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

Unlike MS this is for old bitches; MS 20-35(40)

Question 6

Signs and Symptoms (LMN)

Answer 6

LMN Signs:
- asymmetrical muscle weakness
- fasciculations
- cramping
- atrophy in hands

Question 7

Signs and Symptoms (UMN)

Answer 7

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

Question 8

Signs and Symptoms (Other)

Answer 8

• Fatigue
• Oral Motor Impairments; bulbar symptoms
• Motor Paralysis with 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

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

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

Physical therapy and ALS; guidelines for an HEP?

Answer 14

• Low-Level Exercise
• Energy conservation techniques

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

Question 15

What is the average course of the disease?

Answer 15

Two to Five Years

(25% of patients surviving longer than 5 years)

Question 16

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

Answer 16

Respiratory Failure

Question 17

Does ALS produce symmetrical or asymmetrical muscle weakness?

Answer 17

Asymmetrical Muscle Weakness

Question 18

What may a spinal tap reveal?

Answer 18

Higher than normal protein count.

Question 19

Is weakness found more distally or proximally?

Answer 19

Distal > Proximal

Question 20

Symptoms in which a worse prognosis may be indicated?

Answer 20

patient often have a worse prognosis with bulbar and respiratory weakness such as swallowing difficulties