A Case of Progressive Multifocal Leikoencephalopathy in a 10 Year-old Girl with Idiopathic CD4+ Lymphocytopenia

초록

objective: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 downregulated by the immune system. Methods: a girl with repetitive histories of frequent respiratory tract infection A 10 year-old girl with repetitive histories of frequent respiratory tract infections was admitted to Inha University Hospital due to dyspnea and fever. She had been diagnosed as primary ciliary dyskinesia at 6 years-old and lymphocyte analysis conducted at that period revealed a decreased CD4+ T cell count. During admission, she developed movement disorders of the right leg and arm with gradual aggravation. On neurologic examination, she showed a motor grade of 2-3 on the right extremities, and her sensory was intact. Cerebrospinal fluid results were normal. Magnetic resonance imaging 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 T2-weighted images. Laboratory tests to identify diseases of infection, malignancy, or autoimmune origin were negative. Biopsy results of the lesion in the brain revealed PML due to JCV. On the 59th hospital day, she expired of acute respiratory distress syndrome due to sepsis. conclusion:We report a case of PML due to JCV in a pediatric patient with idiophathic CD4+ lymphocytopenia.