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Neurophysiological evidence for cerebellar dysfunction in primary focal dystonia
  1. J T H Teo1,
  2. B P C van de Warrenburg2,
  3. S A Schneider1,
  4. J C Rothwell1,
  5. K P Bhatia1
  1. 1
    Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, UK
  2. 2
    Department of Neurology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
  1. Dr J T H Teo, Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London WC1N 3BG, UK; jteo{at}ion.ucl.ac.uk

Abstract

Recent studies have suggested that there may be functional and structural changes in the cerebellum of patients with adult onset primary focal dystonia. The aim of this study was to establish whether there is any neurophysiological indicator of abnormal cerebellar function, using the classic eyeblink conditioning paradigm. This paradigm at short intervals is dependent on the olivo-cerebellar circuit and does not require cerebral and basal ganglia structures. Eyeblink conditioning was performed by pairing an auditory tone with a supraorbital nerve stimulus with a delay interval of 400 ms in 12 patients with primary focal dystonia (seven cervical dystonias, five focal hand dystonias) and eight healthy controls. Healthy controls produced more conditioned eyeblink responses than patients with focal dystonia, indicating an abnormality of associative learning in this patient population. This study provides neurophysiological evidence for functional changes in the olivo-cerebellar pathway of patients with primary focal dystonia. Further work needs to be done to determine if these changes are primary, secondary or epiphenomenal to the disease.

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Footnotes

  • A supplementary table is published online only at http://jnnp.bmj.com/content/vol80/issue1

  • Funding: JTHT was supported by the Wellington Fund, Institute of Neurology. SAS was supported by the Brain Research Trust. BPCvdeW was supported by the Niels Stensen Foundation and the Dr Jan Meerwaldt Foundation.

  • Competing interests: None.

  • Ethics approval: The study was carried out with the approval of the local ethics committee.

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