Anti-NMDA Receptor Encephalitis: Clinical Features and Basic Mechanisms

2018 ◽  
pp. 235-260 ◽  
Author(s):  
David R. Lynch ◽  
Amy Rattelle ◽  
Yi Na Dong ◽  
Kylie Roslin ◽  
Amy J. Gleichman ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Clinical Features ◽  
Nmda Receptor Encephalitis

Frequency and temporal sequence of clinical features in adults with anti-NMDA receptor encephalitis presenting with psychiatric symptoms

2018 ◽  
Vol 49 (16) ◽  
pp. 2709-2716 ◽  
Author(s):  
Ronald J. Gurrera
Keyword(s):  
Nmda Receptor ◽  
Clinical Features ◽  
Psychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Signs And Symptoms ◽  
Nmda Receptor Encephalitis ◽  
Nmdar Encephalitis ◽  
Unexplained Fever ◽  
Study Inclusion

AbstractBackgroundAnti-NMDA receptor (NMDAr) encephalitis is the most common autoimmune encephalitis in adults. It mimics psychiatric disorders so often that most patients are initially referred to a psychiatrist, and many are misdiagnosed. Without prompt and effective treatment, patients are likely to suffer a protracted course with significant residual disability, or death. This study focuses on the frequency and chronology of salient clinical features in adults with anti-NMDAr encephalitis who are likely to be first evaluated by a psychiatrist because their presentation suggests a primary psychiatric disorder.MethodsA systematic search of PubMed and EMBASE databases identified published reports of anti-NMDAr encephalitis associated with prominent behavioral or psychiatric symptoms. After eliminating redundancies, the frequencies and relative timing of clinical features were tabulated. Signs and symptoms were assigned temporal ranks based on the timing of their first appearance relative to the first appearance of other signs and symptoms in each patient; median ranks were used to compare temporal sequencing of both individual features and major symptom domains.ResultsTwo hundred thirty unique cases (185 female) met study inclusion criteria. The most common features were seizures (60.4%), disorientation/confusion (42.6%), orofacial dyskinesias (39.1%), and mutism/staring (37.4%). Seizures, fever, and cognitive dysfunction were often the earliest features to emerge, but psychiatric features predominated and sequencing varied greatly between individuals.ConclusionsClinicians should consider anti-NMDAr encephalitis when new psychiatric symptoms are accompanied by a recent viral prodrome, seizures or unexplained fever, or when the quality of the psychiatric symptoms is unusual (e.g. non-verbal auditory hallucinations).

Clinical Features, Treatment, and Outcome of 500 Patients with Anti-NMDA Receptor Encephalitis (PL01.001)

Neurology ◽  
2012 ◽  
Vol 78 (Meeting Abstracts 1) ◽  
pp. PL01.001-PL01.001 ◽  
Author(s):  
M. Titulaer ◽  
L. McCracken ◽  
I. Gabilondo Cuellar ◽  
E. Martinez-Hernandez ◽  
F. Graus ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Clinical Features ◽  
Nmda Receptor Encephalitis

scholarly journals Heterogeneity of clinical features and corresponding antibodies in seven patients with anti-NMDA receptor encephalitis

2015 ◽  
Vol 10 (4) ◽  
pp. 1283-1292 ◽  
Author(s):  
KURT-WOLFRAM SÜHS ◽  
FLORIAN WEGNER ◽  
THOMAS SKRIPULETZ ◽  
CORINNA TREBST ◽  
SAID BEN TAYEB ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Clinical Features ◽  
Nmda Receptor Encephalitis

Early electro-clinical features may contribute to diagnosis of the anti-NMDA receptor encephalitis in children

2013 ◽  
Vol 124 (12) ◽  
pp. 2354-2361 ◽  
Author(s):  
Cyril Gitiaux ◽  
Hina Simonnet ◽  
Monika Eisermann ◽  
Dorothee Leunen ◽  
Olivier Dulac ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Clinical Features ◽  
Nmda Receptor Encephalitis

Corrigendum to “Early electro-clinical features may contribute to diagnosis of the anti-NMDA receptor encephalitis in children” [Clin. Neurophysiol. 124 (2013) 2354–2361]

2014 ◽  
Vol 125 (6) ◽  
pp. 1288
Author(s):  
Cyril Gitiaux ◽  
Hina Simonnet ◽  
Monika Eisermann ◽  
Dorothee Leunen ◽  
Olivier Dulac ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Clinical Features ◽  
Nmda Receptor Encephalitis

scholarly journals Clinical features of seronegative, but CSF antibody-positive, anti-NMDA receptor encephalitis

2020 ◽  
Vol 7 (2) ◽  
pp. e659 ◽  
Author(s):  
Mar Guasp ◽  
Yasmina Módena ◽  
Thaís Armangue ◽  
Josep Dalmau ◽  
Francesc Graus
Keyword(s):  
Nmda Receptor ◽  
Clinical Features ◽  
Serum Antibody ◽  
Independent Variables ◽  
Nmda Receptor Encephalitis ◽  
Retrospective Assessment ◽  
Nmdar Encephalitis ◽  
Paired Serum ◽  
Detectable Serum ◽  
Hospital Clinic

ObjectiveTo determine the frequency of anti-NMDA receptor encephalitis without detectable serum NMDAR antibodies and to compare the clinical features of these patients with those with NMDAR antibodies in serum and CSF.MethodsThis is a retrospective assessment of serum antibody status and clinical features of 489 patients with anti-NMDAR encephalitis, defined by the presence of NMDAR antibodies in the CSF, and available paired serum/CSF samples examined at Hospital Clínic-Institut d’Investigacions Biomèdiques August Pi I Sunyer, Barcelona, between January 2007 and December 2017. NMDAR antibodies were determined with rat brain immunostaining, in-house cell-based assay (CBA), and a commercial CBA. Patients were considered seronegative if NMDAR antibodies were undetectable with the 3 indicated techniques.ResultsSerum NMDAR antibodies were not detected in 75 of 489 (15%) patients. Compared with the 414 seropositive patients, the seronegative were older (23.5 years [interquartile range (IQR): 17–43] vs 20.5 [IQR: 14–31]; p < 0.0001) and less frequently women (39 [52%] vs 313 [76%]; p < 0.001) and had less tumors (6 [9%] vs 128 [32%]; p < 0.001). In multivariate analysis, older age at diagnosis (odds ratio [OR]: 1.35 [per decade]; 95% confidence interval [CI]: 1.10–1.67), absence of tumor (OR: 0.14; 95% CI: 0.05–0.43), and less need for intensive care unit admission (OR: 0.35; 95% CI: 0.18–0.69) were independent variables associated with the absence of serum NMDAR antibodies. Time to diagnosis, treatment with immunotherapy, relapses, and outcome were similar in seronegative and seropositive patients.ConclusionsNMDAR antibodies are not detected in the serum of 15% of the patients with anti-NMDAR encephalitis. These patients appear to be older and have milder neurologic symptoms with less frequency of tumors.

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