huntington’s disease i: symptoms and circuits neurobiology of disease

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

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Bevan et al. 2002 TINS 25:525 Hamani et al. 2004 Brain 127:4

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Somer 2003

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Up and Down States

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Stern et al. 1997J. Neurophys. 77:1697

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