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Case Reports in Neurological Medicine
Volume 2016, Article ID 2510573, 4 pages
Case Report

CNS Vasculitis Associated with Waldenström Macroglobulinemia

1Department of Medicine, University of Hawaii, Honolulu, HI, USA
2Department of Rheumatology, Straub Clinics and Hospital, Honolulu, HI, USA
3Department of Oncology, Straub Clinics and Hospital, Honolulu, HI, USA
4Department of Neurology, Harbor-UCLA Medical Center, Torrance, CA, USA
5Department of Neurology, Straub Clinics and Hospital, Honolulu, HI, USA

Received 6 June 2016; Accepted 26 September 2016

Academic Editor: Dominic B. Fee

Copyright © 2016 Tanawan Riangwiwat et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Waldenström macroglobulinemia (WM) is an indolent B cell lymphoproliferative disorder with monoclonal IgM secretion. We present a patient with WM who presented with multifocal acute cortical ischemic strokes and was found to have central nervous system (CNS) vasculitis. Workup was negative for cryoglobulins and hyperviscosity syndrome. Immunosuppression with intravenous steroids and cyclophosphamide stabilized the patient’s mental status and neurologic deficits. On followup over 7 years, patient gained independence from walking aids and experienced no recurrences of CNS vasculitis. To our knowledge, CNS vasculitis in a WM patient, in the absence of cryoglobulins, has not been reported. Immunosuppression is the preferred treatment.