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Neuropathy, Hereditary Sensory, Type IV

Abstract

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Synonyms of Neuropathy, Hereditary Sensory, Type IV

Familial Dysautonomia, Type II
Hereditary Sensory and Autonomic Neuropathy IV
HSAN IV
HSN IV
Insensitivity to Pain, Congenital, with Anhydrosis; CIPA
Neuropathy, Congenital Sensory, with Anhydrosis

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 these diseases is complicated, and sometimes a source of disagreement among the experts.

Hereditary sensory neuropathy type IV (HSN4) 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 small, unmyelinated nerve fibers and portions of the spinal cord that control responses to pain and temperature as well as other involuntary or automatic body processes. Sweating is almost completely absent with this disorder. Mental retardation is usually present.

The disorder is inherited as an autosomal recessive trait. The gene involved is located on chromosome 1.

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 IV

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