What is ALS?
A neurodegenerative disease causing progressive muscle weakness due to motor neuron degeneration
What is another name for ALS?
Lou Gehrig’s disease
What is the cause of ALS?
Unknown
Is ALS fatal?
Yes
What is the usual cause of death in ALS?
Respiratory failure
Average survival after diagnosis of ALS?
~3 years
Typical age range for ALS diagnosis?
40–70 years (peak 60–69)
Which gender is more affected by ALS?
Males
Is there a genetic component to ALS?
Yes (familial form ~10%)
What lifestyle factor is associated with ALS?
Smoking
What neurons are affected in ALS?
Upper and lower motor neurons
Where does motor neuron degeneration occur?
Spinal cord
What happens after motor neuron death?
Axonal degeneration
What is the excitotoxicity hypothesis?
Excess glutamate leads to neuron death
What neurotransmitter is involved in ALS pathophysiology?
Glutamate
What was found elevated in ALS patients?
Glutamate in CSF
Initial symptoms of ALS?
Muscle weakness from upper/lower motor neuron dysfunction
What is the spinal form of ALS?
Weakness begins in arms/legs and progresses
Early motor symptoms of ALS?
Spasticity
Progressive symptoms of ALS?
Muscle atrophy and worsening weakness
Bulbar symptoms of ALS?
Difficulty chewing
Other ALS symptoms?
Cognitive/behavioral changes
Final stage complication of ALS?
Respiratory failure
What drug is FDA-approved for ALS?
Riluzole
