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Multifocal neutrophilic meningoencephalitis: a novel disorder responsive to anakinra

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Abstract

We report a 57-year-old man with recurrent meningoencephalitis resulting in bouts of altered consciousness, encephalopathy, tremors, focal seizures, and paraparesis. The neurological manifestations were accompanied by fever and leukocytosis in the absence of other systemic manifestations. MRI abnormalities of the brain, brainstem, spinal cord and meninges and CSF pleocytosis and elevated protein were observed. Exhaustive studies failed to reveal an etiology. Brain biopsy revealed nodules of neutrophils and macrophages, but no vasculitis. The lesions were not vasocentric as would be expected with neuro-Behcet’s disease and neuro-Sweet’s disease. The disorder was responsive to high-dose corticosteroid therapy and, ultimately, to anakinra, an IL-1α and IL-1β receptor antagonist.

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Funding

There were no sources of funding for this case report.

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Authors and Affiliations

  1. Department of Neurology, Perelman School of Medicine, Perelman Center for Advanced Medicine, University of Pennsylvania, 3400 Convention Avenue, Philadelphia, PA, 19104, USA

    Joseph R. Berger & Clyde Markowitz

  2. Division of Rheumatology, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, USA

    Peter A. Merkel

  3. Division of Clinical Epidemiology, Department of Biostatistics, Epidemiology, and Informatics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, USA

    Peter A. Merkel

  4. Division of Neuropathology, Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, USA

    Rachel A. Kolster & Zissimos Mourelatos

Authors
  1. Joseph R. Berger
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  2. Clyde Markowitz
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  3. Peter A. Merkel
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  4. Rachel A. Kolster
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  5. Zissimos Mourelatos
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Contributions

JB was responsible for acquisition of data, the initial draft of the manuscript, and editing the manuscript. CM was responsible for acquisition of data and editing the manuscript. PM was responsible for acquisition of data and editing the manuscript. RK was responsible for acquisition of data and editing the manuscript. ZM was responsible for acquisition of data and editing the manuscript.

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Correspondence to Joseph R. Berger.

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The authors report no conflicts of interest relevant to this manuscript.

Ethical statement

The subject of this case report and his family have provided expressed written consent for its publication.

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The authors have obtained informed consent from the patient to describe his illness.

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Berger, J.R., Markowitz, C., Merkel, P.A. et al. Multifocal neutrophilic meningoencephalitis: a novel disorder responsive to anakinra. J Neurol 268, 2995–2999 (2021). https://doi.org/10.1007/s00415-021-10431-x

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  • DOI: https://doi.org/10.1007/s00415-021-10431-x

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