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KMID : 0391520150230020071
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Journal of the Korean Child Neurology Society
2015 Volume.23 No. 2 p.71 ~ p.74
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A Case of Progressive Multifocal Leukoencephalop in a 10-year-old Girl
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Choi Mi-Ran
Kang Ben
Kwon Young-Se
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Abstract
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Progressive multifocal leukoencephalopathy (PML) is a fatal viral disease caused by the John Cunningham virus (JCV) and is characterized by progressive inflammation of the white matter at multiple locations in the brain. A 10-year-old girl with repetitive histories of frequent respiratory tract infections was admitted to our hospital due to dyspnea and fever. She had been diagnosed with primary ciliary dyskinesia at 6 years of age, and a lymphocyte analysis at that period revealed a decreased CD19+ cell count. During admission, she developed a motor deficit of the right leg and arm with gradual aggravation. Magnetic resonance imaging showed high signal intensity on the T2-weighted images and low signal intensity on the T1-weighted images in the lower mid-brain, pons, medulla, both cerebral peduncles and right cerebellum
with partial extension into the left cerebellar hemisphere. Laboratory tests to identify the diseases of infection, malignancy, and autoimmune origin returned negatives. Biopsy results of the lesion in the cerebellum revealed PML due to JCV. On
the 59th day of hospitalization, she expired of acute respiratory distress syndrome due to sepsis.
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KEYWORD
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Progressive multifocal leukoencephalopathy, John Cunningham virus, brain biopsy
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