Definition: 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)
PRIMARY DYSAUTONOMIAS is also available
Other names Riley-Day Syndrome; Neuropathy, Hereditary and Autonomic, Type III; Hereditary-Sensory and Autonomic Neuropathy Type I; HSAN Type III; Riley Day Syndrome; Hereditary Sensory and Autonomic Neuropathy Type I; HSAN Type IIIs; Type III Hereditary Sensory Neuropathy, Dominant; Type 3 Hereditary Sensory Neuropathy, Dominant; Hereditary Sensory and Autonomic Neuropathy 3; Hereditary Sensory Neuropathy, Type 3, Dominant; Familial Dysautonomia; Hereditary Sensory Neuropathy, Dominant, Type III; Hereditary Sensory Neuropathy, Dominant, Type 3; Hereditary Sensory Neuropathy Type 3; HSAN 3; HSAN (Hereditary Sensory and Autonomic Neuropathy); Dominant Hereditary Sensory Neuropathy, Type III
