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autosomaal recessieve complexe spastische paraparese door disfunctie van Kennedy-route (aandoening)
autosomaal recessieve complexe spastische paraparese door disfunctie van Kennedy-route
autosomaal recessieve complexe spastische paraplegie door disfunctie van Kennedy-'pathway'
autosomaal recessieve complexe spastische paraplegie door disfunctie van Kennedy-reactiepad
autosomaal recessieve complexe spastische paraplegie door disfunctie van Kennedy-route
Autosomal recessive complex spastic paraplegia due to Kennedy pathway dysfunction
A rare genetic neurological disorder with characteristics of progressive spastic paraparesis and delayed gross motor development with an onset in infancy or early childhood. Patients also show variable degrees of intellectual disability, speech delay and dysarthria. Other reported features include microcephaly, seizures, bifid uvula with or without cleft palate and ocular anomalies. Brain imaging shows white matter abnormalities in the periventricular and other regions.
Id1186734006
StatusPrimitive
Clinical courseprogressief
Has interpretationgestoord
Interpretsintellectueel vermogen
Has interpretationgestoord
InterpretsAdaptation behavior
InterpretsMovement
Has interpretationafwezig
InterpretsMovement observable
SNOMED CT to Orphanet simple map506353
SNOMED CT to ICD-10 extended map
TargetG11.4
RuleTRUE
AdviceALWAYS G11.4 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified