primary autosomal recessive microcephaly

ORPHA: 2512

Overview

primary microcephaly characterized by microcephaly present at birth, where the brain is small but has normal architecture, and nonprogressive mental retardation that has material basis in an autosomal recessive mutation

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with primary autosomal recessive microcephaly, sourced from HPO and Orphanet clinical annotations.

Thin upper lip vermilionMicrocephalySloping foreheadUpslanted palpebral fissureGlobal developmental delayGrowth delayGray matter heterotopiaShort statureSevere intellectual disabilityVesicoureteral refluxUnilateral renal agenesisAgenesis of corpus callosumPachygyriaHyperreflexiaVentriculomegalyAbnormal cortical bone morphologyHypoplasia of the frontal lobes

Classification & Codes

Orphanet Code

ORPHA:2512
primary autosomal recessive microcephaly
OrphanetORPHA:2512
Treatments0 drug(s)
Symptoms on record17 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO