Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of chronic inflammatory demyelinating polyradiculoneuropathy concomitant with acquired von Willebrand syndrome

Maki Ueda, M.D., Nobutoshi Kawamura, M.D., Ph.D., Takahisa Tateishi, M.D., Hiroshi Shigeto, M.D., Ph.D., Yasumasa Ohyagi, M.D., Ph.D. and Jun-ichi Kira, M.D., Ph.D.

Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University

We report a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) concomitant with acquired von Willebrand syndrome. A 33-year-old man developed motor and sensory polyneuropathy with electrophysiological conduction slowing. At this time, M-protein was absent. He was diagnosed with CIDP and received intravenous immunoglobulin and subsequent oral corticosteroids, which resulted in almost complete remission for over 10 years. At the age of 44, he presented with chronic anemia. Laboratory tests and colonoscopy revealed that he had acquired von Willebrand syndrome with monoclonal gammopathy of undetermined significance (IgG λtype) and colon cancer. Bleeding symptoms were resolved with intravenous immunoglobulin, but not with supplementation of factor VIII. Shortly after successful excision of the cancer, CIDP and acquired von Willebrand syndrome simultaneously recurred. Intravenous immunoglobulin produced rapid improvement of both neurological and hematological abnormalities. Concurring CIDP and acquired von Willebrand syndrome in the present case may indicate that the conditions have a partly common immunological background including monoclonal gammopathy and a potential common autoantibody-mediated mechanism. Alternatively, dysfunction of von Weillebrand factor may increase blood-nerve barrier permeability, inducing the recurrence of CIDP.
Full Text of this Article in Japanese PDF (269K)

(CLINICA NEUROL, 51: 334|337, 2011)
key words: chronic inflammatory demyelinating polyradiculoneuropathy, acquired von Willebrand syndrome, monoclonal gammopathy of undetermined significance, intravenous immunoglobulin

(Received: 1-Nov-10)