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Adult‐onset hereditary sensory and autonomic neuropathy accompanied by anosmia but without skin ulceration
Author(s) -
Sakae N.,
Yamada T.,
Arakawa K.,
Taniwaki T.,
Ohyagi Y.,
Furuya H.,
Ohnishi A.,
Kira J.
Publication year - 2001
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1034/j.1600-0404.2001.00051.x
Subject(s) - anosmia , anhidrosis , medicine , familial dysautonomia , orthostatic vital signs , sensory neuropathy , sensation , sensory system , dermatology , surgery , psychology , neuroscience , covid-19 , blood pressure , infectious disease (medical specialty) , disease
We report a novel type of hereditary sensory and autonomic neuropathy (HSAN) with adult onset in a Japanese family. One male and 2 females of 6 siblings were affected. They developed anosmia initially at the ages of 20–50 years, followed by anhidrosis and sensory loss. Skin ulceration was absent. Both superficial and deep sensation were impaired in the most distal parts of all 4 limbs. Orthostatic hypotension was present in all patients. This is a unique subtype of HSAN distinct from the HSAN I–V described by Dyck.