Isolated congenital microcephaly

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Isolated congenital microcephaly

ORPHA:199642Malformation syndrome

What it is

A rare neurological disorder characterized by a reduced head circumference at birth with no gross anomalies of brain structure. It can be an isolated finding or it can be associated with seizures, developmental delay, intellectual disability, balance disturbances, hearing loss or vision problems.

Key facts

Age of onset
Antenatal, Neonatal
Classified as
Malformation syndrome

Genes reported in subtypes

ANKLE2ASPMCDK5RAP2CDK6CENPECEP135CEP152CITCOPB2CPAPDPP6KIF14KNL1LMNB1MCM7MCPH1METTL5MFSD2ANCAPD3NUP37PDCD6IPPHC1PYCR2SARS1SASS6STILTAF13TRAPPC10TRAPPC14WARS1WDR62

Orphanet records these genes on 2 more specific entries under this disorder, not on this entry itself:

A well-studied disease is usually split into subtypes, and the gene is curated on whichever one the evidence belongs to. Open a subtype to see which gene goes with it.

ICD-10 codes

Q02filed under a broader ICD-10 category — shared with 9 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Cross-references

GARD 3603MEDDRA 10027534MONDO 0016056UMLS C0025958

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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