Norrie disease

MeSH: C537849ORPHA: 649

Overview

genetic disorder that primarily affects the eye and almost always leads to blindness

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 Norrie disease, sourced from HPO and Orphanet clinical annotations.

CryptorchidismThin vermilion borderMicrocephalyMalar flatteningAbnormal cochlea morphologyMacrotiaSensorineural hearing impairmentProtruding earNarrow nasal bridgeDeeply set eyeGlaucomaCataractAbnormal chorioretinal morphologyRetinal detachmentMicrophthalmiaHypotelorismAbnormal pupil morphologyBlindnessNystagmusSclerocorneaOptic atrophyAtypical behaviorPsychosisAutismMotor stereotypyIrritabilityHallucinationsAnxietyDiabetes mellitusDelayed pubertyEctopia lentisSeizureHypotoniaHypertoniaMuscle weaknessHyperreflexiaFailure to thriveMigraineCerebral cortical atrophyClonusEEG abnormalitySleep disturbanceDevelopmental regressionScoliosisCachexiaAbnormal vitreous humor morphologyVenous insufficiencyIntellectual disabilityAttention deficit hyperactivity disorderAplasia/Hypoplasia of the cerebellumHypoplasia of the irisAnterior chamber synechiaeCorneal opacityRemnants of the hyaloid vascular systemAbnormal retinal vascular morphologyAplasia/Hypoplasia of the lensAbnormal diencephalon morphologyAbnormality of immune system physiologyAbnormal helix morphologyNeoplasm of the eyeErectile dysfunctionSelf-injurious behaviorUterine ruptureVascular neoplasm

Classification & Codes

MeSH Code

C537849

Orphanet Code

ORPHA:649
Norrie disease
MeSHC537849
OrphanetORPHA:649
Treatments0 drug(s)
Symptoms on record64 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO