INTRAVASCULAR LYMPHOMATOSIS OF THE NERVOU S-SYSTEM (IVL-NS)

We report on a typical case of intravascular lymphomatosis, a rarely d iagnosed, generalised intravascular lymphoma usually of the B-cell typ e. In most cases there is a lack of clear haematological findings but in more than 50% intravascular lymphomatosis presents with symptoms of the central nervous system. Every rapidly progressive neurological de ficit, especially the association of a subacute dementia with a spinal syndrome may suggest IVL-NS. However, careful examination may detect minor features for a systemic process in 25-80% i.e. B-symptoms elevat ion of ESR and LDH. Neurological imaging demonstrates multifocal lesio ns in the CNS with affinity to the deep white matter consistent with a microvascular or demyelinating disease. Angiographically IVL-NS may m imic cerebral vasculitis. CSF findings are nonspecific. Because diagno sis can only be made histologically, a cerebral biopsy should be under taken in suspected cases. Usually the course of the disease is fatal. Therapy involving steroids, combination polychemotherapy or radiation met with only minor success.