Amyotrophic Lateral Sclerosis (ALS) Flashcards

1
Q

What is ALS

and

What causes ALS

A

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*
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2
Q

What attributes to UMN signs and what can this cause?

A

Demyelination of corticospinal and corticobulbar tracts.

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

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3
Q

Etiology

A

-UNKOWN-

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

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4
Q

Who has a higher incidence rate men or women?

A
  • Higher incidence in men
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5
Q

Typical Age of Onset?

A
  • disease typically begins between 40-70 y/o
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6
Q

Signs and Symptoms (LMN)

A

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

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7
Q

Signs and Symptoms (UMN)

A

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

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8
Q

Signs and Symptoms (Other)

A
  • Fatigue
  • Oral Motor Impairments
  • Motor Paralysis
  • Eventual Respiratory Paralysis
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9
Q

Treatment Options

A
  • Supportive Care
  • Symptom Management

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

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10
Q

What is bulbar involvement characterized by?

A
  • 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.
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11
Q

What laboratory or imaging studies would confirm diagnosis?

A

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).

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12
Q

What is a primary indicator of ALS?

A

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

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13
Q

What remains preserved throughout the course of ALS?

A
  • Sensation
  • Eye Movement
  • Bowel and Bladder Function
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14
Q

What pharmacological intervention may be available?

A

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

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15
Q

Physical therapy and ALS

A
  • 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
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16
Q

What is the average course of the disease?

A

Two to Five Years

(25% of patients surviving longer than 5 years)

17
Q

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

A

Respiratory Failure

18
Q

Does ALS produce symmetrical or asymmetrical muscle weakness?

A

Asymmetrical Muscle Weakness