ICD-11 code : LD90.4;
Preferred Label : Rett syndrome;
ICD-11 definition : A condition, so far found only in girls, 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.;
Origin ID : 201200685;
UMLS CUI : C0035372;
Currated CISMeF NLP mapping
- ICD-10 Mapping
- Semantic type(s)
- UMLS correspondences (same concept)
A condition, so far found only in girls, 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.
