Progressive multifocal leukoencephalopathy complicating untreated chronic lymphatic leukemia: case report and review of the literature.

A 58-year old female with a four-year history of previously untreated CLL at Binet stage A complained about word finding problems, impaired vision, and gait unsteadiness. Concerning her CLL she was asymptomatic and had never required any specific treatment. Her neurological examination disclosed cognitive alterations, homonyme hemianopia to the right, aphasia, and mild right-sided hemiparesis. Cerebral MRI showed a hyperintense lesion on T2 weighted images without contrast enhancement. CSF examination revealed normal findings, including CSF protein, cell count, cytology and PCR-analysis was negative for the presence of JC virus DNA. On follow-up MRI, performed 2 weeks later, the T2 lesion was further enlarging. Subsequent stereotactic brain biopsy was diagnostic for PML revealing abnormal oligodendrocytes staining positive against antibodies specific for simian vacuolating virus 40. In addition, repeated CSF analyses for JC-Virus DNA in the course of the disease became positive. After confirmation of diagnosis treatment with mirtazapine (30 mg/d) and mefloquine (250 mg/d) was initiated. Rapid clinical progression correlated to further worsening on MRI. Therefore this treatment was terminated after 16 days and the regime was changed to a five-day courses of cytarabine (2 mg/kg/d) combined with intrathecal administration of liposomal cytarabine (50 mg). Due to further clinical progression with global aphasia, blindness and severe right-sided hemiparesia, medication was stopped. The Patient died three and a half months after onset of symptoms.