another (termed saltatory conduction). It also serves to conserve energy for the nerve because depolarization occurs only at the nodes. Disruption of the myelin sheath and active demyelination slows neural transmission and causes nerves to fatigue rapidly. With severe disruption, conduction block occurs with resulting disruption of function.

An acute inflammatory event emerges. Edema and infiltrates (e.g., monocytes, macrophages, and microglia) surround the acute lesion and can cause a mass effect (abnormally high pressures), further interfering with the conductivity of the nerve fiber. Conceivably, this inflammation (which gradually subsides) may, in part, account for the pattern of fluctuations in function that characterize this disease. With repeat attacks, the anti-inflammatory processes become less effective and are unable to keep up. During the early stages of MS, oligodendrocytes (myelin-producing cells) survive the initial insult and can produce remyelination. This process is often incomplete and, as the disease becomes more chronic, stalls altogether. Eventually the oligodendrocytes become involved and myelin repair cannot occur. One form of MS, primary-progressive MS, appears to be associated exclusively with disease of the oligodendrocytes.⁶ Demyelinated areas eventually become filled with fibrous astrocytes and undergo a process called gliosis. Gliosis refers to the proliferation of neuroglial tissue within the CNS and results in glial scars (plaques). At this stage, the axon itself becomes interrupted and undergoes neurodegeneration. This is believed to be the main cause of permanent neurological disability. In advanced cases, there are both acute and degenerative lesions of varying size scattered throughout the CNS (brain, brainstem, cerebellum, and spinal cord). Lesions primarily affect white matter early, with lesions of gray matter evident in more advanced disease (type 1 lesions). Lesions may also include small perivascular areas of demyelination (type 2 lesions) and pial surface lesions (type 3). Brain atrophy, the loss of axons and myelin throughout the brain, is evident even in early stages of the disease and is progressive.¹ There are certain areas of predilection, such as the optic nerves, periventricular white matter, spinal cord (corticospinal tracts, posterior white columns), and cerebellar peduncles.⁶

MS is highly variable and unpredictable from person-to-person and within a given individual over time. At one end of the continuum, there is benign MS, defined as disease in which the patient remains fully functional in all neurological systems 15 years after onset. Benign MS affects fewer than 20% of cases. At the other end of the continuum, there is malignant MS (Marburg disease), a relatively rare disease course characterized by rapid onset and almost continual progression leading to significant disability or death within a relatively short time after onset.

There are four major disease courses (clinical subtypes) of MS. Relapsing-remitting MS (RRMS) is the most common course, affecting approximately 85% of patients with MS. It is characterized by discrete attacks or relapses, defined as periods of acute worsening of neurological function. Relapses are followed by remissions, defined as periods without disease progression and partial or complete abatement of signs and symptoms. Before the advent of disease-modifying medications, the majority of patients with RRMS went on to develop secondary-progressive MS (SPMS). SPMS begins with a relapsing-remitting course followed by progression to steady and irreversible decline with or without occasional acute attacks. Primary-progressive MS (PPMS) is a rare form occurring in about 10% of cases. It is characterized by a nearly continuous worsening of the disease from the onset without distinct attacks. Patients may experience modest fluctuations. Progressive-relapsing MS (PRMS) begins with a progressive disease course from the onset and steady deterioration (similar to PPMS), but with occasional acute attacks. It affects approximately 5% of patients with MS. Because the course of the disease may alter (RRMS to SPMS), clinicians need to be alert to changes in signs or symptoms in terms of severity, frequency, and impact on function.^1,7 Box 16.1 summarizes the clinical subtypes of multiple sclerosis.

Exacerbating Factors

MS relapses (exacerbations) are defined by new and recurrent MS symptoms lasting more than 24 hours but generally of longer duration that are unrelated to another etiology. Several exacerbating factors have been identified. Avoiding these factors is important in ensuring the patient's optimal function. An individual whose overall health deteriorates is more likely to have a relapse than one who remains healthy. Viral or bacterial infections (e.g., cold, flu, urinary tract infection, sinus infection) and diseases of major organ systems (e.g., hepatitis, pancreatitis, asthma attacks) are associated with relapses of disease. There is also a modest link between stress and acute attacks. Both major life stress events (divorce, death, losing a job, trauma) and minor stresses (exhaustion, dehydration, malnutrition, and sleep deprivation) can affect the immune system and an already compromised nervous system.

Pseudoexacerbation refers to the temporary worsening of MS symptoms. The episode typically comes and goes quickly, usually within 24 hours. The overwhelming majority of individuals with MS demonstrate an adverse reaction to heat, known as Uthoff's symptom. Anything that raises the body temperature can bring on a pseudo-attack. External heat stressors include sun exposure, hot muggy environmental temperatures, or a hot bath. Internal elevations in temperature can be produced by fever or prolonged exercise. The effects are usually immediate and dramatic in terms of reduced function and increased fatigue. Most pseudo-attacks resolve within 24 hours of cooling off and/or the end of a fever.

Symptoms of MS vary considerably, depending on the location of specific lesions. Early symptoms typically include minor visual disturbances (e.g., episodes of double vision) and paresthesias progressing to numbness, weakness, and fatigability. In more advanced stages, patients demonstrate multiple symptoms with varying involvement. Common MS symptoms are presented in Box 16.2.^8,9 The onset of symptoms can develop rapidly over a course of minutes or hours; less frequently, onset is insidious, occurring over a period of weeks or months. An early remission may lead the individual to postpone initial neurological workup for months or longer.

Sensory

Complete loss of any single sensation (anesthesia) is rare. Focal deficits can produce limited areas of diminished sensation. Altered sensations are far more common and can include paresthesias (pins-and-needles sensation) or numbness of the face, body, or extremities. Disturbances in position sense are also common, as are lower extremity (LE) impairments of vibratory sense.⁹

Pain

Approximately 80% of patients with MS experience pain, with clinically significant pain occurring in about 55%. Almost half experience chronic pain.¹⁰ Patients often experience acute, paroxysmal pain characterized by sudden and spontaneous onset. The pains are described as intense, sharp, shooting, electric shock–like, and burning. The most common types are trigeminal neuralgia, paroxysmal limb pain, and headache. Trigeminal neuralgia (tic douloureux) results from demyelination of the sensory division of the trigeminal nerve innervating the face, cheek, and jaw. Eating, shaving, or simply touching the face may trigger painful episodes. A common sign of posterior column damage in the spinal cord is Lhermitte's sign in which flexion of the neck produces an electric shock–like sensation running down the spine and into the LEs. Paroxysmal limb pain presents as abnormal burning, aching pain (dysesthesias) that can affect any part of the body but is more common in the LEs. It is the most common type of pain in MS and is worse at night and after exercise. It can be aggravated by temperature elevations. Hyperpathia, a hypersensitivity to minor sensory stimuli, can occur. For example, a light touch or light pressure stimulus elicits a severe pain reaction. Headache is more frequent in MS than in the general population and can be migraine or tension type. Chronic neuropathic pain can result from demyelinating lesions in spinothalamic tracts or in the sensory roots. It is more common in patients with minimum disability and is described as a burning pain similar to pain described by individuals with disk herniation. Musculoskeletal pain associated with muscle and ligament strain can develop from mechanical stress, abnormal postures, and immobility, often the result of weak muscles, powerful spasticity, and tonic spasms. Anxiety and fear can worsen pain symptoms.¹⁰

Visual

Visual symptoms are common with MS and are found in approximately 80% of patients. Involvement of the optic nerve produces altered visual acuity; blindness is rare. Optic neuritis, inflammation of the optic nerve, is a common problem, and produces an icepick-like pain behind the eye with blurring or graying of vision or blindness in one eye. A scotoma or dark spot may occur in the center of the visual field. Neuritis rarely affects both eyes and is usually self-limiting. Vision generally improves within 4 to 12 weeks. Damage to the optic nerve will also affect light reflexes. Marcus Gunn pupil often develops in individuals with MS who have had an episode of optic neuritis. Shining a bright light into the healthy eye will produce reflex contraction in both eyes (consensual light reflex). If the light is then shone in the affected eye only, a paradoxical widening (dilation) of both pupils occurs.

Eye movements can be disturbed in a variety of ways. Nystagmus is common in patients with MS and results from lesions affecting the cerebellum or central vestibular pathways. This involves involuntary cyclical movements of the eyeball (horizontal or vertical) that develop when the patient looks to the sides or vertically (gazeinduced nystagmus) or when the patient moves the head. Internuclear ophthalmoplegia (INO) produces incomplete eye adduction (lateral gaze palsy) on the affected side and nystagmus of the opposite abducting eye with gaze to one side. It is caused by demyelination of the pontine medial longitudinal fasciculus (MLF). Additional impairments in conjugate gaze and control of eye movements may also be present with brainstem lesions affecting cranial nerves III, IV, and VI or the MLF. Diplopia, double vision, occurs when the muscles that control the eyes are not well coordinated. Visual disturbances frequently remit and are seldom the primary cause of disability. The effects of impaired vision on balance and movement should be carefully examined.¹¹

Motor

Patients with corticospinal lesions demonstrate signs and symptoms of upper motor neuron (UMN) syndrome. Paresis, spasticity, brisk tendon reflexes, involuntary flexor and extensor spasms, clonus, Babinski's sign, exaggerated cutaneous reflexes, and loss of precise autonomic control all characterize UMN involvement. (See discussion in Chapter 5, Examination of Motor Function: Motor Control and Motor Learning.)

Weakness

Patients with UMN syndrome demonstrate movements that are slow, stiff, and weak, the result of loss of orderly recruitment and reduced firing rate modulation of motor neurons. Reduced muscle strength, power, and endurance, along with impaired synergistic relationships, are evident. Patients with cerebellar lesions demonstrate asthenia or generalized muscle weakness along with ataxia. Patients can also experience muscle weakness secondary to inactivity. Muscle weakness can vary from a mild paresis, often transient at first, to total paralysis of the involved extremities.⁹

Spasticity

Spasticity is an extremely common problem in patients with MS, occurring in 75% of all cases. Spasticity can range from mild to severe, depending on the duration of the disease, number of relapses, and worsening symptoms in recent months. It occurs in the muscles of the upper extremities (UEs) and particularly the LEs. Clinical indications of spasticity include impaired voluntary control of movement (abnormal co-contraction); increased deep tendon reflexes (DTRs); clonus; and decreased range of motion (ROM). Spasticity also results in increased fatigue, impaired functional mobility, and impaired activities of daily living (ADL). Spasticity can cause pain, disabling contractures, abnormal posturing, problems in maintaining skin integrity, and falls. For some patients, spasticity can be beneficial to sitting and standing.¹¹ spasticity fluctuates on a daily basis and can be exacerbated by certain factors, such as fatigue, stress, overheating (fever, environmental), infections, or noxious stimuli (e.g., pain, bladder, renal, bowel, skin lesions/injury).¹² Certain antidepressant agents (serotonin-reuptake inhibitors such as fluoxetine, sertraline, and paroxetine) can exacerbate spasticity.¹³ Spasticity does not typically abate during spontaneous remissions. In patients with advanced disease, spasticity can be quite disabling and difficult to manage.¹²

Fatigue

Fatigue has been defined by the Panel on Fatigue of the MS Council for Clinical Practice Guidelines as "a subjective lack of physical and/or mental energy that is perceived by the individual or caregiver to interfere with usual and desired activities."^{13, p. 1} Fatigue comes on abruptly without warning and typically worsens throughout the day. Patient complaints may include feelings of overwhelming tiredness, exhaustion, and weakness together with difficulty concentrating and mental dullness.⁸ Fatigue is a daily event, experienced by 75% to 95% of individuals with the disease. Approximately 50% to 60% of patients report that fatigue is one of their most troubling symptoms. Patients consistently report that fatigue interferes with physical functioning (79% of patients), overall role performance (67% of patients), social participation, and perceived health status. Severity of disease does not seem to be related to fatigue severity; that is, individuals mildly affected by disease (ambulatory patients) report disabling fatigue as often as more severely disabled patients.¹⁴ Fatigue is the result of central activation failure (central fatigue). Aggravating factors contributing to fatigue include physical exertion, exposure to heat and humidity (reported by 92% of patients), disturbed or reduced sleep, depression, low self-esteem and mood disorders, and medical conditions (e.g., respiratory infection). Side effects of medications also affect fatigue, including analgesics, anticonvulsants, antidepressants, antihistamines, antihypertensive agents, and anti-inflammatory agents.¹² Sense of mastery (control) is a strong psychosocial predictor of fatigue. Individuals with a low sense of mastery reported significantly more fatigue and fatigue-related distress.¹⁵

Coordination and Balance

Demyelinating lesions in the cerebellum and cere bellar tracts are common in MS, producing cerebellar symptoms. Clinical manifestations include ataxia, postural and intention tremors, hypotonia, and truncal weakness. Ataxia is a general term used to describe uncoordinated movements characterized by dysmetria, dyssynergia, and dysdiadochokinesia. Progressive ataxia of the trunk and LEs is often apparent. During sitting or standing, when a limb or the body must be supported against gravity, the patient typically presents with postural tremor (shaking, back-and-forth oscillatory movements). Intention (action) tremors are involuntary, rhythmic, shaking movements that occur when purposeful movement is attempted and results from the inability of the cerebellum to dampen motor movements (see Chapter 6, Examination of Coordination and Balance). Tremors vary in severity from slight, barely perceptible quivering (fine tremor) to wide oscillations (gross tremors). Severe tremors impose significant limitations in performance of functional activities, particularly in such areas as eating, speaking clearly, writing, personal hygiene, and walking. Tremor can be exacerbated by stress, excitement, and anxiety, all adrenalin-releasing conditions producing a temporary aroused condition.⁹ Severe numbness of the feet can contribute to difficulty with standing balance or walking (sensory ataxia).

Lesions affecting the cerebellum (archicerebellum) or central vestibular pathways can produce vestibular dysfunction. Patients may experience symptoms of dizziness, disequilibrium, vertigo, nausea, and so forth. Symptoms are precipitated or made worse by movements of the head or eyes (see Chapter 21, Vestibular Disorders).

