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Neuropathy, Hereditary Sensory, Type I

Abstract

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NORD is very grateful to Thomas Bird, MD, Professor of Neurology and Head of the Division of Neurogenetics, University of Washington, and Research Neurologist, Seattle VA Medical Center, for assistance in the preparation of this report.

Synonyms of Neuropathy, Hereditary Sensory, Type I

  • HSAN1
  • Neuropathy, Hereditary Sensory and Autonomic, Type I HSAN1
  • Neuropathy, Hereditary Sensory Radicular, Autosomal Dominant

Disorder Subdivisions

  • No subdivisions found.

General Discussion

The hereditary sensory neuropathies (HSN) include 4-6 similar but distinct inherited, degenerative disorders of the nervous system (neurodegenerative) that frequently progress to loss of feeling, especially in the hands and feet. The classification of the hereditary sensory neuropathies is complicated, and the experts do not always agree. This report deals with HSN type I. There is a separate report in NORD's Rare Disease Database dealing with HSN type II. One other type of hereditary sensory neuropathy, HSN-III, is related to, or identical with, familial dysautonomia (Riley-Day syndrome). Another type, HSN-IV, is related to, or identical with, a form of Charcot-Marie-Tooth disorder.

Hereditary sensory neuropathy Type I (HSN1) is a rare genetic disorder characterized by the loss of sensation (sensory loss), especially in the feet and legs and, less severely, in the hands and forearms. The sensory loss is due to abnormal functioning of the sensory nerves that control responses to pain and temperature and may also affect the autonomic nervous system that controls other involuntary or automatic body processes.

The disorder is inherited as an autosomal dominant trait, and the mutated gene has been identified and tracked to a site on chromosome 9.

HSNs of various types may attack a single nerve (mononeuropathy) or many nerves simultaneously (polyneuropathy). The resulting symptoms may involve sensory, motor, reflex, or blood vessel (vasomotor) functions.

Organizations related to Neuropathy, Hereditary Sensory, Type I

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