Infect Chemother.  2010 Jun;42(3):198-202. 10.3947/ic.2010.42.3.198.

A Case of Immune Reconstitution Inflammatory Syndrome in AIDS-related Progressive Multifocal Leukoencephalopathy after Antiretroviral Therapy

• Ju SH
• Kim TH
• Na JS
• Song HS
• Yu IK
• Yoon HJ

• KMID: 2170311
• DOI: http://doi.org/10.3947/ic.2010.42.3.198

Abstract

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system due to JC virus. In acquired immunodeficiency syndrome (AIDS) patients, JC virus infects myelin-producing oligodendrocytes causing a non-inflammatory lytic reaction leading to demyelination and brain death. We herein report a case of a 56-years-old AIDS man who developed immune reconstitution inflammatory syndrome and died while undergoing highly active antiretroviral therapy. In this patient, the PML involved the brainstem, causing mental confusion followed by recurrent aspiration, adult respiratory distress syndrome, and eventually to early death.

Keyword

Progressive multifocal leukoencephalopathy; Acquired immunodeficiency syndrome; Highly active antiretroviral therapy; Immune reconstitution inflammatory syndrome

MeSH Terms

Acquired Immunodeficiency Syndrome
Antiretroviral Therapy, Highly Active
Brain Death
Brain Stem
Central Nervous System
Demyelinating Diseases
Humans
Immune Reconstitution Inflammatory Syndrome
JC Virus
Leukoencephalopathy, Progressive Multifocal
Oligodendroglia
Respiratory Distress Syndrome, Adult

Figure

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  Figure 1 Initial brain MRI. Focal patchy high and low signal intensity lesion are seen at right anterior cerebellar hemisphere on T2-weighted image (A) and T1-weighted image (B). (C) Postcontrast-T1-signal intensity. There is no demonstrable enhancement at the right anterior cerebellar hemisphere.

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  Figure 2 MR spectroscopy. MR spectroscopy show slightly increased choline peak (3.2 ppm), lactate peak (1.35 ppm), and decreased N-acetylaspartate (NAA) peak (2.0 ppm), suggestive of active demyelinating disease.

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  Figure 3 Follow-up brain MRI 3 weeks later. (a) T2-weighted image. Interval increased extent of previously seen patchy high signal intensity lesion is seen at the right anterior cerebellar hemisphere, which shows interval aggravation. (b) T1-weighted image. Interval increased extent of previously seen patchy low signal intensity lesion is seen at the right anterior cerebellar hemisphere, which shows interval aggravation. (c) Postcontrast-T1-signal intensity. There is no demonstrable enhancement at the right anterior cerebellar hemisphere.

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