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Headache, Confusion, and Somnolence in a 27-Year-Old Woman

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

A previously healthy 27-year-old woman presented to the emergency department with 2 weeks of headache, photophobia, vomiting, and confusion. She denied fevers or chills. She was born in Guatemala and had immigrated to the US approximately 1 year ago, without subsequent international travel. She took no medications. On examination, she was afebrile (36.8 °C) and hemodynamically stable, with heart rate 70/min and blood pressure 95/53 mm Hg. She was somnolent and confused without focal neurologic deficits. Serum white blood cell count was elevated to 14.9 ×109/L (86% segmented neutrophils, 9% lymphocytes, 5% monocytes, 0% basophils/eosinophils). A computed tomography scan of her brain was notable for diffuse ventriculomegaly and sulcal effacement consistent with hydrocephalus and cerebral edema. Given her diminished level of consciousness, a ventriculostomy was placed emergently. Laboratory analysis of cerebrospinal fluid (CSF) obtained from the ventriculostomy was notable for 40 red blood cells/mm3; 3 nucleated cells/mm3; protein level, 11 mg/dL; and glucose level, 73 mg/dL. Magnetic resonance imaging (MRI) of the brain with and without contrast demonstrated ventriculomegaly of the lateral and third ventricles, with a 0.4 × 0.4 × 0.6–cm nonenhancing lesion obstructing the aqueduct of Sylvius (Figure 1A). No abnormal meningeal enhancement or parenchymal lesion was identified. On hospital day 5, in the midst of an ongoing infectious diseases workup, the measured intracranial pressure and rate of CSF drainage from the ventriculostomy suddenly decreased. Repeat MRI demonstrated resolved hydrocephalus, a patent aqueduct of Sylvius, and the aforementioned nonenhancing lesion now located in the fourth ventricle (Figure 1B).

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A previously healthy 27-year-old woman presented to the emergency department with 2 weeks of headache, photophobia, vomiting, and confusion. She denied fevers or chills. She was born in Guatemala and had immigrated to the US approximately 1 year ago, without subsequent international travel. She took no medications. On examination, she was afebrile (36.8 °C) and hemodynamically stable, with heart rate 70/min and blood pressure 95/53 mm Hg. She was somnolent and confused without focal neurologic deficits. Serum white blood cell count was elevated to 14.9 ×109/L (86% segmented neutrophils, 9% lymphocytes, 5% monocytes, 0% basophils/eosinophils). A computed tomography scan of her brain was notable for diffuse ventriculomegaly and sulcal effacement consistent with hydrocephalus and cerebral edema. Given her diminished level of consciousness, a ventriculostomy was placed emergently. Laboratory analysis of cerebrospinal fluid (CSF) obtained from the ventriculostomy was notable for 40 red blood cells/mm3; 3 nucleated cells/mm3; protein level, 11 mg/dL; and glucose level, 73 mg/dL. Magnetic resonance imaging (MRI) of the brain with and without contrast demonstrated ventriculomegaly of the lateral and third ventricles, with a 0.4 × 0.4 × 0.6–cm nonenhancing lesion obstructing the aqueduct of Sylvius (Figure 1A). No abnormal meningeal enhancement or parenchymal lesion was identified. On hospital day 5, in the midst of an ongoing infectious diseases workup, the measured intracranial pressure and rate of CSF drainage from the ventriculostomy suddenly decreased. Repeat MRI demonstrated resolved hydrocephalus, a patent aqueduct of Sylvius, and the aforementioned nonenhancing lesion now located in the fourth ventricle (Figure 1B).

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Article Information

Corresponding Author: Tianyi Niu, MD, Department of Neurosurgery, The Warren Alpert School of Medicine, Brown University, Rhode Island Hospital, 593 Eddy St, APC 6, Providence, RI 02903 (rahul.sastry@gmail.com).

Published Online: October 9, 2020. doi:10.1001/jama.2020.10167

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for providing permission to share her 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 [and Self-Assessment requirements] 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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