title: Adult-onset hereditary sensory and autonomic neuropathy accompanied by anosmia but without skin ulceration.
abstract: 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.
authors.affiliation: Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan. sakae@neuro.med.kyushu-u.ac.jp
authors.forename: N
authors.initials: N
authors.lastname: Sakae
authors.forename: T
authors.initials: T
authors.lastname: Yamada
authors.forename: K
authors.initials: K
authors.lastname: Arakawa
authors.forename: T
authors.initials: T
authors.lastname: Taniwaki
authors.forename: Y
authors.initials: Y
authors.lastname: Ohyagi
authors.forename: H
authors.initials: H
authors.lastname: Furuya
authors.forename: A
authors.initials: A
authors.lastname: Ohnishi
authors.forename: J
authors.initials: J
authors.lastname: Kira
mesh_terms: D000328:Adult; D017668:Age of Onset; D005260:Female; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000473:pathology*; D006801:Humans; D007007:Hypohidrosis; D007024:Hypotension, Orthostatic / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D000857:Olfaction Disorders / Q000209:etiology*; D012678:Sensation Disorders