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Admission diagnoses of patients later diagnosed with autoimmune encephalitis

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Abstract

Background

Since the detection of autoantibodies against neuronal surface antigens, autoimmune encephalitis (AE) has been more frequently diagnosed, especially in patients with symptoms typical of limbic encephalitis, such as seizures, short-term memory deficits, or psychosis. However, the clinical spectrum of AE may be much wider, making correct clinical diagnosis difficult.

Methods

We retrospectively analysed symptoms and admission diagnoses at first clinical presentation in 50 AE patients. We included patients with a clinical diagnosis of AE for whom a FDG-PET imaging was available. Final diagnoses were re-evaluated by a blinded investigator according to the most recent consensus suggestions published in 2016 for AE diagnostic criteria. We additionally describe two patients with Morvan syndrome who showed CASPR2 antibodies.

Results

In 40 patients (80.0%), the clinical presentation at first admission was typical for AE. Ten patients (20.0%) initially suffered from atypical symptoms; among these patients, isolated headache and cerebellar dysfunction were most frequent (three patients each). However, an initial diagnosis of suspected encephalitis was only reached in 16 patients (32.0%), nine (18.0) of which were suspected to have infectious encephalitis, and seven (14.0%) patients were suspected to have AE. In 34 patients (68.0%), a diagnosis other than encephalitis was considered, (e.g., epilepsy, psychiatric diseases, transient ischemic attack, dementia, meningitis, and cerebellitis).

Conclusions

These data show the broad spectrum of initial symptoms of AE; the correct initial diagnosis of AE is often missed or delayed. Hence, clinicians in neurological and psychiatric hospitals should consider AE in the differential diagnosis of cases with atypical clinical presentations.

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Authors and Affiliations

  1. Department of Neurology, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany

    Annette Baumgartner, Sebastian Rauer & Tilman Hottenrott

  2. Department of Psychiatry and Psychotherapy, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany

    Annette Baumgartner

  3. Institute of Laboratory Medicine and Department of Neurology, University Hospital Schleswig-Holstein, Kiel, Germany

    Frank Leypoldt

  4. Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

    Friederike Ufer

  5. Clinical Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria

    Harald Hegen & Florian Deisenhammer

  6. Department of Neurology, Charité University Medicine Berlin and German Center for Neurodegenerative Diseases (DZNE), Berlin, Germany

    Harald Prüss

  7. Department of Neurology, Ulm University, Ulm, Germany

    Jan Lewerenz

  8. Medical Care Center, Neurology, Konstanz, Germany

    Oliver Stich

Authors
  1. Annette Baumgartner
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  2. Sebastian Rauer
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  3. Tilman Hottenrott
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  6. Harald Hegen
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  8. Jan Lewerenz
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  9. Florian Deisenhammer
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  10. Oliver Stich
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Corresponding author

Correspondence to Annette Baumgartner.

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Conflicts of interest

S.R. received consulting and lecture fees, grant and research support from Bayer Vital GmbH, Biogen Idec, Merck Serono, Novartis, Sanofi-Aventis and Teva. He is a founding member of ravo Diagnostika GmbH, Freiburg. F.L. disclosures receiving speakers honoraria from Grifols, Teva, Biogen, Merck, Roche and Fresenius. He is employed by an academic institution offering commercial antibody testing. H.H. has participated in meetings sponsored by received speaker honoraria or travel funding from Bayer, Biogen, Merck, Novartis, Sanofi-Genzyme, Teva, and received honoraria for acting as consultant for Teva. J.L. has received honoraria for speaking and travel grants from Bayer, TEVA, CHDI and the Movement Disorders Society. F.D. has participated in meetings sponsored by or received honoraria for acting as an advisor/speaker for Biogen Idec, Celgene, Genzyme-Sanofi, Merck, Novartis Pharma, Roche, and TEVA ratiopharm. His institution has received research grants from Biogen and Genzyme Sanofi. He is section editor of the MSARD Journal (Multiple Sclerosis and Related Disorders). A.B., T.H., F.U., H.P. and O.S. report no conflicts of interest with this study. None of the authors has any financial or personal relationships with individuals or organisations that could inappropriately influence this submission.

Ethical standard

The authors confirm that the study is in accordance with ethical standards. The local ethics committee approved the study.

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Baumgartner, A., Rauer, S., Hottenrott, T. et al. Admission diagnoses of patients later diagnosed with autoimmune encephalitis. J Neurol 266, 124–132 (2019). https://doi.org/10.1007/s00415-018-9105-3

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  • DOI: https://doi.org/10.1007/s00415-018-9105-3

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