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Patients with primary cervical dystonia have evidence of discrete deficits in praxis
  1. Britt Sofie Hoffland1,2,
  2. Dorinda Snik1,2,
  3. Kailash P Bhatia1,
  4. Elena Baratelli1,
  5. Petra Katschnig1,3,
  6. Petra Schwingenschuh1,3,
  7. Sebastian Crutch4,
  8. Bart P van de Warrenburg2,
  9. Mark J Edwards1
  1. 1Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, UCL, London, UK
  2. 2Department of Neurology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
  3. 3Department of Neurology, Division of Special Neurology, Medical University Graz, Graz, Austria
  4. 4Dementia Research Group, Institute of Neurology, UCL, London, UK
  1. Correspondence to Dr M J Edwards, NIHR Clinician Scientist, Sobell Department, Institute of Neurology, UCL, Queen Square, London WC1N 3BG, UK; m.edwards{at}ion.ucl.ac.uk

Abstract

Background Functional imaging and electrophysiological data from patients with primary dystonia reveal widespread abnormalities in brain areas associated with higher motor functions but to date there has been little investigation of the functional consequences of these abnormalities. The aim of this study was to use a battery of tests of praxis, based on those tests used in routine clinical examination, to uncover evidence of higher motor dysfunction in patients with primary cervical dystonia.

Methods Praxis was assessed in 13 patients with primary cervical dystonia without hand involvement and in 29 age and sex matched controls. A semiquantitative praxis assessment was used which combined timed tests of meaningful and meaningless movements with copying of transitive and intratransitive hand movements and pantomime of tool use. Control tasks consisted of evaluation of motor speed, strength and a number of additional cognitive tasks.

Results Patients made significantly more errors in copying meaningless gestures and were slow in the performance of meaningless sequences of hand movements. Copying meaningful gestures and performance of meaningful sequences of hand movements were normal.

Conclusion This study has identified a discrete deficit in praxis in dystonia patients and suggests additional functional consequences from the widespread pathophysiological abnormalities seen in primary dystonia.

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Footnotes

  • Funding BPvdW and BSH are supported by a grant from the Prinses Beatrix Fonds. DS is supported by a scholarship from the Prinses Beatrix Fonds. MJE is supported by a grant from the National Institutes of Health Research. SC is supported by an Alzheimer's Research Trust Research Fellowship.

  • Competing interests None.

  • Ethics approval This project was approved by the Joint Ethics Committee of the Institute of Neurology and the National Hospital for Neurology and Neurosurgery.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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