Huntington’s Disease I: Symptoms and Circuits Neurobiology of Disease.

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Presentation transcript:

Huntington’s Disease I: Symptoms and Circuits Neurobiology of Disease

Extrapyramidal Motor Symptoms Hyperkinetic/hypokinetic Rigidity, bradykinesia, akinesia Tremor (resting-PD, intention-cerebellar) Dystonia (Torsion Spasm) Chorea (“dance”)/ choreatic dyskinesia / athetosis (“unfixed,” “changeable”) / Ballismus/hemiballismus Tics (Tourette)

“When either or both of the parents have shown manifestations of the disease…one or more of the offspring invariably suffer of the disease, if they live to adult life. But if by any chance these children go through life without it, the thread is broken and the grandchildren and great-grandchildren of the original shakers may rest assured that they are free from disease” George Huntington M.D. 1872

Bevan et al TINS 25:525 Hamani et al Brain 127:4

Somer 2003

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Up and Down States Stern et al J. Neurophys. 77:1697

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