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Chapter 17: Amyotrophic Lateral Sclerosis

Vanina Dal Bello-Haas

LEARNING OBJECTIVES

LEARNING OBJECTIVES

  1. Describe the epidemiology, risk factors, etiology, pathogenesis, diagnosis, and general prognosis of amyotrophic lateral sclerosis (ALS).

  2. Compare and contrast the Milano-Torino (MiToS) functional staging and King's clinical staging systems for staging disease progression.

  3. Differentiate among impairments related to lower motor neuron, upper motor neuron, and bulbar pathology.

  4. Discuss the medical and health-care management of individuals with ALS.

  5. Outline a framework for rehabilitation for individuals with ALS.

  6. Describe the components of the physical therapy examination for ALS.

  7. Describe the role of the physical therapist in the management of individuals with ALS and the factors that influence intervention options.

  8. Compare and contrast overwork damage and disuse atrophy as it relates to ALS.

  9. Describe considerations that must be considered when designing an exercise program for an individual with ALS.

  10. Describe common impairments associated with ALS and the physical therapy interventions to address these impairments.

  11. Determine the goals and expected outcomes for an individual with ALS based on physical therapist examination findings.

  12. Design a physical therapy plan of care for the individual with ALS.

INTRODUCTION

Motor neuron diseases (MNDs) include a heterogeneous spectrum of inherited and sporadic (no family history) clinical disorders of the upper motor neurons (UMNs), lower motor neurons (LMNs), or a combination of both1 (Table 17.1).1 Amyotrophic lateral sclerosis (ALS)*, also known as Lou Gehrig disease, is the most common and devastatingly fatal MND among adults. ALS is typically characterized by the degeneration and loss of motor neurons in the spinal cord, brainstem, and brain, resulting in a variety of UMN and LMN clinical signs and symptoms.2 Clinical, genetic, postmortem, neuropathological, and imaging evidence supports that ALS should be regarded as a multisystem, heterogeneous disorder or syndrome rather than solely a motor disease, with variable involvement of extramotor networks and connections.

Table 17.1Motor Neuron Disorders
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Table 17.1 Motor Neuron Disorders

Subtype

Nervous System Pathology

Amyotrophic lateral sclerosis

Degeneration of the corticospinal tracts, neurons in the motor cortex and brainstem, and anterior horn cells in the spinal cord

Primary lateral sclerosis

Degeneration of UMNs

Progressive bulbar palsy

Degeneration of motor neurons of cranial nerves IX to XII

Progressive muscular atrophy

Loss or chromatolysis of motor neurons of the spinal cord and brainstem

Adapted in part from Rowland.1

UMN = upper motor neurons.

* The term MND is used to describe the disease in the United Kingdom, whereas the term ALS is used in North America and Europe. In Europe, ALS is also called Charcot disease.

EPIDEMIOLOGY

The worldwide incidence of ALS has been reported to be 0.6 to 3.8 cases per 100,000 people per year,3 with incidence differing based on ancestral origin; for example, the incidence rate for populations of European descent is about 2.1 to ...

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