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Headache, Cognitive Decline, and a Curious Rim-Enhancing Lesion

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 75-year-old man presented with several weeks of intermittent headache that had progressed to severe, persistent headache with right eye pain. He had hypertension, hyperlipidemia, diabetes, and atrial fibrillation. He denied tobacco, alcohol, or drug use. A neurological examination was notable for disorientation to the month, deficits in short-term memory, and an upward drift of the left arm. Cranial nerve, sensory, coordination, and deep tendon reflex examinations were normal. He walked with a slightly wide but steady gait. His serum red and white blood cell counts, coagulation profile, and electrolyte levels were within normal limits. Blood cultures had negative results. A 24-hour electroencephalogram showed right hemispheric slowing and multiple right centroparietal seizures of 1 minute each, without noticeable clinical correlates. A magnetic resonance image (MRI) of the brain was remarkable for a large T2-hyperintense lesion in the right temporoparietal region, with an increased diffusion-weighted imaging (DWI) signal (low apparent diffusion coefficient), a low signal on susceptibility-weighted imaging, increased T1 signal intensity in and around the lesion, and a contrast-enhancing rim with leptomeningeal enhancement (Figure 1). A smaller satellite lesion with a similar MRI profile was noted inferiorly in the right anterior temporal lobe. A computed tomography scan of the chest, abdomen, and pelvis did not show any metastatic disease. A cerebrospinal fluid (CSF) profile showed a normal red blood cell count and protein and glucose levels and a mildly elevated white blood cell count of 11 cells/μL (to convert to cells × 109/L, multiply by 0.001). The fluid was absent oligoclonal bands and had negative bacterial and fungal culture results, a meningitis-encephalitis polymerase chain reaction panel, and a paraneoplastic panel. Cytology and flow cytometry had negative results. The patient then underwent surgical biopsy of the temporoparietal lesion.

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A 75-year-old man presented with several weeks of intermittent headache that had progressed to severe, persistent headache with right eye pain. He had hypertension, hyperlipidemia, diabetes, and atrial fibrillation. He denied tobacco, alcohol, or drug use. A neurological examination was notable for disorientation to the month, deficits in short-term memory, and an upward drift of the left arm. Cranial nerve, sensory, coordination, and deep tendon reflex examinations were normal. He walked with a slightly wide but steady gait. His serum red and white blood cell counts, coagulation profile, and electrolyte levels were within normal limits. Blood cultures had negative results. A 24-hour electroencephalogram showed right hemispheric slowing and multiple right centroparietal seizures of 1 minute each, without noticeable clinical correlates. A magnetic resonance image (MRI) of the brain was remarkable for a large T2-hyperintense lesion in the right temporoparietal region, with an increased diffusion-weighted imaging (DWI) signal (low apparent diffusion coefficient), a low signal on susceptibility-weighted imaging, increased T1 signal intensity in and around the lesion, and a contrast-enhancing rim with leptomeningeal enhancement (Figure 1). A smaller satellite lesion with a similar MRI profile was noted inferiorly in the right anterior temporal lobe. A computed tomography scan of the chest, abdomen, and pelvis did not show any metastatic disease. A cerebrospinal fluid (CSF) profile showed a normal red blood cell count and protein and glucose levels and a mildly elevated white blood cell count of 11 cells/μL (to convert to cells × 109/L, multiply by 0.001). The fluid was absent oligoclonal bands and had negative bacterial and fungal culture results, a meningitis-encephalitis polymerase chain reaction panel, and a paraneoplastic panel. Cytology and flow cytometry had negative results. The patient then underwent surgical biopsy of the temporoparietal lesion.

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Article Information

Corresponding Author: Charles B. Beaman, MD, PhD, Department of Neurology, Columbia University Irving Medical Center, 710 W 168th St, New York, NY 10032 (cb3333@cumc.columbia.edu).

Published Online: March 8, 2021. doi:10.1001/jamaneurol.2021.0161

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient’s son for granting permission to publish this information.

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AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 credit toward the CME of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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