Amyotrophic Lateral Sclerosis (ALS)

Diagnosis

Chronic degenerative disease that produces both upper and lower motor neuron impairments

Symptoms are caused by demyelination, axonal swelling, and atrophy within the:

Cerebral cortex

Premotor areas

Sensory cortex

Temporal cortex

Damaged Structures

Rapid degeneration and demyelination in the giant pyramidal cells in the cerebral cortex, affecting:

Areas of the corticospinal tracts

Cell bodies of the lower motor neurons in the gray matter

Anterior horn cells

Areas within the precentral gyrus of the cortex

Rapid degeneration causes denervation of muscle fibers, muscle atrophy, and weakness

Contributing Factors

Genetic Inheritance as an Autosomal Dominant Trait

Slow-acting virus

Metabolic Disturbances

Risk is higher in men and usually occurs between 40 to 70 years of age

Clinical Presentation

Asymmetrical muscle weakness, cramping, and atrophy, usually found in the hands

Muscle weakness due to degeneration, eventually causing significant fasciculations, atrophy, and wasting of the muscles

Weakness spreads distal to proximal throughout the course of the disease

Incoordination of movement

Spasticity

Clonus

Positive Babinski Reflex

Dysarthria

Dysphagia

Emotional Lability

Fatigue

Oral motor impairment

Motor Paralysis

Eventual Respiratory Paralysis

Imaging and Tests

Electromyography to assess fibrillation and muscle fasciculations

Muscle biopsy to verify lower motor neuron involvement rather than muscle disease

Spinal tap may reveal a higher protein content in some patients with ALS

CT scan will appear normal until late in disease process

Likely Additional Findings

Paralysis of Vocal Cords

Swallowing Impairment

Contractures

Decubiti

Breathing difficulty that requires ventilatory support

Medical Management

Pharmacological intervention may include Riluzole (Rilutek), a drug that appears to have an effect on the progression of the disease; however, its long-term effects are unknown

Symptomatic therapy may include anticholinergic, antispasticity, and antidepressant medications

Physical, occupational, speech, respiratory, and nutritional therapies may be warranted

Physical Therapy Management

Focus on quality of life

Low-Level Exercise Program

ROM

Mobility Training

Assistive/Adaptive Devices

Wheelchair Prescription

Bronchial Hygiene

Energy Conservation Techniques

Patient, Family, and Caregiver Training/Education

Home care regimen

A low-level exercise program may be indicated as long as the patient does not exercise to fatigue

Family involvement is encouraged to support the patient through the course of the disease and assist with mobility, pacing skills, energy conservation techniques, and overall safety

During the late stage of disease, family and caregivers must be competent with positioning, bronchial hygiene, ROM, and assistance with mobility

Prognosis

Therapy may assist with current issues; however, it will not hinder the progression of ALS

ALS is usually a rapidly progressing neurological disease with an average course of 2-5 years, with roughly a quarter of patients surviving longer than 5 years

If a patient is diagnosed before 50 years of age, the disease usually follows a long-term course

Death usually occurs from respiratory failure