ALS Flashcards
how common is ALS?
Most common form of spinocerebellar degenerative diseases
what is ALS?
progressive Degeneration of the neurons
Devastating neurologic disorder that selectively affects motor fx
what is the survival rate of ALS?
Survival up to 2-5 years after diagnosis
How does pt with ALS die typically?
Death comes secondary caused by respiratory failure
what is the average age of onset of ALS?
Between ages 40-70
What are the UMN (cerebral cortex) clinical manifestations?
Weakness Spasticity Impaired fine motor function Dysarthria (decreased articulation) Dysphagia (decreased swallowing) Dysphonia (decreased speaking)
what are the LMN (brainstem + anterior horn cells in spinal cord) clinical manifestations?
Fasciculations - twitching
Weakness
Muscle atrophy
Hyporeflexia (lack of reflexes)
what remains intact in ALS?
Sensory system Ocular movements Bowel function Bladder function Intellect
EVERYTHING BUT MOTOR
what is early pt presentation of ALS?
Progressive weakness and atrophy of distal muscles
Regional spread of clinical weakness to nearby spinal cord levels
Eventual UMN and LMN involvement
what is advanced pt presentation of ALS?
Motor muscles of the diaphram, palate, pharynx, tongue, neck and shoulders- therefore decreased respiration, chewing, swallowing and speech
what is worked on in rehab for ALS?
Quality of life issues
Supportive management esp. with immobility
Psychological support
what sex does ALS affect more?
2x as many men as women
what is the nutritional deficiency etiology of ALS?
disturbance in enzyme metabolism
Excessive glutamate response causing excitotoxicity killing UMN + LMN