Early onset Huntington disease: a neuronal degeneration syndrome

  title={Early onset Huntington disease: a neuronal degeneration syndrome},
  author={Sara H Seneca and Dominique Fagnart and Kathelijn Keymolen and Willy Lissens and Dani{\`e}le Hasaerts and Sara Debulpaep and Brigitte Desprechins and Inge Liebaers and Linda J De Meirleir},
  journal={European Journal of Pediatrics},
Huntington disease (HD) is an autosomal dominant, lethal neurodegenerative disorder of the central nervous system, caused by an uncontrolled expansion of a CAG dynamic mutation in the coding region of the IT15gene. Although a majority of patients have a midlife onset of the disease, in a small number of patients the disease manifests before 20 years of age. In adults, HD is mainly characterised by involuntary movements, personality changes and dementia. By contrast, in children a dominant… CONTINUE READING
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A new model for prediction of the age of onset and penetrance for Huntington ’ s disease based on CAG length

  • BR Leavitt, CL Wellington, MR Hayden
  • Clin Genet
  • 2004

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