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Research paper
Risk factors for spinal cord lesions in dystonic cerebral palsy and generalised dystonia
  1. Emilie Guettard1,2,
  2. Damien Ricard3,
  3. Emmanuel Roze1,2,
  4. Alexis Elbaz4,
  5. Mathieu Anheim1,2,5,
  6. Stéphane Thobois6,
  7. Jean-Francois Lepeintre7,
  8. Damien Galanaud8,
  9. Christian Mazel9,
  10. Marie Vidailhet1
  1. 1AP-HP, Department of Neurology, Groupe Hospitalier Pitié-Salpêtrière, Paris, France
  2. 2Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière (CRICM), CNRS UMR 7225, UPMC Université Paris 6/Inserm UMR_S 975, AP-HP, Paris, France
  3. 3Service de Neurologie, Hôpital d'Instruction des Armées du Val-de-Grâce, Paris, France
  4. 4INSERM, U708, Neuroepidemiology, Paris, France
  5. 5Department of Medical Genetics, Groupe Hospitalier Pitié-Salpêtrière, Paris, France
  6. 6Université Lyon I, Hospices Civils de Lyon, Hôpital Neurologique Pierre Wertheimer, Service de Neurologie C, Lyon, France
  7. 7Neurosurgery Department, Hôpital FOCH, Suresnes Cedex, France
  8. 8Department of Neuroradiology, Groupe Hospitalier Pitié-Salpêtrière, Paris, France
  9. 9Université Paris XIII, Institut Mutualiste Montsouris, Département de Chirurgie Orthopédique, 42 Boulevard Jourdan, Paris, France
  1. Correspondence to Professor Marie Vidailhet, Department of Neurology, CRICM UPMC/Inserm UMR_S 975, CNRS UMR 7225, Pitié-Salpêtrière Hospital, Paris, France; marie.vidailhet{at}psl.aphp.fr

Abstract

Background Cervical myelopathy (CM) in patients with cerebral palsy (CP) is underdiagnosed as symptoms of spinal cord lesions, being similar to those due to dystonia, may be overlooked or identified late. The aim of this study is to identify the risk factors and clinical characteristics of CM in patients with generalised dystonia, including dystonic CP.

Methods The authors conducted a case–control study to identify early clinical signs of CM in consecutive patients with generalised dystonia. The authors compared the clinical characteristics and symptoms of those who developed CM (cases) and those who did not (controls). The same clinical information on possible neurological manifestations of CM was collected for cases and controls at the date of the last visit.

Results Out of 54 patients, 17 (31%) developed symptomatic CM during the study period. In all cases, CM occurred after the age of 36 years. 81% of cases and 35% of controls had a Burke–Fahn–Marsden movement subscore for the neck >4. Age (OR per 10 years=2.3, 95% CI 1.4 to 4.2, p=0.006) and severity of neck dystonia (OR=7.7, 95% CI 1.7 to 49.6, p=0.005) were the main risk factors of CM. Gait disorders and falls, wasting of hand muscles and bladder disorders were the best clinical clues of CM.

Conclusions As severity of cervical dystonia and age are the major risk factors for spinal cord lesions, dystonic patients, including patients with dystonic CP, should be screened for CM from the third decade of life onwards. Early recognition of CM is crucial for functional prognosis and impact on autonomy.

  • Cervical myelopathy
  • cerebral palsy
  • cervical dystonia
  • generalised dystonia
  • spinal cord lesion
  • neurooncology, dystonia
  • chorea
  • paediatric movement disorders
  • Gilles de la Tourette
  • Tourette syndrome
  • cerebellar ataxia
  • cerebellar degeneration
  • heredit spastic paraplegia
  • Parkinson's disease
  • cerebellar disease
  • clinical neurology
  • Parkinson
  • functional imaging
  • movement disorders
  • pet
  • motor control
  • *new 1998

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Footnotes

  • Competing interests None.

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

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