Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

A case of acute autonomic and sensory neuropathy (AASN) with antibody against a mixture of galactocerebroside and phospholipids

Teruyuki Ishikura, M.D.1), Kazushiro Takata, M.D., Ph.D.1), Makoto Kinoshita, M.D., Ph.D.1), Kei Fukada, M.D., Ph.D.1), Jinichi Sawada, M.D.1) and Takanori Hazama, M.D.1)

1)Department of Neurology, Osaka General Medical Center

A 62-year-old woman presented with paresthesia of limbs, gait disturbance, urinary retention and constipation following upper respiratory infection. Neurological examination revealed gait disturbance due to loss of position sense in her extremities with intact muscle power, and autonomic failure represented by orthostatic hypotension, constipation and autonomic bladder. Cerebrospinal fluid analysis showed normal cell counts with elevated protein levels. Nerve conduction study showed sensory nerve impairment with almost normal motor nerve conduction in her upper and lower extremities. Sympathetic skin response of both hands was unresponsive, indicating autonomic nervous dysfunction. We diagnosed her as having acute autonomic and sensory neuropathy (AASN) and treated her with intravenous immunoglobulin, which ameliorated her symptoms enabling her to walk without any assistance at the time of discharge. Screening tests of serum autoantibodies revealed positivity of antibody against a mixture of galactocerebroside (Gal-Cer) and phospholipids. According to previous literature, no specific antibodies have been identified in AASN. This case, therefore, suggests a possible role of anti-Gal-Cer antibody in the pathogenesis of AASN.
Full Text of this Article in Japanese PDF (406K)

(CLINICA NEUROL, 57: 33|36, 2017)
key words: galactocerebroside, acute autonomic and sensory neuropathy, Guillain-Barre syndrome, anti-ganglioside antibody, phospholipidic acid

(Received: 14-May-16)