An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: mechanisms and models

Lancet Neurol. 2019 Nov;18(11):1045-1057. doi: 10.1016/S1474-4422(19)30244-3.

Josep Dalmau ¹, Thais Armangué ², Jesús Planagumà ³, Marija Radosevic ³, Francesco Mannara ³, Frank Leypoldt ⁴, Christian Geis ⁵, Eric Lancaster ⁶, Maarten J Titulaer ⁷, Myrna R Rosenfeld ⁸, Francesc Graus ³

Affiliations

1 Neuroimmunology Programme, Institut d'Investigacions Biomèdiques August Pi i Sunyer Hospital Clínic, University of Barcelona, Barcelona, Spain; Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA; Centro de Investigación Biomédica en Red para Enfermedades Raras, Madrid, Spain; Institució Catalana de Recerca i Estudis Avançats, Barcelona, Spain. Electronic address: [email protected].
2 Neuroimmunology Programme, Institut d'Investigacions Biomèdiques August Pi i Sunyer Hospital Clínic, University of Barcelona, Barcelona, Spain; Pediatric Neuroimmunology Unit, Department of Neurology, Sant Joan de Déu Children's Hospital, University of Barcelona, Barcelona, Spain.
3 Neuroimmunology Programme, Institut d'Investigacions Biomèdiques August Pi i Sunyer Hospital Clínic, University of Barcelona, Barcelona, Spain.
4 Institute of Clinical Chemistry, University Hospital Schleswig-Holstein, Kiel, Germany; Department of Neurology, Christian-Albrechts-Universität zu Kiel, Kiel, Germany.
5 Translational Neuroimmunology, Department of Neurology, Jena University Hospital, Jena, Germany.
6 Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA.
7 Department of Neurology, Erasmus University Medical Center, Rotterdam, Netherlands.
8 Neuroimmunology Programme, Institut d'Investigacions Biomèdiques August Pi i Sunyer Hospital Clínic, University of Barcelona, Barcelona, Spain; Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA.

PMID: 31326280 DOI: 10.1016/S1474-4422(19)30244-3

Abstract

The identification of anti-NMDA receptor (NMDAR) encephalitis about 12 years ago made it possible to recognise that some patients with rapidly progressive psychiatric symptoms or cognitive impairment, seizures, abnormal movements, or coma of unknown cause, had an autoimmune disease. In this disease, autoantibodies serve as a diagnostic marker and alter NMDAR-related synaptic transmission. At symptom onset, distinguishing the disease from a primary psychiatric disorder is challenging. The severity of symptoms often requires intensive care. Other than clinical assessment, no specific prognostic biomarkers exist. The disease is more prevalent in women (with a female to male ratio of around 8:2) and about 37% of patients are younger than 18 years at presentation of the disease. Tumours, usually ovarian teratoma, and herpes simplex encephalitis are known triggers of NMDAR autoimmunity. About 80% of patients improve with immunotherapy and, if needed, tumour removal, but the recovery is slow. Animal models have started to reveal the complexity of the underlying pathogenic mechanisms and will lead to novel treatments beyond immunotherapy. Future studies should aim at identifying prognostic biomarkers and treatments that accelerate recovery.

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