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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 El Escorial and Awaji-Shima diagnostic criteria for ALS.

  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 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 taken into account 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). Amyotrophic lateral sclerosis (ALS),* commonly known as Lou Gehrig’s disease, is the most common and devastatingly fatal MND among adults. “Pure” 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 It is important to note that ALS is increasingly being considered a multisystem disorder or syndrome, with variable pathological involvement of extra-motor networks and connections, in addition to the LMNs and UMNs.

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 upper motor neurons
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

*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’s disease.

EPIDEMIOLOGY

It is estimated that 30,000 individuals in the United States have ALS at any one time and 15 cases are diagnosed every day. Except in a very few high-incidence areas, such as Guam, Western New Guinea, and the Kii Peninsula of Japan (geographical foci, Western Pacific form of ALS), the overall incidence of ALS ...

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