Lymphomatosis Cerebri Research Articles

Lymphomatosis cerebri as a cause of white matter dementia

Primary central nervous system lymphoma most often presents as a solitary, isolated lesion in immunocompetent patients. Rarely, the disease presents as a diffuse, infiltrating condition without formation of a cohesive mass, a pattern called lymphomatosis cerebri. We present 3 immunocompetent individuals who developed rapidly progressive dementia. Magnetic resonance imaging features mimicked other disorders of white matter and prompted preoperative diagnoses of Binswanger's disease (subcortical ischemic vascular dementia), unknown leukoencephalopathy, viral infection, or infiltrating glioma. Neuropathologic examination at biopsy (Poon T, Matoso I, Tchertkoff V, Weitzner I Jr, Gade M. CT features of primary cerebral lymphoma in AIDS and non-AIDS patients. J Comput Assist Tomogr . 1989;13:6-9) and autopsy (Schwaighofer BW, Hesselink JR, Press GA, Wolf RL, Healy ME, Berthoty DP. Primary intracranial CNS lymphoma: MR manifestations. Am J Neuroradiol . 1993;10:725-9) demonstrated nonnecrotic, diffusely infiltrating, large-cell B-cell lymphoma of white matter, with relative sparing of gray matter, and without significant leptomeningeal involvement or bulky periventricular disease at autopsy. Microglial and astrocytic reactions, but only subtle myelin pallor, were evident as individual tumor cells permeated the entire brain and spinal cord, albeit with considerable variation in cell density. Individual tumor cells could be identified from the optic nerve to spinal cord, documenting the "whole-brain" nature of the disease. CD20 immunostaining was necessary to fully appreciate the extent of individual lymphoma cell percolation through the white matter. The neurobehavioral deficits manifested by these patients demonstrate that lymphomatosis cerebri is an additional neoplastic cause of white matter dementia and can be added to the growing list of disorders responsible for this syndrome.

Current Status of Treatment for Malignant Lymphomas

In cats, lymphoma (lymphosarcoma) is the most common neoplasm affecting the spinal cord and the second most common intracranial tumour. Although lymphoma commonly develops in the spinal cord as a part of a multicentric process, a primary form may occur. Lymphoma can exhibit a wide range of morphological patterns, including intraparenchymal brain mass, lymphomatosis cerebri, intravascular lymphoma, lymphomatous choroiditis and meningitis, extradural, intradural-extramedullary or intramedullary lymphoma in the spinal cord, or neurolymphomatosis in the peripheral nerves. Lymphoma may occur as a paraneoplastic disorder associated with peripheral neuropathies. Magnetic resonance imaging (MRI) and computed tomography (CT) are the techniques of choice for morphological assessment of nervous system lesions in vivo. However, biopsy should be performed to achieve a definitive diagnosis. Knowledge of the different morphological patterns expressed by lymphoma in the nervous system of cats allows veterinary clinicians to suspect lymphoma and to arrange appropriate diagnostic procedures, including immunophenotype and clonality studies, along with therapeutic protocols and prognostic evaluations.