ALS

Question 1

What is ALS?

Answer 1

Amyotrophic lateral sclerosis - a rare, progressive neuro disorder characterized by the loss of upper and lower motor neurons.

Question 2

What is the survival rate of ALS?

Answer 2

Death usually occurs around 3 years after diagnosis

Question 3

What is the etiology of ALS?

Answer 3

Unknown

Question 4

What are the risk factors for ALS?

Answer 4

40-70 Y, male, genetics play a small role (~10%), smoking

Question 5

What is the patho behind ALS?

Answer 5

Motor neurons in the brainstem / spinal cord gradually degenerate therefore electrical and chemical messages from the brain cannot reach the muscles.

Question 6

What type of neurotransmitter is glutamate?

Answer 6

Excitatory neurotransmitter

Question 7

What is the potential role that glutamate plays in ALS?

Answer 7

Excessive levels of glutamate initiate a cascade of events that lead to neuronal death.
Elevated glutamate levels in CSF are seen with patients with ALS and anti-glutaminergic drugs improve the survival rate.

Question 8

What are the clinical manifestations of ALS?

Answer 8

Weakness of upper extremities (sometimes begins in the legs, involves spasticity), dysarthria, dysphagia, drooling, cognitive / behavioral changes, constipation, sleep problems, breathing problems (most common cause of death is respiratory failure)