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Hereditary Sensory and Autonomic Neuropathies (HSANs): An Overview

Introduction

Hereditary Sensory and Autonomic Neuropathy (HSAN) or Hereditary Sensory Neuropathy (HSN) is a medical condition with clinical manifestation involving the peripheral sensory nerves (see Table H-5). There are five different clinical entities described under hereditary sensory and autonomic neuropathies. All are characterized by progressive loss of peripheral sensory nerves function. Their incidence has been estimated to be about 1 in 25,000 of population. The distribution of these diseases is worldwide.

TABLE H-5Hereditary Sensory and Autonomic Neuropathies (HSAN)
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TABLE H-5 Hereditary Sensory and Autonomic Neuropathies (HSAN)
Type Inheritance Eponym/Synonym
HSAN Type I Autosomal dominant Hick Syndrome
Denny-Brown Syndrome
Thévenard Syndrome
Hereditary Sensory
Radicular
Neuropathy
HSAN Type II Autosomal recessive Neurogenic
Acroosteolysis
Giaccai Syndrome
HSAN Type III Autosomal recessive Familial Dysautonomia
Riley-Day Syndrome
HSAN Type IV Autosomal recessive Familial Dysautonomia Type II
Swanson Syndrome
Congenital Sensory
Neuropathy with Anhidrosis
HSAN Type V Autosomal recessive Strümpell-Lorrain Syndrome
Familial Spastic Paraplegia
Posterior column ataxia Autosomal dominant Biemond Syndrome
Congenital
Anesthesia
Ataxic neuropathy Autosomal dominant Sensory Ataxic Neuropathy

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