title: A case of acute autonomic and sensory neuropathy (AASN) with antibody against a mixture of galactocerebroside and phospholipids.
abstract: 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.
authors.affiliation: Department of Neurology, Osaka General Medical Center.
authors.forename: Teruyuki
authors.initials: T
authors.lastname: Ishikura
authors.forename: Kazushiro
authors.initials: K
authors.lastname: Takata
authors.forename: Makoto
authors.initials: M
authors.lastname: Kinoshita
authors.forename: Kei
authors.initials: K
authors.lastname: Fukada
authors.forename: Jinichi
authors.initials: J
authors.lastname: Sawada
authors.forename: Takanori
authors.initials: T
authors.lastname: Hazama
mesh_terms: D000208:Acute Disease; D001323:Autoantibodies / Q000097:blood*; D001342:Autonomic Nervous System Diseases / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D015415:Biomarkers / Q000097:blood; D005260:Female; D005699:Galactosylceramides / Q000276:immunology*; D020275:Guillain-Barre Syndrome / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage; D008875:Middle Aged; D010743:Phospholipids / Q000276:immunology*; D011984:Sensory Receptor Cells; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D005699:Galactosylceramides; D016756:Immunoglobulins, Intravenous; D010743:Phospholipids