(38) Motor Neuron Diseases Flashcards Preview

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Flashcards in (38) Motor Neuron Diseases Deck (16):
1

Spinal motor atrophy is called ___ in...
infants
juveniles
adults

infants = Werdnug-hoffman
juveniles = Wolfkart-Kugelberg
adults = adult onset spinal muscle atrophy

2

pediatric spinal motor atrophy is usually (sporadic, AR, AD) and adult onset is usually (sporadic, AR, AD)

kids = AR
adults = sporadic

3

mutation assc with Werdnug-hoffman? Wolfkart-Kugelberg

5q; absent SNM1

**size of SNM2 determines if see neonatal or juvenile form

4

spinal motor atrophy presents with (proximal or distal) weakness

proximal

5

spinal motor atrophy affects UMN or LMN

LMN
*fasiculations, hypotonia

6

pathophys of spinal mortor atrophy

anterior horn cells die leading to denervation

7

adult motor neuron disease that may progress to ALS

progressive bulbar palsy and progressive lateral sclerosis

8

tongue and palate weakness/atrophy

presentation of bulbar palsy or ALS

9

adult motor neuron disease that primarily affect UMN (little sign of atrophy or denervation)

progressive lateral sclerosis

10

pts with this may present with LMN lesion signs in upper limbs and UMN lesion signs in lower limbs

cervical spondylosis = protruding discs
*upper limb = nerve root compression
lower limb = compression of spinal cord and corticospinal tracts

11

anterior horn cell histo that suggests ALS

de-myelination, Bunina eosinophillic inclusions (ubiquitin accumulations)

12

etiology of ALS

most commonly SOD mut on chrom 21

familial ALS without FTD = expansion on chrom 9 of C90RF72 gene

13

likely cause of neuronal degeneration in ALS

glutamate mediated excitotoxicity secondary to free radical injury of neurons

14

what is the treatment for ALS

Riluzole (glutamate antagonist)

+ symptomatic relief (botox to dec drooling, stool softeners, anti-depressants, bracing)

15

ALS muscle weakness is usually first seen

in the hands

16

ALS preserves

sensory, bladder and bowel function