Hereditary sodium channelopathy-related…

Caring for someone with this diagnosis? Eleplan keeps diagnoses, medications, documents and every specialist in one plan.

Start free with Eleplan

Hereditary sodium channelopathy-related small fibers neuropathy

ORPHA:306577Disease

What it is

A rare, genetic, peripheral neuropathy disorder due to gain-of-function mutations in voltage-gated sodium channels present in the small peripheral nerve fibers characterized by neuropathic pain of varying intensity (often beginning in the distal extermities and with a burning quality) associated with autonomic dysfunction (e.g. orthostatic dizziness, palpitations, dry eyes and mouth), abnormal quantitative sensory testing, and reduction in intraepidermal nerve fiber density. Large fiber functions (i.e. normal strength, tendon reflexes, and vibration sense) and nerve conduction studies are typically normal.

Key facts

Prevalence
<1 / 1 000 000
Age of onset
No data available
Inheritance
Autosomal dominant
Classified as
Disease

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Genes

SCN10ADisease-causing germline mutation(s) (gain of function)
SCN11ADisease-causing germline mutation(s) (gain of function)
SCN9ADisease-causing germline mutation(s) (gain of function)

ICD-10 codes

G99.1filed under a broader ICD-10 category

Cross-references

MONDO 0017629OMIM 133020OMIM 615551UMLS C5925033

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.

Powered by Eleplan

A rare diagnosis is just the start. Eleplan keeps the whole care plan in one place.

Diagnoses, medications, documents, appointments, and the whole care team — organized and always in sync, so you are not retelling the same story to every new specialist. With Ellie, your AI care assistant, on top of it. Free to start.