LD90.2

ICD-11 MMS 2026-01

Pelizaeus-Merzbacher-like disease

Description

Pelizaeus-Merzbacher-like disease (PMLD) is an autosomal recessive leukodystrophy sharing identical clinical and radiological features as X-linked Pelizaeus-Merzbacher disease (PMD).

Index terms

  • Pelizaeus-Merzbacher-like disease
  • Pelizaeus-Merzbacher-like due to AIMP1 mutation
  • Pelizaeus-Merzbacher-like due to GJC2 mutation
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  • Pelizaeus-Merzbacher-like due to HSPD1 mutation
  • Pelizaeus-Merzbacher-like due to SLC16A2 mutation

Exclusions from above levels

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

Sibling codes

  • LD90.0 – Angelman syndrome
  • LD90.1 – Early-onset parkinsonism - intellectual deficit
  • LD90.3 – Prader-Willi syndrome
  • LD90.4 – Rett syndrome
  • LD90.Y – Other specified conditions with disorders of intellectual development as a relevant clinical feature
  • LD90.Z – Conditions with disorders of intellectual development as a relevant clinical feature, unspecified

Foundation entity

Pelizaeus-Merzbacher-like disease – http://id.who.int/icd/entity/1101042369

Part of: LD90: Conditions with disorders of intellectual development as a relevant clinical feature › 20: Developmental anomalies