N‐Methyl‐D‐Aspartate Receptor Antibodies in Autoimmune Encephalopathy Alter Oligodendrocyte Function

2020 ◽  
Vol 87 (5) ◽  
pp. 670-676 ◽  
Author(s):  
Carlos Matute ◽  
Ana Palma ◽  
María Paz Serrano‐Regal ◽  
Estibaliz Maudes ◽  
Sumanta Barman ◽  
...  
2014 ◽  
Vol 20 (5) ◽  
pp. 568-569 ◽  
Author(s):  
Diana Aguiar de Sousa ◽  
Patrícia Pita Lobo ◽  
Ana Castro Caldas ◽  
Miguel Coelho ◽  
Luísa Albuquerque

2018 ◽  
Vol 76 (1) ◽  
pp. 2-5 ◽  
Author(s):  
Mariana Espinola-Nadurille ◽  
Paola Bautista-Gomez ◽  
Jose Flores ◽  
Veronica Rivas-Alonso ◽  
Rodrigo Perez-Esparza ◽  
...  

ABSTRACT Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a form of autoimmune encephalopathy that presents with a wide variety of symptoms, including neuropsychiatric manifestations. The authors’ aim for this study was to analyze the results of paraclinical studies of patients with a diagnosis of anti-NMDAR encephalitis and the association between symptom onset and diagnosis, and start of immunotherapy. Retrospective data of 29 patients with anti-NMDAR encephalitis were gathered and analyzed. Abnormal EEG was found in 27 patients (93.1%), whereas MRI was abnormal in 19 patients (65.5%). In contrast, an inflammatory pattern on CSF analysis was found in only 13 patients (44.8%). The absence of pleocytosis or increased proteins in the CSF was associated with a longer time from symptom onset to diagnosis and treatment (p = 0.003). The authors conclude that noninflammatory CSF may delay the correct diagnosis and start of immunotherapy in anti-NMDAR encephalitis. In the presence of suggestive clinical features, extensive studies including EEG are recommended.


2018 ◽  
Vol 17 (01) ◽  
pp. 041-044 ◽  
Author(s):  
Li-Ying Liu ◽  
Yang-Yang Wang ◽  
Ling-Yu Pang ◽  
Gui-Xia Zhang ◽  
Li-Ping Zou

AbstractAnti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is under-recognized in children and adults. It is characterized by prominent neurologic and psychiatric features at first onset. Autonomic manifestations are rarer in children than in adults. We report a 3-year-old male patient with rare manifestation of severe autonomic dysfunctions of the gastrointestinal system and atypical prodromal symptoms. The serum and CSF of the patient were positive for anti-NMDA receptor antibodies, and his electroencephalogram results showed an “extreme delta brush” pattern. The patient had completely recovered by the sixth-month assessment after receiving first-line immunomodulatory treatment. Thus, anti-NMDAR encephalitis should be considered in the differential diagnosis of children with mental status changes and widespread autonomic dysfunction, particularly if symptoms are accompanied by gastrointestinal dysmotility and anhidrosis.


2018 ◽  
Vol 89 (10) ◽  
pp. A18.3-A18
Author(s):  
Lim H ◽  
Whittam D ◽  
Jackson E ◽  
Williams N ◽  
O’Connell D ◽  
...  

BackgroundThe association of N-methyl d-aspartate receptor-antibodies (NMDAR-Abs) and encephalitis is now well recognised. MethodsRetrospective review of frequency of diagnosis and outcomes in encephalitis with NMDAR-Abs identified at the Walton Centre between 2012–2017.ResultsNMDAR-Abs were detected in 29/1131 (3%) of sera and/or CSF samples. Of 20 (69%) patients with encephalitis, 50% were identified in the last two years. Median onset age was 34(17–75) years and 60% (12/20) were female. Median symptom duration before diagnosis was 24(2–720) weeks between 2012–2015, improving to 14(1–96) weeks between 2015–2016. Five patients (25%) had an infectious prodrome (one prior HSV-1 encephalitis). Psychiatric/cognitive symptoms, seizures, and movement disorders were present in 80% (16/20), 70% (14/20), and 55% (11/20) of patients respectively. Three patients had ovarian teratomas.Electroencephalograms were abnormal in 65% (15/23) and MRI brain in 37% (7/19) patients. Unmatched intrathecal oligoclonal bands and CSF pleocytosis were present in 31% (5/16) and 60% (15/25) of samples respectively. Immunotherapy was beneficial in 73% (11/15) of patients. Two patients died (sepsis and multi-organ failure) and two improved spontaneously. At a median follow-up of 9 (5–180) months, 69% (11/16) of patients had an mRS ≤2. ConclusionAlthough the recognition of NMDAR-Ab encephalitis has improved, there is still a significant delay to diagnosis. The majority of patients have good outcomes.


2010 ◽  
Vol 38 (2) ◽  
pp. 679-682 ◽  
Author(s):  
Gerard Davies ◽  
Sarosh R. Irani ◽  
Cordelia Coltart ◽  
Gordon Ingle ◽  
Yogen Amin ◽  
...  

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