title: [Chronic recurrent polyneuropathy in a patient with autoimmune hemolytic anemia associated with antibodies to gangliosides GM1, GA1, and GD1b].
abstract: We report a case of chronic recurrent polyneuropathy with warm-type autoimmune hemolytic anemia (AIHA) associated with antibodies to gangliosides GM1, GA1 and GD1b. There have been few reports of AIHA associated with polyneuropathy. A 39-year-old man was admitted in May 1991 because of severe hemolytic anemia. Direct Coombs' test was positive of IgG1 and complement type. The treatment was started with prednisolone, the hematological abnormalities disappeared after 6 months, but thereafter, he developed muscle weakness of all limbs and AIHA within 7 weeks. On neurological examination, he was unable to arise from a chair and could not stand, had muscle atrophy and numbness of the distal part of all limbs and areflexia. Cerebrospinal fluid was acellular and protein content was 117 mg/dl. Electrodiagnostic studies showed mild slowing of nerve conduction velocities associated with severe decrease in amplitudes of compound muscle action potential and multifocal conduction block. A sural nerve biopsy specimen showed moderate loss of small myelinated fibers, but not of unmyleinated fibers. AIHA was substantially improved by methylprednisolone (1,000 mg/day) for 3 days, but neurological symptoms remained unchanged. A month later, he was given 2 courses of intravenous gammaglobulin infusion (0.4 g/kg/day) for 5 days. He was able to walk after the second course. In May 1992, the mild motor-sensory polyneuropathy recurred and gammaglobulin infusion was effective for neurological symptoms. We detected the high level of anti-GM1 antibody titer in the patient's serum by ELISA, the level of the antibody titers correlated with neurological manifestation. A thin-layer chromatography with immunostaining showed the presence of anti-GM1, GA1 and GD1b antibodies in his serum. We grouped the reported cases of AIHA into cold and warm types, according to its hemolytic temperature, and characterized the neurological manifestations in each type. We also discussed the pathogenic role of anti-gangliosides antibodies in this case.
authors.affiliation: Department of Neurology, Saitama Medical School.
authors.forename: K
authors.initials: K
authors.lastname: Nomura
authors.forename: A
authors.initials: A
authors.lastname: Kaneko
authors.forename: A
authors.initials: A
authors.lastname: Iwasaki
authors.forename: H
authors.initials: H
authors.lastname: Ino
authors.forename: K
authors.initials: K
authors.lastname: Hirashima
authors.forename: K
authors.initials: K
authors.lastname: Hamaguchi
mesh_terms: D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications* / Q000276:immunology; D001323:Autoantibodies / Q000378:metabolism*; D002908:Chronic Disease; D005732:Gangliosides / Q000276:immunology*; D006801:Humans; D008297:Male; D009422:Nervous System Diseases / Q000209:etiology*; D012008:Recurrence
chemical_list: D001323:Autoantibodies; D005732:Gangliosides