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Learning from history: Lord Brain and Hashimoto’s encephalopathy
  1. Grace F Crotty1,2,
  2. Colin Doherty3,
  3. Isaac H Solomon4,
  4. James D Berry2,
  5. Martin A Samuels1
  1. 1 Department of Neurology, Brigham and Women's Hospital, Boston, Massachusetts, USA
  2. 2 Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts, USA
  3. 3 Department of Neurology, St James's Hospital, Dublin, Ireland
  4. 4 Department of Pathology, Brigham and Women's Hospital, Boston, Massachusetts, USA
  1. Correspondence to Dr Grace F Crotty, Department of Neurology, Brigham and Women's Hospital, Boston, MA 02114, USA; gcrotty{at}partners.org

Abstract

We present two historic cases of severe encephalopathy associated with antithyroid antibodies. The first was published by Lord Brain of Eynsham, and the second was from our department’s archives. Although both cases are from archival sources, they continue to inform current clinical care. We briefly review the poorly defined entity, Hashimoto’s encephalopathy, and discuss diagnostic advances for autoimmune encephalopathy and for Creutzfeldt-Jakob disease. We advocate for giving a trial of corticosteroids to patients with ‘encephalopathy, not otherwise specified’ while awaiting antibody results or more definitive testing. Our case, initially diagnosed as having Creutzfeldt-Jakob disease, responded remarkably (with video evidence) to a trial of corticosteroids.

  • Hashimoto’s encephalopathy
  • thyroid disease
  • Creutzfeldt-Jakob disease
  • steroids
  • autoimmune neurology

https://doi.org/10.1136/practneurol-2018-002107

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    Footnotes

    • Contributors GC involved in conception of the work; acquisition and analysis of data; initial draft and revision and approved final version. CD involved in conception of the work; acquisition and analysis of data; initial draft and revision and approved final version. JA involved in conception of the work; acquisition and analysis of data; initial draft and revision and approved final version. MS involved in conception of the work; acquisition and analysis of data; initial draft and revision and approved final version.

    • 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 for publication Obtained.

    • Provenance and peer review Commissioned. Externally peer reviewed by Simon Mead, London, UK and Mike Zandi, London, UK.

    Linked Articles

    • Editors’ commentary
      Highlights from this issue
      Phil E M Smith Geraint N Fuller
      Practical Neurology 2019; 19 277-277 Published Online First: 11 Jul 2019. doi: 10.1136/practneurol-2019-002364

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