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Adult-onset hereditary sensory and autonomic neuropathy accompanied by anosmia but without skin ulceration

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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.

Keywords: ageusia; anhidrosis; anosmia; axonal degeneration; hereditary sensory and autonomic neuropathy; orthostatic hypotension

Document Type: Research Article

Affiliations: 1: Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812–8582, Japan, 2: Department of Neurology, University of Occupational and Environmental Health, Kitakyushu 807–8555, Japan

Publication date: 2001-11-01

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