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Dystonia

Last updated: December 21, 2020

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Dystonia is a movement disorder characterized by sustained or intermittent muscle contractions causing involuntary movements, fixed postures, or both. The disorder may be idiopathic, hereditary, or acquired. Acquired dystonia is most commonly due to drugs (antipsychotics), metabolic disorders, cerebrovascular disease, or traumatic brain injury. Dystonia is classified according to the anatomic distribution of the symptoms into focal, multifocal, segmental, and generalized dystonia. Treatment involves pharmacologic management with levodopa or anticholinergic agents, as well as treatment of the underlying cause if possible. Focal dystonias respond well to periodic botulinum toxin injections in the affected muscle.

Most cases of acute dystonia are caused by antipsychotic drugs.

Dystonia disorders are classified according to the distribution of symptoms in the body.

Focal dystonia

  • Affects a single region of the body
  • The majority of cases are idiopathic.
  • Onset typically in adulthood (usually > 30 years)
  • Patients may have sensory tricks (geste antagonistes) with which they are able to suppress spasms (e.g., touching the affected region).

Conditions

  • Spasmodic torticollis: cervical dystonia
    • Abnormal head movements or fixed head posture
    • Geste antagoniste: a voluntary maneuver that temporarily reduces the severity of dystonic postures or movements
  • Blepharospasm: eye dystonia: (e.g., increased blinking or involuntary eye closure); usually bilateral, symmetrical
  • Spasmodic dysphonia: laryngeal dystonia or voice dystonia
    • Voice breaks or strained voice (most common) [2]
    • Weak, breathy voice
  • Oromandibular dystonia: : involuntary movements of the tongue, jaw, and/or face (e.g., jaw clenching, jaw opening)
  • Writer's dystonia (writer's cramp): non-painful contractions of hand muscles that are provoked by specific tasks (e.g., writing)

Segmental dystonia

Generalized dystonia

  • Affects the trunk and at least two additional regions of the body
  • Typically early onset (< 21 years of age)

Conditions

Other types of dystonia [4]

  • Multifocal dystonia: affects ≥ 2 nonadjacent regions of the body
  • Hemidystonia: affects the limbs of one half of the body

All treatments can be used for generalized dystonia, but botulinum toxin injections are typically reserved for focal dystonia. [5]

  1. Albanese A, Bhatia K, Bressman SB, et al. Phenomenology and classification of dystonia: a consensus update.. Mov Disord. 2013; 28 (7): p.863-73. doi: 10.1002/mds.25475 . | Open in Read by QxMD
  2. Cloud LJ, Jinnah HA. Treatment strategies for dystonia. Expert Opin Pharmacother. 2010; 11 (1): p.5-15. doi: 10.1517/14656560903426171 . | Open in Read by QxMD
  3. Campbell D . The management of acute dystonic reactions. Aust Prescr. 2001; 24 : p.19-20. doi: 10.18773/austprescr.2001.011 . | Open in Read by QxMD
  4. Dressler D. Botulinum toxin for treatment of dystonia. European Journal of Neurology. 2010; 17 : p.88-96. doi: 10.1111/j.1468-1331.2010.03058.x . | Open in Read by QxMD
  5. Spasmodic Dysphonia. https://www.nidcd.nih.gov/health/spasmodic-dysphonia. Updated: March 6, 2017. Accessed: March 30, 2017.
  6. Kamm C. Early onset torsion dystonia (Oppenheim's dystonia) . Orphanet J Rare Dis. 2006; 1 : p.48. doi: 10.1186/1750-1172-1-48 . | Open in Read by QxMD
  7. Dystonias Fact Sheet. https://www.ninds.nih.gov/disorders/patient-caregiver-education/fact-sheets/dystonias-fact-sheet. . Accessed: December 21, 2020.