LD20.4

ICD-11 MMS 2026-01

Syndromes with brain calcifications as a major feature

Description

A group of rare familial syndromes characterised by abnormal calcification of cerebral tissue and typically first manifesting in adult life with any of a wide range of neuropsychiatric disorders.

Index terms

  • Syndromes with brain calcifications as a major feature
  • cerebral calcification
  • cerebral cortex calcification
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  • Bilateral striopallidodentate calcinosis
  • Brain calcification, Rajab type
  • Familial idiopathic basal ganglia calcification
  • Fahr disease
  • Fahr syndrome
  • Primary familial brain calcification

Exclusions from above levels

  • LD2F.13 – Meckel syndrome
  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

  • LD20.0 – Syndromes with cerebellar anomalies as a major feature
  • LD20.1 – Syndromes with lissencephaly as a major feature
  • LD20.2 – Syndromes with microcephaly as a major feature
  • LD20.3 – Syndromes with holoprosencephaly as a major feature
  • LD20.Y – Other specified syndromes with central nervous system anomalies as a major feature
  • LD20.Z – Syndromes with central nervous system anomalies as a major feature, unspecified

Foundation entity

Syndromes with brain calcifications as a major feature – http://id.who.int/icd/entity/1361836748

Part of: LD20: Syndromes with central nervous system anomalies as a major feature › 20: Developmental anomalies