Sandhoff disease

ICD-10: E75.0MeSH: D012497ORPHA: 796

Overview

lysosomal storage disorder from the GM2 gangliosidosis family, characterised by central nervous system degeneration

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 Sandhoff disease, sourced from HPO and Orphanet clinical annotations.

MacrocephalyFull cheeksHearing impairmentBlindnessSeizureAtaxiaMuscle weaknessFailure to thriveCongestive heart failureSplenomegalyRecurrent respiratory infectionsHepatomegalyMotor deteriorationSkeletal dysplasiaKyphosisAbnormal glycosphingolipid metabolismProgressive psychomotor deteriorationCherry red spot of the maculaAbnormality of movement

Classification & Codes

ICD-10 Code

E75.0

MeSH Code

D012497

Orphanet Code

ORPHA:796
Sandhoff disease
ICD-10E75.0
MeSHD012497
OrphanetORPHA:796
Treatments0 drug(s)
Symptoms on record19 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO