Structured Summary
Abstract
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)
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Classification
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MeSH Record
Synonyms
21 entry terms
- Dominant Hereditary Sensory Neuropathy, Type III
- Dysautonomia, Familial
- HSAN (Hereditary Sensory and Autonomic Neuropathy) Type III
- HSAN 3
- HSAN III
- HSAN Type III
- HSAN3
- HSN-III
- Hereditary Sensory Neuropathy Type 3
- Hereditary Sensory Neuropathy, Dominant, Type 3
- Hereditary Sensory Neuropathy, Dominant, Type III
- Hereditary Sensory Neuropathy, Type 3, Dominant
- Hereditary Sensory and Autonomic Neuropathy 3
- Hereditary-Sensory and Autonomic Neuropathy Type III
- Neuropathy, Hereditary Sensory And Autonomic, Type III
- Neuropathy, Hereditary and Autonomic, Type III
- Riley-Day Syndrome
- Type 3 Hereditary Sensory Neuropathy, Dominant
- Type III Hereditary Sensory Neuropathy, Dominant
- Hereditary Sensory and Autonomic Neuropathy Type III
- Riley Day Syndrome
MeSH Record
Aspects Covered
34 allowable subheadings
Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.
MeSH Record
Indexing Annotation
PRIMARY DYSAUTONOMIAS is also available
MeSH Record
History Note
1981; uss AUTONOMIC DYSFUNCTION 1963-1980
MeSH Hierarchy
Tree Numbers
MeSH Record
NLM Classification
WL 600
AMA Style
References
- National Library of Medicine. Familial Dysautonomia. Medical Subject Headings (MeSH). 2026. Unique ID D004402. http://id.nlm.nih.gov/mesh/2026/D004402
- Familial Dysautonomia. In: Wikipedia. https://en.wikipedia.org/wiki/Familial_dysautonomia
- Familial Dysautonomia. In: Wikidata. https://www.wikidata.org/wiki/Q2325854