Dejerine–Sottas disease

ICD-10: G60.0ORPHA: 3115

Overview

a rare hereditary neurological disorder characterised by damage to the peripheral nerves and resulting progressive muscle wasting

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 Dejerine–Sottas disease, sourced from HPO and Orphanet clinical annotations.

Gait disturbanceNystagmusAreflexiaPes cavusTalipes equinovarusGait ataxiaLimb ataxiaPostural tremorClumsinessUnsteady gaitImpaired vibratory sensationScoliosisKyphoscoliosisDecreased motor nerve conduction velocitySomatic sensory dysfunctionDistal amyotrophyAcute demyelinating polyneuropathySensorimotor neuropathyImpaired pain sensationLower limb muscle weaknessImpaired temperature sensationFrequent fallsUrinary bladder sphincter dysfunctionGenu valgumSkeletal muscle atrophyBabinski signIntrinsic hand muscle atrophy

Classification & Codes

ICD-10 Code

G60.0

Orphanet Code

ORPHA:3115
Dejerine–Sottas disease
ICD-10G60.0
OrphanetORPHA:3115
Treatments0 drug(s)
Symptoms on record27 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO