Syndrome of fixed dystonia in adolescents--short term outcome in 4 cases

Anirban Majumdar, Jesús López-Casas, Pilar Poo, Jaume Colomer, Marta Galvan, Lokesh Lingappa, Clare Short, Philip E Jardine, Emilio Fernández-Alvarez

Research output: Contribution to journalArticle (Academic Journal)peer-review

6 Citations (Scopus)

Abstract

We describe the clinical features, investigations and outcome of 4 adolescents aged 13, 16, 17 and 19 years, with fixed dystonia. The diagnosis was made within 6 months of the onset of symptoms. One patient had an identifiable traumatic precipitant. All the affected extremities had pain, sudomotor and vascular changes which were consistent with complex regional pain syndrome. The extremities affected by dystonia were the foot and the hand. The dystonia spread to affect other extremities in one patient. One patient had hemifacial spasm. Examination of the central and peripheral nervous system and allied investigations failed to reveal an organic cause. Common genetic causes for dystonia were excluded. The response to physical treatments for the affected extremities, such as Botulinum Toxin and surgery was poor. In all our cases there were significant psychological and psychiatric factors. Three patients fully met the criteria for psychogenic dystonia and responded well to psychological intervention. Fixed dystonia in adolescents is an uncommon disorder of unknown aetiology, usually presenting in girls, which can be very disabling and difficult to treat. The affected parts of the body are usually painful and show vascular changes. The condition is allied to CRPS. Treatment with multidisciplinary approach including psychological measures and physiotherapy is more likely to be successful and may prevent unnecessary physical measures.

Original languageEnglish
Pages (from-to)466-72
Number of pages7
JournalEuropean Journal of Paediatric Neurology
Volume13
Issue number5
DOIs
Publication statusPublished - Sep 2009

Keywords

  • Adolescent
  • Anxiety
  • Autonomic Nerve Block
  • Bites and Stings
  • Botulinum Toxins, Type A
  • Complex Regional Pain Syndromes
  • Diazepam
  • Dystonia
  • Female
  • Foot
  • Hemifacial Spasm
  • Humans
  • Magnetic Resonance Imaging
  • Mental Disorders
  • Muscle Relaxants, Central
  • Neurologic Examination
  • Obsessive-Compulsive Disorder
  • Tetany
  • Tomography, X-Ray Computed
  • Treatment Outcome
  • Young Adult
  • Case Reports
  • Journal Article

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