Progressive multifocal leukoencephalopathy in a patient with idiopathic CD4+ cells deficit

Progressive multifocal leukoencephalopathy (PML) is a fatal neurological disease affecting the central nervous system. JC polyomavirus is the agent related to this disease. PML usually occurs in patients with HIV infection or other immunodeficiencies. We report a case of PML in a patient with idiopathic CD4+ cells deficit. The symptoms began with right arm hyposthenia followed by right hemiplegia. Blood analyses were normal, the only abnormal value was a marked decrease in CD4+ cells count with normal CD8+ cells. The magnetic resonance imaging (MRI) of the brain, showed multiple non-homogeneous lesions without enhancement in the left callous circumvolution and in the sub-cortical left frontal white matter. In the following two weeks, the patient had relevant progression in neurological deficits and a subsequent MRI demonstrated significant worsening. Because of the rapid clinical progression, we decided to start therapy with Cidofovir. The patient, after one month of admission, was slowly worsening in neurological functions.