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Start free with EleplanHereditary sensory and autonomic neuropathy type 1
ORPHA:36386Disease
Also called Hereditary sensory and autonomic neuropathy type I · HSAN1
What it is
A rare slowly progressive neurological disorder characterized by prominent predominantly distal sensory loss, autonomic disturbances in some patients, autosomal dominant inheritance, and juvenile or adulthood disease onset.
Key facts
- Age of onset
- All ages
- Inheritance
- Autosomal dominant
- Classified as
- Disease
Signs and symptoms
Very common80–99%
6Common30–79%
13Sometimes5–29%
10- Cough
- Distal amyotrophy
- EMG: slow motor conduction
- Gastroesophageal reflux
- Hearing impairment
- Inability to walk
- Motor axonal neuropathy
- Neuropathic arthropathy
and 2 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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