The Anti-NMDA Receptor Encephalitis Foundation Newsletter

Objective To construct a grading score that predicts neurologic function 1 year after diagnosis of anti-NMDA receptor (NMDAR) encephalitis.

Methods Three hundred eighty-two patients with detailed information and functional status at 1 year were studied. Factors associated with poor status (defined as modified Rankin Scale score ≥3) were identified and incorporated into a multivariate logistic regression model. This model was used to develop a 5-point prediction score, termed the anti-NMDAR Encephalitis One-Year Functional Status (NEOS) score.

Results Intensive care unit admission ( p < 0.001), treatment delay >4 weeks ( p = 0.012), lack of clinical improvement within 4 weeks ( p < 0.001), movement disorder ( p = 0.001), central hypoventilation ( p < 0.001), elevated CSF white blood cell count ( p < 0.001), elevated CSF protein level ( p = 0.027), and abnormal MRI ( p = 0.002) were associated with 1-year functional status in univariate analysis. Intensive care unit admission, treatment delay >4 weeks, lack of clinical improvement within 4 weeks, abnormal MRI, and CSF white blood cell count >20 cells/μL were independent predictors for outcome in multivariate regression modeling. These 5 variables were assigned 1 point each to create the NEOS score. NEOS score strongly associated with the probability of poor functional status at 1 year (3% for 0 or 1 point to 69% for 4 or 5 points, p < 0.001).

Conclusions The NEOS score accurately predicts 1-year functional status in patients with anti-NMDAR encephalitis. This score could help estimate the clinical course following diagnosis and may aid in identifying patients who could benefit from novel therapies.

 




Objective To assess intensive care unit (ICU) complications, their management, and prognostic factors associated with outcomes in a cohort of patients with autoimmune encephalitis (AE).

Methods This study was an observational multicenter registry of consecutively included patients diagnosed with AE requiring Neuro-ICU treatment between 2004 and 2016 from 18 tertiary hospitals. Logistic regression models explored the influence of complications, their management, and diagnostic findings on the dichotomized (0–3 vs 4–6) modified Rankin Scale score at hospital discharge.

Results Of 120 patients with AE (median age 43 years [interquartile range 24–62]; 70 females), 101 developed disorders of consciousness, 54 autonomic disturbances, 42 status epilepticus, and 39 severe sepsis. Sixty-eight patients were mechanically ventilated, 85 patients had detectable neuronal autoantibodies, and 35 patients were seronegative. Worse neurologic outcome at hospital discharge was associated with necessity of mechanical ventilation (sex- and age-adjusted OR 6.28; 95% CI, 2.71–15.61) tracheostomy (adjusted OR 6.26; 95% CI, 2.68–15.73), tumor (adjusted OR 3.73; 95% CI, 1.35–11.57), sepsis (adjusted OR 4.54; 95% CI, 1.99–10.43), or autonomic dysfunction (adjusted OR 2.91; 95% CI, 1.24–7.3). No significant association was observed with autoantibody type, inflammatory changes in CSF, or pathologic MRI.

Conclusion In patients with AE, mechanical ventilation, sepsis, and autonomic dysregulation appear to indicate longer or incomplete convalescence. Classic ICU complications better serve as prognostic markers than the individual subtype of AE. Increased awareness and effective management of these AE-related complications are warranted, and further prospective studies are needed to confirm our findings and to develop specific strategies for outcome improvement.

AE=
: autoimmune encephalitis;
AED=
: antiepileptic drug;
DWI=
: diffusion-weighted imaging;
eCRF=
: electronic case report form;
FDG-PET=
: fluorodeoxyglucose PET;
FLAIR=
: fluid-attenuated inversion recovery;
GENERATE=
: German Network for Research in Autoimmune Encephalitis;
ICU=
: intensive care unit;
IGNITE=
: Initiative of German Neurointensive Trial Engagement;
IQR=
: interquartile range;
mRS=
: modified Rankin Scale;
RE=
: receptor encephalitis;
SE=
: status epilepticus

 




There are now compelling reasons to think that at least one of the girls may have suffered from a much-misunderstood neurological condition…

 




Seizure. 2018 Dec 19;65:20-24. doi: 10.1016/j.seizure.2018.12.015.[Epub ahead of print] Review…

 




For people with encephalitis, rapid treatment of their acute brain inflammation is critical for avoiding devastating physical and cognitive deficits. But appropriate treatment requires identifying the culprit causing the symptoms.

 




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.

 




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The Francis McNaughton Memorial Prize for Clinical Research and The André Barbeau Memorial Prize for Basic Research The Canadian Neurological Society invites expanded abstract submissions for its two prizes: the Francis McNaughton Memorial Prize for Clinical Research and the André Barbeau Memorial Prize for Basic Research. These prizes were designed to encourage neurology trainees to undertake research projects. Prizes are awarded for the best submitted expanded, structured abstract, based on work done during neurology residency or in post-residency training. Contestants must be a member in good standing with any one of the five Societies of the CNSF (a Junior member or an Active member within two years of receiving their certificate). Contestants must submit a letter from their program chair indicating that the work was done by the resident and was principally the work of that resident. The Francis McNaughton and André Barbeau prizes will each consist of: Presentation of your work during the Grand Plenary session at the Congress A Prize Certificate $1,000 cash Full registration to the Congress Travel to the Congress (economy fare) 3 nights’ accommodation Additional $500 prize awards may be presented. New for 2019 – the Anti-NMDA Receptor Encephalitis Foundation in association with the CNS is offering an additional $1500 prize for the best paper submitted on Autoimmune Encephalitis The prize will consist of: Presentation of your work at the Congress, highlighted as a prize-winning abstract A Prize Certificate $1500 cash intended to defray costs related to attending the Congress General Requirements for all CNS Prizes Contestants need not be the sole authors, but should have been primarily responsible for the work being presented. Contestants must submit a structured abstract, expanded up to, but not exceeding 3 pages, which is to include any figures, tables, and necessary references. Submissions longer than 3 pages of single spaced typing will not be considered. The format followed should consist of Background, Materials and Methods, Results, and Conclusions. The authors should bear in mind, in the background section, that not all judges will be experts in the subject of the research paper. Contestants must submit a small biography which indicates where the candidate is in their residency or in a diploma program (if applicable), and listing other work that he/she has done. Contestants must also submit their basic abstract to the CNSF Annual Congress, on the official online abstract submission site. Submission Details All Society Prize submissions must be received by January 31, 2019. Clearly indicate which Society prize you are submitting for. Submissions should be sent as three separate PDF files: Letter from their program chair (for McNaughton and Barbeau prizes) Biography Expanded Abstract Applicants for these prizes should send their submissions to: Canadian Neurological Society c/o marika-fitzgerald@cnsfederation.org

 




New Prize offered by The Anti-NMDA Receptor Encephalitis Foundation at the 54th annual Congress of the Canadian Neurological Sciences Federation…

 




A Sydney teenager who was wrongly diagnosed with a string of mental illnesses has survived a serious brain disease and graduated dux of her school. Mariana Tana, 18, was afflicted by auditory and visual hallucinations, seizures, nausea and headaches at the age of 15. Even ambulance officers didn’t believe her. “They said that’s not how seizures usually work and I was just putting it on,” she said. The symptoms were so severe Ms Tana was unable to attend school and attempted to take her own life. Psychiatrists diagnosed different conditions including schizophrenia, bipolar and multiple personality disorder. “It was frightening, I convinced myself I was crazy,” Ms Tana said. “That what I was seeing was so out of the ordinary that there’s something mentally wrong with me.” But the problem was physical, not mental, and in some cases it’s fatal. Ms Tana was referred to Professor David Brown, the Director of Immunopathology at NSW Health Pathology’s Institute of Clinical Pathology and Medical Research at Westmead Hospital. He discovered antibodies in her blood and suspected they were attacking healthy brain cells. He diagnosed an extremely rare and relatively newly identified autoimmune disease, anti-NMDA receptor encephalitis that causes inflammation of the brain. The case was challenging because Ms Tana didn’t present with typical signs of autoimmune encephalitis. The condition was first described 10 years ago — it continues to baffle doctors and inspired the best-selling book, Brain on Fire, that was also made into a Netflix drama produced by Charlize Theron. Ms Tana was prescribed a range of treatments that sometimes carry serious side effects. “I often say to patients these drugs suppress your immune system,” Professor Brown said. “You can die from them. [But] the nature of these illnesses if they’re not picked up early enough is that the damage can become permanent.” Slowly, Ms Tana’s condition improved after she was prescribed a cocktail of medication and received a blood plasma treatment. “It was life changing,” she said. “I didn’t have as many physical symptoms. I wasn’t in pain all of the time. I still had headaches but they weren’t as bad. And I could do day-to-day things.” After dropping out of school, Ms Tana returned to her studies and completed the coursework for Year 11 in two weeks. She went on to complete year 12 as dux of her school, SEDA College in Redfern. “It was so exciting for me to feel good after so long of not thinking I’d achieve anything,” she said. She is hoping to study writing and counselling at university. Professor Brown said more research is needed, because a small number of patients being treated for psychosis for the first time may instead be suffering from encephalitis. “There are people for whom the diagnosis comes too late and end up with permanent disability because of possible delayed treatment,” he said. He is proud of his patient. “To see someone go from not being able to go to school to going back to school, topping her class, is great.”

 




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