Definition: A group of inherited disorders characterized by degeneration of dorsal root and autonomic ganglion cells, and clinically by loss of sensation and autonomic dysfunction. There are five subtypes. Type I features autosomal dominant inheritance and distal sensory involvement. Type II is characterized by autosomal inheritance and distal and proximal sensory loss. Type III is DYSAUTONOMIA, FAMILIAL. Type IV features insensitivity to pain, heat intolerance, and mental deficiency. Type V is characterized by a selective loss of pain with intact light touch and vibratory sensation. (From Joynt, Clinical Neurology, 1995, Ch51, pp142-4)
Other names Sensory and Autonomic Neuropathies, Hereditary; Sensory Neuropathy, Hereditary; Pain Insensitivity with Anhidrosis, Congenital; Neuropathies, Hereditary Sensory and Autonomic; Insensitivity to Pain with Anhidrosis, Congenital; HSN Type II; HSN Type I; HSAN Type V; HSAN Type IV; HSAN Type II; HSAN Type I; HSAN; Type V, HSAN; Type IV, HSAN; Type I, HSN; Type I, HSAN; Type Acroosteolysis, Giaccai; Sensory Neuropathy, Congenital; Sensory Neuropathies, Hereditary; Sensory Neuropathies, Congenital
