autosomal dominant optic atrophy plus syndrome

MeSH: C535351ORPHA: 1215

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 autosomal dominant optic atrophy plus syndrome, sourced from HPO and Orphanet clinical annotations.

Progressive visual lossOptic atrophySensorineural hearing impairmentProgressive external ophthalmoplegiaAbnormality of visual evoked potentialsBilateral ptosisMyopathyLimb-girdle muscle weaknessMitochondrial myopathyAbsent brainstem auditory responsesFatigueAbnormal retinal nerve fiber layer morphologyEMG: impaired neuromuscular transmissionSensory neuropathyConstriction of peripheral visual fieldAtaxiaSpastic paraplegiaPes cavusAbsent Achilles reflexEMG: chronic denervation signsMotor axonal neuropathyPeripheral neuropathyTemporal optic disc pallorDiabetes mellitusCardiomyopathyMigraine

Classification & Codes

MeSH Code

C535351

Orphanet Code

ORPHA:1215
autosomal dominant optic atrophy plus syndrome
MeSHC535351
OrphanetORPHA:1215
Treatments0 drug(s)
Symptoms on record26 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO