8A02.11

ICD-11 MMS 2026-01

Dystonia-plus

Description

This is a group of heterogenous syndromes present with dystonia – a disorder of involuntary muscle contractions – along with other clinical features, but not in tandem with a neurodegenerative disease. Examples include myoclonus dystonia and dopa responsive dystonia.

Index terms

  • Dystonia-plus
  • Myoclonus-dystonia
  • Dopa-responsive dystonia
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  • DRD - [dopa-responsive dystonia]
  • DYT5
  • Segawa syndrome
  • Autosomal dominant dopa-responsive dystonia
  • Autosomal dominant DRD - [dopa-responsive dystonia]
  • Autosomal dominant Segawa syndrome
  • DYT5a
  • HPD - [ hereditary progressive dystonia] with marked diurnal fluctuation
  • Hereditary progressive dystonia with marked diurnal fluctuation
  • Autosomal recessive dopa-responsive dystonia
  • Autosomal recessive DRD - [dopa-responsive dystonia]
  • Autosomal recessive Segawa syndrome
  • Tyrosine hydroxylase deficiency

Exclusions from above levels

  • 8D21 – athetoid cerebral palsy
  • 6A06 – Stereotyped movement disorder
  • 05 – Endocrine, nutritional or metabolic diseases
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  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 19 – Certain conditions originating in the perinatal period
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • 8A02.10 – Drug-induced dystonia
  • 8A02.12 – Dystonia associated with heredodegenerative disorders
  • 8A02.1Y – Other specified secondary dystonia
  • 8A02.1Z – Secondary dystonia, unspecified

Foundation entity

Dystonia-plus – http://id.who.int/icd/entity/378693810

Allowed postcoordination axes

Part of: 8A02.1: Secondary dystonia › 8A02: Dystonic disorders › 08: Diseases of the nervous system