Flashcards in 13: HD Clinical Deck (9):
HD motor dysfunction examples
motor impersistence (can't maintain sustained muscular contraction). eye movement abnormalities. dystonia, parkinsonism. incoordination, unsteadiness, immobility, dysarthria, dysphagia
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
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
experimental HD treatment?
ACR16 - DA modulating drug
goal in HD treatment
delay/prevent onset of symptomatic HD in the asymptomatic individual
2 animal models
intrastriatal kainic acid or quinolic acid
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.
inhibits mitochondrial succinate dehydrogenase, blocked by NMDA-antagonists. associated with striatal pathology similar to HD