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Benign Hereditary Chorea

  • Roongroj Bhidayasiri
  • Daniel Tarsy
Chapter
Part of the Current Clinical Neurology book series (CCNEU)

Abstract

Benign hereditary chorea (BHC) is a relatively rare autosomal dominant disorder which produces childhood onset chorea, usually under age 5, with a stable or only slightly progressive course. In some cases, chorea decreases in adolescence or early adulthood. The clinical picture is apparently not identical in all families and myoclonus, dystonia, gait disturbance, and tremor have also occasionally been present. Linkage to a region on chromosome 14q13.1-q21.1 has been reported in several families. It is possible that the phenotype may also be due to other yet unidentified genetic causes. Unlike Huntington’s disease (HD), BHC is not associated with behavioral changes or dementia. Brain MRI is normal.

Keywords

Behavioral Change Primary Care Physician Clinical Picture Early Adulthood SPECT Imaging 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Supplementary material

Benign Hereditary Chorea.mp4 (MP4 16,584KB)

The patient displays continuous low-amplitude and largely distal choreiform movement of her arms, hands, fingers, ankles, and toes.

References

  1. 1.
    Schrag A, Quinn NP, Bhatia KP, Marsden CD. Benign hereditary chorea-entity or clinical syndrome? Mov Disord. 2000;15:80–288.Google Scholar
  2. 2.
    Breedveld GJ, Percy AK, MacDonald ME, et al. Clinical and genetic heterogeneity in benign hereditary chorea. Neurology. 2002;59:579–84.PubMedCrossRefGoogle Scholar
  3. 3.
    Maahajnah M, Inbar D, Steinmetz A, et al. Benign hereditary chorea: clinical, neuroimaging, and genetic findings. J Child Neurol. 2007;22:1231–4.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  • Roongroj Bhidayasiri
    • 1
    • 2
  • Daniel Tarsy
    • 3
  1. 1.Chulalongkorn Center of Excellence on Parkinson’s Disease and Related DisordersChulalongkorn University HospitalBangkokThailand
  2. 2.Department of NeurologyDavid Geffen School of Medicine at UCLALos AngelesUSA
  3. 3.Department of NeurologyHarvard Medical School Beth Israel Deaconess Medical CenterBostonUSA

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