J Korean Rheum Assoc.  2008 Jun;15(2):159-164. 10.4078/jkra.2008.15.2.159.

Progressive Multifocal Leukoencephalopathy in a Patient with Systemic Lupus Erythematosus

• Ko HY
• Min JK
• Kim HK
• Lee HY
• Shim YS
• Kim JY

• KMID: 1270233
• DOI: http://doi.org/10.4078/jkra.2008.15.2.159

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.

Keyword

Progressive multifocal leukoencephalopathy; JC virus; SLE

MeSH Terms

Humans

Figure

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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.

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  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).

Reference

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