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A difficult case
Encephalitis associated with anti-mGluR5 antibodies
  1. Denison Alves Pedrosa1,
  2. João Henrique Fregadolli Ferreira1,
  3. Rene Gleizer1,
  4. Rafael Bernhart Carra2,
  5. Rachel Marin de Carvalho2,
  6. Verena Endmayr3,
  7. Romana Hoftberger3,
  8. Lívia Almeida Dutra1
  1. 1 Hospital Israelita Albert Einstein, São Paulo, Brazil
  2. 2 Hospital Municipal Dr Moysés Deutsch, São Paulo, Brazil
  3. 3 Department of Neurology, Medical University of Vienna, Wien, Austria
  1. Correspondence to Lívia Almeida Dutra, Hospital Israelita Albert Einstein, Sao Paulo, SP, Brazil; livia.dutra{at}einstein.br

Abstract

A 30-year-old woman had 5 days of visual hallucinations, nystagmus, memory impairment and mutism. On examination, she was disorientated with reduced attention span, gaze-evoked nystagmus, paratonia and abnormal frontal reflexes. Cerebrospinal fluid (CSF) showed 80 cells, protein 0.41 g/L and glucose 3.2 mmol/L (plasma glucose 5.0 mmol/L). MR scan of the brain showed involvement of limbic and extra-limbic regions and brainstem. Commercial cell-based assays were negative, but tissue-based assays showed neuropil staining, and cell-based assays for anti-metabotropic glutamate receptor 5 (mGluR5) antibodies were positive in serum and CSF. Six months later, she was diagnosed with Hodgkin’s lymphoma. This case emphasises the broader clinical spectrum of anti-mGluR5 encephalitis, challenging its initial characterisation as Ophelia syndrome. It underscores the significance of interpreting commercial cell-based assays and advocates for tissue-based assay testing followed by cell-based assay testing in serum and CSF for diagnosing rare autoimmune encephalitis.

  • PARANEOPLASTIC SYNDROME
  • HALLUCINATIONS
  • IMAGE ANALYSIS
  • NEUROIMMUNOLOGY

Data availability statement

No data are available.

https://doi.org/10.1136/pn-2024-004089

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    Data availability statement

    No data are available.

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    Footnotes

    • Contributors DAP, JHFF and LAD contributed to drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; study concept or design; analysis or interpretation of data. RG, RBC, RMdC and RH contributed to major role in the acquisition of data; study concept or design; analysis or interpretation of data.

    • 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 RH reports speaker’s honoraria from Novartis and Biogen. LAD reports receiving funding from Fleury Laboratory for the BrAIN (Braziian Autoimmune Encephalitis Network) Project and BrAIN Registry (Brazilian National Registry on Autoimmune Encephalitis). The other authors report no disclosures relevant to the manuscript.

    • Provenance and peer review Not commissioned. Externally peer reviewed by Sophie Binks, Oxford, UK.

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

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