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Infantile cerebral and cerebellar atrophy with postnatal progressive microcephaly (disorder)
Infantile cerebral and cerebellar atrophy with postnatal progressive microcephaly
A rare central nervous system malformation syndrome with characteristics of progressive microcephaly with profound motor delay and intellectual disability, associated with hypertonia, spasticity, clonus, and seizures, with brain imaging revealing severe cerebral and cerebellar atrophy, and poor myelination. This phenotype is caused by homozygous mutation in the MED17 gene on chromosome 11.
infantiele cerebrale en cerebellaire atrofie met postnatale progressieve microcefalie
Id770725000
StatusPrimitive
Associated morphologyAtrophy
Finding siteCerebrum
Has interpretationBelow reference range
InterpretsHead circumference
Clinical courseProgressive
Associated morphologyAtrophy
Finding siteCerebellar structure
DHD Diagnosis thesaurus reference set
ICD-10 complex map reference set
TargetQ04.3
RuleTRUE
AdviceALWAYS Q04.3 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified