Primary cerebral angioimmunoblastic T-cell lymphoma.

Case Report A 71-year-old woman with a past medical history of chronic alcoholism and depression presented with a subacute confusional state and was admitted to the Department of Neurology (Hôpital Pierre Wertheimer, Bron, France) in November 2008. At admission, the patient was somnolent, apathetic, and confused. Her spatiotemporal orientation and cognitive function were impaired. She suffered from a moderately painful headache but presented no meningeal syndrome. An initial neurologic examination was performed, which did not reveal any focal abnormality or cranial nerve palsy. A general examination of the patient was normal except for weight loss and a low-grade fever (38.3°C), which disappeared in time. No peripheral lymphadenopathy, splenomegaly hepatomegaly, and/or rash were noted. The patient had a WHO performance status of 3. All laboratory tests results, including C-reactive protein levels, lactate dehydrogenase levels, and CBC, were normal. HIV testing was negative. A diagnosis of metabolic alcoholor drug-related disorders was rejected. Brain computed tomography (CT) revealed extensive hypodensity in the right temporal lobe. Brain magnetic resonance imaging (MRI) revealed hyperintense lesions in the right temporal lobe on T2-weighted fluid-attenuated inversion recovery sequences (Fig 1A) and diffusion-weighted sequences. After gadolinium injection, the signal was enhanced in the lesion periphery, which suggested the presence of mild edema (Fig 1B). MRI also revealed leptomeningeal contrast enhancement in the right temporal region. Some lesions were also detected in the contralateral insular cortex. CSF analysis revealed high total protein levels (1.37 mg/L), a normal CSF-to-serum glucose ratio, and increased cellularity (42 cells/ L) with pleocytosis (lymphocytes, lymphoplasmacytic cells, and plasma cells). No atypical cell or infectious agent was identified. Oligoclonal immunoglobulin G bands were present. Because of a strong suspicion of herpes simplex virus meningoencephalitis, an intravenous administration of acyclovir was initiated. However, no herpes simplex virus DNA was detected in the CSF by polymerase chain reaction (PCR), and this treatment was stopped. The results of a full infection screening of the serum and CSF by PCR were negative, except for Epstein-Barr virus (EBV) DNA in the CSF (3.0 log copies/mL). The results of the immunologic tests were also negative. An electroencephalogram revealed an irregular diffuse abnormal slow-wave activity. After stimulation, an asymmetrical activity of periodic biphasic waves, which were clearly predominant over the right hemisphere, was observed. Contrast-enhanced CT of the chest, abdomen, and pelvis was normal and did not indicate the presence of a malignant tumor. Combined CT and [F]fluorodeoxyglucose (FDG) positron emission tomography (PET) was performed 4 days after the brain MRI (Fig 2). Axial and coronal slices of FDG PET revealed a hypermetabolic focus (Fig 2, arrows) in the right temporal lobe that corresponded to known brain lesions. No extracerebral abnormality was identified. Bone marrow aspiration did not show infiltration by lymphoma or any other neoplastic disease. The dilated eye examination, including a slit-lamp evaluation, was normal.