L-2-hydroxyglutaric aciduria

ORPHA: 79314

Overview

2-hydroxyglutaric aciduria that involves damage to cerebellum affecting movement coordination resulting in problems with balance and muscle coordination (ataxia)

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 L-2-hydroxyglutaric aciduria, sourced from HPO and Orphanet clinical annotations.

SeizureInfectious encephalitisIntellectual disabilitySevere intellectual disabilityMacrocephalyAtypical behaviorHypotoniaSpastic tetraparesisAbnormality of extrapyramidal motor functionNeoplasm of the nervous systemAplasia/Hypoplasia of the cerebellumAphasia

Classification & Codes

Orphanet Code

ORPHA:79314
L-2-hydroxyglutaric aciduria
OrphanetORPHA:79314
Treatments0 drug(s)
Symptoms on record12 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO