원발성 섬모 이상운동증 환자에서 발생한 진행성 다소성 백질뇌증 1례

초록

Background: Progressive multifocal leukoencephalopathy (PML) is a rare and usually fatal viral disease that is characterized by progressive inflammation of the white matter of the brain at multiple locations. The cause of PML is a type of polyomavirus called the John Cunningham virus (JCV), which is normally present and downregulated by the immune system. Case: The patient was a 10 year old female with repetitive histories of frequent sinusitis, pneumonia and otitis media. She was diagnosed as primary ciliary dyskineisa in 2006. She was admitted with chief complaints of dyspnea and fever, which had developed a day before. During admission, she developed movement disorders of the right leg and arm with gradual aggravation. On neurologic examination, she showed a motor grade 2-3 of the right extremities, and her sensory was intact. Cerebrospinal fluid results were normal. MRI results showed high signal intensity in the lower midbrain, pons, medulla, both cerebral peduncles and right cerebellum with partial extension into the left cerebellar hemisphere on T2W1 and FLAIR images. Laboratory tests to rule out other similar disease, such as anti-Sm antibody, anti-histone antibody, rheumatoid factor, etc. were negative. Biopsy results of the lesion in the brain revealed progressive multifocal leukoencephalopathy due to JC virus. On the 59th day of admission, she expired due to acute respiratory distress syndrome and sepsis. Conclusion: We report a case of progressive multifocal leukoencephalopathy due to JC virus in a pediatric patient.