Progressive Multifocal Leukoencephalopathy in a Patient with Systemic Lupus Erythematosus

Hoon Young Ko; Jun-Ki Min; Hye-Kang Kim; Hee-Yeon Lee; Yong-Soo Shim; Jee-Young Kim

doi:10.4078/jkra.2008.15.2.159

Journal List > J Korean Rheum Assoc > v.15(2) > 1003618

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Ko, Min, Kim, Lee, Shim, and Kim: Progressive Multifocal Leukoencephalopathy in a Patient with Systemic Lupus Erythematosus

Original Article

J Korean Rheum Assoc 2008;15(2):159-164.

Published online: 20 June 2008

DOI: https://doi.org/10.4078/jkra.2008.15.2.159

Progressive Multifocal Leukoencephalopathy in a Patient with Systemic Lupus Erythematosus

Hoon Young Ko, Jun-Ki Min^∗, Hye-Kang Kim, Hee-Yeon Lee, Yong-Soo Shim^∗, Jee-Young Kim^∗∗∗

Department of Internal Medicine, Seoul, Korea

^∗Department of Holy Family Hospital, Bucheon, Korea

^∗∗Department of Neurology, The Catholic University of Korea College of Medicine, Seoul, Korea

^∗∗∗Department of Radiology, The Catholic University of Korea College of Medicine, Seoul, Korea

Received 14 March 2008 Accepted 28 April 2008

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Progressive multifocal leukoencephalopathy (PML) is a rare, serious, and usually fatal demyelinating disease that occurs predominantly in severely immunosuppressed patients. The disease is caused by the infection of oligodendrocytes with JC virus that is widely distributed as a latent infection in the general populations. PML has been described mainly in patients infected with the human immunodeficiency virus. However, other immune-suppressed patients including malignancies and organ transplants can be affected with JC virus infection. Recently it is suggested that rheumatologic diseases, including systemic lupus erythematosus (SLE), rheumatoid arthritis, dermatomyositis, polymyositis, wegener's granulomatosis be known to be at risk of developing PML. We report a case of PML in a patient with SLE.

Keywords: Progressive multifocal leukoencephalopathy, JC virus, SLE

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Fig. 1.

MR images demonstrate patchy low signal intensity on T1-weighted image (A) and high signal intensity on T2-weighted image (B) in left middle cerebellar peduncle. FLAIR image (C) and diffusion image (D) showing lesions with increased signal affecting left middle cerebellar peduncle. ADC map (E) showing high value in corresponding lesion seen on the FLAIR image. Follw-up T2-weighted image (F) demonstrating further extension of the previous lesions.

Fig. 2.

Sections of brain tissue. (A) Hematoxylin and eosin stained section show some atypical oligodendrocytes with infiltraion of lymphocytes and foamy macrophages (×400, H&E). (B) Luxol fast blue stained section show destroyed myelin fiber (×200, Luxol Fast Blue). (C) Electron Microscopic finding show 33 nm-sized viral particles (×76,000).

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