PMID: 11921119Mar 29, 2002Paper

Selective loss of striatal preprotachykinin neurons in a phenocopy of Huntington's disease

Movement Disorders : Official Journal of the Movement Disorder Society
E K RichfieldAnton Reiner

Abstract

Phenocopies of Huntington's disease (HD) are individuals with a family history, clinical symptoms, and occasionally pathological evidence of HD but without an expanded CAG repeat within the HD gene. We report on an HD phenocopy with selective loss of preprotachykinin (PPT) neurons, dysfunction of surviving PPT neurons, preservation of preproenkephalin (PPE) neurons within the striatum, and greater loss of immunohistochemical staining for substance P in terminals of striatal neurons projecting to the substantia nigra, than in those projecting to the internal pallidal segment. This case demonstrates the existence of one type of striatal lesion that may produce a clinical picture similar to HD, and raises the possibility of a rare hereditary disease that mimics HD.

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Citations

Jan 11, 2005·Neurotoxicity Research·Tomás PalomoRichard M Kostrzewa
Apr 15, 2003·The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques·M SuttonBrown, O Suchowersky
May 12, 2007·Brain Pathology·Marion Maat-SchiemanSjoerd van Duinen
Jul 18, 2008·Handbook of Clinical Neurology·Jean Paul G VonsattelMaria Del Pilar Amaya
Mar 12, 2005·Journal of the Neurological Sciences·Adrian DanekRuth H Walker
Dec 16, 2003·Movement Disorders : Official Journal of the Movement Disorder Society·Ruth H WalkerRussell L Margolis
Sep 8, 2006·Movement Disorders : Official Journal of the Movement Disorder Society·Ruth H WalkerEva Andermann
Nov 10, 2007·Current Opinion in Neurology·Edward J Wild, Sarah J Tabrizi

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