hereditary sensory and autonomic neuropathy type 1

ORPHA: 36386

Overview

hereditary sensory neuropathy characterized by slowly progressing, prominent, predominantly distal sensory loss and autonomic disturbances with juvenile or adult onset and autosomal dominant inheritance

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 hereditary sensory and autonomic neuropathy type 1, sourced from HPO and Orphanet clinical annotations.

Inability to walkOsteomyelitisPathologic fractureNeuropathic arthropathyDistal amyotrophyMotor axonal neuropathyCoughEMG: slow motor conductionMuscle weaknessGait imbalanceAbnormality of the autonomic nervous systemDistal sensory impairmentDecreased amplitude of sensory action potentialsImpaired ability to dress oneselfHyperkeratosisPenetrating foot ulcersPoor wound healingDistal muscle weaknessSteppage gaitImpaired distal tactile sensationPain insensitivityHypohidrosis or hyperhidrosisFoot dorsiflexor weaknessLimb painImpaired temperature sensationTrophic changes related to painSkin ulcerHearing impairmentGastroesophageal reflux

Classification & Codes

Orphanet Code

ORPHA:36386
hereditary sensory and autonomic neuropathy type 1
OrphanetORPHA:36386
Treatments0 drug(s)
Symptoms on record29 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO