distal hereditary motor neuronopathy type 5
ORPHA: 139536
Overview
autosomal dominant distal hereditary motor neuronopathy characterized by muscle weakness and wasting, predominantly confined to the hands, and often exclusively involve thenar and/or interosseus dorsalis I eminences
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 distal hereditary motor neuronopathy type 5, sourced from HPO and Orphanet clinical annotations.
Unsteady gaitImpaired vibratory sensationFirst dorsal interossei muscle weaknessThenar muscle atrophyFirst dorsal interossei muscle atrophyThenar muscle weaknessCold-induced hand crampsUpper limb muscle weaknessDistal amyotrophyMotor polyneuropathyDistal lower limb muscle weaknessHyperreflexiaPes cavusHammertoePes valgusAbnormal motor nerve conduction velocity
Classification & Codes
Orphanet Code
ORPHA:139536distal hereditary motor neuronopathy type 5
| Orphanet | ORPHA:139536 |
| Treatments | 0 drug(s) |
| Symptoms on record | 16 signs |
| Status | published |
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO