Recurrent primary central nervous system lymphoma mimicking neurodegenerative disease--an autopsy case report

A 42-year-old female presented with recurrent primary central nervous system lymphoma mimicking the roentgenographic appearance of diffuse brain degeneration. Betamethazone was administered, but her condition worsened. Biopsy of a swollen neck lymph node demonstrated lymphoma cells. Computed tomography and magnetic resonance (MR) imaging showed no contrast-enhanced lesions. T2-weighted MR imaging revealed diffuse, high intensity areas in the white matter of the bilateral cerebrum, basal ganglia, and brainstem. Despite chemotherapy for systemic lymphoma, she died of respiratory arrest. Histological examination of autopsy specimens showed diffuse infiltration of lymphoma cells in the perivascular space of the cerebral cortex, but little neovascularization. The absence of contrast enhancement may have been due to preservation of the blood-brain barrier. Histological confirmation of roentgenographic findings of brain degeneration is important in patients treated for primary central nervous system lymphoma.