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Neurological manifestations of adult-onset Still’s disease—case-based review

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Abstract

Adult-onset Still’s disease (AOSD) is a rare inflammatory disorder in which pathophysiology is yet to be fully understood. We report the case of a 66-year-old male that presents with fever, arthralgia, and laboratory abnormalities suggestive of a systemic inflammatory disease. During a diagnostic workout, the patient developed neurological symptoms, namely a sudden confounding syndrome and hearing loss that improved with corticosteroid therapy. After exclusion of malignancy, infection—namely nervous system infection—and other rheumatic diseases, AOSD diagnosis was made using the Yamaguchi criteria. In some rare cases, neurological symptoms are present and the diagnosis may become even more challenging if the clinicians are not aware of these rare manifestations of AOSD. Therefore, the authors present the case of a patient with neurological manifestations of AOSD.

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Acknowledgments

The authors thank every health professional in Centro Hospitalar Barreiro-Montijo EPE for the contribution to this report.

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Author notes

  1. Mariana Santos and Daniela Rodrigues contributed equally to this work.

Authors and Affiliations

  1. Department of Cardiology of Centro Hospitalar Barreiro-Montijo EPE, Barreiro, Portugal

    Mariana Santos, Helder Santos, Inês Almeida & Hugo Miranda

  2. Department of Internal Medicine of Centro Hospitalar Barreiro-Montijo EPE, Barreiro, Portugal

    Daniela Rodrigues, Catarina Parente, Catarina Carvalho, Rodrigo Rufino, António Cardoso & Martinho Fernandes

Authors
  1. Mariana Santos
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  2. Daniela Rodrigues
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  3. Helder Santos
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  4. Catarina Parente
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  5. Catarina Carvalho
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  6. Rodrigo Rufino
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  7. Inês Almeida
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  8. Hugo Miranda
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  9. António Cardoso
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  10. Martinho Fernandes
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Corresponding author

Correspondence to Mariana Santos.

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Informed consent was obtained from the patient for publication of this case report. A copy of the informed consent is available to the editors of the journal.

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Santos, M., Rodrigues, D., Santos, H. et al. Neurological manifestations of adult-onset Still’s disease—case-based review. Clin Rheumatol 40, 407–411 (2021). https://doi.org/10.1007/s10067-020-05244-8

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  • DOI: https://doi.org/10.1007/s10067-020-05244-8

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