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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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Mobile isolated intraventricular neurocysticercosis

C. Extract the lesion surgically

The key to the correct diagnosis is the MRI finding of a mobile intraventricular mass that had provoked obstructive hydrocephalus. The abrupt paroxysmal onset of hydrocephalus as a result of transient ventricular obstruction by a mobile mass has a small differential diagnosis that is limited to intraventricular tumors and free-floating cystic lesions. A mobile intraventricular cyst is essentially pathognomonic for the diagnosis of neurocysticercosis.1

Neurocysticercosis is the most common helminthic infection of the central nervous system and remains a major cause of seizures and hydrocephalus, both in endemic areas and in the US.2,3 The infection is transmitted via ingestion of Taenia solium eggs and spreads to the central nervous system from the stomach by way of vascular and lymphatic channels. The robust immunologic response to the cyst, which has a terminal life span, results in cerebral edema and associated symptoms.2 Although any neurologic deficit is possible, focal seizures are the most common manifestation.

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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.

References
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Del Brutto  OH , Nash  TE , White  AC  Jr ,  et al.  Revised diagnostic criteria for neurocysticercosis.   J Neurol Sci. 2017;372:202-210. doi:10.1016/j.jns.2016.11.045PubMedGoogle ScholarCrossref
2.
White  AC  Jr , Coyle  CM , Rajshekhar  V ,  et al.  Diagnosis and treatment of neurocysticercosis: 2017 Clinical Practice Guidelines by the Infectious Diseases Society of America (IDSA) and the American Society of Tropical Medicine and Hygiene (ASTMH).   Clin Infect Dis. 2018;66(8):e49-e75. doi:10.1093/cid/cix1084PubMedGoogle ScholarCrossref
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Proaño  JV , Madrazo  I , García  L , García-Torres  E , Correa  D .  Albendazole and praziquantel treatment in neurocysticercosis of the fourth ventricle.   J Neurosurg. 1997;87(1):29-33. doi:10.3171/jns.1997.87.1.0029PubMedGoogle ScholarCrossref
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Nash  TE , Ware  JM , Mahanty  S .  Intraventricular neurocysticercosis: experience and long-term outcome from a tertiary referral center in the United States.   Am J Trop Med Hyg. 2018;98(6):1755-1762. doi:10.4269/ajtmh.18-0085PubMedGoogle ScholarCrossref
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Apuzzo  MLJ , Dobkin  WR , Zee  C-S , Chan  JC , Giannotta  SL , Weiss  MH .  Surgical considerations in treatment of intraventricular cysticercosis: an analysis of 45 cases.   J Neurosurg. 1984;60(2):400-407. doi:10.3171/jns.1984.60.2.0400PubMedGoogle ScholarCrossref
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Schantz  PM , Moore  AC , Muñoz  JL ,  et al.  Neurocysticercosis in an orthodox Jewish community in New York City.   N Engl J Med. 1992;327(10):692-695. doi:10.1056/NEJM199209033271004PubMedGoogle ScholarCrossref
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Tsang  VC , Brand  JA , Boyer  AE .  An enzyme-linked immunoelectrotransfer blot assay and glycoprotein antigens for diagnosing human cysticercosis (Taenia solium).   J Infect Dis. 1989;159(1):50-59. doi:10.1093/infdis/159.1.50PubMedGoogle ScholarCrossref
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Bergsneider  M , Holly  LT , Lee  JH , King  WA , Frazee  JG .  Endoscopic management of cysticercal cysts within the lateral and third ventricles.   J Neurosurg. 2000;92(1):14-23. doi:10.3171/jns.2000.92.1.0014PubMedGoogle ScholarCrossref
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Colli  BO , Martelli  N , Assirati  JA  Jr , Machado  HR , de Vergueiro Forjaz  S .  Results of surgical treatment of neurocysticercosis in 69 cases.   J Neurosurg. 1986;65(3):309-315. doi:10.3171/jns.1986.65.3.0309PubMedGoogle ScholarCrossref
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Citow  JS , Johnson  JP , McBride  DQ , Ammirati  M .  Imaging features and surgery-related outcomes in intraventricular neurocysticercosis.   Neurosurg Focus. 2002;12(6):e6. doi:10.3171/foc.2002.12.6.7PubMedGoogle Scholar
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