20 - MN disease Flashcards Preview

NEURO 410 B > 20 - MN disease > Flashcards

Flashcards in 20 - MN disease Deck (21):
1

signs of MN degeneration: upper vs. lower

upper = some weakness, normal bulk, no fasiculations, increased tone and reflexes aka spasticity. lower = lots of weakness, atrophy, fasiculations, decreased tone and reflexes

2

3 anatomic syndromes

UMN = primary lateral sclerosis. LMN = progressive muscular atrophy. upper and lower = ALS

3

clinical features of ALS? what is affected? what is spared? survival?

muscle wasting, weakness, fasiculations, spasticity, cramps. limb, bulbar and respiratory affected. spared: sensation, sphincter function. 2 - 5 years.

4

bulbar clinical features

speech. chewing. swallowing. coughing, chocking, excess saliva. weight loss. dehydration.

5

lower extremity clinical features

foot drop. difficulty with chairs and stairs

6

upper extremity clinical features

hand: find motor activities like writing. shoulder: frozen shoulder, proximal arm weakness. axial: stooped posture

7

respiratory clinical features

dyspnea aka shortness of breath. orthopnea aka difficulty breathing when lying down. sleep disordered breathing

8

cognitive clinical features? prevalence? results?

frontotemporal: executive dysfunction and behavioural impairment. up to 50%. impacts decision making capacity + reduced survival.

9

VBM in findings in ALS?

decreased grey matter volume in motor cortex, frontal and temporal lobes

10

MRS findings in ALS?

decreased NAA (neuronal marker). increased glial marker mI. decrease NAA to mI ratio. so degeneration, but increase glia.

11

extra-motor pathology: where?

frontotemporal lobar degeneration

12

spectrum of ALS and FTD: linked by?

molecular link: ubiquinated cytoplasmic inclusions in FTD and ALS both contain TDP 43 protein

13

most common genetic cause

C9ORF72

14

MRI findings for ALS?

qualitative: low sensitivity + specificity but will see atrophy, hyperintensity of corticospinal tract and hypointense ribbon in motor cortex

15

goal of disease management?

maximize quality of life + death

16

disease modifying treatment: drug name? effect?

riluzole: antiglutamatergic, prolongs survival 3 - 6 months

17

3 important interventions in ALS

BiPAP (ventilation). PEG (feeding tube). multisiciplinary care.

18

ceftriaxone: what? effects?

beta-lactam antibiotic: stimulates glutamate transporter. didn't work in clinical trials but had been promising in in vivo/vitro neuroprotection

19

no effective treatment yet...what is required?

objective, sensitive + indirect measure of disease burden and drug efficacy: biomarkers

20

take home: ALS is a ___ disease? what poses challenges to finding treatment?

multisystem disease. clinical and pathogenic heterogeniety pose challenges to finding treatment.

21

take home: important goal? 4 important interventions?

maximize quality of life. riluzole, biPAP, PEG, multidisciplinary care