ALS Flashcards
What are other names for ALS?
- Lou Gehrigs disease
- Maladie de Charcot
What is ALS?
Mixed UMN disease
- Involvement of voluntary muscles, sparing the eye muscles & urinary sphincter
What is ALS caused by?
Degeneration of neurons in the cervical spine & medulla
- Etiology unknown
What is the pathology of ALS?
Degeneration of anterior horn in SC & in brain stem & corticospinal tracts
What is the defining feature of ALS?
Both upper & lower motor neurons in the motor cortex of brain, stem, SC
What do the motor neurons develop? What does it lead to?
Protein inclusion in the cell –> apoptosis
What causes high glutamate levels?
Cell death –> high levels of Ca due to high levels of excitatory neurotransmitters
What is an anti-glutamingergic drug?
Riluzole
- The only med tx for ALS
How does ALS present?
- Weakness, atrophy of the hands w/ spasticity & hyperreflexia of the lower motor neuro deficits
- Dysarthria & dysphagia
- Tongue atrophy & fasciculations
What are some of the “first” sx?
Drop foot
Walking
Speech
Dexterity
Bular onset ALS
Speech
Swallow
What sx occur in later stages?
Swallowing - aspiration
Intercostal - poor lung fxn
How do you dx ALS?
- No definite testing
- UMN & LMN signs
- MRI brain & cervical spine
- EMG
- CSF (protein elevation), HIV, Lyme
How do you tx ALS?
- Riluzole (ongoing): inhibits release of gluatmate
- Supportive (tracheostomy, gastrostomy)
What is the px of ALS?
Most die within 5 years & are dx around 50-60 years