11/16/2004 Case 1: Primary T-cell lymphoma of CNS

Clinical History:

A 70 year old man developed a rapidly progressive dementia. MRI showed basal ganglionic enhancement. A brain biopsy was done to rule out Creutzfeldt-Jakob Disease; it did not demonstrate spongiform encephalopathy. Prior to the biopsy the patient was minimally responsive; after it he was improved, and was able to walk out of the hospital. However he again soon deteriorated, was transferred to a nursing home, and died, about 7 months after the onset of symptoms. Autopsy was limited to the brain.

Diagnostic Notes:

Dr Sharer pointed out that the biopsy slide had previously been briefly shown at a prior Neuroplex meeting. It showed suboptimal histological preparation because of the formic acid treatment used given the presumptive diagnosis of CJD. The biopsy raised a question of encephalitis. The autopsy slide distributed came from one of several areas of gross softening in the basal ganglia or midbrain. It showed a perivascular and somewhat diffuse mononuclear cell infiltrate, resembling a lymphoma or encephalitis. Immunostains showed that the cells were LCA/CD45+, CD20, CD3, CD30, CD56, and CD5 all -; and CD4 strongly + while CD8 all -. The diagnosis was therefore one of primary T cell CNS non-Hodgkin's lymphoma. Discussion ranged over the literature on dementia as a presenting sign of primary CNS lymphoma, and on the differential diagnosis. Dr Zagzag mentioned his published cases of mycosis fungoides spread to the nervous system. It was also discussed that the patient's improvement after the biopsy must have been related to treatment with steroids as a standard perioperative precaution.

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