[Intravascular lymphomatosis of the nervous system--case report and review of the literature].
We report on a typical case of intravascular lymphomatosis, a rarely diagnosed, generalised intravascular lymphoma usually of the B-cell type. 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 deficit, 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 elevation of ESR and LDH. Neurological imaging demonstrates multifocal lesions in the CNS with affinity to the deep white matter consistent with a microvascular or demyelinating disease. Angiographically IVL-NS may mimic cerebral vasculitis. CSF findings are nonspecific. Because diagnosis can only be made histologically, a cerebral biopsy should be undertaken in suspected cases. Usually the course of the disease is fatal. Therapy involving steroids, combination polychemotherapy or radiation met with only minor success.
Heesen
C
C
Neurologische Klinik, Universität Hamburg.
Bergmann
M
M
Figge
C
C
Löschke
S
S
Feldmann
M
M
D000368:Aged; D001158:Arteries / Q000473:pathology; D001921:Brain / Q000098:blood supply*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D016393:Lymphoma, B-Cell / Q000175:diagnosis* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D013116:Spinal Cord / Q000098:blood supply*; D014057:Tomography, X-Ray Computed; D019043:Vascular Neoplasms / Q000175:diagnosis* / Q000473:pathology; D014680:Veins / Q000473:pathology
Die intravaskuläre Lymphomatose des Nervensystems (IVL-NS)--Kasuistik und Literaturübersicht.