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

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 siblin gs were affected. They developed anosmia initially at the ages of 20-50 yea rs, followed by anhidrosis and sensory loss. Skin ulceration was absent. Bo th 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.