13: HD Clinical Flashcards Preview

NEURO 410 B > 13: HD Clinical > Flashcards

Flashcards in 13: HD Clinical Deck (9):
1

HD motor dysfunction examples

motor impersistence (can't maintain sustained muscular contraction). eye movement abnormalities. dystonia, parkinsonism. incoordination, unsteadiness, immobility, dysarthria, dysphagia

2

HD: pathology - 3 main things? degree of pathology related to?

striatal atrophy. neuronal loss + gliosis. diffuse cortical changes, primarily frontal. degree of pathology realted to duration of symptomatic HD

3

selective neuronal loss in HD?

striatal PROJECTIOn neurons affected aka medium spiny neurons that are GABAergic. ones projecting to GPe and SN are most vulnerable while those to GPi degenerate more gradually. interneurons spared

4

experimental HD treatment?

ACR16 - DA modulating drug

5

goal in HD treatment

delay/prevent onset of symptomatic HD in the asymptomatic individual

6

2 animal models

intrastriatal kainic acid or quinolic acid

7

excitotoxic hypothesis?

intrastriatal injection of excitotoxic amino acids mimics characteristic pathology of HD; toxicity can be prevented by NMDA antagonists BUT acute striatal lesion unlike slow cell loss associated with NDDs.

8

2 NPA

inhibits mitochondrial succinate dehydrogenase, blocked by NMDA-antagonists. associated with striatal pathology similar to HD

9

HD: impaired mito. energy metabolism = ?

increased neuronal sensitivity to endogenous glutamate = excitotoxicity and loss of neurons