8A03.10

ICD-11 MMS 2026-01

Friedreich ataxia

Description

Friedreich ataxia is an autosomal recessive ataxia characterised by difficulties to coordinate movements, associated with neurological signs (dysarthria, loss of reflexes, decrease of deep sensation, pes cavus and scoliosis), cardiomyopathy and sometimes diabetes mellitus. It is due to a mutation in the frataxin gene.

Index terms

  • Friedreich ataxia
  • Friedreich-like ataxia with selective vitamin E deficiency
  • AVED - [isolated vitamin E deficiency]
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  • Familial isolated vitamin E deficiency

Exclusions from above levels

  • http://id.who.int/icd/entity/155258022 – Metabolic disorders
  • http://id.who.int/icd/entity/76906748 – Cerebral palsy
  • 6A06 – Stereotyped movement disorder
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  • 05 – Endocrine, nutritional or metabolic diseases
  • 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

  • 8A03.11 – Ataxia due to Cerebrotendinous xanthomatosis
  • 8A03.12 – Ataxia due to Refsum disease
  • 8A03.13 – Ataxia due to abetalipoproteinemia
  • 8A03.14 – Hereditary episodic ataxia
  • 8A03.15 – Ataxia due to mitochondrial mutations
  • 8A03.16 – Spinocerebellar ataxia
  • 8A03.1Y – Other specified hereditary ataxia
  • 8A03.1Z – Hereditary ataxia, unspecified

Foundation entity

Friedreich ataxia – http://id.who.int/icd/entity/980686666

Part of: 8A03.1: Hereditary ataxia › 8A03: Ataxic disorders › 08: Diseases of the nervous system