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A healthy 24-year-old man presented with 1 week of headache, gait imbalance, and coordination difficulties. His mother reported that he had experienced personality changes. He denied any fevers, chills, night sweats, or weight loss. He reported smoking marijuana and denied tobacco and intravenous drug use. He is sexually active, with multiple male partners.
On examination, he was alert and oriented, with a temperature of 36.3°C, blood pressure of 131/90 mm Hg, heart rate of 94/min, respiratory rate of 18/min with pulse oximetry of 98% on ambient air, and a body mass index of 28.1 (calculated as weight in kilograms divided by height in meters squared). Neurologic examination was significant for hyperreflexia in all extremities, bilateral shoulder weakness, and right-sided pronator drift. Laboratory results showed a white blood cell count of 3600/μL (73.5% neutrophils, 16.4% lymphocytes, 7.8% monocytes, 1.4% eosinophils, 0.6% basophils); the remainder of the complete blood cell count and comprehensive metabolic profile was unremarkable. The result of a human immunodeficiency virus (HIV) type 1 antibody test was positive, and CD4 cell count was 73/mm3. Results of serologic testing for Toxoplasma gondii IgG were negative. Computed tomography (CT) imaging of the brain showed a left caudate nucleus and lentiform nucleus lesion with mass effect on the left lateral ventricle (Figure, left panel). Fluid-attenuated inversion recovery magnetic resonance imaging (MRI) of the brain confirmed the presence of an aggressive mass lesion extending through the corpus callosum (Figure, right panel). CT of the chest, abdomen, and pelvis and spinal MRI were unremarkable.
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Primary CNS lymphoma (PCNSL)
B. Obtain a brain biopsy
The key to the diagnosis in this case was an imaging finding of an aggressive solitary lesion suggesting malignancy. The most common malignant brain mass in patients with AIDS is PCNSL, particularly in individuals with CD4 cell counts less than 100/mm3. Brain tumors arising from CNS tissue and metastatic malignancies are possible but less likely. Since PCNSL is an Epstein-Barr virus (EBV)–associated B-cell lymphoma, a positive CSF polymerase chain reaction (PCR) assay is highly suggestive of the diagnosis. In this case, a lumbar puncture (option C) was contraindicated because of imaging consistent with increased intracranial pressure. Other causes of brain mass in patients with human immunodeficiency virus (HIV) are toxoplasma encephalitis and progressive multifocal leukoencephalopathy. The latter does not manifest with a mass effect as seen in this case, but toxoplasma encephalitis can present as a solitary, aggressive mass on MRI. This patient had a negative toxoplasma IgG serologic status, making toxoplasmosis unlikely. Had he presented subacutely, had multiple ring-enhancing lesions on MRI, and/or had a positive toxoplasma serologic status, a treatment trial with 10 to 14 days of sulfadiazine and pyrimethamine for toxoplasmosis would be reasonable before considering a brain biopsy (option D). However, the rapid progression, lack of above-mentioned features, and possibility of PCNSL required more urgent diagnosis and treatment. Antiretroviral therapy (ART) is clearly indicated and was given to the patient (option A); outcomes with PCNSL improve when the viral load declines and CD4 cells recover. However, confirming the diagnosis (option B) is vital before initiating antitumor therapy.
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Corresponding Author: N. Cary Engleberg, MD, Division of Infectious Diseases, Department of Internal Medicine, University of Michigan Health System, 3119P Taubman Center Box 5378, 1500 E Medical Center Dr, Ann Arbor, MI 48109 (email@example.com)
Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.
Additional Contributions: We thank the patient’s mother for providing permission to share the patient’s information.
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