Preferred Label : Atypical Rett syndrome;
ICD-11 definition : Atypical Rett syndrome is a neurodevelopmental disorder in which patients don''t fulfill
the diagnostic criteria for typical Rett syndrome. Subvariants include: early-onset
seizure type (Hanefeld variant) with seizures in the first months of life with subsequent
development of Rett features; congenital variant (Rolando variant), the most severe
form, with onset of classic Rett features during the first three months of life; 'forme
fruste', a milder variant with onset in early; late childhood regression form with
a more gradual and later onset regression of language and motor skills; preserved
speech variant (Zappella variant) with recovery of some verbal and manual skills.;
Origin ID : 605088126;
Automatic exact mappings (from CISMeF team)
Atypical Rett syndrome is a neurodevelopmental disorder in which patients don''t fulfill
the diagnostic criteria for typical Rett syndrome. Subvariants include: early-onset
seizure type (Hanefeld variant) with seizures in the first months of life with subsequent
development of Rett features; congenital variant (Rolando variant), the most severe
form, with onset of classic Rett features during the first three months of life; 'forme
fruste', a milder variant with onset in early; late childhood regression form with
a more gradual and later onset regression of language and motor skills; preserved
speech variant (Zappella variant) with recovery of some verbal and manual skills.
