ALS

Question 1

most-common adult-onset progressive motor neuron disease

Answer 1

ALS

Question 2

Familial ALS (FALS) present in what % of cases?

Answer 2

5-10%, inherited autosomal trait

Question 3

Sinaki & Mulder Stage I

Answer 3

Independent with mobility & ADLs
mild weakness, clumsiness
tx: home/work modification, psych support, sub-max exercise (watch fatigue)

Question 4

Sinaki & Mulder Stage II

Answer 4

Ambulatory, may need AD or other equipment
MODerate, selective weakness
slight decrease in independence with ADLs (ex: climbing stairs, raising arms)

Question 5

Sinaki & Mulder Stage III

Answer 5

Ambulatory, but may need WC with community mobility
isolated severe weakness (ex: foot drop, weak grip)
cervical muscle fatigue –> soft collar
tx: deep-breathing ex, chest stretching

Question 6

Sinaki & Mulder Stage IV

Answer 6

WC level mobility, perform some ADLs
severe weakness of UE or LE
tx: isometric contractions, motorized WC? monitor skin

Question 7

Sinaki & Mulder Stage V

Answer 7

WC dependent, difficulty moving in bed
Progressive weakness - severe LE, mod-severe UE
s/s: pain, fasciluations, mm spasms, spasticity
poor head control –> semi rigid collar
tracheostomy?

Question 8

Sinaki & Mulder Stage VI

Answer 8

bed-bound
progressive respiratory distress
modified diet, suction, airway clearance, “head drop”
tx: similar to Hospice care

Question 9

LMN signs

Answer 9

hyporeflexia, weakness, hypotonicity, fasciculations, mm cramps

Question 10

UMN signs

Answer 10

hyperreflexia, weakness, hypertonicity, pathologic reflexes (clonus, Babinski, Hoffman’s)

Question 11

Bulbar signs

Answer 11

dysphagia, dysarthria, pseudo bulbar palsy (emotional lability), siallorhea,