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

Maki Ueda, Nobutoshi Kawamura, Takahisa Tateishi, Hiroshi Shigeto, Yasumasa Ohyagi, Jun Ichi Kira

Research output: Contribution to journalArticlepeer-review

Abstract

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 λ type) 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.

Original languageEnglish
Pages (from-to)334-337
Number of pages4
JournalClinical Neurology
Volume51
Issue number5
DOIs
Publication statusPublished - May 2011

All Science Journal Classification (ASJC) codes

  • Clinical Neurology

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