Dentatorubral pallidoluysian atrophy in South Wales.

@article{Wardle2008DentatorubralPA,
  title={Dentatorubral pallidoluysian atrophy in South Wales.},
  author={Mark Wardle and Elisa Majounie and Nigel W. Williams and Anne Elizabeth Rosser and Huw R Morris and Neil P. Robertson},
  journal={Journal of neurology, neurosurgery, and psychiatry},
  year={2008},
  volume={79 7},
  pages={804-7}
}
BACKGROUND Dentatorubral pallidoluysian atrophy (DRPLA) is a rare, autosomal dominant, clinically heterogeneous neurodegenerative disorder characterised clinically by progressive dementia, ataxia, chorea, myoclonic epilepsy and psychiatric disturbance and pathologically by combined degeneration of the dentatorubral and pallidoluysian systems. DRPLA has a marked ethnic predilection, most commonly reported in Japan and thought to be rare in Caucasian populations. METHODS We describe the… CONTINUE READING
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