Gait and Mobility

Individuals with MS experience difficulty walking as a result of muscle weakness, fatigue, spasticity, impaired balance, impaired sensation, visual problems, and ataxia. Approximately half of patients with RRMS will require some form of assistance during walking within 15 years of their diagnosis. Staggering, uneven steps, poor foot placement, uncoordinated limb movements, and frequent loss of balance characterize ataxic gait. It is often mistaken for drunkenness, a finding that frequently results in the patient revealing the disease for the first time ("outing"). Severe LE extensor spasticity may produce a scissoring gait pattern. Gait and balance impairments increase the risk of falls and fall injury. Approximately half of patients with MS report recent falls. Fear of falling is associated with self-imposed restrictions in mobility and contributes to disability and social isolation.⁸

Speech and Swallowing

Speech problems are the result of muscle weakness, spasticity, tremor, or ataxia and affect as many as 40% of individuals with MS. Dysarthria is characterized by slurred or poorly articulated speech with low volume, unnatural emphasis, and slow rate. Dysphonia is characterized by changes in vocal quality including harshness, hoarseness, breathiness, or hypernasal sounds. Poor coordination of the tongue and oral muscles can also result in dysphagia, difficulty in swallowing. Signs of swallowing dysfunction include difficulty chewing and maintaining a lip seal, inability to swallow (ingest food), and spitting or coughing during or after meals. Aspiration pneumonia is a serious complication that can develop if foods or liquids are inhaled into the trachea. Signs of this include a wet voice quality with gurgling or sounds of congestion, and fever. The patient is also at risk for poor nutritional intake and dehydration and may experience weight loss. Poor coordination of breath control and posture contributes to speech and feeding difficulties.¹⁶

Cognitive

Cognitive symptoms in MS are common, with approximately 50% of patients demonstrating measurable impairments. Only 10% of patients experience problems severe enough to interfere with daily activities. Cognitive impairments are related to the specific location of the lesions rather than to the overall severity of the disease, its course, or the patient's disability status. Cognitive functions likely to be affected in MS include short-term memory, attention and concentration, information processing, executive functions (concept formation, abstract reasoning, problem solving, and planning and sequencing), visuospatial functions, and verbal fluency. Long-term memory, conversational skills, and reading comprehension are typically intact. Focal frontal lobe lesions can produce cognitive inflexibility and poor impulse control. Significant mental deterioration (global dementia) is relatively rare and may be seen in rapidly progressing disease (malignant MS) or in patients with significant cerebral lesions. Secondary factors that can influence cognition include depression, fatigue, medications, and co-morbid conditions (e.g., cardiovascular or cerebrovascular disease). Level of cognitive dysfunction is a major factor in determining quality of life, social functioning, employment status, and function.^8,17

Depression

Clinical depression is common in patients with MS, with at least 50% of individuals experiencing a major depressive episode. Depressive symptoms can include feelings of hopelessness or despair, diminished interest or pleasure in activities, changes in appetite and significant weight loss or gain, insomnia or hypersomnia (daytime sleepiness), feelings of lethargy or worthlessness, fatigue or loss of energy, decreased concentration, and recurrent thoughts of death and suicide.¹⁸ It can occur as a direct result of MS lesions, as a side effect of some drugs (e.g., corticosteroids, possibly interferon), or as a psychological reaction to the stresses of this far-reaching and unpredictable disease.¹⁹ Anxiety, denial, anger, aggression, or dependency can also occur. Patients with MS face enormous issues related to the ambiguity of their health status, the unpredictable course of disease activity, unpredictable future status, and the loss of effective functioning during the prime of their lives. Feelings of learned helplessness and low self-efficacy are common and have been linked to depression.²⁰ Moreover, many of the symptoms of MS (tremor, scanning speech, incontinence) are socially embarrassing, causing additional emotional distress.

Emotional

Affective disorders occur in approximately 10% of cases and can include changes in mood, feelings, emotional expression, and control. Pseudobulbar affect (PBA), also known as involuntary emotional expression disorder or emotional incontinence, is characterized by sudden and unpredictable episodes of crying, laughing, or other emotional displays. It may occur when the disease damages the area of the brain that controls normal expression of emotion. Euphoria consists of an exaggerated feeling of well-being, a sense of optimism incongruent with the patient's incapacitating disability. Bipolar affective disorders (alternating periods of depression and mania) can also occur. Affective symptoms have been linked to more advanced disease and greater intellectual impairment.^21,22

Bladder

Urinary bladder dysfunction occurs in about 80% of patients. Demyelinating lesions affecting the lateral and posterior spinal tracts unmask the sacral reflex arc producing loss in volitional and synergistic control of the micturition reflex. Types of bladder dysfunction in MS can include a small, spastic bladder (a failure to store problem), a flaccid or big bladder (a failure to empty problem), or a dyssynergic bladder. The dyssynergic or conflicting bladder represents a problem with coordination between the bladder contraction and sphincter relaxation. Common symptoms include urinary urgency, urinary frequency, hesitancy in starting urination, nocturia (frequency at night), dribbling, and incontinence. The severity of bladder symptoms is associated with severity of other neurological symptoms, particularly pyramidal tract involvement. Progressive loss of functional mobility (e.g., hand skills, sitting balance and transfer skills, ambulation) contributes to personal hygiene problems, emotional distress, and functional incontinence (inability to toilet or manage dysfunction). Emptying dysfunction with large residual urine volume increases the risk of recurrent urinary tract infections (UTIs) and kidney damage from frequent UTIs.²³

Bowel

Constipation is the most common bowel complaint in MS and results from lesions affecting control of the gastrocolic reflex. It is associated with the presence of spasticity of the pelvic floor muscles and is also a frequent consequence of inactivity, lack of fluid intake, poor diet and bowel habits, depression, and medication side effects. Bowel impaction is a serious complication that requires immediate attention. Diarrhea and incontinence are less problematic but can also occur as a result of loss of rectal control, sphincter abnormalities, or other, secondary problems (e.g., gastroenteritis, inflammatory bowel disease).²⁴

Sexual

Sexual dysfunction is common, affecting as many as 91% of men and 72% of women. In women, symptoms can include changes in sensation, vaginal dryness, trouble reaching orgasm, and loss of libido. In men, symptoms can include impotence, decreased sensation, difficulty or inability to ejaculate, and loss of libido. Sexual activity is also affected by the appearance of other symptoms such as spasticity, uncontrollable spasms, pain, weakness and fatigue, bladder or bowel incontinence, losses in functional mobility, and changes in self-image. Psychological factors have a large impact on function. Sexual dysfunction has tremendous functional and psychosocial implications for both patient and partner.²⁵

The diagnosis of MS is made by the neurologist based on a careful medical history, a complete neurological examination, and supportive laboratory tests. Evidence of damage must be present in at least two separate areas of the CNS (dissemination of lesions in space) and damage must have occurred at two separate points in time at lease 1 month apart (dissemination of lesions in time). In addition, other possible diagnoses must be ruled out. The revised 2010 McDonald Criteria of the International Panel on Diagnosis of MS has resulted in earlier diagnosis of MS with improved specificity and sensitivity (Tip-Sheet is available at www.nationalmssociety.org/).^{26, 27}

Laboratory tests used to help confirm the diagnosis include magnetic resonance imaging (MRI), evoked potentials (EP), and lumbar puncture (LP) with cerebrospinal fluid (CSF) analysis.

MRI is highly sensitive for detecting MS plaques in the white matter of the brain and spinal cord (Fig. 16.1). New lesions with active inflammation that occur during the preceding 6 weeks or so are seen as areas of increased signal intensity, "bright spots." Contrast-enhanced T1-weighted images (gadolinium-enhanced) are used to detect more long-term disease activity (i.e., loss of myelin and axons, gliosis). These lesions are seen as "black holes" on the MRI; the darker the lesion, the more extensive the tissue damage. Approximately 95% of patients with clinically defined MS have well-defined MRI changes. Excessive MRI activity includes three or more enhancements on repeat scans separated by at least quarterly intervals. Lesions revealed on MRI do not always correlate with clinical disability. "Silent attacks" documented by MRI changes outnumber the attacks that cause active symptoms such as paralysis or vision loss by 10:1. Misdiagnosis can occur, because 5% of individuals with confirmed MS do not exhibit MRI changes. In addition, other diseases can cause similar lesions evident on MRI (e.g., disseminated encephalomyelitis) and some healthy individuals can exhibit bright spots on MRI. Neurology guidelines call for MRIs to be performed at predefined intervals to document disease progression and response to disease-modifying medications.^28,29

Up to 90% of individuals with MS demonstrate abnormal EP. The presence of demyelinating lesions on visual, auditory, and somatosensory pathways produces slowed conduction. Of the three, visual evoked potentials (VEPs) have been found to be the most helpful in the diagnostic process.²⁶

Patients with MS show elevated total immunoglobulin (IgG) in CSF and the presence of oligoclonal IgG bands (seen in 90% to 95% of patients) in response to inflammatory demyelinating lesions. Patients with PPMS have higher levels of immunoglobulins in spinal fluid than patients with RRMS.²⁶

A number of medications are used to help treat and prevent relapses and slow the progression of neurological disability. Medications are also given to provide symptom relief.

Management of Acute Relapses

Corticosteroid therapy (methylprednisolone) is used to treat acute disease relapses (exacerbations), shortening the duration of the episode. These drugs exert powerful anti-inflammatory and immunosuppressive effects, including diminished swelling within the CNS, decreased T-cell activation, limited immune cell penetration of the CNS, and enhanced apoptosis of activated immune cells. The drugs do not modify the disease course or degree of recovery. Typically, corticosteroids are given in high doses (500 to 1,000 mg/day), administered intravenously for a brief course (e.g., 3 to 5 days), followed by tapered dosage of oral medication over a period of 1 to 3 weeks. There are a number of potential adverse side effects, including mood changes, increased blood pressure, fluid retention, hyperglycemia, acne, and insomnia. Chronic use is associated with hypertension, diabetes, aseptic femoral necrosis, osteopenia, and peptic ulcer.³⁰

Plasmapheresis (plasma exchange) may be used to enhance recovery from an acute relapse in patients who fail to respond to steroids. It is used for an exacerbation of RRMS and is not recommended for PPMS or SPMS.

Disease-Modifying Therapeutic Agents

Since 1993 the U.S. Food and Drug Administration (FDA) has approved drugs to reduce disease activity in people with MS. Synthetic interferon drugs (interferon beta-1b [Betaseron, Extavia], interferon beta 1-a [Avonex and Rebif]) are first-line injectable drugs that have substantial immunomodulating properties. These are close copies of a naturally occurring human chemical, interferon beta. Interferons slow down the immune system response by reducing inflammation, swelling, and rapid proliferation of T and B cells. They also block activated T cells from crossing the blood–brain barrier and damaging myelin. Other disease-modifying drugs include glatiramer acetate (Copaxone), fingolimod (Gilenya), natalizumab (Tysabri), and mitoxantrone (Novantrone). Daily pills have also been approved by the FDA to treat MS (e.g. Aubagio, Tecficlera). Patients may show reduced relapses, reduced number of new lesions on MRI, and reduced severity of attack as evidenced by acquired neurological deficits. For patients with new and suspected MS (clinical isolated syndrome [CIS]), the medications may delay time to a second clinical episode and a confirmed diagnosis of MS. Continued, frequent relapses or excessive MRI activity may indicate the need to switch drug therapy to higher doses or combination therapies. These agents cannot, however, reverse existing deficits. All of these medications are contraindicated for women who are pregnant or trying to become pregnant, or who are breastfeeding.^20,31 Table 16.1 presents an overview of the disease-modifying drugs.

Common adverse effects of the injectable interferon drugs include injection-site skin reactions (soreness, redness, pain, bruising, or swelling) and flu-like symptoms following injection that lessen over time (fever, chills, sweating, muscle aches, and fatigue). Injection sites are varied to reduce adverse effects. Rare and more severe adverse reactions include depression, allergic reactions, and liver reactions. Copaxone can produce similar injection-site reactions and an initial flushing reaction immediately after injection (anxiety, chest pain, palpitations, shortness of breath). It has the advantage of not causing flu-like symptoms or depression. Patients receive Novantrone by intravenous (IV) infusion in a medical facility and must be closely monitored for serious heart and liver damage. Tysabri poses serious risks for a rare brain infection (progressive multifocal leukoencephalopathy [PML]). An additional disadvantage is the significant annual cost of these drugs (in the thousands of dollars) that may not be covered fully by private insurance plans.^30,31

Problems with adherence using immunomodulating agents are well documented, especially for the injectable medications. Health professionals can have a significant impact on promoting acceptance and maintenance of immunomodulating therapy. In discussions with the patient the therapist needs to determine the patient's understanding of the treatment benefits and risks, perception of his or her illness, general mood and level of self-esteem, lifestyle and daily living situation, and level of family and community support. Professionals need to support the patient's hope for a positive outcome from drug therapy and emphasize the benefits of early treatment and the importance of consistency in management.³²

Management of Symptoms

Pharmacological agents are used for symptomatic relief of a wide range of symptoms in MS. The clinician should have a thorough understanding of the medications the patient is taking, the expected benefit, and potential adverse reactions.

Spasticity

Management of spasticity and spasms includes the use of muscle relaxants. Oral baclofen (Lioresal) is commonly used and is highly effective in reducing muscle tone and decreasing the frequency of spasms and clonus. Dosage is progressed gradually to obtain optimal effects. Other examples of oral agents include tizanidine (Zanaflex), dantrolene sodium (Dantrium), and diazepam (Valium). The reduction in spasticity must be balanced with the possibility of adverse effects from overdosing that may include sedation (drowsiness), weakness, and fatigue. The therapist must be alert to these changes and communicate with the physician to achieve optimal dosing for rehabilitation. The therapist must also recognize that at times spasticity can be used to enhance function, substituting for lack of strength. For example, extensor spasticity can be used to assist standing during a stand-pivot transfer. Significant reduction of spasticity with medications might serve to produce loss of function. Carbamazepine (Tegretol) can be effective in reducing paroxysmal (sudden, sharp-onset) spasms. Patients who do not adequately respond to standard drug treatment (e.g., those with intractable spasticity or spasms) may benefit from intrathecal administration of baclofen directly into the CSF of the lumbar spine via a catheter. A programmable implanted pump controls the dosage. Significant reduction in spasticity and spasms has been reported in the LEs and trunk with less improvement reported in the UEs. Adverse effects can include sedation, dizziness, impaired vision, and impaired speech. Pump failure, infection, and lead displacement can also occur.^8,33

Botulinum toxin (BT) injections are used to provide localized relief of muscle tone and spasms. Efficacy is short term and generally lasts up to 3 months. Excessive use of BT can overly weaken muscles, so it is typically reserved for patients with significant problems. Physical therapy stretching of the limb for at least 4 weeks after injection is an important adjunct. Phenol injections have also been used but are more unpredictable in degree and duration of response and are associated with sensory side effects.⁸

Surgical intervention may be considered for patients with intractable spasticity. The typical surgical candidate presents with spastic paralysis for many years resulting in a nonfunctional limb and serious complications (e.g., contractures and skin breakdown). Interventions include severing tendons (tendonotomy), nerves (neurectomy), or nerve roots (rhizotomy).⁸

Pain

A variety of drugs are available to manage pain and clinical decisions are based on type of pain. Tricyclic antidepressants are used to treat burning, central neuropathic pain similar to the use with peripheral neuropathic pain. Paroxysmal pain responds to carbamazepine (Tegretol), amitriptyline (Elavil), phenytoin (Dilantin), diazepam (Valium), or gabapentin (Neurontin). Dysesthesias are managed with low doses of amitriptyline (Elavil), imipramine (Tofranil), or desipramine (Nor-pramin). Antiepileptic drugs (carbamazepine) are used with trigeminal neuralgia. The discomfort and pain associated with spasticity and spasms may be managed with over-the-counter or prescription anti-inflammatory drugs. Sometimes pain can be managed with mild painkillers (acetaminophen or ibuprofen). Strong opioids (oxycodone, methadone, morphine) have limited effectiveness and are not typically prescribed.^10,30

Fatigue

Amantadine (Symmetrel) and modafinil (Provigil) have demonstrated moderate benefit in managing MS-related fatigue in some patients. In patients receiving disease-modifying agents, glatiramer acetate is associated with less fatigue than interferon beta-1b.⁸

Tremor

Agents to decrease tremor have been used with varying degrees of success. Some patients respond well to a single drug, some to combinations of drugs, and some find no benefit. Medications used to decrease tremor include hydroxyzine (Atarax, Vistaril), clonazepam (Klonopin), propranolol (Inderal), buspirone (Buspar), ondansetron (Zofran), and primidone (Mysoline). Dizziness and vertigo can be managed with antinausea drugs (meclizine [Antivert]) or with scopolamine patches. Severe tremor can also be treated with deep brain stimulation, which involves implanting electrodes into the thalamus.²⁸

Cognitive and Emotional Impairments

Cognitive rehabilitation training has been used to improve function in patients with MS. Compensatory training strategies (e.g., memory aids, organizational tools) can improve function in everyday activities together with modifying the home environment to limit distractions. Medications approved for the treatment of Alzheimer's disease (donepezil [Aricept]) have been shown to provide only modest benefits in memory deficits and verbal learning in some patients.⁸

Depression can be managed effectively with antidepressant medications (e.g., fluoxetine [Prozac], Paxil, sertraline [Zoloft]). Some antidepressants can also decrease fatigue. Patients with pseudobulbar affect can be effectively treated with the antidepressant medication amitriptyline (Elavil). Professional counseling and participation in support groups often help the patient cope with the stresses of this unpredictable disease. Exercise and an active lifestyle are important components to reduce depression and anxiety.

