Motor Neuron Diseases

Question 1

Spinal muscular atrophy

Answer 1

5q gene deletion
premature death of alpha motor neurons in SC and brainstem
weakness of muscles

Question 2

Type 1 SMA

Answer 2

acute infantile
never sits, cant control head, can’t suck/cry
fatal

Question 3

Type 2 SMA

Answer 3

chronic infantile
delayed milestone, but can sit (not walk)
variable prognosis

Question 4

Type 3 SMA

Answer 4

chronic juvenille
can stand and walk but can’t go up stairs. may lose ability to walk, develop scoliosis, and bulbar dysfunction
typical life expectancy

Question 5

Type 4 SMA

Answer 5

adult
lose ability to walk
typical life expectancy

Question 6

Post polio

Answer 6

virus attacks motor neurons
adult (30s)
affects muscles previously affected

Question 7

ALS

Answer 7

degeneration of coricospinal tract UMN and LMN

distal then proximal weakness

Question 8

Classifications of ALS

Answer 8

suspected ALS: UMN or LMN only in 1 or more regions
probable ALS: LMN and UMN signs in 2 regions
definite ALS: LMN and UMN signs in 3 regions