sensory ataxic neuropathy-dysarthria-ophthalmoparesis syndrome

MeSH: C537583ORPHA: 70595

Overview

Sensory ataxic neuropathy-dysarthria-ophthalmoparesis syndrome is characterised by adult-onset severe sensory ataxic neuropathy, dysarthria and chronic progressive external ophthalmoplegia

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 sensory ataxic neuropathy-dysarthria-ophthalmoparesis syndrome, sourced from HPO and Orphanet clinical annotations.

PtosisOphthalmoparesisNystagmusDysarthriaHyporeflexiaMyoclonusAbnormal vestibular functionGait ataxiaIncreased circulating lactate concentrationPositive Romberg signImpaired vibratory sensationRagged-red muscle fibersSensory ataxic neuropathyIncreased variability in muscle fiber diameterProximal muscle weaknessImpaired distal proprioceptionAtrophy/Degeneration involving the spinal cordBilateral sensorineural hearing impairmentAbnormal thalamic MRI signal intensityUpgaze palsyAbnormal cerebellar cortex morphologyCataractDepressionSeizureAreflexiaDilated cardiomyopathyMigraineMemory impairmentGastroparesisIntestinal pseudo-obstructionCognitive impairment

Classification & Codes

MeSH Code

C537583

Orphanet Code

ORPHA:70595
sensory ataxic neuropathy-dysarthria-ophthalmoparesis syndrome
MeSHC537583
OrphanetORPHA:70595
Treatments0 drug(s)
Symptoms on record31 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
sensory ataxic neuropathy-dysarthria-ophthalmoparesis syndrome | OrphanDrug