Bladder and Bowel Impairments

Urinary problems require a complete urodynamic workup to identify the specific cause of the problem and to arrive at the appropriate course of treatment. Treatment for an overactive, spastic bladder (storage dysfunction) typically involves pharmacological management with anticholinergic medications (propantheline [Pro-Banthine], oxybutynin [Ditropan], imipramine [Tofranil]) to regulate bladder emptying. Adverse effects can include dry mouth, tachycardia, blurred vision, and accommodation disturbances. Dietary recommendations include drinking 8 glasses of fluid per day (water) while limiting intake of caffeine or alcohol. A flaccid bladder (emptying dysfunction) is managed with alternate techniques for emptying, including instruction in the Crede maneuver (the application of manual downward pressure over the lower abdomen) or intermittent self-catheterization (ISC) performed four to five times per day. Pharmacological agents may include cholinergic stimulation with urecholine. Dietary recommendations include limiting intake of citrus juices while drinking cranberry juice daily or taking cranberry tablets. A dyssynergic bladder (combined dysfunction) is managed with alpha-adrenergic blocking agents (e.g., terazosin [Hytrin], prazosin [Minipress], tamsulosin [Flomax]) and antispasticity agents (e.g., baclofen [Lioresal], tizanidine hydrochloride [Zanaflex]). On rare occasions when bladder symptoms cannot be controlled with medication and/or ISC, continuous catheterization (indwelling or Foley catheter; condom, or Texas catheter) or surgical urinary diversion (suprapubic catheter) may be necessary. For example, the patient with advanced disease and significant ataxia of the UEs may be unable to manually perform self-catheterization. Urinary tract infections result from retention of urine in the bladder and from catheterization procedures. Antibiotic therapy is the mainstay of treatment.⁸

Constipation is a common problem and may be associated with medications that can exacerbate constipation (e.g., antihypertensives, analgesics/narcotics, tricyclic antidepressants, anticholinergics, diuretics, sedatives/tranquilizers, antacids). It is typically managed with dietary changes including increased fluid intake (six to eight glasses daily) and fiber in the diet. Bulk-forming supplements (Metamucil, FiberCon, Citrucel, Benefiber), or stool softeners (Colace [docusate], Surfak [docusate], miraLax [polyethylene glycol]) can also be used. Regular or continuous use of stimulant laxatives and enemas is not recommended. Bowel training may also include manual disimpaction. Incontinence management includes dietary changes such as avoidance of irritants (caffeine, alcohol); adjustment of medications used to reduce spasticity, which can contribute to the problem; or addition of medications to control bowel spasms (tolterodine [Detrol], propantheline [Pro-Banthine]).³⁴

The chronicity of this disease, along with its variable and unpredictable course, may lead some to view individuals with MS as poor rehabilitation candidates. Although the disease or its direct impairments cannot be altered, there is strong evidence to support rehabilitation in producing significant gains in enhancing levels of activity and participation.^{35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50} In a Cochrane Database Systematic Review of multidisciplinary rehabilitation for adults with MS, researchers identified 10 trials (9 randomized controlled trials [RCTs] and 1 controlled clinical trial [CCT] with 954 participants and 73 caregivers) that met the inclusion criteria. Support was strong for producing both short- and long-term gains (up to 12 months) following rehabilitation in activity and participation for both inpatient and outpatient programs. Low-intensity programs conducted over longer time periods produced stronger evidence in improving quality of life. Evidence of benefits to caregivers was limited. The researchers concluded that suggestions for "best dose" of therapy or optimal therapy are not possible given the current level of clinical trials.⁵¹

According to the National MS Society's Medical Advisory Board, rehabilitation referral should be initiated whenever there is an "abrupt or gradual worsening of function or an increase in impairment that has a significant impact on the individual's mobility, safety, independence, and/or quality of life."^{52, p. 1}

Individuals with neurodegenerative diseases such as MS benefit from restorative intervention, aimed at remediating or improving impairments, activity limitations, and participation restrictions.⁵³ Direct CNS impairments are not responsive to intervention whereas indirect impairments caused by evolving multisystem dysfunction from inactivity and disuse can be modified (Fig. 16.2). For example, strength training can result in meaningful improvements in balance and gait. Goals and outcome statements reflective of restorative intervention focus on remediating impairments and regaining functional independence while promoting self-management skills. As the disease progresses, important goals and outcomes also include assisting the patient in effective coping skills by promoting acceptance and adjustment to limitations and disabilities and enhancing quality of life. The enhancement of quality of life may in fact be the most meaningful outcome for patients in the face of chronic neurodegenerative disease.

Preventative intervention is aimed at minimizing potential complications, impairments, activity limitations, or disabilities as the disease progresses. Preventative interventions for the patient with MS are geared toward decreasing the duration and severity of symptoms or delaying the emergence of disease sequelae through early detection and intervention, termed secondary prevention. Prevention is also aimed at minimizing the degree of disability, termed tertiary prevention. Goal and outcome statements reflective of preventative intervention focus on promotion of health, wellness, and fitness, and preservation of optimal function.⁵³

Compensatory intervention is aimed at modifying the task, activity, or environment to maintain optimal function within the scope of existing impairments and limitations. Goal and outcome statements reflective of compensatory intervention focus on regaining/maintaining function.⁵³

Maintenance therapy is defined as a series of occasional clinical, educational, and administrative services designed to maintain the patient's current level of function. Individuals with MS who benefit from maintenance therapy typically are in the late stages of the disease (Expanded Disability Status Scale [EDSS] stages 7.0 to 9.5; the EDSS is discussed later in the section titled Tests and Measures). Maintenance programs are not well funded by insurance and require careful documentation. Medicare, which covers services for the elderly and the disabled, covers maintenance therapy if the professional skills of a therapist (specialized knowledge and judgment) are needed to maintain a person's condition because of identified dangers.⁵⁴ For example, risk of secondary impairment and loss of functional capa bilities is reduced or safety of caregivers is enhanced. A variety of interventions are used to achieve goals and outcomes, including limited direct interventions, patient/client-related instruction, and supportive counseling. The therapist tapers the frequency of the visits as the patient or family/caregivers are able to assume independent self-management of the care plan.

A coordinated interdisciplinary team is necessary to oversee the comprehensive examination and management needed to address the patient's complex and multifaceted problems. The team typically includes the physician, nurse, physical therapist, occupational therapist, speech-language pathologist, nutritionist, and social worker. As with any team, the patient is the central figure, with family and caregivers being key members. The ideal rehabilitation program considers the patient's disease history, course, and symptoms, including impairments, activity limitations, and disability. Of equal importance are the patient's abilities (assets), priorities, and resources (e.g., family, home, community). The focus is on long-range planning with anticipated episodes of care, including hospital-based, outpatient, and home/community-based care. Dal Bello-Haas⁵⁵ discusses a continuum of care based on disease stage (early, middle, and late) for individuals with neurodegenerative diseases. Considerations for the patient with MS are presented in Table 16.2.

Because many different areas of the CNS may be affected, it is imperative that a careful examination is performed to determine the extent of neurological and functional involvement. Subsequent reexaminations at specified intervals are used to distinguish change in status as well as effects of treatment. It may not always be possible to differentiate change in status associated with remission of symptoms from treatment outcomes. Considering the variability of symptoms of any individual patient, it is often beneficial to observe performance over a period of a few days to obtain a representative sample of baseline functioning. Fatigue and exacerbating factors should be taken into account when scheduling the examination.

Physical therapy examination data can be obtained through the patient's history and systems review and relevant tests and measures. The selection of examination procedures and level of inquiry are determined by the patient's unique status. The severity of problems, stage of disease (early/mild, middle/moderate, and late/advanced), age, setting of rehabilitation, and other factors must all be taken into account in structuring the examination.

Patient/Client History

Data obtained through interview with the patient/family and review of the medical record will provide information on general demographics, medical/surgical history, social and employment history, family history, living environment, general health status, and social and health habits. The patient's current/primary complaints and current functional status and activity level should be ascertained. Coexisting health problems and medications should also be identified.

Systems Review

Data obtained from the history and medical record will inform the systems review and help focus selection of appropriate tests and measures (Box 16.3). Review of the following systems is included: (1) cardiovascular, (2) integumentary, (3) musculoskeletal, (4) neuromuscular, and (5) cognitive, emotional, and communication.

Tests and Measures

The following are specific areas and relevant tests and measures that can be used to examine function in patients with MS (for more detailed descriptions, see earlier chapters in this text focusing on examination).

Cognition

Memory function, attention, concentration, conceptual reasoning, problem solving, and speed of information processing should be examined, as well as the effects of fatigue on cognitive performance. An expert panel convened by the Consortium of MS Centers in 2001 developed the Minimal Examination of Cognitive Function in MS (MACFIMS). This 90-minute battery of seven neuropsychological tests examines processing speed/working memory, learning and memory, executive function, visual–spatial processing, and word retrieval.⁵⁶ A brief screen of cognitive function can be achieved using the Mini-Mental Status Examination (MMSE).⁵⁷

Affective and Psychosocial Function

Emotional stability should be examined. The presence of emotional lability, euphoria, emotional dysregulation, or depression (symptoms, severity, length, effect on functional performance); the level of stress and anxiety; coping strategies; and presence of sleep disorders should be documented. A useful instrument is the Beck Depression Inventory.⁵⁸ As previously mentioned, it is imperative that the physical therapist be familiar with the patient's medications, because numerous medications will have effects on the affective and psychosocial domain.

Sensation

Given the extent of variability in sensory deficits in individuals with MS, a detailed examination of superficial and deep sensations should be completed (see Chapter 3, Examination of Sensory Function). Additionally, sensory deficits and their effects on quality of life (QOL) in individuals with MS can be quantified by using the Nottingham Sensory Assessment⁵⁹ or Guy's Neurological Disability Scale.⁶⁰ The latter is a comprehensive multidimensional scale designed to assess the wide range of disability in patients with MS. It is a questionnaire driven by patient interview and can be administered by any health care personnel.⁶¹

Pain

The presence of acute, paroxysmal pain (Lhermitte's sign, dysesthesias) and chronic pain including pain behaviors and reactions during specific movements and provoking stimuli should be documented. The McGill Pain Questionnaire⁶² or the Neuropathic Pain Scale,⁶³ developed to assess distinct pain qualities associated with neuropathic pain, can be used. (See discussion in Chapter 25, Chronic Pain.)

Visual Acuity

Acuity, tracking, and accommodation should be examined; the presence of visual deficits (blurred vision, field defects [scotoma], diplopia) should be documented.

Cranial Nerve Integrity

Motor and sensory cranial nerve function should be examined; the presence of deficits (optic pain [optic neuritis], oculomotor dyscontrol, dysphagia, impaired gag reflex, trigeminal neuralgia) should be documented.

Range of Motion

Passive range of motion (PROM) and active range of motion (AROM) should be examined; the presence of specific range of motion (ROM) impairments should be documented.

Muscle Performance

Functional strength using manual muscle testing (MMT) and dynamometers (isokinetic, grasp and pinch dynamometers) should be examined; strong spasticity may be a contraindication to standard MMT positions.

Fatigue

The frequency, duration, and severity of fatigue should be examined; precipitating factors, activity levels, and efficacy of rest attempts should be documented.⁶⁴ The Modified Fatigue Impact Scale (MFIS) developed by Fisk et al^65,66 is a structured, self-report, 21-item questionnaire addressing the effects of fatigue on cognitive, physical, and psychosocial function using a 5-point ordinal scale with 0 equal to never and 4 equal to almost always. Each area (subscale) can be scored separately; the total MFIS score range is from 0 to 84 (see Appendix 16.A). The MFIS can be downloaded in PDF format (www.nationalmssociety.org/MUCS_fatigue.asp). An abbreviated version of the MFIS has five items (the MFIS-5). Additionally, the Fatigue Scale for Motor and Cognitive Functions (FSMC) is a 20-item scale developed as a measure of cognitive and motor fatigue for people with MS.⁶⁷ To measure fatigue during the examination and treatment process, the Visual Analog Scale—Fatigue can be used as a single-item self-report of fatigue.⁶⁸

Temperature Sensitivity

The degree of temperature sensitivity and its effect on fatigue and weakness should be examined. A tympanic membrane thermometer (ear thermometer) can be used before, during, and after moderate-intensity exercise. A determination of the correlation between temperature changes and worsening of neurological symptoms can be made. This transient increase in symptoms following a raise in core temperature has been more accurately termed a pseudoexacerbation, and occurs due to transient increased blockade of nerve conduction in demyelinated fibers.^69,70

Motor Function

The therapist should examine for the presence of corticospinal signs (paresis, spasticity, hyperactive DTRs, positive Babinski's sign, and involuntary spasms [flexor or extensor]). The Amended Motor Club Examination (AMCA) was developed to examine the nature and degree of motor and functional deficits in patients with MS.⁷¹

Spasticity can be examined using a subjective rating scale. The Ashworth Spasticity Scale⁷² led to the more widely used Modified Ashworth Scale.⁷³ These are ordinal scales designed to measure tone intensity; the modified Ashworth has an additional grade at the lower end allowing for more discrete rating. A determination should be made of the differences between lower limbs versus upper limbs; right and left sides; and factors that influence tone.

The therapist should examine for the presence of cerebellar signs (ataxia, intention tremor, nystagmus, dysarthria). The effects of position change (e.g., sitting-to-standing) may produce an increase in ataxic movements with increased demands for postural stability and should be documented.

The therapist should examine for the presence of vestibular dysfunction (dizziness, vertigo, nystagmus, blurred vision with head and body movements, and postural imbalance; see Chapter 21, Vestibular Disorders).

Posture

Static and dynamic postural control in various different positions (e.g., sitting, standing) should be examined. The presence of postural abnormalities and postural tremor should be documented. Instruments can include posture grids, plumb lines, and still photography with light-emitting diodes.

Balance, Gait, and Locomotion

The therapist should examine static and dynamic balance, reactive and anticipatory control, sensory interaction, and synergistic strategies. Useful instruments include the Clinical Test for Sensory Interaction in Balance,⁷⁴ dynamic posturography,^75,76 the Berg Balance Scale,^77,78 the Tinetti Performance Oriented Mobility Examination (POMA),⁷⁹ and the Balance Evaluation Systems Test (BESTest).⁸⁰

Gait parameters and characteristics should be examined including gait speed, kinematics, stability, safety, and endurance. Examination of patients with significant ataxia can be enhanced by use of videotaped performance. Useful tests include timed walk tests (10-Meter Walk Test or 6-Minute Walk Test); the Dynamic Gait Index; ⁸¹ and the Ambulation Index (AI).⁸² The Rivermead Visual Gait Examination (RVGA) was developed to examine gait in patients with MS.⁸³

Alignment and fit, safety, practicality, and ease of use of orthotic and assistive devices should be examined along with energy conservation and expenditure. Wheelchair skills, including functional mobility, management, safety, transfers, and energy conservation and expenditure, should be examined.

Aerobic Capacity and Endurance

Vital signs (heart rate, blood pressure, respiratory rate) and breathing patterns should be examined at rest and during exercise. Exertional symptoms (dyspnea; elevated blood pressure, heart rate, respiratory rate) and perceived exertion during and after activity should be documented. Useful scales include the Rating of Perceived Exertion Scale (the Borg RPE Scale)⁸⁴ and the Dyspnea Scale.⁸⁵

Skin Integrity and Condition

Skin integrity and condition should be examined. Areas of insensitivity, bruising, moisture buildup, and skin breakdown should be examined and documented, along with level of urinary continence; bed and wheelchair positioning; effectiveness of pressure-relieving compensatory strategies and pressure-relieving devices (PRDs); and cognitive status and safety awareness.

Functional Status

An examination of functional mobility skills (FMS), basic activities of daily living (BADL), and instrumental activities of daily living (IADL) is indicated, together with social functioning and community and work adaptive skills. A commonly used instrument for patients undergoing active rehabilitation is the Functional Independence Measure (FIM)^86,87 (see Chapter 8, Examination of Function).

Environment (Home, Community, and Work)

Physical space for barriers, access, and safety should be examined; a specific task-analysis (patient performance-based examination) in relevant environments (home, work) may be included (see Chapter 9, Examination of the Environment).

General Health Measures

General health measures are used to examine outcomes across a broad spectrum of global or long-term health outcomes. Instruments involve self-report of the patient's perceptions of limitations and quality of life (e.g., physical and social function, general health and vitality, emotional well-being, bodily pain, and so forth). The Health Status Questionnaire (SF-36)⁸⁸ is widely acknowledged as the gold standard of generic measures of health status. The properties of this instrument have been investigated in patients with MS. Freeman et al⁸⁹ found limitations in evaluating change in moderate to severely disabled patients participating in inpatient rehabilitation with significant floor and ceiling effects in four of eight SF-36 dimensions. The Sickness Impact Profile⁹⁰ and the Examination of Motor and Process Skills⁹¹ are also general health measures.

Disease-Specific Measures

Disease-specific measures are designed to examine attributes common in a specific disease entity. Items are included to provide information about the disease process and outcomes, and ideally document clinically meaningful change over time. Thus, the instruments have greater responsiveness or sensitivity to change than general health measures.

Expanded Disability Status Scale (EDSS) for Patients with Multiple Sclerosis

In 1955, Kurtzke developed a 10-point scale for rating overall disability in MS (the Disability Status Scale or DSS).⁹² This scale was expanded in 1983 to increase its clinical sensitivity by including half-point increments, becoming the EDSS (Appendix 16.B).⁹³ This scale has been widely adopted by clinicians and has been used as a standard in MS research. Based on a standard neurological examination, patients are first graded on presenting symptoms in seven specific functional systems (pyramidal, cerebellar, brainstem, sensory, bowel and bladder, visual, mental), plus other functions. Functional system scores (FSS) are obtained using an ordinal clinical rating scale ranging from 0 to 5 or 6. The EDSS is based on the grades obtained from the FSS and uses a 0 to 10 ordinal scale graded in half-point increments, with 0 equal to normal neurological function and 10.0 equal to death owing to MS. For example, patients classified in EDSS step 2.5 demonstrate minimal disability in two FSS (two FSS grade 2, others 0 or 1). The EDSS focuses on ambulation as the primary indicator of disability (see scores 3.0 through 6.5 for levels of ambulation; patients with scores 7.0 or greater are unable to walk). Criticisms of the EDSS include its lack of sensitivity to changes that do not include functional mobility (ambulation) and problems in interrater reliability with patients whose performance is less impaired (scores in the lower ranges, ambulation less impaired).⁹⁴ Copies of the EDSS can be obtained from the National Multiple Sclerosis Society at www.nationalmssociety.org/MUCS_FSS.asp.

The Minimum Record of Disability (MRD)

The Minimum Record of Disability (MRD) was developed by the International Federation of Multiple Sclerosis Societies in 1985.⁹⁵ This instrument has three subscales: the EDSS with FSS, the Incapacity Status Scale (ISS), and the Environmental Status Scale (ESS). It is widely used and includes classification of dysfunction according to World Health Organization (WHO) terminology. Thus, the instrument addresses impairments (the FS and EDSS), disability (ISS), and handicap (ESS). The ISS includes 16 items that address functional disability in ADL. The ESS measures social performance, including work status, financial status, and economic status, and place of residence, personal assistance, transportation, community assistance, and social activity. Solari et al⁹⁶ examined the validity of the self-administered version of the MRD and found that determination of ambulatory ability, ADL skills, and social activities was both accurate and cost-effective.

MS Functional Composite (MSFC)

The MS Functional Composite (MSFC) is a 21-item test that includes 3 different functional subtests: the Timed 25-Foot Walk (T25FW), the 9-Hole Peg Test (9HPT), and the Paced Auditory Serial Addition Test (PASAT). The MSFC Administration and Scoring Manual can be obtained from the National Multiple Sclerosis Society at www.nationalmssociety.org/search-results/index.aspx?q=MSFC&x=28&y=14&start=0&num=20.

Multiple Sclerosis Quality of Life—54 (MSQOL-54)

The Multiple Sclerosis Quality of Life—54 (MSQOL-54) is a multidimensional health-related quality-of-life measure that combines both generic and MS-specific items into a single instrument.⁹⁷ The generic items are from the SF-36 to which 18 items were added to provide more information regarding MS-specific issues.⁹⁸ No overall summary score is used: the MSQOL-54 consists of 12 subscales, 2 combined summary scores, and 2 single-item measures. The subscales are physical function, role limitations—physical, role limitations—emotional, pain, emotional well-being, energy, health perceptions, social function, cognitive function, health distress, overall quality of life, and sexual function. The summary scores are the physical health composite summary and the mental health composite summary. The single-item measures are satisfaction with sexual function and change in health.

MS Quality of Life Inventory (MSQLI)

The Consortium of Multiple Sclerosis Centers, Health Science Research Subcommittee, developed the MS Quality of Life Inventory (MSQLI) as a comprehensive outcomes examination package.⁹⁹ It includes a battery of 10 self-report scales (138 items) that provide information about health-related quality of life in MS, including the Health Status Questionnaire (SF-36), Modified Fatigue Impact Scale (MFIS), MOS Pain Effects Scale (PES), Sexual Satisfaction Scale (SSS), Bladder Control Scale (BLCS), Bowel Control Scale (BWCS), Impact of Visual Impairment Scale (IVIS), Perceived Deficits Questionnaire (PDQ), Mental Health Inventory (MHI), and MOS Modified Social Support Survey (MSSS). The battery can be administered in approximately 45 minutes in most cases. Abbreviated versions of some of the scales can reduce the set to 81 items requiring approximately 30 minutes to administer. A User's Manual can be obtained from www.nationalmssociety.org/for-professionals/researchers/clinical-study-measures/msqli/index.aspx.

Functional Examination of Multiple Sclerosis (FAMS)

The Functional Examination of MS (FAMS) is a 59-item index of health-related quality-of-life measures developed by Cella et al.¹⁰⁰ There are six subscales (mobility, symptoms, emotional well-being [depression], general contentment, thinking/fatigue, and family/social well-being). The mobility subscale strongly correlates with the EDSS.

Multiple Sclerosis Impact Scale (MSIS-29)

The MS Impact Scale (MSIS-29) measures the physical and psychological impact of MS.¹⁰¹ The scale was primarily developed for community-based populations though testing with hospital-based populations (patients admitted for inpatient rehabilitation, IV corticosteroid treatment for MS relapses, and patients admitted with PPMS) revealed consistency of psychometric properties.¹⁰²

The Neurology Section Multiple Sclerosis Outcome Measures Taskforce of the American Physical Therapy Association (APTA) has compiled a list of measures for each relevant International Classification of Functioning, Disability, and Health (ICF) category together with instrument analysis, recommendations for use, and relevant references. The document can be found at www.neuropt.org/files/FINAL_EDGE_DOCUMENT.pdf.

Goals and Outcomes

The general goals and outcomes for patients with progressive disorders of the CNS, adapted from the Guide to Physical Therapist Practice,⁵³ are presented in Box 16.4. These general goals will provide the basis for development of specific anticipated goals and expected outcomes for an individual patient.

The preferred practice pattern for patients with MS from the Guide to Physical Therapist Practice is 5E, Impaired Motor Function and Sensory Integrity Associated with Progressive Disorders of the Central Nervous System.⁵³ In this document the reader will find relevant information on patient/client diagnostic classification; ICD-9-CM codes; examination components; considerations for evaluation, diagnosis, and prognosis; and suggested interventions. Thus the Guide to Physical Therapist Practice serves as a primary resource to help physical therapists design an appropriate plan of care (POC) and document the services provided and outcomes achieved.

Management of Sensory Deficits and Skin Care

In patients with MS, inflammation causes a disruption in neuronal signaling, causing a variety of sensory symptoms.¹⁰³ Strategies should be instituted to increase awareness of sensory deficits, compensate for sensory loss, and promote safety.¹⁰⁴ It is important to remember that sensory deficits may remit, so ongoing examination is necessary. The success of compensatory training strategies depends on the availability of other intact sensory systems. For example, visual compensation techniques can be instituted when deficits in proprioception produce imbalance and place the patient at risk for falls.

If multiple sensory systems are involved (e.g., vision is also impaired), sensory compensatory strategies are not likely to be successful.

Patients with proprioceptive losses demonstrate impairments in movement control and motor learning. They require increased use of other sensory systems, especially vision. Tapping, verbal cueing, and/or biofeedback can all be effective forms of augmented feedback. Proprioceptive loading through exercise, light tracking resistance, resistance bands or weights, and the use of a pool may heighten residual proprioceptive function and improve movement awareness.

Visual loss will interfere with movement and postural control. Blurred vision, especially at night or in low light situations, can occur after episodes of optic neuritis. When individuals with MS have to stand in an upright position in the dark, the likelihood of falls increases.¹⁰⁵ It is therefore important to instruct the patient to maintain adequate lighting at all times (e.g., use of a bright light at night) and reducing clutter to improve safety. Adding color contrast between items in the environment (e.g., stair markings) can also improve safety. Double vision is frequently the result of impaired coordination and weak eye muscles. It can be controlled by placing a patch over one eye and is an important strategy for improving reading, driving, or watching television. However, eye patching should not be used all the time, because it will prevent possible adaptation of the CNS. Eye patching also interferes with depth perception. The symptoms of visual blurring and double vision also fluctuate and can be heightened with fatigue, an increase in temperature, stress, and infection. Management of these symptoms is important to improve vision.¹⁰⁴ If low vision persists, the patient should be referred to a low-vision specialist or one of the national service organizations that provide help to individuals with vision impairments (National Association for Visually Handicapped, National Federation of the Blind, American Foundation for the Blind).

One of the most common early sensory symptoms of MS is decreased sensitivity to touch.¹⁰³ Patients with MS face an increased risk of developing pressure ulcers owing to the symptoms of MS, including loss of sensation, immobility, loss of bowel and bladder control, and a catabolic nutritional state.¹⁰⁴ Changes in skin turgor, static posturing, and prolonged pressure over bony prominences increase the likelihood of skin breakdown. Approximately 20% of patients with MS will develop a pressure ulcer during the course of the disease.⁸ Patients may not feel the discomfort of a prolonged position or may be unable to shift position because of weakness or spasticity. In addition, spasticity and/or spasms may cause friction effects between the skin and supporting surfaces. Awareness, protection, and care of desensitized parts should be taught early in the rehabilitation process and consistently reinforced by all members of the team. It has been demonstrated that patient education programs result in 50% reductions in pressure sore incidence.¹⁰⁶ The patient/family/caregiver should be educated in the following principles of skin care:

• The skin should be kept clean and dry. Soiled skin should be cleansed and dried promptly.

• The skin should be inspected regularly (at least once a day) and carefully, with particular attention to persistent areas of redness and over bony prominences.

• Clothing should be breathable and comfortable (soft, not too loose or wrinkled, or too tight). Seams, buttons, and pockets should not press on the skin, particularly in weight-bearing areas.

• Regular pressure relief is essential. Patients should be instructed to change their position or be changed frequently, typically every 2 hours in bed and every 15 to 30 minutes when sitting in a wheelchair.¹⁰⁷

Pressure-relieving devices (PRDs) may be necessary to protect insensitive areas and should be implemented as appropriate. These can include mattresses (water, gel, air, or alternating pressure) to distribute body weight and reduce shear and friction in bed. Sheepskins, air or foam cushions, cuffs, and/or boots may be necessary to protect body areas prone to breakdown (shoulder blades, elbows, ischial tuberosities, sacrum, trochanters, knees, malleoli, or heels). Cushions (foam; fluid or air pressure– relieving cushions) are necessary for patients who spend prolonged periods of time sitting in their wheelchair. When evaluating a PRD it is imperative that a pressure mapping system be used to determine its effectiveness and to ensure that the areas of high pressure are adequately protected.^107,108,109

Prevention is the best strategy. Important measures for maintaining skin integrity and function include maintaining good nutrition and drinking plenty of fluids. Studies suggest that for patients with MS with pressure sores, there are increased requirements for specific nutrients; particularly zinc and iron supplementation should be considered.¹¹⁰ The patient must be cautioned against activities that might traumatize the skin. Dragging, bumping, or scraping body parts during a transfer or bed mobility activities can injure the skin. Thermal injury can result from contact with hot water or hot objects. If nonblanchable skin redness develops (lasting longer than 30 minutes), patients should be instructed to stay off the area until the redness disappears. If the redness does not disappear within 24 hours, the individual should seek medical attention. Blisters, blue areas, or open sores indicate more serious injury and require immediate attention. This may include systemic antibiotic therapy for infection and wound management techniques (cleansing and débridement, topical antibiotic agents, and protective dressings).⁸

Management of Pain

Pain can be classified into four categories: pain directly from MS, pain secondary to other symptoms of MS, pain as a result of drug treatment for MS, and pain independent of MS. The management of pain depends on an accurate determination of its causes. Musculoskeletal strain or joint malalignment from chronically weakened muscles are important considerations and are responsive to physical therapy intervention. Patients may experience relief of pain with regular stretching or exercise, massage, and ultrasound. Postural retraining and correction of faulty movement patterns along with orthotic and/or adaptive seating devices can reduce malalignment and pain. Stabbing pain from Lhermitte's sign may be relieved with a soft cervical collar to limit neck flexion. Hydrotherapy or pool therapy using lukewarm water may have a beneficial effect on painful dysesthesias. Pressure stockings or gloves can also be used to relieve pain, converting the sensation of pain to one of pressure. Neutral warmth may be an additional factor in the pain relief experienced with stockings or gloves. Patients with chronic pain may benefit from referral to a total management approach for chronic pain, for example, the multidisciplinary pain clinic (see Chapter 25, Chronic Pain). Stress management techniques, relaxation training, biofeedback, and meditation are often helpful in reducing both anxiety and pain. The use of transcutaneous electrical nerve stimulation (TENS) to modulate pain in patients with MS has had conflicting results, with some patients experiencing improvement and some a worsening of symptoms.^9,10

Exercise Training

Muscle weakness and decreased endurance are common findings in patients with MS. In addition, patients with MS often adopt a sedentary lifestyle and limit their physical activity. The benefits of exercise have been firmly established in terms of producing meaningful physiological and psychological changes, improving function while lessening disability, and enhancing quality of life.^{111,112,113,114,115,116,117,118,119,120,121,122,123,124,125,126,127,128} In a Cochrane Database Systematic Review of Exercise Therapy for MS, researchers identified nine high-quality RCTs (260 participants).¹²⁸ Six trials compared exercise versus no exercise while three trials compared two types of exercise therapy. The researchers found strong evidence in favor of exercise therapy for improving muscle power, exercise tolerance, and mobility-related activities. Moderate evidence was found for improving emotional mood. There were no adverse effects on fatigue and perception of handicap. The researchers found no evidence that a specific exercise therapy program was more successful in improving level of activity and participation than another. Individuals with minimal to moderate impairments (i.e., EDSS scores between 1 and 6) demonstrate the best exercise tolerance. This speaks to the need to institute exercises early in the course of the disease. Exercise responses of the patient with MS are influenced by a host of factors that require careful attention during exercise, including fatigue, spasticity, incoordination, impaired balance, sensory loss (numbness), tremor, and heat intolerance. Depression may affect adherence to an exercise program. Therapists therefore need to provide constant reinforcement and a positive environment.

Individuals with MS will vary greatly in their responses to exercise. The focus and pace of therapy must be readjusted according to the patient's specific abilities and needs at that time. Patients with RRMS who are experiencing an exacerbation should not exercise until remission is evident. Exercise therapy can be reinstituted when the deterioration has stabilized and no new symptoms are appearing. Patients with PPMS can exercise within the limits of their capabilities as exercise may slow further deterioration and optimize remaining function.^129,130 Box 16.5 Evidence Summary presents a summary of selected research on exercise and MS.

Strength and Conditioning

Maximal muscle force during sustained isometric or isokinetic exercise is lower for persons with MS secondary to reduced ability to activate muscles (reduced force/unit muscle mass), reduced muscle metabolic responses, and muscle weakness secondary to muscle fiber atrophy, spasticity, and disuse.¹²⁹ Determining an appropriate exercise prescription to improve strength and endurance is challenging and needs to be carefully individualized for each patient. Prescription is based on four interrelated elements: frequency of exercise, intensity of exercise, type of exercise, and time or duration (the FITT equation). The following guidelines can be used:¹³⁰

• Exercise sessions should be scheduled on alternate (non-endurance) days and during optimal times, such as in the morning, when body core temperatures tend to be lowest and before fatigue sets in. Patients with greater neurological involvement may require more frequent exercise (e.g., daily exercise time).

• Resistance training modes can include weight machines, free or pulley weights, latex resistance bands, or isokinetic machines.

• Circuit training, in which improved work capacity is developed through the use of various different stations that alternate work between UEs and LEs, distributes the load among muscles and may prove beneficial for reducing the likelihood of fatigue.

• Sessions should involve discontinuous work, carefully balancing exercise with adequate rest periods.

• Progression is generally slower than with healthy individuals.

• Precautions should be taken to prevent the deleterious effects of overwork. Exercising to the point of fatigue is contraindicated and can result in worsening of symptoms, most notably increased weakness. This may have additional adverse effects on the continuing motivation of the patient.

• Precautions should be taken to monitor the effects of fatigue. Time to fatigue varies greatly among individuals with MS and is not correlated with the level of physical impairment or disability.

• Precautions should be taken to manage core body temperature and prevent overheating.^131,132 Environmental temperatures should be carefully controlled. Air conditioning is a medical necessity in many climates. Additional cooling can be achieved through the use of fans, wet neck wraps, spray bottles for misting the skin with cool water, and immersion in cool water with aquatic exercises. Surface cooling devices have emerged as effective tools in managing body temperatures, controlling fatigue, and improving function. These include cooling suits or vests.^133,134,135

• Precautions should be taken with certain impairments. Tactile and proprioceptive losses or incoordination and tremors may make the use of some equipment (e.g., free weights) unsafe. Visual feedback, when intact, should be used to monitor exercise performance. An alternative suggestion would be to use synchronized arm/leg ergometers to control limb movements.

• Precautions should be taken with cognitive and memory impairments. Individuals may require written or posted exercise instructions/diagrams including reminders of the number of repetitions, proper form, and correct use of equipment.

• Functional training activities (e.g., closed chain exercises) can be used to promote strength and functional endurance. Individuals with ataxia and balance problems may require the use of more stable postures (e.g., modified plantigrade, quadruped, or supported sitting).

• Group exercise classes can provide valuable motivation and social support. The therapist's primary role is one of educator and group leader. Successful management of group classes requires careful, individualized examination of group members to determine specific goals and exercises.

• Outcome measures can include isokinetic dynamometry, MMT (may be unreliable if spasticity is present), functional tests (e.g., sit-to-stand), fatigue (MFIS), and quality-of-life measures (HRQL).

Aerobic Conditioning

Individuals with MS demonstrate expected physiological responses to submaximal aerobic exercise; that is, heart rate (HR), blood pressure (BP), and oxygen uptake (VO₂) all increase in a linear fashion in response to increasing workloads. Respiratory responses (respiratory rate [RR] and minute ventilation) also increase.¹²⁹ However, HR and BP responses may be blunted if cardiovascular dysautonomia is present. A direct relationship exists between the duration and extent of disease and the likelihood of autonomic cardiovascular dysfunction. Patients with MS can also demonstrate respiratory muscle dysfunction (weakness, dyssynergia), contributing to reduced exercise tolerance.

Exercise tolerance and maximal aerobic power (VO_2max) are reduced in individuals with reduced cardiorespiratory fitness secondary to physical inactivity. Decreased physical work capacity, decreased vital capacity, increased HR at rest and in response to exercise, decreased muscular strength, increased fatigue, increased anxiety, and depression are common findings.

Determining an appropriate exercise prescription to improve cardiovascular conditioning needs to be carefully individualized for each patient. While predicting exercise capacity and cardiorespiratory fitness is challenging for individuals with MS, recent studies have shown that peak VO₂ and exercise capacity can be predicted through submaximal testing. ^136,137 The following guidelines for clinical exercise testing can be used.¹³⁰

• The preferred mode is either an upright or recumbent leg cycle ergometer. A recumbent device is indicated if sitting balance is impaired. Combination leg and arm ergometry or UE ergometry alone may be necessary in the presence of significant LE involvement. Toe clips and heel straps are recommended to control foot placement especially in patients with spasticity, tremor, or weakness.

• Performance measures include HR, ratings of perceived exertion (RPE), BP, and expired gas analysis (VO₂). Using the RPE scale, peripheral (muscles, joints) exertion is consistently rated as more stressful (higher) than central (cardiopulmonary) exertion.

• A continuous or discontinuous protocol (3- to 5-minute stages) can be used; the discontinuous protocol is indicated with symptomatic disease, especially fatigue.

• A submaximal test should be used. Most individuals with MS can achieve 70% to 85% of their age-predicted maximal heart rate (HR_max).

• Recommendations for increasing workloads for each stage are 12 to 25 watts for LE work and 8 to 12 watts for combined UE and LE work.

• Termination criteria include achievement of peak HR, peak VO₂, volitional fatigue, significant BP changes (systolic blood pressure [SBP] greater than 250 mm Hg or diastolic blood pressure [DPB] greater than 115 mm Hg or a hypotensive response), or a decrease in oxygen uptake with increasing work rate.

• Precautions should be taken to monitor for attenuated HR or BP responses during exercise. A category-ratio RPE scale can be used to estimate central and peripheral exertion.⁸⁴

• Precautions should be taken to manage core body temperature and prevent overheating (e.g., use of a fan for cooling).

• Precautions should be taken to monitor the effects of fatigue.

• Precautions should be taken to prevent the deleterious effects of overwork.

• Precautions should be taken with certain medications that can affect results: amantadine hydrochloride (HCl) may temporarily reduce fatigue; baclofen and amitriptyline HCl may cause muscle weakness; prednisone can also cause muscle weakness along with reduced sweating and hypertension.

• Morning is the optimal time for testing.

Prescription is again based on the four interrelated elements of the FITT equation. Recommendations for exercise programming to improve aerobic conditioning include the following:¹³⁰

• Recommended training frequency is 3 to 5 days/week, on alternate days. Daily exercise at lower levels of intensity is recommended for individuals with more limited exercise capacities (e.g., 3 to 5 metabolic equivalent [METs]).

• Training intensity should be limited to 60% to 85% HR_peak or 50% to 70% peak VO₂.

• Recommended duration is 30 minutes per session or, for more involved individuals, three 10-minute sessions per day.

• Type of exercise can include cycling, walking, swimming, or water aerobics.

• Circuit training may prove best for optimizing training.

• Individuals with balance problems or sensory loss will require non–weight-bearing activities.

• Exercise precautions: discussed in previous section.

• Outcome measures include graded exercise test results (GXT), HR (which may be difficult to monitor with dysautonomia; sensory loss in the fingers may make self-monitoring difficult), tests of lung function (forced vital capacity [FVC]), body composition, RPE, fatigue (FI, MFIS), functional status, and quality-of-life measures (HRQL).

Patient education is particularly important because the overall success of a fitness program is influenced by the individual's level of understanding of the basic principles of training, independence in self-monitoring, and skill in decision making relative to level of impairment and exercise modifications required, as well as lifestyle and general health and safety considerations.

Flexibility Exercises

Stretching and ROM exercises are necessary to ensure adequate joint motion and to counteract the effects of spasticity (Fig.16.3). Sedentary or inactive persons who are dependent on wheelchairs often develop tightness in hip flexors, adductors, hamstrings, and plantarflexors. Limited overhead ROM is seen with tightness in the pectoralis major/minor, and latissimus dorsi and is associated with a slumped, forward posture. Patients confined to bed typically present with tightness in hip/knee extensors, adductors, and plantarflexors. Stretching and ROM exercises should be performed daily. For adequate stretching, holding at end range should be a minimum of 30 to 60 seconds repeated for a minimum of 2 repetitions. The use of orthoses or dynamic splinting is an appropriate option for prevention and in some cases reversal of contractures.^138,139 Considering the gait deviations and difficulty with transfers/bed mobility that arise from limited ROM and spasticity, it is important to also include aggressive trunk ROM to allow for full function of the core musculature, most notably the quadratus lumborum (Fig. 16.4). More active patients may benefit from Tai Chi, which provides additional important benefits of relaxation and balance training. ROM measurement using goniometry is an appropriate outcome measure.

Management of Fatigue

With approximately 75% of individuals with MS describing persistent or sporadic symptoms, fatigue is among the most debilitating. Fatigue is characterized by overwhelming sleepiness, excessive tiredness, and sense of weakness that comes on suddenly and severely. Aversion to activity for fear of bringing on fatigue is also common. The resultant lowered activity levels have important implications for diminished health status and deconditioning. Therapists are faced with a balancing act, on one hand prescribing exercise, while on the other hand avoiding overwork and the development of fatigue. Aerobic exercise training (previously discussed) and energy effectiveness strategies (EES) are central to any intervention plan to lessen fatigue.¹³ During exercise prescription and physical therapy sessions, it is imperative that a skilled therapist recognize the difference between MS-related fatigue and the expected exercise-related fatigue. MS-related fatigue during exercise is often associated with thermal stress, which can be offset with adequate rest and the use of cooling and precooling treatments during exercise.^132,133,134

Patients are instructed to keep an activity diary in which they record how they slept the night before, daily activities by hour, and how costly those activities were. For each activity, they can be asked to rate their level of fatigue (F), the value or importance of the activity (V), and satisfaction perceived with performance of the activity (S) by assigning a number between 1 and 10 with 1 being very low and 10 being very high. For example, the activity might be fixing lunch. Scores reported for this activity might be F = 7, V = 3, and S = 2. Aggravating factors associated with increasing fatigue (e.g., heat stress) and MS symptoms that appear or worsen during the day are also recorded. An MS Daily Activity Diary is presented in Appendix 16.C.¹⁴⁰

Based on this information, therapists can initiate training sessions, teaching energy effectiveness strategies. Energy conservation refers to the adoption of strategies that reduce overall energy requirements of the task and overall level of fatigue. These can include modifying the task or modifying the environment to ensure successful completion of daily activities. For example, a motorized scooter or powered wheelchair can be considered for community or home mobility to help conserve energy and maintain independence. Other mobility equipment such as walkers, crutches, or orthotics can also be considered. Activities that are difficult or have high energy needs can be broken down into component parts, requiring accurate activity analysis. Activity pacing refers to the balancing of activity with rest periods interspersed throughout the day. For the patient with chronic fatigue, rest–activity ratios are developed, with periodic rest periods planned in advance. Time-outs with complete rest should be instituted if an activity becomes exhaustive. Overall levels of energy can be improved if patients learn to set priorities and limit their activities, saving their energy for those activities that are truly important to them (e.g., activities that are enjoyable and meaningful in terms of the individual's lifestyle). The occupational therapist addresses EES and can provide valuable suggestions in terms of planning, work simplification, and developing energy-efficient activities for self-care and home management. The vocational rehabilitation counselor can provide useful strategies for behavioral modification and vocational rehabilitation. Team efforts with the physical therapist and others are important for consistency and reinforcement. Weekly review of activities and recommended modifications is used to evaluate progress. The MFIS should be administered on a regular basis to monitor ongoing fatigue status (see Appendix 16.A). Finally, stress management techniques are important components of symptom management.

The occupational and physical therapist should complete a direct environmental examination of the home and/or job site (see Chapter 9, Examination of the Environment). A number of adaptations may be considered to improve efficiency and safety, including air-conditioning, home, or work modifications, or ergonomic equipment. The patient/family/caregivers should be educated as to the importance of these recommendations for improved function. Periodic review of equipment and environmental modifications is also recommended.

Proper communication between the occupational and physical therapist and the patient's physician can ensure that the correct doses of the pharmacological treatments are in place, as the therapist has the unique opportunity of observing the patient in various circumstances and activity levels.

Management of Spasticity

Although spasticity varies greatly from person to person, muscles that typically demonstrate strong tone include the antigravity muscles. For example, in the LEs, the quadriceps, hip adductors, and plantarflexors are often spastic while in the UEs the elbow, wrist, and finger flexors together with shoulder adductors are spastic. Individuals with MS typically demonstrate stronger spasticity in the LEs than the UEs. Spasticity is functionally limiting and contributes to the development of a number of secondary impairments such as contractures, postural deformity, and decubitus ulcers.

A variety of physical therapy interventions can be used, including cryotherapy, hydrotherapy, therapeutic exercise, stretching, positioning, or any combination thereof. The responses to these interventions must be monitored closely and carefully balanced with pharmacological interventions. The therapist must closely monitor the effects of the antispasticity medications prescribed and optimize physical therapy interventions with the dosing cycle. For example, patients on baclofen will respond better to stretching techniques if they are applied in the middle of the dosing cycle rather than at the end or beginning. Physical therapists must also recognize contributing factors that affect tone and respond appropriately. For example, infection or fever that increases tone may require a referral to the physician. It is important to reduce or eliminate all factors that can aggravate spasticity (e.g., heat, humidity, stress).

Topical cold (ice packs or wraps) or hydrotherapy (cool bath) can temporarily reduce spasticity by decreasing tendon reflex excitability and clonus and by slowing conduction of impulses in nerves and muscles. The effects of cryotherapy are relatively short-lived, although some patients may experience enhanced ability to move that lasts for minutes or hours. It is important to remember that some patients, particularly those with intact sensation, may react to the unpleasant sensation of cold with fight or flight (autonomic nervous system) responses, such as increased HR, increased RR, or nausea. Cryotherapy is contraindicated in these patients.

Stretching and ROM exercises begun early in the course of the disease and continued daily can help patients maintain joint integrity and mobility in the presence of spasticity. Combining stretching with movements using rhythmic rotation (gentle rotation of the limb) or proprioceptive neuromuscular facilitation (PNF) stretching techniques (hold–relax active contraction [HRAC], contract–relax active contraction [CRAC]) is effective in gaining ROM.¹⁴¹ See Chapter 10, Strategies to Improve Motor Function, for a discussion of these techniques. Maintained stretch, held for 30 minutes to 3 hours, also can be used to decrease stretch reflex activity. Maintained stretch can be achieved with prolonged positioning (e.g., tilt table standing with toe wedges), low-load weights applied using skin traction, or serial casts. Air splints also provide an effective mechanism to maintain limbs in lengthened, out of spasticity positions. Patients/family members/clients should be taught stretching exercises as part of a home exercise program (HEP). Fast, ballistic stretching movements are contraindicated, because spasticity is velocity sensitive. Stretching movements need to proceed slowly to gradually achieve the desired range.

Active exercises at slow or self-selected speeds should focus on expanding the available ROM. Emphasis on contracting the antagonist muscles can assist through mechanisms of reciprocal inhibition. Electrical stimulation of muscles antagonist to the spastic muscles can also be used to decrease spasticity. Movements that encourage abnormal postures should be discouraged. Patients with abnormal co-contraction may benefit from exercises focused on improving motor control (timing exercises) or biofeedback. Tai Chi, yoga, and aquatic exercises combined with cool water temperatures (less than 85°F [29.44°C]) can also be helpful in producing desired relaxation.¹²

Functional activities aimed at reducing tone should concentrate on trunk and proximal segments, because many patterns of hypertonus seem to be fixed from the action of the stronger proximal muscles. Extensor tone seems to predominate, so activities that stress LE flexion with trunk rotation are generally the most effective. For example, lower trunk rotation (LTR) in hook-lying can be effective in reducing proximal extensor tone. One very effective strategy is to position the patient in hook-lying with a therapy ball under the flexed legs and gently rock the ball back and forth. Moving from quadruped position to side-sitting can also be effective in reducing extensor tone in some patients as the activity combines LTR with prolonged inhibitory pressure on the quadriceps.¹⁴²

For the patient with limited functional mobility (EDSS levels of 7.0 or above) positioning out of abnormal spastic postures is an important component of the management program. In general, prolonged or static positioning in any fixed posture can be deleterious to the patient with strong spasticity and should be avoided. For example, the patient who remains in bed all day with the LEs positioned in extension, adduction, and plantarflexion may be unable to flex enough at the hips and knees to sit in a wheelchair. Similarly, the feet will remain fixed in plantarflexion and cannot be positioned on the footpedals. A positioning schedule using varied positions (in bed, chair, or wheelchair) will help keep the patient from getting stuck in any one posture. Mechanical positioning devices (e.g., resting splints, toe spreader, finger spreader, ankle splint) are helpful in maintaining position and preserving joint structures.

Management of Coordination and Balance Deficits

Cerebellar deficits (ataxia, postural instability) are common in MS. Impairments in somatosensory, visual, and vestibular systems are also common and result in disordered proprioception. Spasms and muscle weakness can affect balance by changing the force and sequence of muscle contraction.¹⁴³ These combined effects result in difficulty in sustaining upright postures, walking, and other functional activities, leading to an increased risk of falls.

Interventions directed at promoting postural control should first focus on static control (holding) in weight-bearing, antigravity postures (e.g., sitting, quadruped, kneeling, modified plantigrade, and standing). Progression through a series of postures is used to gradually increase postural demands by varying the base of support (BOS), raising the center of mass (COM), and increasing the number of body segments (degrees of freedom) that must be controlled. Specific exercise techniques that can be used to promote stability include joint approximation applied through proximal joints (shoulders or hips) or spine, and rhythmic stabilization (PNF). Patients with significant ataxia will not be able to hold steady and may benefit from the application of the technique of PNF dynamic reversals (slow reversals), progressing through decrements of range.¹⁴¹ Dynamic postural control can be challenged by incorporating activities such as weight shifting and UE reaching (Fig. 16.5) or LE stepping. In sitting, a resisted PNF chop pattern that combines UE movements with trunk movements (flexion with rotation and extension with rotation) is an excellent activity. This can be progressed to more advanced dynamic activities with the patient sitting on a therapy (Swiss) ball as opposed to sitting on a hard flat surface (Fig. 16.6). Core musculature is engaged while the demands on ankle and knee musculature are minimized. This allows for a more focused core balance program that can be progressed to standing.^144,145

An important goal of therapy is to promote safe and functional balance. Effective training should involve a variety of everyday functional tasks that challenge balance. Figure 16.7 demonstrates a dynamic postural control activity of combined stepping and reaching. Figure 16.8 demonstrates sit-to-stand movement transitions. As training progresses, tasks are modified (e.g., wide base to narrow base to tandem stance, stable surface to moveable surface) to promote adaptation of skills. Sensory contexts are also varied to promote adaptive control in various different perceptual contexts (e.g., eyes open to eyes closed, firm surface to thick foam surface).¹⁴² Patients with MS and central vestibular dysfunction may benefit from vestibular rehabilitation to improve impaired balance and disability due to dizziness or disequilibrium (Fig. 16.9). See Chapter 21, Vestibular Disorders, for additional discussion.¹⁴⁶

The pool is an important therapeutic medium to practice static and dynamic postural control in both sitting and standing, as well as walking. Water provides graded resistance that slows down the patient's ataxic movements, while the buoyancy aids in upright balance. Water aerobics have been shown to be effective in improving strength, decreasing muscular fatigability, increasing endurance, and improving quality of life in patients with MS.^120,147 When recommending aquatic exercise programs it is imperative to assess the individual's tolerance to heat. It is commonly recommended that persons with MS exercise in a pool that is 80° to 85°F (26.66° to 29.44°C).

Biofeedback training using augmented feedback can be used to improve balance function. Augmented visual feedback¹⁴⁸ and augmented proprioceptive feedback (e.g., whole body vibration platform training)¹⁴⁹ have been used to improve function in patients with MS. Training on a moveable force platform (e.g., SMART Balance Master® [NeuroCom International, Inc., Clackamas, OR 97015]) can also improve balance. The added biofeedback from visual and/or auditory feedback displays on force platform training machines is especially useful for patients with somatosensory deficits. The patient with ataxia needs to learn how to reduce excess postural sway (frequency and amplitude) and to control center of alignment position. Prolonged latencies (onset of responses) should be expected. Additionally, with advancements in technology, whole body vibration¹⁵⁰ and household video game platforms (i.e., Nintendo Wii) have become useful tools for balance training for individuals with neurological conditions.^151,152

Control of ataxic limb movements (tremor and dysmetria) can be achieved through proprioceptive loading and light resistance. For example, the therapist can use PNF extremity patterns using the technique of dynamic reversals with light tracking resistance to modulate force output and reciprocal actions of muscles. Ataxic movements have sometimes been helped by the application of latex resistance bands or light weights to stabilize movements. Velcro® weight cuffs (wrist or ankle), weighted boots, or a weighted jacket or belt can reduce tremors of the limbs or trunk. The extra weight will also increase energy expenditure, and must therefore be carefully balanced against the increased fatigue they might cause. Weighted canes or walkers can be used to reduce ataxic UE movements that interfere with the use of an assistive device during ambulation. Weighted spoons or forks can be used to enhance eating. For patients with significant tremor, these devices may mean the difference between dependent and independent function. External devices (braces or splints) can be used to stabilize ataxic limbs but also have the undesirable effect of adding weight to limb movements. Air splints can also stabilize limb movements and should be considered, because they are lighter and less energy costly. A soft cervical collar can be used to stabilize head and neck tremors. All these strategies, however, should be viewed as temporary and compensatory. Once the devices are removed, ataxic movements will return or in some cases may actually temporarily worsen.

Unwanted movements are worse under conditions of stress, anxiety, and excitement. The increased arousal, the result of adrenalin pumping through the system, increases existing tremors while decreasing function. Stress management techniques are therefore an important component of the POC. In general, patients do better in a low-stimulus environment that allows full concentration on control of movements. They benefit from augmented feedback (verbal cueing of knowledge of results and knowledge of performance; biofeedback) and repetition to improve motor learning. The patient with MS is often restricted in practice by neuromuscular fatigue and neurological deficits that impair sensory feedback, attention, memory, and concentration. The successful therapist will need to carefully identify the patient's resources and abilities and capitalize on them to maximize motor learning.

Locomotor Training

Walking ability is frequently impaired. However, at least 65% of patients with MS are still walking after 20 years.⁹ Early gait problems often include poor balance and heaviness of one or more limbs. Patients frequently report difficulty lifting their legs (hip flexor weakness). Weak dorsiflexors are also common, resulting in foot drop. Problems with foot clearance may result in a circumducted gait pattern, among other gait deviations. Later problems evolve owing to clonus, spasticity, sensory loss, and/or ataxia. Weakness generally extends to include the quadriceps and hip abductors. Quadriceps weakness typically results in hyperextension of the knee and forward flexion of the trunk with increased lumbar lordosis. Hip abductor weakness results in a Trendelenburg gait pattern with a strong lateral lean to the weak side.

A well-designed exercise program of tone management, stretching, and strengthening exercises can improve walking. Standing and walking activities should stress safety and maintaining a stable BOS; maximum weight-bearing through the LEs; and adequate weight transfer and forward progression with trunk, limb, and pelvic kinematics consistent with safe walking. Verbal and manual cueing can assist the patient in the correct mechanics of gait. A variety of functional activities should be practiced. These include walking forward and backward, side-stepping, and cross-stepping (Fig. 16.10). Braiding (a PNF activity that combines side-stepping and cross-stepping) is a complex, higher-level walking activity. Stair climbing, negotiating curbs and ramps, navigation around obstacles, and walking on varied surfaces should also be practiced for safety in community mobility. See Chapter 11, Locomotor Training, for further discussion. As previously mentioned, the pool is an important medium that can be used to assist training of the more involved patient with ataxia while reducing tone and fatigue.

Locomotor training (LT) using an antigravity treadmill or a treadmill training (TT) with body weight support (BWS) has been the focus of increasing attention in the literature and used extensively to improve gait in patients with spinal cord injury and stroke (see discussion in Chapter 10 and Evidence Summary Boxes in Chapter 15, Stroke, and Chapter 20, Traumatic Spinal Cord Injury). In studies involving persons with MS using LT and BWS, improvements in muscle strength, spasticity, endurance, balance, walking speed, and quality of life have been reported. Level of effort was reduced while detrimental effects on fatigue are not evident.^153,154,155 Robot-assisted treadmill training (RATT) with BWS has also been used and when compared to conventional BWS TT, patients in both groups experienced similar improvements in outcome measures.^156,157 When RATT was compared to conventional gait training, no difference in outcomes was found between the groups.¹⁵⁸ In summary, LT with BWS is an activity-dependent intervention that is feasible and safe and has the potential to result in significant improvements in function for patients with MS.

Orthotics and Assistive Devices

Patients with MS typically require orthotic devices as ambulation skills decline. Ankle-foot stability can be achieved by the addition of an ankle-foot orthosis (AFO). Improvements in energy efficiency and safety are also important outcomes. AFOs are prescribed for foot drop, poor knee control (especially hyperextension), minimal to moderate spasticity, and poor somatosensation. The most common type used is the standard polypropylene AFO, which is lightweight and has the added benefit of cosmesis (Fig. 16.11). An AFO with an articulated joint can be prescribed to provide more rigid control for the ankle with the addition of a plantarflexion stop. Functional electrical stimulation (FES) devices have become prevalent in treatment and compensation for foot drop with improvements in walking performance and satisfaction reported. Patients also experienced fewer falls and reduced fatigue.^159,160,161 In order to effectively use any orthotic or FES device for foot drop, an individual must have adequate hip flexion strength. Relative contraindications to the prescription of these devices include severe spasticity, foot edema, and weakness (nonfunctional grades of LE muscles, especially hip flexors). Although knee-ankle-foot orthoses (KAFOs) can provide additional stabilization control of the knee, they are rarely used because of the increased energy expenditure required.

Canes, forearm crutches, or a walker may be necessary to compensate for deficits in fatigue, strength, sensory loss (numbness), or balance (Fig. 16.12). For many patients acceptance of an assistive device involves full recognition of their disability. They need to be convinced that use of these devices is far safer than "wall walking" or "furniture walking." Devices also provide recognition to the community at large that patients are not staggering or losing their balance because they are "drunk," a frequent occurrence with many patients. The devices may be the difference between community participation or remaining homebound because of fear of falling. Patients should be encouraged to try out different devices to determine which works best for them. For example, the patient with significant fatigue levels may benefit from a large-wheeled walker with locking hand brakes and a seat that allows for frequent rests. Recent technological advancements have brought a new breed of mechanical upright walking devices that have integrated computer chips, sensors, and motors to aid an individual in ambulation. Cosmesis is an important factor in promoting acceptance. There are many innovations in assistive technology that make the choices easier. For example, designer canes now come in many different colors and styles, including clear Lucite. ABLEDATA (www.able-data.com) is a federally funded project that offers product information, resources, and links to manufacturers.¹⁶²

As the disease progresses, many patients benefit from a wheeled mobility device (powered scooter or wheelchair). The course and progression of the disease and presenting symptoms should be taken into consideration when deciding on a device. For patients with adequate trunk stability, UE function, and appropriate visual, perceptual, and cognitive skills, a scooter provides needed mobility while conserving energy. Scooters also do not carry the same negative stigma as that of a wheelchair. Both three- and four-wheel scooters are available. Four-wheel scooters have superior outdoor and uneven terrain performance, but are not as easily transported. Features that should be recommended include a seat that rotates for easy mounting and dismounting, easy dismantling for loading into the car, and steering mechanisms that minimize the work of the UEs. One disadvantage of scooters is that seating cannot always be customized. They are often not designed for prolonged sitting or for patients with moderate to severe postural instability. Some new three-wheeled scooters are designed to turn in very small areas while others have a wide turning radius and may not be suitable for in-home use. The individual with MS must be adequately educated on the safety precautions of a scooter, because the trunk strength and stability requirements are significantly higher than those of a power wheelchair.

A wheelchair should be considered when postural demands necessitate increased support. A standard wheelchair requires additional energy expenditure and coordination for propulsion. When prescribing a manual wheelchair to an individual with MS it is important to include education on proper wheelchair propulsion for both preservation of shoulder strength and energy conservation. A power wheelchair should be considered when impairments prevent or limit manual propulsion or when fatigue is a major limiting factor in mobility (Fig. 16.13). However, they are more costly and require specialized transportation by a wheelchair-accessible van or bus. Most patients will navigate using a joystick. For patients with impaired hand strength and sensation, the joystick can be adjusted to increase sensitivity. Wheelchair seating should ensure proper alignment of the pelvis, trunk and head, and limbs while enhancing function. Common malalignments include posterior tilting of the pelvis (sacral sitting) with kyphosis, typically the result of spasticity in the hamstring muscles. This can be improved with the addition of a seat cushion. Postural alignment can also be assisted by the addition of contoured seating (custom built). A solid back support and adjustable lateral trunk supports may be needed to enhance postural alignment and upright sitting. Footrests should be positioned to ensure that the thighs are parallel to the floor. If extensor spasms are strong, they can actually propel the patient out of the chair. A strong lap belt that secures firmly around the pelvis is necessary for safety. For patients with strong adductor spasticity, a medial knee block (pommel) may be necessary. Heel loops and straps may be required to maintain foot position on the footrests. Patients who no longer demonstrate adequate trunk and head stability require an alternate seating design. A tilt-in-space wheelchair with head/neck support is a better option than a reclining wheelchair with high back and elevating leg rests. The former maintains the normal hip sitting angle; the latter produces extension of the hips and may feed into strong extensor spasticity. Elevating leg rests tend to stretch hamstring muscles and may cause posterior pelvic tilting when spasticity is present. The reclining wheelchair with elevating leg rests also creates greater environmental access problems. Motorized control of the seat back available in tilt-in-space wheelchairs will allow the patient to make easy adjustments in position, thus preventing skin breakdown. See Chapter 32, The Prescriptive Wheelchair, for additional discussion.

Patients should be instructed in transfer and wheelchair mobility/management skills. A transfer board or hydraulic lift may be necessary as UE function deteriorates. Attention to good sitting posture and pressure-relief techniques is essential to maintain alignment and prevent skin breakdown. Patients should be encouraged to balance time in the wheelchair with other activities, such as walking or exercising, and should be extra diligent in stretching muscles that tend to contract as a result of prolonged sitting (e.g., hip and knee flexors).

One of the constraints the therapist will have to deal with is financial reimbursement for the changing mobility needs of the patient with chronic MS. Private or public insurance organizations require a statement of medical necessity for payment. Because symptoms are not static in MS but rather typically exacerbate or remit, the therapist needs to provide clear and convincing documentation of need, stressing improved function and safety. Many third-party payers will not reimburse for new wheelchairs prescribed within specified time intervals or may be hesitant to finance expensive specialty wheelchairs such as the tilt-in-space chair or a second lightweight chair for traveling. The therapist will need to provide careful documentation of potential adverse outcomes to justify the cost of the new chair. For example, a likely deleterious outcome for a patient who is denied reimbursement for a tilt-in-space chair may be skin breakdown. The costs of nursing and surgical care for decubitus ulcers can then be compared to the cost of the new wheelchair, which can be justified as a preventive measure. It is equally important to anticipate future needs as they relate to rate of disease progression when ordering equipment.

Functional Training

Functional training should focus on problem solving and the development of appropriate decision making skills required to meet the challenges of being disabled. Skills should be adapted and practiced to ensure safe performance in both the home and community environments. Training in functional mobility skills (e.g., bed mobility, transfers, locomotion) is typically directed by the physical therapist whereas ADL (e.g., dressing, personal hygiene, bathing, toileting, grooming, and feeding) and IADL (e.g., cooking, laundry, and bed making) training is directed by the occupational therapist and training in communication skills by the speech-language pathologist. Close communication and coordination among team members is necessary to ensure that training methods are consistently applied and successful. Full participation of the patient in all phases of planning and training will increase personal involvement while decreasing dependency and passivity.

The majority of patients with MS will use multiple adaptive devices. This requires careful attention to appropriate prescription of devices and environmental modifications to assist the patient in conserving energy and maintaining function. Adaptive equipment can include bed or bathroom grab bars, overhead trapeze, raised seats, transfer board, or hydraulic lift. Appropriate positional and functional splints to facilitate writing or typing and plates and cups with lips to minimize spills are often helpful in assisting with hand function. Long-handled shoehorns, reachers, button hooks, sock aids, or Velcro^® closures can assist in dressing. Effective communication may require built-up writing utensils or a universal cuff for written communication or more sophisticated computerized devices. Patients with severe speech problems may require voice amplification devices, electronic aids, or computer-assisted alternative communication systems. The team must recognize when a device is indicated, and assist the patient in acceptance and in learning how to use the device before significant deterioration of function occurs.

Management of Speech and Swallowing

Impairments in communication and swallowing have been identified in individuals with MS.¹⁶³ The scientific literature reports that 44% of MS patients experience impairments of speech and voice in the early onset of their disease, and as many as 35% to 43% of MS patients can acquire voice, chewing, and swallowing disorders.¹⁶⁴ Respiratory deconditioning, characterized by reduced diaphragmatic support and shortening of the intercostal muscles, contributes to speech disorders and increases the likelihood of respiratory infections. Thus, collaborating with a speech-language pathologist to develop a resistive breathing training (RBT) program paired with activities to improve trunk stability, head control, and sitting balance is an important component of the POC for patients with MS. Improved respiration can be facilitated through the implementation of prolonged phonation exercises, resistive breathing exercises, and incentive spirometry. The therapist should focus on diaphragmatic and segmental chest expansion, expiratory training, and volitional and effortful coughing.¹⁶⁵

When dysphagia or difficulty in swallowing occurs, physical therapists should work closely with speech-language pathologists to preserve safety of swallowing. Often a detailed examination is needed to investigate the deceptively complex swallowing mechanism. Diagnostic methods include Videofluoroscopic Swallowing Studies (VFSS) and Flexible Endoscopic Evaluation of Swallowing (FEES).¹⁶⁶ The role of the physical therapist is important in assisting with improving sitting position, body posture, and head control. An upright body posture with a slightly forward and downward-pointing chin position can be helpful in achieving a safe swallow and preventing aspiration.¹⁶⁷ Application of transcutaneous neuromuscular electric stimulation (NMES) to the submental muscles (suprahyoid triangle) to facilitate muscle reeducation was cleared by the FDA in late 2002, after the submission of data from over 800 patients (adults and children). NMES of the submental muscles paired with selected oral–motor exercises and swallowing maneuvers (e.g., Mendelsohn maneuver, effortful swallow, super-supraglottic swallow) can improve the strength, ROM, and coordination of the swallowing musculature.¹⁶⁵

Thermal-tactile stimulation (TTS) is a sensory technique whereby stimulation is provided to the anterior faucial pillars to improve swallowing reflexes and the pharyngeal phase of swallowing. Anecdotally, the use of cold and icy beverages such as a shakes, fruit slushes, and ice chips provide heightened sensory input, which can improve the initiation of swallowing for many patients. Some patients also benefit from alternating small (teaspoon sized) sips of liquids with their food during mealtime. Most patients should be discouraged to engage in consecutive swallowing because it increases demand on the respiratory system and decreases airway protection. Resistive sucking through a straw can also be helpful. Thick liquids such as honey-thick and nectar-thick liquids can provide some resistance to facilitate muscle strengthening.¹⁶⁸ Moist foods (with sauces, broth, water, or milk) are easier to manage than dry ones. Semisolid and pureed foods are easier than regular solids. Foods that irritate the throat (e.g., vinegar) and crumbly or stringy foods (e.g., cake, cookies, potato chips, celery, cheeses) should be avoided. Patients also benefit when instructed to focus their effort on eating and never attempting to talk during active eating (mastication). Maintaining a quiet and peaceful environment during meals is helpful in improving attention and focus. Fatigue can also affect food intake. Many patients with MS benefit from reducing the size of their meals and eating smaller, more nutrient-dense meals throughout the day. Percutaneous endoscopic gastrostomy (PEG) feeding tubes and/or nasogastric tubes may become medically necessary for individuals with severe dysphagia.¹⁶⁹ For overall safety, it is important that family members, caregivers, and health care providers alike are educated in the use of the Heimlich maneuver in the event of an emergency.¹⁷⁰

Cognitive Training

Cognitive impairments can present major difficulties for the patient and for the rehabilitation team in general. Referral to a neuropsychologist may be indicated to determine the patient's strengths and weaknesses and to assist in the adaptive process. Compensatory strategies for memory deficits can be helpful. These include the use of memory aids, timing devices, and environmental strategies. Memory can be assisted by using a memory notebook to log daily events and reminders. With the increasing availability of mobile technologies, mobile devices have proven helpful for individuals with cognitive impairment related to MS. Trials have shown that use of a personal digital assistant (PDA) device significantly improves functional performance of everyday tasks. Patients who have incorporated mobile devices into their daily routines note improvement in organization and self-efficacy that positively affected their daily lives.¹⁷¹ A pill dispenser can assist the patient in maintaining a correct medication schedule. Cueing devices such as an alarm clock, bell timer, or watch alarm can help patients remember when to do certain tasks (e.g., taking medications, performing pressure relief). Structuring and labeling the environment is also an effective strategy to assist memory (e.g., labeled drawers, cabinets). Directions for functional tasks (e.g., transfers, self-stretching techniques) should be carefully written down for both patients and caregivers. Complex tasks can be broken down with clear written directions provided for each step. Directions can be posted in different areas of the home (e.g., steps to follow for toilet or tub transfer posted in the bathroom). Additional cognitive strategies that may be helpful include mental rehearsal, requesting assistance, maximizing alertness, avoidance of difficult situations, and mental exercises. Poor follow-through should be expected among patients with severe cognitive deficits, because often there is very little insight. In this situation, the efforts of family and caregivers must be fully maximized.¹⁷

Cognitive-behavioral therapy (CBT) for patients with MS can yield significant improvement in the ability to deal with distress, debilitating symptoms, impairment and disease exacerbation, and progression.¹⁷² As with healthy individuals, regular physical activity can have a positive effect on cognitive function, self-efficacy, and quality of life.^173,174 A computer-based home training program used in conjunction with other treatments has been shown to improve function in patients with MS.¹⁷⁵

Individuals with MS and their families experience a variety of losses such as loss of social functioning, interpersonal relationships, employment status, independence, and functional skills. Disabilities emerge as the disease progresses over time. Various different psychosocial adaptations can be seen, including anger, denial, depression, and so forth. The unique feature of a relapsing-remitting disease course is that it requires continual readjustment every time a new set of symptoms appears. Patients who appear well adjusted at one stage may regress as the disease worsens. The uncertainty of MS produces significant cognitive and emotional stress. Patients often feel out of control and unsure of themselves. Living with MS requires not only initial acceptance, but also a tremendous flexibility to deal with this lack of closure. Patients also experience the cumulative effects of smaller, everyday stresses that are associated with fluctuating symptoms, inability to perform ADL, dependency on others, architectural barriers, and so forth. Many factors play a role in determining how an individual reacts to MS. These include the overall effect of the disease on daily life functioning, previous coping skills, perceived self-efficacy, extent of social support, and spiritual well-being. They may experience attitudes of "wait and see" or "nothing can be done." This may explain why many individuals with MS do not take medications to help control their MS despite medical guidelines recommending disease-modifying drugs. The longer they are influenced by these attitudes, the less likely they are to seek help. Learned helplessness, low self-efficacy, and lack of environmental mastery have been identified as major factors contributing to depression and fatigue.²⁰

As mentioned, depression does not necessarily correlate to the severity of the disease. For example, a person with mild disease can be severely depressed, whereas the person with severe disability is not. The therapist must be alert to the signs of depression and intervene as appropriate. Chapter 26, Psychosocial Disorders, presents a complete discussion of this topic.

Despite the negative psychosocial effects of the disease, studies indicate that while initial diagnosis was met with negative reactions, over time positive changes in terms of values and outlook often occur. Interventions that target reexamination of the individual's role and identity result in an increased appreciation for life and can assist individuals with MS in better managing the disease and enjoying their lives.¹⁷⁶ Self-efficacy is the belief that an individual will be able to deal with particular situations that may contain novel, unpredictable, and stressful elements. Strategies that enhance self-efficacy and self-management (elements of CBT) empower the patient with MS.¹⁷⁷ Additional interventions include education, involvement in goal-setting and treatment planning, wellness forums, and support or psychotherapy groups. The use of stress reduction techniques (e.g., relaxation techniques, meditation, and exercise) can also be helpful in promoting effective coping. Family and multidisciplinary support are key elements in effective psychosocial management.^178,179

The primary roles of the clinician can be categorized as caring professional, expert teacher, and competent practitioner. A positive, affirming attitude can effectively influence patients' attitudes and assist them to view rehabilitation from a more positive perspective. The development of a strong collaborative relationship with the patient and family/caregivers in which there is respect, compassion, and effective communication is key to successful rehabilitation outcomes.¹⁷⁷ The overall focus should be on the maintenance of hope and encouragement tempered with realism.

As an educator, the therapist has an important role in assisting the patient and family/caregivers in providing information on the following:

• The disease process, clinical manifestations, and their significance in terms of management

• Prevention of secondary complications, indirect impairments, and activity limitations

• The rehabilitation process, the POC, and its specific interventions

• The HEP, including interventions that can be carried out independently

• Monitoring the effects and possible adverse reactions of medications

• Use of assistive devices and adaptive equipment

• General health and stress management techniques

• Community resources

Prompt referral to community resources including a support group can provide a necessary stabilizing base for patients and their families/caregivers. Within this environment individuals can gain accurate and useful information about the disease, discuss common problems and methods of coping, and share anxieties and resources. Thus, it provides a valuable forum to assist in the continual adjustment process. The National Multiple Sclerosis Society (www.nationalmssociety.org) provides education, emotional support, and a variety of programs and services to individuals with MS and their families through their local chapters.¹⁸⁰ Web-based resources are provided in Appendix 16.D.

