Medical terminology · Neurology

Dysautonomia, Familial

MeSH — National Library of Medicine · Retrieved 2026-10-13

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 periphera

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Reference details

MeSH descriptor
D004402
Vocabulary edition
2026
Tree positions
C10.177.575.300; C10.500.250.309; C10.574.500.493.250; C10.668.829.800.175.250; C16.131.666.310.309; C16.320.400.415.309

Scope and 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)

Entry terms and synonyms

Dysautonomia, Familial HSAN 3 Hereditary Sensory Neuropathy Type 3 Hereditary Sensory and Autonomic Neuropathy 3 HSAN III Familial Dysautonomia HSAN3 HSN-III Neuropathy, Hereditary Sensory And Autonomic, Type III HSAN Type III Riley-Day Syndrome Riley Day Syndrome Hereditary-Sensory and Autonomic Neuropathy Type III Hereditary Sensory and Autonomic Neuropathy Type III Neuropathy, Hereditary and Autonomic, Type III Dominant Hereditary Sensory Neuropathy, Type III Hereditary Sensory Neuropathy, Dominant, Type 3 Hereditary Sensory Neuropathy, Dominant, Type III Hereditary Sensory Neuropathy, Type 3, Dominant HSAN (Hereditary Sensory and Autonomic Neuropathy) Type III Type 3 Hereditary Sensory Neuropathy, Dominant Type III Hereditary Sensory Neuropathy, Dominant

Classification tree positions

C10.177.575.300 C10.500.250.309 C10.574.500.493.250 C10.668.829.800.175.250 C16.131.666.310.309 C16.320.400.415.309

Sources and reuse

Medical vocabulary definition and classification record.
Retrieved: 2026-10-13. Text has been organized for reading; this import has not been independently reviewed by a clinician. It is not individualized medical advice.
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