Signs of rapidly progressive dementiain a case of intravascular lymphomatosis

Intravascular lymphomatosis (IVL), a raretype of non–Hodgkin’s lymphoma, is an uncommoncause of progressive dementia, usually followed by deathwithin a few months of onset of clinical disease. Oftenthis aggressive tumor is only diagnosed at autopsy, becauseof misleading clinical features mimicking a broadspectrum of syndromes and the absence of circulatinglympoma cells in the blood, bone marrow or cerebrospinalfluid in many cases. Here we present IVL in a78–year–old woman with findings leading to the clinicaldiagnosis of vascular dementia with sudden beginningand positive 14–3–3 protein in the CSF, commonly reportedin Creutzfeldt–Jakob disease (CJD).