Progressive multifocal leukoencephalopathy and idiopathic CD4 lymphocytopenia

Abstract

Idiopathic CD4 lymphocytopenia (ICL) is a syndrome described in patients with low counts of CD4 cells and no other causes for immunosuppression. A few cases of progressive multifocal leukoencephalopathy (PML) have been described in association with this entity. There is no effective treatment for any of them, and the clinical course and outcome are unpredictable. We report on a case of ICL with PML and review the literature, trying to identify the clinical features and the prognosis clues associated to these entities together. A 72-year-old man presented with acute onset gait instability that progressed to a severe cerebellar syndrome with cognitive decline. A cranial MRI showed findings consistent with PML, this diagnosis being confirmed by CSF analyses. Absolute number of CD4+ was 242 cells/μL. An extensive work-up including HIV tests was negative. Ten cases of PML and ICL have previously been reported. Factors contributing to the different outcomes are unknown. Although an effective treatment does not exist for PML, it has been recently demonstrated in vitro that several 5HT2A-receptor antagonists block the JC virus infection. Our patient greatly improved and remains stable 34 months after onset; we describe the potential role of mirtazapine in the treatment of PML.