Progressive multifocal leucoencephalopathy (PML) is a rare disorder occurring when a strain of papovavirus (JC virus) infects the central nervous system. It results in a generally quick and fatal outcome. It is associated with cell mediated immune deficient diseases but some few cases were reported in immunocompetent hosts. Since 1981, it has been commonly associated with AIDS. In AIDS related PML, long term survival without real neurological improvement has been reported in patients treated with highly active antiretroviral therapy (HAART).¹ Few cases of improvement with cidofovir or cytosine arabinoside have been described in AIDS related or non-AIDS-related PML,^2,3 but in larger trials in AIDS related PML, no clinical benefit was found.⁴ As a whole, the treatment of this progressive demyelinating disease remains controversial, in particular in the rare cases of non-AIDS-related PML. We describe a patient with an underlying haematological disease, without clear cut immune cell deficiency, who developed rapidly progressive PML. The patient showed clinical, virological, and imaging improvement when treated with an association of intravenous and intrathecal cytosine arabinoside combined with intravenous cidofovir.

A 48 year old man presented with progressive multiple lymphadenopathies, hepatosplenomegaly, weight loss, and blood cell count abnormalities. Fine needle aspiration cytology of lymphadenopathy diagnosed marginal zone B cell lymphoma. There was also bone marrow and blood proliferation. A few weeks after the diagnosis, the patient noticed rotatory vertigo and visual problems suggestive of a right homonymous hemianopia. Because of dissemination and the large …