[A case of chronic demyelinating polyneuropathy with benign IgM anti-myelin-associated glycoprotein paraproteinenia--transient improvement with weekly plasmapheresis].
We report a case of chronic demyelinating polyneuropathy accompanying benign IgM monoclonal gammopathy treated with plasmapheresis, which brought the improvement of the neurological signs and conduction velocities of peripheral nerves. A 60-year-old man developed numbness in the hands and legs and unstable gait. These symptoms became worse slowly. Three years after the onset, he was admitted to Matsuyama Red Cross Hospital because mild clumsiness in the hands was added. The neurological examinations revealed marked loss of both superficial and deep sensations of the glove-stocking type, mild weakness of distal muscles and hyporeflexia in the upper and lower extremities, and mild sensory ataxia. On the laboratory examinations, the serum showed a marked increase of IgM with a monoclonal IgM of lambda light chain. The cytological examination of the bone marrow showed no evidence of malignancy. Marked decrease of nerve conduction velocities was noted in the electrophysiological examinations of the peripheral nerves. Segmental demyelination and widely spaced major dense lines of myelin were observed in the histological examinations of the sural nerve. The immunological examination revealed the antibody activity of IgM against myelin-associated glycoprotein in the patient's serum. He was treated with double-filtration plasmapheresis of 2 liters once a week for 3 months as inpatient. During this treatment, the concentration of IgM in the serum was kept to be much lower than before the treatment, and the sensory disturbances, grasping powers and nerve conduction velocities were mildly improved at the end of the treatment. After discharge, he was treated with monthly plasmapheresis of 2 liters for 3 months.(ABSTRACT TRUNCATED AT 250 WORDS)
Muramoto
T
T
Department of Neurology, Matsuyama Red Cross Hospital.
Yamashita
Y
Y
Ohnishi
A
A
Saito
T
T
Kajiyama
K
K
Shirokuchi
A
A
D001323:Autoantibodies / Q000378:metabolism*; D002908:Chronic Disease; D003711:Demyelinating Diseases / Q000209:etiology* / Q000628:therapy; D006801:Humans; D007075:Immunoglobulin M / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D009185:Myelin Proteins / Q000276:immunology*; D018992:Myelin-Associated Glycoprotein; D010265:Paraproteinemias / Q000150:complications* / Q000276:immunology; D010956:Plasmapheresis; D011115:Polyneuropathies / Q000209:etiology* / Q000628:therapy
D001323:Autoantibodies; D007075:Immunoglobulin M; D009185:Myelin Proteins; D018992:Myelin-Associated Glycoprotein