Hereditary Sensory and Autonomic Neuropathies
| 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) |
| Notes: |
do not confuse with HEREDITARY MOTOR AND SENSORY NEUROPATHIES; for X refs to HSAN types: HSAN TYPE III is see DYSAUTONOMIA, FAMILIAL |
| Also Called: |
Hereditary Sensory Radicular Neuropathy,HSAN Type IV,HSAN Type V,HSN Type I,HSN Type II,Sensory Neuropathy, Hereditary |
| Previously Indexed: |
Peripheral Nerve Diseases (1966-1978),Peripheral Nerves (1966-1978),Sensation (1966-1978) |
Hereditary Sensory and Autonomic Neuropathies Categories.
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Dysautonomia, Familial - An autosomal disorder of the peripheral and autonomic nervous systems limited to individuals of Ashkenazic Jewish descent. Clinical manifestations are present at birth and include diminished lacrimation, defective thermoregulation, orthostatic hypotension (HYPOTENSION, ORTHOSTATIC), fixed pupils, excessive SWEATING, loss of pain and temperature sensation, and absent reflexes. Pathologic features include reduced numbers of small diameter peripheral nerve fibers and autonomic ganglion neurons. (From Adams et al., Principles of Neurology, 6th ed, p1348; Nat Genet 1993;4(2):160-4) |
Hereditary Sensory and Autonomic Neuropathies Definitions and Terms
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