Rett syndrome

Description

A condition in which apparently normal early development is followed by partial or complete loss of speech and of skills in locomotion and use of hands, together with deceleration in head growth, usually with an onset between seven and 24 months of age. Loss of purposive hand movements, hand-wringing stereotypies, and hyperventilation are characteristic. Social and play development are arrested but social interest tends to be maintained. Trunk ataxia and apraxia start to develop by age four years and choreoathetoid movements frequently follow. Severe mental retardation almost invariably results.

Index terms

Rett syndrome

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
LD90.2 – Pelizaeus-Merzbacher-like disease
LD90.3 – Prader-Willi 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

Rett syndrome – http://id.who.int/icd/entity/201200685

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