Amyotrophic Lateral Sclerosis (ALS)

Question 1

What is ALS

and

What causes ALS

Answer 1

Chronic degenerative disease that produces (BOTH) upper and lower motor neuron impairments

Significant loss of:
- anterior horn cells in the spinal cord
and
- motor cranial nuclei in lower brainstem produces:

• Weakness*
• Muscle Atrophy*

Question 2

What attributes to UMN signs and what can this cause?

Answer 2

Demyelination of corticospinal and corticobulbar tracts.

Causes:
- denervation of muscles fibers
- muscle atrophy and weakness

Question 3

Etiology

Answer 3

-UNKOWN-

Theories:
- Genetic Inheritance (autosomal dominant trait)
- Slow acting virus
- Metabolic Disturbances
- toxicity of lead and aluminium

Question 4

Who has a higher incidence rate men or women?

Answer 4

• Higher incidence in men

Question 5

Typical Age of Onset?

Answer 5

• disease typically begins between 40-70 y/o

Question 6

Signs and Symptoms (LMN)

Answer 6

LMN Signs:
- asymmetrical muscle weakness
- fasciculations
- cramping
- atrophy in hands
(Weakness: Distal > Proximal)

Question 7

Signs and Symptoms (UMN)

Answer 7

UMN Signs:
- incoordination of movement
- spasticity
- clonus
- (+) Babinski Reflex

Question 8

Signs and Symptoms (Other)

Answer 8

• Fatigue
• Oral Motor Impairments
• Motor Paralysis
• Eventual Respiratory Paralysis

Question 9

Treatment Options

Answer 9

• Supportive Care
• Symptom Management

All branches of rehab are involved in focusing on increase QOL.

Question 10

What is bulbar involvement characterized by?

Answer 10

• Dysarthria
• Dysphagia
• Emotional Lability: rapid, often exaggerated changes in mood, where strong emotions or feelings (uncontrollable laughing or crying, or heightened irritability or temper) occur.

Question 11

What laboratory or imaging studies would confirm diagnosis?

Answer 11

Electromyography: assesses fibrillation and muscle fasciculations.

Muscle Biopsy: to verify lower motor neuron involvement

Spinal Tap: May reveal higher protein content

CT Scan: May appear normal until late in the disease process (so not as helpful).

Question 12

What is a primary indicator of ALS?

Answer 12

A patient that presents with motor impairment (without) sensory impairment is a primary indicator of ALS.

Question 13

What remains preserved throughout the course of ALS?

Answer 13

• Sensation
• Eye Movement
• Bowel and Bladder Function

Question 14

What pharmacological intervention may be available?

Answer 14

Riluzole (Rilutek)
- may have an affect on the progression of the disease
- long term effects unknown

Question 15

Physical therapy and ALS

Answer 15

• Low-Level Exercise
• Energy conservation techniques

HEP may be indicated; however patients SHOULD NOT exercise to fatigue; promoting further weakness

• PT does not hinder progression of ALS

Question 16

What is the average course of the disease?

Answer 16

Two to Five Years

(25% of patients surviving longer than 5 years)

Question 17

What is the main cause of death for patients with ALS?

Answer 17

Respiratory Failure

Question 18

Does ALS produce symmetrical or asymmetrical muscle weakness?

Answer 18

Asymmetrical Muscle Weakness