Differential diagnosis of chorea

S. A. Schneider, R. H. Walker, K. P. Bhatia

Research output: Chapter in Book/Report/Conference proceedingChapterpeer-review


Causes of chorea are multifold and include inherited (genetic) and acquired forms. Among the genetic disorders, Huntington's disease remains most important. However, other disorders with an indistinguishable clinical presentation are being increasingly recognized, and are referred to as the Huntington's disease like (HDL) syndromes. These include HDL1 due to mutation of the PRNP gene (chr. 20p12); HDL2 due to mutation of the JPH3 gene (chr. 16q24); HDL3 mapped to chr. 4 (gene unknown) and HDL4/SCA17 due to mutation of the TBP gene (chr. 6q27). Other disorders discussed here include dentatorubropallidoluysian atrophy; the spinocerebellar ataxias; neuroferritinopathy; pantothenate-kinase associated neurodegeneration; chorea-acanthocytosis and McLeod syndrome. We review the growing list of recognized genetic (and acquired) disorders and discuss the clinical approach and useful factors to arrive at the etiological diagnosis.

Original languageEnglish
Title of host publicationNeuroacanthocytosis Syndromes II
PublisherSpringer-Verlag Berlin Heidelberg
Number of pages22
ISBN (Electronic)9783540716938
ISBN (Print)9783540716921
StatePublished - 2008


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