ALS

Question 1

what is ALS

Answer 1

most common MND - aka lou gehrig’s

rapidly progressive neurodegenerative that affects MN and corticospinal tracts

combined upper and lower motor signs

Question 2

UMN findings in ALS

Answer 2

spasticity

hyperreflexia

pathological reflex

Question 3

LMN findings in ALS

Answer 3

muscle weakness

atrophy

fasciculations

cramps

abdominal cramps - red flag

Question 4

bulbar findings in ALS

Answer 4

spastic dysarthria: monotone and low pitch; UMN

flaccid dysarthria: wet and nasal; LMN

dysphagia

PBA

respi dysfunction

Question 5

what is ALS cachexia

Answer 5

weight loss, atrophy and reduced calorie intake

reduced calorie intake - d/t dysphagia

atrophy - d/t muscles not being used since MN is damaged

Question 6

hierarchy of initial sx in ALS

Answer 6

leg weakness - balance prob and sprains

arm weakness - deltoids

bulbar

generalized weakness - rare

Question 7

_____ is a prominent feature of ALS

Answer 7

fasciculations that develop insidiously and slowly

Question 8

usually normal in ALS

Answer 8

mental status

sensory CN function

sensation

cerebellar - except balance prob d/t weakness

CN 3,4,6

onufrowicz nuclues - bowel and bladder

spinocerebellar tracts

Question 9

onset if classic ALS

Answer 9

UMN, LMN and bulbar

Question 10

onset if progressive bulbar palsy

Answer 10

bulbar

Question 11

onset if progressive lateral sclerosis

Answer 11

UMN onset

Question 12

onset if progressive muscular atrophy

Answer 12

LMN

Question 13

epidemiology in ALS

Answer 13

M > F but if F affected worse prog

58 yo - rare adolescence

Question 14

inc risk of ALS

Answer 14

smokers > alcohol

high fat intake

high glutamate intake

Question 15

discuss progressive/primary lateral sclerosis

Answer 15

affects corticospinal and spares LMN

spinal onset and rarely bulbar

initial: LE spasticity –> pathological reflexes and detrusor hyperactivity

LACK OF LMNL sx

Question 16

discuss hereditary spastic paraplegia

Answer 16

affects CST and DCML - mild to mod

progressive spasticity
LE weakness
hypertonic bladder
impaired vibration

Question 17

discuss tropical spastic paraparesis

Answer 17

usually in tropical areas - carribean, equatorial areas, mga south

optic neuropathy, cerebellar ataxia or polyneuropathy

viral - (+) serology

Question 18

discuss progressive muscular atrophy

Answer 18

chromatolysis of MN of SC and brainstem - ant horn cell

distal leg weakness and atrophy –> UE>LE

bulbar wala initially

Question 19

discuss progressive bulbar palsy

Answer 19

affects CN 9 and 10

dysphagia, dec gag, weakness of palate, facial and tongue fasciculations

pneumonia - usual cause of death

Question 20

parts of CNS usually affected by ALS

Answer 20

motor cortex

corticospinal tracts

ant horn cell

Question 21

poor prognosticating factors of ALS

Answer 21

> 40-60 yo

bulbar or pulmonary affectation

short tine bet onset and dx

predominance of LMN

women

Question 22

pharmacologic tx for ALS

Answer 22

riluzole - 100 mg qd

can only slow

Question 23

main characteristic of ALS treated by PT

Answer 23

weakness and fatigue

Question 24

resistance training in ALS

Answer 24

12 wk mod resistance program

but not to point of exhaustion

Question 25

signs of over exhaustion

Answer 25

weaker 30 mins p exercise

excessive soreness 24-48 hrs p exercise

severe cramps, heaviness and shortness of breath

Question 26

aerobic training in ALS

Answer 26

improves physical and mental

pool therapy - midchest height at 92-95F

low impact aerobics

in-patient: hallway amb