Motor Neuron Diseases

Question 1

T/F motor neuron diseases can affect UMN and LMN

Answer 1

True

Question 2

Motor neuron diseases can be both what?

Answer 2

Inherited or sporadic

Question 3

Examples of motor neuron diseases

Answer 3

ALS
Post-polio syndrome
Spinal muscular atrophy

Question 4

Symptoms of ALS

Answer 4

extremity, trunk, and/or bulbar weakness
muscle twitching, spasticity, and/or cramping
dysarthria and/or dysphagia
respiratory compromise

Question 5

Structures affected in ALS

Answer 5

motor cortex
corticospinal and corticobulbar tracts
brainstem
anterior horn spinal neurons

Question 6

Etiology of ALS can be both what?

Answer 6

Familial (10%)
Sporadic (90%)

Question 7

Pathophysiology of ALS

Answer 7

complex interaction of environmental and genetic factors disrupt metabolic pathways
(hard to pinpoint)

Question 8

Typical onset presentation of ALS

Answer 8

Spinal/Limb onset
Bulbar onset

Question 9

Spinal/Limb onset

Answer 9

asymmetric, painless weakness of a limb

Question 10

Bulbar onset

Answer 10

dysarthria, dysphagia, tongue twitching

Question 11

Negative symptoms of ALS

Answer 11

lack of progression
sensory impairment
visual decline
bowel/bladder dysfunction
imaging, emg r/o

Question 12

Factors for longer survival times in ALS

Answer 12

younger
spinal/limb onset
UMN predominance

Question 13

Factors of shorter survival times in ALS

Answer 13

older
bulbar or respiratory onset
onset of FTD and/or behavior changes
LMN predominance

Question 14

Onset age in PLS

Answer 14

younger

Question 15

Limbs affected first in PLS

Answer 15

lower extremities

Question 16

Rate of progression in PLS

Answer 16

slower rate

Question 17

What function is spared in PLS?

Answer 17

respiratory function

Question 18

Is PLS fatal?

Answer 18

Not necessarily, average course lasts >20 years

Question 19

Describe PLS

Answer 19

UMN degeneration with LMN sparing

Question 20

Describe PMA

Answer 20

LMN degeneration with UMN sparing

Question 21

Rate of progression of PMA

Answer 21

slightly slower than ALS

Question 22

Progression of brachial amyotrophic diplegia

Answer 22

Typically proximal to distal

Question 23

Progression of leg amyotrophic diplegia

Answer 23

Typically asymmetrical, distal to proximal progression

Question 24

Describe PBA

Answer 24

Degeneration of lower cranial nerves (IX-XII) with subsequent limb involvement

Question 25

Kennedy’s Disease is also known as

Answer 25

Spinal Bulbar Muscular Atrophy

Question 26

Symptoms of Kennedy’s disease

Answer 26

Muscular weakness and cramping (proximal > distal)
Dysarthria and/or Dysphagia
Fasciculations
Tremor
Enlarged breasts and/or infertility

Question 27

Symptoms of post-polio syndrome

Answer 27

Progressive weakness and atrophy
Fatigue (general and musc)
Breathing/Swallowing problems
Cold intolerance
Muscle and joint pain

Question 28

Treatment considerations in PPS

Answer 28

Joint protection
Energy conservation strategies
Fall prevention
Light to Mod aerobic intensity

Question 29

Describe SMA

Answer 29

Family of inherited motor neuron disorders