Subject(s)
Lymphoma/blood , Lymphoma/cerebrospinal fluid , Vascular Neoplasms/blood , Vascular Neoplasms/cerebrospinal fluid , CD79 Antigens/genetics , Central Nervous System Neoplasms/diagnosis , Cerebrospinal Fluid/chemistry , Female , Humans , Liquid Biopsy , Lymphoma/diagnosis , Lymphoma/genetics , Middle Aged , Mutation , Vascular Neoplasms/diagnosis , Vascular Neoplasms/geneticsABSTRACT
TITLE: Demencia rapidamente progresiva con criterios de enfermedad de Creutzfeldt-Jakob: un caso de linfoma intravascular cerebral.
Subject(s)
Akinetic Mutism/etiology , Brain Neoplasms/diagnosis , Creutzfeldt-Jakob Syndrome/diagnosis , Dementia/etiology , Diagnostic Errors , Lymphoma, B-Cell/diagnosis , Vascular Neoplasms/diagnosis , 14-3-3 Proteins/cerebrospinal fluid , Aged , Brain Neoplasms/cerebrospinal fluid , Brain Neoplasms/complications , Brain Neoplasms/pathology , Delayed Diagnosis , Disease Progression , Electroencephalography , False Negative Reactions , Fatal Outcome , Humans , Lymphoma, B-Cell/cerebrospinal fluid , Lymphoma, B-Cell/complications , Lymphoma, B-Cell/pathology , Male , Neuroimaging , Neurologic Examination , Neuropsychological Tests , Vascular Neoplasms/cerebrospinal fluid , Vascular Neoplasms/complications , Vascular Neoplasms/pathologyABSTRACT
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. DESIGN: 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. SETTING: Neurological and neuropathological examinations were performed at tertiary referral hospitals. PATIENTS: 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.
