The neurological masquerade of intravascular lymphomatosis.
BACKGROUND
Intravascular lymphomatosis (IVL) is an uncommon systemic disease characterized by occlusion of small vessels by malignant lymphomatous cells. Central nervous system involvement usually presents as subacute encephalopathy, dementia, seizures, or multifocal cerebrovascular events.


OBJECTIVE
To increase awareness about IVL, an uncommon cause of neurological disease.


METHODS
This is a retrospective case series of 8 pathologically proved cases of IVL with neurological disease. Patients were part of a pathological series collected between April 1962 and October 1998 at Indiana University School of Medicine and the Armed Forces Institute of Pathology, Washington, DC.


METHODS
Neurological and neuropathological examinations were performed at tertiary referral hospitals.


METHODS
Eleven patients were diagnosed pathologically as having IVL, but 3 were not included in this evaluation because of a lack of appropriate clinical information. Of the final sample (n = 8), there were 4 men and 4 women (mean +/- SD age, 62.9 +/- 9.9 years).


RESULTS
All 8 patients had focal neurological deficits, 7 had encephalopathy or dementia, 5 had epileptic seizures, and 2 had myelopathy. Death occurred at a mean of 7.7 months (range, 1-24 months) after the onset of symptoms. All patients had elevated cerebrospinal fluid protein levels, 4 had pleocytosis, and 2 had an elevated IgG level in their cerebrospinal fluid. Of the 4 patients who underwent a brain biopsy, 1 was diagnosed as having IVL before death.


CONCLUSIONS
Intravascular lymphomatosis is an uncommon disease with a myriad of potential neurological manifestations. Diagnosis requires a high index of suspicion and a pathological examination. If diagnosed early, aggressive chemotherapy is potentially curative, although the overall prognosis remains dismal.
Beristain
Xabier
X
Department of Neurology, Indiana University School of Medicine, 545 Barnhill Dr, Emerson Hall, Suite 125, Indianapolis, IN 46202, USA. xberista@iupui.edu
Azzarelli
Biagio
B
D000328:Adult; D000368:Aged; D001706:Biopsy; D001921:Brain / Q000473:pathology; D001927:Brain Diseases / Q000209:etiology; D002556:Cerebrospinal Fluid Proteins / Q000032:analysis; D003704:Dementia / Q000209:etiology; D004827:Epilepsy / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007964:Leukocytosis / Q000209:etiology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000097:blood / Q000134:cerebrospinal fluid / Q000150:complications* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000209:etiology*; D012189:Retrospective Studies; D013118:Spinal Cord Diseases / Q000209:etiology; D019043:Vascular Neoplasms / Q000134:cerebrospinal fluid / Q000150:complications* / Q000473:pathology
D002556:Cerebrospinal Fluid Proteins; D007074:Immunoglobulin G