ALS Flashcards
ALS
Amyotrophic Lateral Sclerosis
ALS Etiology
Progressive neuro disorder characterized by the loss of motor neurons
-upper + lower motor neurons
Death usually around 3 years after dx. (RF generally)
Unknown etiology
ALS: RF
- 40-60yo
- Male
- Genetics (10%)
- Smoking
ALS: Pathogenesis
Motor neurons in the brainstem and spinal cord gradually degenerate
Electrical and chemical messages from the brain do not reach the muscles
ALS: what could cause it?
Excitatory Hypothesis
-Excessive levels of glutamate initiate a cascade that leads to neuron death
Glutamate: Excitatory NT
Why do we think this?
- Elevated glutamate levels in the CSF
- Antiglutaminergic drug (Riluzole) improves survival
Clinical Manifestations
Weakness of upper extremities
- Sometimes begins in legs
- Muscle wasting
- Spasticity
- Dysarthria (trouble speaking), dysphagia, drooling
- Gate
- Cognitive and behavioral changes
- Constipation
- Sleep problems
- Breathing issues
Riluzole (Rilutek)
Class: Glutamate inhibitor
Indication: only drug for ALS
MOA: Glutamate Antagonist
-Reduces damage to neurons
SE: dizziness, GI upset, Hepatotoxicity
Does increase life expectancy (3-6 months)
