Last reviewed: November 2017
Last updated: February  2017

Summary

Definition

History and exam

Key diagnostic factors

  • simultaneous contraction of agonist and antagonist muscles
  • muscle pain
  • appearance or worsening of dystonia with action
  • blepharospasm
  • cervical torticollis
  • hand spasms
  • foot spasms

Other diagnostic factors

  • twisting of the affected body part
  • geste antagoniste (sensory trick)
  • spread to another body part
  • parkinsonism
  • myoclonus
  • tremor, weakness, or spasticity
  • reflex sympathetic dystrophy
  • Kayser-Fleischer rings on slit-lamp examination
  • acute presentation (within 5 days of exposure to antidopaminergic agent)
  • acute worsening of pre-existing generalized dystonia

Risk factors

  • FHx of dystonia
  • repetitive activity of affected region
  • birth injury and delayed development in childhood
  • exposure to antidopaminergic agents
  • trauma
  • genetic mutation
  • Ashkenazi Jewish ethnicity
  • structural lesion of the basal ganglia
  • parkinsonian syndrome

Diagnostic investigations

1st investigations to order

  • levodopa responsiveness
Full details

Investigations to consider

  • cranial MRI
  • serum ceruloplasmin
  • 24-hour urine copper
  • DYT1 gene testing
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Ludy C. Shih

Assistant Professor of Neurology

Beth Israel Deaconess Medical Center

Harvard Medical School

Boston

MA

Disclosures

LCS is an unpaid secretary/treasurer at the Tremor Research Group. LCS receives funding from the NIH Dystonia Coalition project for participant enrollment.

Director of the Huntington's Disease Clinic

Instructor of Neurology

Parkinson's Disease and Movement Disorders Center

Beth Israel Deaconess Medical Center

Boston

MA

Disclosures

SF is employed by the Beth Israel Deaconess Medical Center, which has received funding from Allergan to support partial fellowship training.

Dr Ludy C. Shih and Dr Samuel Frank would like to gratefully acknowledge Dr David K. Simon and Dr Daniel Tarsy, previous contributors to this monograph. DKS has received consulting fees from the Gerson Lehrman Group. DKS is an author of a reference cited in this monograph. DT has received an unrestricted grant from Allergan and Medtronic being used for education. He received unrestricted funds for patient education from Allergan, Boehringer Ingelheim, Valeant, and Teva Neurosciences. He has received research funds from Solvay and Neurogen. DT is an author of a number of references cited in this monograph.

Peer reviewers VIEW ALL

Reader in Clinical Neurology

Honorary Consultant

Institute of Neurology

National Hospital for Neurology and Neurosurgery

London

UK

Disclosures

PDL declares that she has no competing interests.

Assistant Professor

Director

Movement Disorder Center

Dept of Neurology

University of Florida

Tampa

FL

Disclosures

ZH has been a consultant for Allergan and a speaker for Novartis and TEVA.

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