The Anti-NMDA Receptor Encephalitis Foundation Newsletter

Objectives Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDARE) is a severe, but treatable disease. This study aims to give a detailed description of electroencephalogram (EEG) results in paediatric and adult patients to improve disease recognition, and analyses the predictive value of the first EEG for the final clinical outcome.

Methods This nationwide cohort study includes patients with N-methyl-D-aspartate receptor antibodies confirmed with cell-based assay and immunohistochemistry in serum and cerebrospinal fluid. EEG recordings were re-evaluated by two experienced neurophysiologists, mixed with control EEGs for blinding. Initial EEG as well as follow-up registrations were analysed.

Results 35 adults and 18 children were included. Only two patients (4%) had a normal EEG. During the first recording, the majority of the patients had normal posterior rhythm (71%), which was associated with better modified Rankin Scale at final outcome (OR 4.74; 95% CI 1.56 to 14.47; p=0.006). In addition, EEGs showed focal (73%) or diffuse (67%) slowing. The first EEG was severely abnormal in 26%. However, 8 of 14 patients with a severely abnormal first EEG still had a favourable outcome. During the course of the disease, extreme delta brushes (EDBs) were present in 6 of 53(11%)patients.

Conclusions The first EEG commonly shows normal posterior rhythm with focal or diffuse slowing. Although the sensitivity of an abnormal EEG is high (96%), normal EEG does not exclude anti-NMDARE. EDBs are only present in severely affected patients. The first EEG recording is predictive of the final clinical outcome.

 




COMMENT| VOLUME 5, ISSUE 9, P693-694, SEPTEMBER 01, 2018 The prevalence of rare diseases in psychiatry Published:May 17, 2018DOI:https://doi.org/10.1016/S2215-0366(18)30182-2 In 1923 in France, an interesting play by author Jules Romains was performed: “Knock, ou le Triomphe de la médicine”. The events of the play concern an ambitious physician named Knock. The young doctor settles down in a country village to succeed the local physician. Soon after his arrival, he finds out that severe diseases are somewhat rare in the village and that he has few patients. Dr Knock feels duped by his predecessor and therefore decides to modify the definition of “disease”. He is determined and successful in convincing the unknowing villagers that they are in fact patients. The result is immediate; everyone in the village is worried about their health following the diagnosis from Dr Knock. Knock is so busy with his new patients that he barely has time anymore to diagnose and administer treatment to the small group of patients with severe diseases. To read this article in full you will need to make a payment Purchase one-time access Or purchase The Lancet Choice Access any 5 articles from the Lancet Family of journals Subscribe to The Lancet Psychiatry Already a print subscriber? Claim online access Already an online subscriber? Sign in Register: Create an account Institutional Access: Sign in to ScienceDirect References Kessler RC Aguilar-Gaxiola S Alonso J et al. The global burden of mental disorders: an update from the WHO World Mental Health (WMH) surveys. Epidemiol Psichiatr Soc. 2009; 18: 23-33 View in Article Moore TJ Mattison DR Adult utilization of psychiatric drugs and differences by sex, age, and race. JAMA Intern Med. 2017; 177: 274-275 View in Article Hyde CL Nagle MW Tian C et al. Identification of 15 genetic loci associated with risk of major depression in individuals of European descent. Nat Genet. 2016; 48: 1031-1036 View in Article Florio AD Munk-Olsen T Bergink V The birth of a psychiatric orphan disorder: postpartum psychosis. Lancet Psychiatry. 2016; 3: 502 View in Article Bouwkamp CG Kievit AJA Markx S et al. Copy number variation in syndromic forms of psychiatric illness: the emerging value of clinical genetic testing in psychiatry. Am J Psychiatry. 2017; 174: 1036-1050 View in Article Yu C Arcos-Burgos M Baune BT et al. Low-frequency and rare variants may contribute to elucidate the genetics of major depressive disorder. Transl Psychiatry. 2018; 8: 70 View in Article Chang HS Chen PM Lu T Hu LY Anti-N-methyl-D-aspartate receptor encephalitis: should psychiatrists shoulder the responsibility for a non-psychiatric disorder?. J Neurol Sci. 2015; 353: 189-190 View in Article Article Info Publication History Published: May 17, 2018 IDENTIFICATION DOI: 10.1016/S2215-0366(18)30182-2 Copyright © 2018 Elsevier Ltd. All rights reserved. ScienceDirect Access this article on ScienceDirect

 




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Background and purpose The aim was to assess the therapeutic potential of bortezomib in the treatment of refractory N‐methyl‐d‐aspartate receptor (NMDAR) antibody encephalitis and its potential in other immune‐mediated, B‐cell‐driven neurological diseases. Methods Two cases of severe NMDAR antibody encephalitis, resistant to first and second line therapy with steroids, intravenous immunoglobulins, plasma exchange, cyclophosphamide and rituximab, were treated with four and five cycles of 1.3 mg/m2 bortezomib at 350 and 330 days following initial presentation. Results Both patients showed significant clinical improvement with reductions of NMDAR antibody titres following bortezomib treatment. This is the first case in the literature where the NMDAR antibody level was undetectable following treatment with bortezomib. Conclusion Bortezomib’s unique ability to target long‐lived autoreactive plasma cells appears to be a useful adjunct to standard second line immunosuppressive therapy in treatment‐refractory NMDAR antibody encephalitis. The drug’s pharmacodynamics, cell targeting and mechanism of action are reviewed, and it is postulated that bortezomib may be useful in a host of B‐cell‐driven neuroimmunological diseases.

 




Julia C. Nantes, Adam G. Thomas, Natalie L. Voets, Jonathan G. Best, Clive R. Rosenthal, Adam Al-Diwani, Sarosh R. Irani, Charlotte J.Stagg…

 




Sometimes all the signs and symptoms point to an autoimmune encephalitis(AE), but the all-important antibody is not found in serum or cerebral spinal fluid. This …Read More…

 




We have just partnered with Smart Patients, to create an online support community for patients and caregivers affected by AE. We receive so many questions on diagnosis, treatment and recovery, we thought we could help more people by bringing them together in a safe online community.

 




Anti- N-methyl- d-aspartate receptor (NMDAR) encephalitis is a common autoimmune encephalitis presenting with psychosis, dyskinesias, autonomic dysfunction and seizures. The underlying autoantibodies…

 




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After a 34-year-old female developed a headache and high fever, she was diagnosed with aseptic meningitis. On admission, neurological examinations revealed cere…

 




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