A significant number of patients with MS (one out of every two patients) will require the assistance of another person at some point in the course of their disease. This places an extra burden on family members and on the financial resources of the patient if outside caregivers must be utilized. The majority of caregivers experience moderate levels of stress associated with their caregiving duties. As the level and duration of physical care increases, caregivers can experience a variety of signs and symptoms, including physical (e.g., fatigue, headache, sleep disturbances, appetite changes), psychological (e.g., anxiety, depression, frustration), social (e.g., family conflicts, decreasing social experiences or "lack of life"), and spiritual changes (e.g., hopeless and meaningless life and work).¹⁸¹ This can also put stress on relationships, as the caregiver is often a spouse and the dependency and the degree of support needed to perform certain tasks can be a source of tension.¹⁸² The therapist will need to be sensitive to these changes and to conflicts, problems, and tensions as they develop. Considerable time and energy will be devoted to counseling and educating caregivers and coordinating home management. Attention should also be paid to the children of patients with MS, because studies have indicated that parental MS has a negative impact on children's psychological well-being. This is due, in part, to lack of understanding about the disease, and education may help ameliorate some of the negative effects.¹⁸³

Timely referral to neurorehabilitation services is the key to successful management of activity limitations, disability, and quality-of-life issues in patients with MS. Too often services are not begun until the individual becomes severely disabled. A comprehensive POC that addresses the needs of the whole patient and emphasizes meaningful functional activities, patient education, and self-management is ideal for such a complex neurodegenerative disorder. Activities that prove attainable and safe ensure patient success and build self-efficacy. Many patients with MS report that they lack the knowledge and skills needed to exercise safely. Promoting self-efficacy, self-management, and mastery can be achieved through supervised programs that focus on regular exercise, activity pacing, energy conservation, and overall healthy behaviors. Comprehensive efforts of the interdisciplinary team are needed to provide the coordinated and continuing care required with anticipated inpatient, outpatient, and home/community episodes of care.

Acknowledgment

The authors wish to thank Marissa A. Barrera, MS, MPhil, MSCS, CCC-SLP for her contributions to this chapter.

Questions for Review

1. What are the pathophysiological processes involved in MS? What are the primary areas of CNS involvement?

2. Differentiate among the various disease courses (clinical subtypes) of MS.

3. How is the diagnosis of MS established? What tests and measures are used to confirm the diagnosis?

4. What is the role of disease-modifying drugs used in the medical management of MS? What are their indications and potential adverse effects?

5. Discuss the Expanded Disability Status Scale (EDSS) for patients with MS and indications for use.

6. Discuss the guidelines for an effective exercise prescription for the patient with MS to improve strength and conditioning.

7. Discuss the guidelines for an effective exercise prescription for the patient with MS to improve aerobic performance.

8. Discuss the problem of fatigue in MS and how it influences the design of an exercise program.

9. What strategies can be used to assist in the psychosocial adjustment of the patient with relapsing-remitting MS?

HISTORY

The patient is a 27-year-old graduate student who was admitted to an acute care facility with a chief complaint of double vision for 2 weeks. She reported that both lower extremities (LEs) seemed weaker recently. Four months earlier, she had noticed persistent tingling of her fingers on the left hand and some numbness on the left side of her face.

Neurological examination showed a scotoma in the upper field of the left eye, weakness of the left medial rectus muscle, horizontal nystagmus on left lateral gaze, and mild weakness of the left central facial muscles. All other muscles had normal strength. The deep tendon reflexes were normal on the right and brisk on the left, and there was a left extensor plantar response. The sensory system was unremarkable. A diagnosis of suspected MS was made. The patient was discharged a few days later, seemingly improved after corticosteroid treatment.

The patient was readmitted to a neurological service 10 months later because she noticed increased difficulty in walking and her speech had become thickened.

NEUROLOGIST REPORT

Patient presents with wide-based ataxic gait, minor slurring of speech, bilateral tremor in the finger-to-nose test, and dysdiadochokinesia. CT scan is within normal limits. MRI scan reveals numerous white areas indicative of lesions. Lumbar puncture shows 56 mg of protein with increased level of gamma-globulin. All other CSF findings are normal. Treatment with high doses of intravenous corticosteroids seemed to improve the neurological symptoms. Patient was discharged home with a referral for outpatient rehabilitation.

Two months later, the patient's symptoms worsened and she is now admitted for intensive rehabilitation.

MEDICATIONS

Prednisone 20 mg po qid

Maalox 30 mL po qid

Valium 10 mg qid

SOCIAL HISTORY

Patient has been living on her own for several years until her recent illness. She has taken a medical leave from graduate school and had returned home to live with her parents. They are both supportive and would like some advice as to how to modify their two-story home. There are five entry stairs with a handrail on both sides. There is a first floor bathroom, and they plan to convert the first floor study into a bedroom. Her parents are both in their early 60s, in good health, and very anxious about their daughter's rapidly deteriorating condition.

PHYSICAL THERAPY EXAMINATION FINDINGS

Mental Status

• Alert, oriented

• Memory: minimal impairment

• At times lacks insight, seems unaware of the seriousness of her condition

• Euphoric at times; other times she is depressed and cries easily

Communication

• Speech is dysarthric, difficult to understand at times

Vision

• Transient double vision

• Gaze-evoked nystagmus to both left and right

• Ocular dysmetria

• Upper field defect of left eye

Endurance/Fatigue

• Moderate impairment

• Tolerance to activity is approximately 10 minutes before rest is required

Skin

• WNL except for small bruise on right lateral malleolus

ROM

• WNL except for 0° right dorsiflexion; 0° to 5° left dorsiflexion

Tone

• Moderate extensor spasticity (2 on the modified Ashworth Scale) in both lower extremities (BLEs), left greater than right

• Occasional extensor spasms, which are a major safety risk when they occur during transfers

Sensation

• Paresthesias in BLEs with moderate proprioceptive losses, ankle joints greater than proximal joints

• Both upper extremities (BUEs): mild decrease in light touch, left greater than right

Strength

• Moderate weakness in BLEs; generally functional muscle grades (able to move against gravity), with the greatest weakness noted at the hips

• Standard MMT positions not used owing to spasticity

• BUEs 3+/5 (fair+) to 4/5 (good) strength

Coordination

• BUEs: Intention tremors with mild limb ataxia; voluntary movements are hypermetric

• RAM are moderately impaired

• BLEs: Movements restricted by spasticity and spasms; unable to test

Balance

• Sitting balance:

  • Static: With eyes open (EO), able to maintain position independently up to 5 minutes with minimal postural tremor; with eyes closed (EC), truncal ataxia is pronounced

  • Dynamic: With EO, able to weight shift to left and right to about 40% of limits of stability (LOS); with EC, experiences loss of balance (LOB) with minimal weight shifts

    Standing balance:

  • Static: Able to maintain standing position in parallel bars with min assist × 1 for up to 3 minutes; during standing, patient is unable to maintain centered alignment; demonstrates moderate postural tremor; with EC, sway is increased dramatically and patient quickly loses her balance

  • Tends to keep her hips and knees stiff in extension/hyperextension

  • Dynamic: Unable to weight shift or step without bilateral handhold

Functional

• Functional Independence Measure (FIM):

  • Eating: FIM 6; requires adaptive equipment

  • Grooming: FIM 6; requires adaptive equipment

  • Bathing: FIM 5; requires setup and adaptive equipment

  • Dressing—upper and lower: FIM 4; minimal assist

  • Toileting: FIM 4; minimal assist for balance

  • Sphincter control—bladder FIM 6; bowel FIM 6

  • Transfers—bed, chair, wheelchair: FIM 4, minimal contact assist for stand pivot transfers

  • Transfers—toilet and tub: FIM 4; minimal contact assist

  • Locomotion—walk: FIM 4; minimal contact assist, uses walker

  • Locomotion—stairs: FIM 2; less than 4 to 6 stairs, maximal assist

  • Locomotion—wheelchair: FIM 5; supervision, uses manual wheelchair for distances up to 150 ft; posture in wheelchair: sacral sitting

  • Requires a lap belt due to extensor spasms, which can cause her to fling out of the chair

  • Communication—expression: FIM 6; requires extra time, mild dysarthria

  • Communication—comprehension: FIM 6; complete understanding, requires extra time for processing

  • Social interaction: FIM 7

  • Problem solving: FIM 6; requires extra time, slight difficulty initiating decisions

  • Memory: FIM 6; slight difficulty remembering daily routines and executing requests without need for repetition

Expanded Disability Status Scale (EDSS) score: 6.5

PATIENT'S GOALS

• She would like to regain ambulation skills and independent living status. She recognizes the need to live with her parents for the time being but sees this as only temporary.

1. Develop a problem list: identify/categorize this patient's problems in terms of direct impairments, indirect impairments, functional limitations, and disability.

2. Identify two outcomes (the remediation of functional limitations and disability) and two goals (remediation of impairments) for this patient.

3. Formulate four treatment interventions that could be used at the start of therapy to achieve the stated outcomes and goals. Provide a brief rationale for each.

4. What strategies can be used to develop self-management skills and promote self-efficacy and quality of life?

Fatigue is a feeling of physical tiredness and lack of energy that many people experience from time to time. But people who have medical conditions like MS experience stronger feelings of fatigue more often and with greater impact than others.

Following is a list of statements that describe the effects of fatigue. Please read each statement carefully, then circle the one number that best indicates how often fatigue has affected you in this way during the past 4 weeks. (If you need help in marking your responses, tell the interviewer the number of the best response.) Please answer every question. If you are not sure which answer to select, choose the one answer that comes closest to describing you. Ask the interviewer to explain any words or phrases that you do not understand.

Name: _____________________________      Date: _____/_____ /_____

ID#: _____________________________       Test: 1 2 3 4

Because of my fatigue during the past 4 weeks…

Instructions for Scoring the MFIS

Items on the MFIS can be aggregated into three sub-scales (physical, cognitive, and psychosocial), as well as into a total MFIS score. All items are scaled so that higher scores indicate a greater impact of fatigue on a person's activities.

Physical Subscale

This scale can range from 0 to 36. It is computed by adding raw scores on the following items: 4 + 6 + 7 + 10 + 13 + 14 + 17 + 20 + 21.

Cognitive Subscale

This scale can range from 0 to 40. It is computed by adding raw scores on the following items: 1 + 2 + 3 + 5 + 11 + 12 + 15 + 16 + 18 + 19.

Psychosocial Subscale

This scale can range from 0 to 8. It is computed by adding raw scores on the following items: 8 + 9.

Total MFIS Score

The total MFIS score can range from 0 to 84. It is computed by adding scores on the physical, cognitive, and psychosocial subscales.

FUNCTIONAL SYSTEMS

Pyramidal Functions

0. Normal.

1. Abnormal signs without disability.

2. Minimal disability.

3. Mild or moderate paraparesis or hemiparesis; severe monoparesis.

4. Marked paraparesis or hemiparesis; moderate quadriparesis; or monoplegia.

5. Paraplegia, hemiplegia, or marked quadriparesis.

6. Quadriplegia.

V. Unknown.

Cerebellar Functions

0. Normal.

1. Abnormal signs without disability.

2. Mild ataxia.

3. Moderate truncal or limb ataxia.

4. Severe ataxia, all limbs.

5. Unable to perform coordinated movements due to ataxia.

V. Unknown.

X. Is used throughout after each number when weakness (grade 3 or more on pyramidal) interferes with testing.

Brainstem Functions

0. Normal.

1. Signs only.

2. Moderate nystagmus or other mild disability.

3. Severe nystagmus, marked extraocular weakness, or moderate disability of other cranial nerves.

4. Marked dysarthria or other marked disability.

5. Inability to swallow or speak.

V. Unknown.

Sensory Functions (revised 1982)

0. Normal.

1. Vibration or figure-writing decrease only, in one or two limbs.

2. Mild decrease in touch or pain or position sense, and/or moderate decrease in vibration in one or two limbs; or vibratory decrease alone in three or four limbs.

3. Moderate decrease in touch or pain or position sense, and/or essentially lost vibration in one or two limbs; or mild decrease in touch or pain and/or moderate decrease in all proprioceptive tests in three or four limbs.

4. Marked decrease in touch or pain or loss of proprioception, alone or combined, in one or two limbs; or moderate decrease in touch or pain and/or severe proprioceptive decrease in more than two limbs.

5. Loss (essentially) of sensation in one or two limbs; or moderate decrease in touch or pain and/or loss of proprioception for most of the body below the head.

6. Sensation essentially lost below the head.

V. Unknown.

Bowel and Bladder Functions (revised 1982)

0. Normal.

1. Mild urinary hesitancy, urgency, or retention.

2. Moderate hesitancy, urgency, retention of bowel or bladder, or rare urinary incontinence.

3. Frequent urinary incontinence.

4. In need of almost constant catheterization.

5. Loss of bladder function.

6. Loss of bowel and bladder function.

V. Unknown.

Visual (or Optic) Functions

0. Normal.

1. Scotoma with visual acuity (corrected) better than 20/30.

2. Worse eye with scotoma with maximal visual acuity (corrected) of 20/30 to 20/59.

3. Worse eye with large scotoma, or moderate decrease in fields, but with maximal visual acuity (corrected) of 20/60 to 20/99.

4. Worse eye with marked decrease of fields and maximal visual acuity (corrected) of 20/100 to 20/200; grade 3 plus maximal acuity of better eye of 20/60 or less.

5. Worse eye with maximal visual acuity (corrected) less than 20/200; grade 4 plus maximal acuity of better eye of 20/60 or less.

6. Grade 5 plus maximal visual acuity of better eye of 20/60 or less.

V. Unknown.

X. Is added to grades 0 to 6 for presence of temporal pallor.

Cerebral (or Mental) Functions

0. Normal.

1. Mood alteration only (does not affect DSS score).

2. Mild decrease in mentation.

3. Moderate decrease in mentation.

4. Marked decrease in mentation; chronic brain syndrome: moderate.

5. Dementia or chronic brain syndrome: severe or incompetent.

V. Unknown.

Other Functions

0. None.

1. Any other neurological findings attributed to MS (specify).

V. Unknown.

EXPANDED DISABILITY STATUS SCALE (EDSS)

INSTRUCTIONS

1. At the top of the day's diary, describe how you slept the night before.

2. Assign a number value from 1 to 10 (1 being very low and 10 being very high) for:

   • Your level of fatigue (F)

   • The value or importance of the activity you are doing (V)

   • The satisfaction you feel with your performance of the activity (S)

     You can compute the "value" of an activity by comparing it to other activities you would like to do during the course of the day.

     For example:

     1 PM: F = 7 V = 3 S = 2 Activity: Fixing lunch standing 15 minutes (hot); Comment: Blurred vision

3. Always describe the physical work done in the Activity section (e.g., stood to shower 10 minutes, went up 20 stairs, walked 200 feet).

4. Note the external temperature of the environment under Activity.

5. List under Comment all MS symptoms as they appear or worsen during the day, including cognitive problems, visual problems, weakness, dizziness, dragging foot, pain, numbness, burning, and so forth.

6. Make notes every hour.

Name: _________________________________________ Date: _____________________________

Describe sleep last night: ______________________________________________________________