AN UNUSUAL COURSE OF PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY IN A PATIENT WITH IDIOPATHIC CD4-LYMPHOCYTOPENIA( T)

A case is reported of idiopathic CD4+T lymphocytopenia with progressiv e multifocal leukoencephalopathy and cervical lymph node tuberculosis. A 57 year old Japanese man presented with cervical lymphadenopathy an d progressive neurological deficits, and six months later he developed akinetic mutism. He had a persistent severely depressed number of cir culating CD4+T lymphocytes in the absence of human immunodeficiency vi rus infection. T1 weighted MRI showed a diffuse decreased signal inten sity Limited to the white matter without mass effect. A brain biopsy s pecimen had a morphology similar to that of progressive multifocal leu koencephalopathy. Polyomavirus antigen was detected in the brain lesio n, and viral DNA was identified in nucleated blood cells and urine. Un usually this serious medical condition has lasted for more than three years without remission. To our knowledge this is the first patient wi th CD4+T lymphocytopenia with progressive multifocal leukoencephalopat hy, suggesting that similar opportunistic infections should be conside red even in previously normal people.