severe growth deficiency-strabismus-extensive dermal melanocytosis-intellectual disability syndrome

ORPHA: 488627

Overview

human disease

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 severe growth deficiency-strabismus-extensive dermal melanocytosis-intellectual disability syndrome, sourced from HPO and Orphanet clinical annotations.

Coarse facial featuresSeizureGlobal developmental delayFailure to thrive in infancyProteinuriaProgressive microcephalyGait disturbanceCerebral cortical atrophyProfound intellectual disabilityPoor speechMultifocal cerebral white matter abnormalitiesBilateral tonic-clonic seizure with focal onsetDecreased glomerular filtration rateChronic kidney diseaseNephrotic syndromeSensorineural hearing impairmentStrabismusVisual impairmentBlue scleraeNystagmusPsychosisAggressive behaviorCyanosisDysarthriaGeneralized hypotoniaAbsent speechHyperreflexiaTubulointerstitial nephritisDysphagiaHypoplasia of the corpus callosumVentriculomegalyGait imbalancePseudobulbar affectVisual hallucinationGenu valgumLateral ventricle dilatationDysplastic corpus callosumMultifocal hyperintensity of cerebral white matter on MRIArachnoid cystBlue nevus

Classification & Codes

Orphanet Code

ORPHA:488627
severe growth deficiency-strabismus-extensive dermal melanocytosis-intellectual disability syndrome
OrphanetORPHA:488627
Treatments0 drug(s)
Symptoms on record40 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO