Motor Neuron Diseases Flashcards

(29 cards)

1
Q

T/F motor neuron diseases can affect UMN and LMN

A

True

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2
Q

Motor neuron diseases can be both what?

A

Inherited or sporadic

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3
Q

Examples of motor neuron diseases

A

ALS
Post-polio syndrome
Spinal muscular atrophy

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4
Q

Symptoms of ALS

A

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

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5
Q

Structures affected in ALS

A

motor cortex
corticospinal and corticobulbar tracts
brainstem
anterior horn spinal neurons

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6
Q

Etiology of ALS can be both what?

A

Familial (10%)
Sporadic (90%)

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7
Q

Pathophysiology of ALS

A

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

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8
Q

Typical onset presentation of ALS

A

Spinal/Limb onset
Bulbar onset

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9
Q

Spinal/Limb onset

A

asymmetric, painless weakness of a limb

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10
Q

Bulbar onset

A

dysarthria, dysphagia, tongue twitching

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11
Q

Negative symptoms of ALS

A

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

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12
Q

Factors for longer survival times in ALS

A

younger
spinal/limb onset
UMN predominance

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13
Q

Factors of shorter survival times in ALS

A

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

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14
Q

Onset age in PLS

A

younger

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15
Q

Limbs affected first in PLS

A

lower extremities

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16
Q

Rate of progression in PLS

17
Q

What function is spared in PLS?

A

respiratory function

18
Q

Is PLS fatal?

A

Not necessarily, average course lasts >20 years

19
Q

Describe PLS

A

UMN degeneration with LMN sparing

20
Q

Describe PMA

A

LMN degeneration with UMN sparing

21
Q

Rate of progression of PMA

A

slightly slower than ALS

22
Q

Progression of brachial amyotrophic diplegia

A

Typically proximal to distal

23
Q

Progression of leg amyotrophic diplegia

A

Typically asymmetrical, distal to proximal progression

24
Q

Describe PBA

A

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

25
Kennedy's Disease is also known as
Spinal Bulbar Muscular Atrophy
26
Symptoms of Kennedy's disease
Muscular weakness and cramping (proximal > distal) Dysarthria and/or Dysphagia Fasciculations Tremor Enlarged breasts and/or infertility
27
Symptoms of post-polio syndrome
Progressive weakness and atrophy Fatigue (general and musc) Breathing/Swallowing problems Cold intolerance Muscle and joint pain
28
Treatment considerations in PPS
Joint protection Energy conservation strategies Fall prevention Light to Mod aerobic intensity
29
Describe SMA
Family of inherited motor neuron disorders