A Case of Chronic Demyelinating Neuropathy associated with             IgG λ Type Monoclonal Gammopathy of Undetermined Significance

J Korean Neurol Assoc > Volume 16(5); 1998 > Article

Journal of the Korean Neurological Association 1998;16(5): 747-751.

단클론성 감마글로부린병증에 의한 만성 탈수초성 신경병증

고진석, 김승현*김주한김명호

한양대학교 의과대학 신경과학교실

A Case of Chronic Demyelinating Neuropathy associated with IgG λ Type Monoclonal Gammopathy of Undetermined Significance

Jin-Seok Ko. M.D., Seung-Hyun Kim. M.D*., Ju-Han Kim. M.D., Myung-Ho Kim. M.D.

Department of Neurology College of Medicine Hanyang University

Abstract

Monoclonal gammopathy of undetermined significance(MGUS) is one of the monoclonal plasma cell disorder. According to the recent reports, polyneuropathy associated with MGUS may be treatable disease due to potential relationship between the monoclonal protein and immune-mediated nerve damage. It is reported that plasmapheresis is highly effective in the treatment of polyneuropathy associated with IgG type MGUS. We present 53-year-old male with slowly progressive motor weakness and sensory change since 1 year ago. Electrophysiologic studies were compatible with demyelinating neuropathy showing conduction block and temporal dispersion in upper and lower extremities. Abnormal arc against anti-λ on serum immunoelectrophoresis and M-band in the gamma region on serum protein electrophoresis were shown. After the combined therapy of plasmapheresis (5 times) and steroid, clinical and electrophysiologic findings were markedly improved. We report a case of demyelinating neuropathy associated with IgG λ type MGUS, which showed good response to combined therapy of plasmapheresis and steroid. Key Words : Monoclonal gammopathy of undetermined significance, IgG λ type, plasmapheresis