Lymphomatosis Cerebri
A 64-year-old male patient presented with gait disturbance and slowness of behavior. At another hospital, cerebrospinal fluid (CSF) analysis and cytology were performed to differentiate several diseases such as ischemic diseases, infectious diseases, infiltrative tumors, and neurodegenerative disorders. But, there were no positive results among the tested. Conservative treatments including antiplatelet drug without steroids under the impression of ischemic diseases were applied for about 2 months. However, his neurological symptoms were aggravated gradually and cognitive functions declined too. He was transferred to our hospital for further evaluation. On neurological examinations, he was drowsy deeply and obeyed only simple commands. He could not stand by himself, even sitting. Kanofsky performance status (KPS) score was 30. He was severely disabled and needed special cares for daily living activities. We had reviewed the clinical data including several images taken from outside meticulously. Brain computed tomography (CT) revealed no specific findings. Brain MRIs showed extensive, diffuse hyper-intense lesions without enhancement involving bilateral cerebral hemispheres and brainstem on both T2 and fluid attenuated inversion recovery (FLAIR)-weighted MRIs (). MR angiograms were normal. Follow-up brain MRIs taken from our hospital revealed further progression of T2 and FLAIR hyper-intense lesions involving the gray and white matters together extending into both thalamus and periventricular deep white matters (). Definite focal enhancement lesions were not seen on enhanced T1-weighted MRI (). Initially, infiltrative tumors such as gliomatosis cerebri were suspected. Since it was difficult to confirm diagnosis with those clinical studies only, a stereotactic biopsy was performed for pathological diagnosis. Two separated targets to avoid false-negative results were selected. One was the lesion within the deep white matter of left frontal lobe and the other was the lesion close to the left frontal horn which showed brighter T2 signal intensity than first one. Both separated targets for biopsy were targeted on the T2 and FLAIR images. Scattered atypical lymphocytes and perivascular lymphoid cells were found in the two specimens. These tumor cells were strongly immunoreactive with CD20 which was consistent with diffuse large B lymphoma (). Despite subsequent high dose corticosteroids for 1 week, there were no further neurological improvements up to 3 months.