Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

A case of acute autonomic, sensory and motor neuropathy (AASMN) -less favorable response of the autonomic dysfunctions to IVIg treatment-

Teruyuki Takahashi, M.D.1), Masato Tamura, M.D.2), Koichi Chida, M.D.2), Katsuhiko Ogawa, M.D.1), Shin-ichi Ono, M.D.1), Hiromasa Tsuda, M.D.2), Yutaka Suzuki, M.D.2) and Tomohiko Mizutani, M.D.2)

1)Department of Neurology, Nihon University Nerima-Hikarigaoka Hospital
2)Division of Neurology, Department of Medicine, Nihon University School of Medicine

We report a rare case of acute autonomic, sensory and motor neuropathy (AASMN). The patient, a 26-year-old woman, developed fever and common cold around January 20, 2001 and was admitted because of abdominal pain due to ileus on January 30. After admission, the patient complained of muscle weakness and numbness in the extremities, difficulty in seeing with the right eye, and dysuria. Neurologically, marked orthostatic hypotension, right tonic pupil, distal dominant moderate muscle weakness in extremities, areflexia in both lower limbs, glove and stocking type of paresthesia, and neurogenic atonic bladder were noted. Sensation to pin prick, light touch, temperature, and vibration were markedly impaired in upper limbs and below the level of the 5th thoracic cord. Cerebrospinal fluid examination revealed albumino-cytologic dissociation. Peripheral nerve conduction study revealed lower limb dominant axonal type impairment of sensory conduction and slight impairment of motor conduction velocity. Clinical autonomic testings revealed dysfunction of both sympathetic and parasympathetic systems. As having AASMN, she was given the intravenous high-dose immunoglobulin (IVIg) therapy twice. After IVIg, the sensory and motor symptoms improved remarkably, but pandysautonomia did not. To our knowledge, this is the first report of AASMN treated by IVIg, and the notable clinical feature in this case was the favorable motor and sensory recovery to IVIg, as opposed to poor autonomic outcome.

(CLINICA NEUROL, 44: 643－647, 2004)
key words: acute autonomic, sensory and motor neuropathy (AASMN), intravenous administration of high-dose immunoglobulin (IVIg), pandysautonomia

(Received: 8-Jan-04)