Annette Baumgartner1,2, Sebastian Rauer3, Tilman Hottenrott3, Frank Leypoldt4, Friederike Ufer5, Harald Hegen6, Harald Prüss7, Jan Lewerenz8, Florian Deisenhammer6, Oliver Stich9. 1. Department of Neurology, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany. annette.baumgartner@uniklinik-freiburg.de. 2. Department of Psychiatry and Psychotherapy, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany. annette.baumgartner@uniklinik-freiburg.de. 3. Department of Neurology, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany. 4. Institute of Laboratory Medicine and Department of Neurology, University Hospital Schleswig-Holstein, Kiel, Germany. 5. Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. 6. Clinical Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria. 7. Department of Neurology, Charité University Medicine Berlin and German Center for Neurodegenerative Diseases (DZNE), Berlin, Germany. 8. Department of Neurology, Ulm University, Ulm, Germany. 9. Medical Care Center, Neurology, Konstanz, Germany.
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.
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.
Authors: George K Tofaris; Sarosh R Irani; Binith J Cheeran; Ian W S Baker; Zameel M Cader; Angela Vincent Journal: Neurology Date: 2012-06-27 Impact factor: 9.910
Authors: Agnes van Sonderen; Marco W J Schreurs; Marienke A A M de Bruijn; Sanae Boukhrissi; Mariska M P Nagtzaam; Esther S P Hulsenboom; Roelien H Enting; Roland D Thijs; Paul W Wirtz; Peter A E Sillevis Smitt; Maarten J Titulaer Journal: Neurology Date: 2016-04-01 Impact factor: 9.910
Authors: Kurt-Wolfram Sühs; Florian Wegner; Thomas Skripuletz; Corinna Trebst; Said Ben Tayeb; Peter Raab; Martin Stangel Journal: Exp Ther Med Date: 2015-08-19 Impact factor: 2.447
Authors: Francesc Graus; Maarten J Titulaer; Ramani Balu; Susanne Benseler; Christian G Bien; Tania Cellucci; Irene Cortese; Russell C Dale; Jeffrey M Gelfand; Michael Geschwind; Carol A Glaser; Jerome Honnorat; Romana Höftberger; Takahiro Iizuka; Sarosh R Irani; Eric Lancaster; Frank Leypoldt; Harald Prüss; Alexander Rae-Grant; Markus Reindl; Myrna R Rosenfeld; Kevin Rostásy; Albert Saiz; Arun Venkatesan; Angela Vincent; Klaus-Peter Wandinger; Patrick Waters; Josep Dalmau Journal: Lancet Neurol Date: 2016-02-20 Impact factor: 44.182
Authors: Paul Whiteley; Ben Marlow; Ritika R Kapoor; Natasa Blagojevic-Stokic; Regina Sala Journal: Front Psychiatry Date: 2021-12-17 Impact factor: 4.157
Authors: Eero Rissanen; Kelsey Carter; Steven Cicero; John Ficke; Marie Kijewski; Mi-Ae Park; Joseph Kijewski; Emily Stern; Tanuja Chitnis; David Silbersweig; Howard L Weiner; Chun K Kim; Jennifer Lyons; Joshua P Klein; Shamik Bhattacharyya; Tarun Singhal Journal: Neurol Neuroimmunol Neuroinflamm Date: 2022-01-28
Authors: Emma G Stafford; Amanda Kortum; Aude Castel; Lauren Green; Jeanie Lau; Peter J Early; Karen R Muñana; Christopher L Mariani; Jeffrey A Yoder; Natasha J Olby Journal: J Vet Intern Med Date: 2019-09-08 Impact factor: 3.333
Authors: Anna E M Bastiaansen; Robin W van Steenhoven; Marienke A A M de Bruijn; Yvette S Crijnen; Agnes van Sonderen; Marleen H van Coevorden-Hameete; Marieke M Nühn; Marcel M Verbeek; Marco W J Schreurs; Peter A E Sillevis Smitt; Juna M de Vries; Frank Jan de Jong; Maarten J Titulaer Journal: Neurol Neuroimmunol Neuroinflamm Date: 2021-08-02