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A Woman With Eosinophilic Brainstem Meningoencephalitis

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 25-year-old woman presented with headache, dysarthria, double vision, and dizziness for 1 week. She denied any history of fever, head trauma, vomiting, diarrhea, abdominal pain, or cough. She had traveled to northeastern Brazil 2 months before the onset of the neurological symptoms.

On examination, she had a mild right-side hemiparesis with hyperreflexia and hemiataxia. She could walk unassisted but presented with a broad base. There were no meningeal signs. She did not present with hepatosplenomegaly of lymphadenopathy. Her cranial magnetic resonance imaging showed a T2-weighted fluid-attenuated inversion recovery hyperintense lesion with heterogeneous gadolinium enhancement in the pons (Figure). There were no signs of diffusion restriction on diffusion-weighted imaging or hemorrhages on susceptibility-weighted imaging.

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B. Neuroschistosomiasis

Although listeriosis is a major cause of rhombencephalitis, the CSF typically does not show eosinophilic meningoencephalitis, and the patient’s blood and CSF cultures were sterile. Beyond listeriosis, other infections (herpesvirus infections and tuberculosis) and autoimmune diseases (Behçet disease, systemic lupus erythematosus, vasculitis, demyelinating diseases, and paraneoplastic syndromes) may cause rhombencephalitis.1 The patient presented with a nodular lesion with an heterogenous, multinodular, contrast-enhancing pattern consistent with a granulomatous disease (eg, infection by mycobacteria, fungi, parasite, or even sarcoidosis).2,3

The differential diagnosis is narrowed largely by the CSF characteristics. Eosinophilic meningoencephalitis is defined as the presence of more than 10 eosinophils/mm3 in CSF and/or eosinophils accounting for more than 10% of cerebrospinal leukocytes.4 The most common cause of eosinophilic meningoencephalitis is invasion of the central nervous system by helminthic parasites, such as Angiostrongylus cantonensis, Gnathostoma species, Schistosoma mansoni, and Taenia solium.5 Eosinophilic granulomatosis with polyangiitis may present eosinophilia and neuromyelitis-optica spectrum disorders may present some eosinophils in the CSF, but we would not expect sufficient eosinophils to fulfill the definition of eosinophilic meningoencephalitis in these diseases.

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Article Information

Corresponding Author: Lucas Fernandes Ferreira, Department of Neurology, Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo, Dr Eneas de Carvalho de Aguiar St 255, Sao Paulo, SP, 05403-000, Brazil (lucasfernandes150@gmail.com).

Published Online: January 10, 2022. doi:10.1001/jamaneurol.2021.4861

Conflict of Interest Disclosures: None reported.

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

References
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2.
Shih  RY , Koeller  KK .  Bacterial, fungal, and parasitic infections of the central nervous system: radiologic-pathologic correlation and historical perspectives: from the radiologic pathology archives.   Radiographics. 2015;35(4):1141-1169. doi:10.1148/rg.2015140317PubMedGoogle ScholarCrossref
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Saleem  S , Belal  AI , El-Ghandour  NM .  Spinal cord schistosomiasis: MR imaging appearance with surgical and pathologic correlation.   AJNR Am J Neuroradiol. 2005;26(7):1646-1654.PubMedGoogle Scholar
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Lo Re  V  III , Gluckman  SJ .  Eosinophilic meningitis.   Am J Med. 2003;114(3):217-223. doi:10.1016/S0002-9343(02)01495-XPubMedGoogle Scholar
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Sawanyawisuth  K , Chotmongkol  V .  Eosinophilic meningitis.   Handb Clin Neurol. 2013;114:207-215. doi:10.1016/B978-0-444-53490-3.00015-7PubMedGoogle ScholarCrossref
6.
Ferrari  TCA , Moreira  PRR .  Neuroschistosomiasis: clinical symptoms and pathogenesis.   Lancet Neurol. 2011;10(9):853-864. doi:10.1016/S1474-4422(11)70170-3PubMedGoogle ScholarCrossref
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Lindholz  CG , Favero  V , Veríssimo  CM ,  et al.  Study of diagnostic accuracy of Helmintex, Kato-Katz, and POC-CCA methods for diagnosing intestinal schistosomiasis in Candeal, a low intensity transmission area in northeastern Brazil.   PLoS Negl Trop Dis. 2018;12(3):e0006274. doi:10.1371/journal.pntd.0006274PubMedGoogle Scholar
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Centers for Disease Control (CDC).  Acute schistosomiasis with transverse myelitis in American students returning from Kenya.   MMWR Morb Mortal Wkly Rep. 1984;33(31):445-447.PubMedGoogle Scholar
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Bruscky  IS , Bruscky  DMV , Melo  FL , Medeiros  ZM , Correia  CDC .  Cerebral mansoni schistosomiasis: a systematic review of 33 cases published from 1989 to 2019.   Trans R Soc Trop Med Hyg. Published online May 26, 2021. doi:10.1093/trstmh/trab067Google Scholar
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Gray  DJ , Ross  AG , Li  YS , McManus  DP .  Diagnosis and management of schistosomiasis.   BMJ. 2011;342:d2651. doi:10.1136/bmj.d2651PubMedGoogle ScholarCrossref
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