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ernstige verstandelijke beperking en progressieve spastische paraplegie (aandoening)
ernstige verstandelijke beperking en progressieve spastische paraplegie
ernstige verstandelijke handicap en progressieve spastische paraplegie
AP4-deficiƫntiesyndroom
ernstige mentale retardatie en progressieve spastische paraplegie
Severe intellectual disability and progressive spastic paraplegia
AP4 deficiency syndrome
A rare complex spastic paraplegia with characteristics of early onset hypotonia that progresses to spasticity, global developmental delay, severe intellectual disability and speech impairment, microcephaly, short stature and dysmorphic features. Patients often become non-ambulatory and some develop seizures and stereotypic laughter.
Id778011005
StatusPrimitive
Clinical courseprogressief
InterpretsMovement
Has interpretationafwezig
InterpretsMovement observable
Has interpretationgestoord
InterpretsAdaptation behavior
Has interpretationgestoord
Interpretsintellectueel vermogen
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetF72.9
TermErnstige zwakzinnigheid; Zonder vermelding van gedragsstoornissen
TargetG11.4
TermHereditaire spastische paraplegie
SNOMED CT to Orphanet simple map280763
SNOMED CT to ICD-10 extended map
TargetG82.1
RuleTRUE
AdviceALWAYS G82.1 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified