ALS Flashcards

1
Q

how common is ALS?

A

Most common form of spinocerebellar degenerative diseases

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2
Q

what is ALS?

A

progressive Degeneration of the neurons

Devastating neurologic disorder that selectively affects motor fx

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3
Q

what is the survival rate of ALS?

A

Survival up to 2-5 years after diagnosis

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4
Q

How does pt with ALS die typically?

A

Death comes secondary caused by respiratory failure

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5
Q

what is the average age of onset of ALS?

A

Between ages 40-70

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6
Q

What are the UMN (cerebral cortex) clinical manifestations?

A
Weakness
Spasticity
Impaired fine motor function
Dysarthria (decreased articulation)
Dysphagia (decreased swallowing)
Dysphonia (decreased speaking)
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7
Q

what are the LMN (brainstem + anterior horn cells in spinal cord) clinical manifestations?

A

Fasciculations - twitching
Weakness
Muscle atrophy
Hyporeflexia (lack of reflexes)

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8
Q

what remains intact in ALS?

A
Sensory system
Ocular movements
Bowel function
Bladder function
Intellect

EVERYTHING BUT MOTOR

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9
Q

what is early pt presentation of ALS?

A

Progressive weakness and atrophy of distal muscles

Regional spread of clinical weakness to nearby spinal cord levels

Eventual UMN and LMN involvement

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10
Q

what is advanced pt presentation of ALS?

A

Motor muscles of the diaphram, palate, pharynx, tongue, neck and shoulders- therefore decreased respiration, chewing, swallowing and speech

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11
Q

what is worked on in rehab for ALS?

A

Quality of life issues
Supportive management esp. with immobility
Psychological support

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12
Q

what sex does ALS affect more?

A

2x as many men as women

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13
Q

what is the nutritional deficiency etiology of ALS?

A

disturbance in enzyme metabolism

Excessive glutamate response causing excitotoxicity killing UMN + LMN

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