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Unusual association of diseases/symptoms
CASE REPORT
Unusual case of anti-N-methyl-D-aspartic acid-receptor (NMDA-R) encephalitis and autoimmune polyglandular syndrome (APS)
  1. Simona Frunza-Stefan1,
  2. Hilary B Whitlatch1,
  3. Gautam G Rao2,
  4. Rana Malek1
  1. 1Department of Medicine, Division of Endocrinology, Diabetes and Nutrition, University of Maryland Medical Center, Baltimore, Maryland, USA
  2. 2Department of Obstetrics, Gynecology and Reproductive Sciences, University of Maryland Medical Center, Baltimore, Maryland, USA
  1. Correspondence to Dr Simona Frunza-Stefan, simonastefan{at}gmail.com, sfrunza{at}som.umaryland.edu

Summary

Anti-N-methyl-D-aspartic acid-receptor (NMDA-R) encephalitis is a novel disease discovered within the past 10 years. It is an autoimmune disease (AD) that has been associated with other ADs, such as Graves’ disease. However, association with autoimmune polyglandular syndromes (APS) has not been previously described. A 58-year-old woman presented with altered mental status and an 8-month history of weight loss, apathy and somnolence. Laboratory evaluation confirmed Graves’ disease with thyrotoxicosis and type 1 diabetes mellitus. Despite treatment, she continued to have a fluctuating mental status. Further diagnostic evaluation included an abdominal MRI that showed a cystic lobular left adnexal mass. Serum anti-NMDA-R antibodies were positive, raising concern for NMDA-R encephalitis. Bilateral salpingo-oophorectomy was performed, with pathology consistent with cystadenofibroma. She had a favourable recovery with marked clinical improvement. Anti-NMDA-R antibodies were negative 2 months following surgery. The concomitant occurrence of APS and anti-NMDA-R encephalitis suggests a shared mechanism of autoimmune pathophysiology.

  • diabetes
  • thyroid disease

https://doi.org/10.1136/bcr-2018-224821

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    Footnotes

    • Contributors SF-S: contributed to the conception and design of the work as well as acquisition of data; major contributor in writing/ drafting the manuscript; gave final approval of the version to be published. HBW: contributed by helping design, conduct, draft and revise the case and also helped with figures, data interpretation and approved the final version. GGR: contibuted to publication by conception, acquisition of data and its interpretation; discussed planning and approved the final vision to be published. RM: contributed by discussing the conception and design, conducted the work, revised it critically for important intellectual content and approved the final version to be published. All authors agreed to be accountable for the article and to ensure that all questions regarding the accuracy or integrity of the article are investigated and resolved.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.