ALS

Question 1

motor deficits seen in ALS? Sensory? Exceptions?

Answer 1

UMN and LMN sparing the EOM. No sensory or cognitive deficits. No autonomic involvement

Question 2

presentation of ALS

Answer 2

fasciculations with eventual atrophy progressing to respiratory failure

Question 3

causes of ALS

Answer 3

defect in superoxide dismutase 1, Gain of function

Question 4

Treatment

Answer 4

Riluzole increases survival by decreasing presynaptic glutamate

Question 5

what tracts does ALS affect

Answer 5

bulbar as well as somatic

Question 6

ALS-frontotemporal dementia: caused by

Answer 6

in familial ALS, hexanucleotide repeat

Question 7

glutamate hypothesis

Answer 7

too much Glu which cant be efficiently taken up by transporters. Acts on NMDA and nonNMDA receptors