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Parkin gene related neuronal multisystem disorder
  1. MWIM Horstink1,
  2. BPC van de Warrenburg1,
  3. M Lammens2,
  4. A Brice3
  1. 1Department of Neurology, University Medical Centre, PO Box 9101, 6500 HB Nijmegen, The Netherlands
  2. 2Department of Pathology, University Medical Centre, PO Box 9101, 6500 HB Nijmegen, The Netherlands
  3. 3Hôpital de la Salpetriére, Paris, France
  1. Correspondence to;
 Dr MWIM Horstink;

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We read with much interest the article on Japanese patients with parkin gene related autosomal recessive juvenile parkinsonism (ARJP) complicated by cerebellar and pyramidal tract dysfunction.1 Recently, we described a Dutch family with parkin gene related ARJP showing typical levodopa responsive parkinsonism. The proband clinically had additional mild gait ataxia and pathologically showed—besides classic parkin gene related ARJP findings—neuronal loss in parts of the spinocerebellar system—namely, Purkinje cell layer, dentate nucleus, and gracile fascicles.2 Just as our Japanese colleagues, we suspected some kind of hereditary multiple system degeneration with predominant parkinsonism until genetic analysis indicated parkin gene related ARJP. Although the non-extrapyramidal abnormalities in the Japanese and in our patients could have been coincidental, the recent Japanese findings seem to confirm that the spinocerebellar and probably also other systems can be affected in parkin gene related ARJP. The fact that the Japanese patients did not respond to levodopa …

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