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Younger Age at Onset Is Associated With Worse Long-term Behavioral Outcomes in Anti-NMDA Receptor Encephalitis | Neurology Neuroimmunology & Neuroinflammation

Younger Age at Onset Is Associated With Worse Long-term Behavioral Outcomes in Anti-NMDA Receptor Encephalitis | Neurology Neuroimmunology & Neuroinflammation | AntiNMDA | Scoop.it
AbstractBackground and Objectives Anti-NMDA receptor encephalitis (anti-NMDARE) is one of the most common causes of encephalitis. It typically presents in adolescence and young adulthood, but little is known about its potential long-term consequences across the lifespan. Adaptive behavior describes an individual's ability to respond and adapt to environmental demands and unanticipated changes in daily routines. In this study, we evaluate the relationship between features from clinical presentation, including age, and long-term adaptive behavior in participants with anti-NMDARE.Methods Cross-sectional informant-reported data were collected between 2017 and 2019 from 41 individuals/caregivers of individuals with anti-NMDARE treated at 3 major academic hospitals. Neurologic disability was assessed by record review using the modified Rankin Scale (mRS). Functional outcomes were assessed using the validated Adaptive Behavior Assessment System, Third Edition (ABAS-3).Results The mean age at the time of study enrollment was 23.4 years (SD 17.0 years), and the mean time from symptom onset to study enrollment was 4.0 years. Seventeen participants were aged <12 years at symptom onset, 19 participants were aged 12–30 years, and 5 participants were aged >30 years. Mean ABAS-3 scores at study enrollment for all participants were in the average range (mean general adaptive composite standard score 92.5, SD 18.7). Individuals aged <12 years at symptom onset had lower mean ABAS-3 scores and were in the below average range compared with those aged 12–30 years at symptom onset, whose mean scores were in the average range (87 vs 99, p < 0.05). Similar differences were seen in 3 of the individual subscales (functional academics, health and safety, and self-care). There were no significant differences in mRS scores between age groups (p > 0.05).Discussion Although anti-NMDARE is associated with an overall favorable outcome, younger age at onset associates with worse long-term adaptive behavior despite no differences in neurologic disability. These findings suggest that the disease may have distinct consequences on the early developing brain. Future studies should evaluate behavioral recovery and quality of life after anti-NMDARE and identify additional factors associated with differential recovery.GlossaryABAS-3=Adaptive Behavior Assessment System, Third Edition; anti-NMDARE=anti-NMDA receptor encephalitis; GAC=general adaptive composite; ICU=intensive care unit; mRS=modified Rankin ScaleAnti-NMDA receptor encephalitis (anti-NMDARE) is now recognized as the most common identified cause of encephalitis in children and young adults, accounting for 40% of cases with an identified etiology and more common than any individual viral etiology.1,2 The disease manifests as subacute behavioral change or cognitive dysfunction often accompanied by reduced consciousness, speech dysfunction, seizures, movement disorder, and autonomic instability.3Recent literature has focused on advancing understanding of the long-term outcomes of individuals with anti-NMDARE. Although patients typically have dramatic and frequent improvement in motor disability,4 studies have also noted specific deficits in cognition, behavior, and psychosocial well-being, as well as substantial caregiver burden, years after initial presentation.5,-,9 Clinical features at the time of presentation that have been associated with poorer long-term outcomes include requirement for intensive care unit (ICU) admission and longer time between diagnosis and treatment initiation.4,10In this study, we aimed to evaluate long-term behavioral function in individuals with anti-NMDARE with focus on adaptive behavior,11 the ability to complete age-expected tasks in everyday environments.12 Adaptive behavior is a valuable outcome that encompasses functional outcomes in a real-world setting beyond what can be measured in performance-based testing. Furthermore, we investigated the association between features of their clinical presentations and long-term outcomes, in particular the role of age at symptom onset, based on earlier observations at a single institution of worse outcomes in those with symptom onset in childhood.13,14MethodsStudy ParticipantsChildren (aged <18 years) and adults (aged ≥18 years) treated for anti-NMDARE at the Johns Hopkins Hospital, Hospital of the University of Pennsylvania, and Children's Hospital of Philadelphia from July 1, 2005, until June 30, 2015, were invited to participate in this study. At the Johns Hopkins Hospital, participants were identified based on chart review of all individuals with a billing diagnosis of encephalitis as part of a prior study.13 At the Hospital of the University of Pennsylvania and Children's Hospital of Philadelphia, participants were identified through a preexisting clinical registry of all individuals with autoimmune encephalitis. At each site, chart review by a neurologist with clinical expertise in autoimmune neurology (A.Y. and E.G.-L.) confirmed that individuals invited to participate in this study met the diagnostic consensus criteria for anti-NMDARE.15 As this study aimed to assess long-term outcomes, it was also required that at least 1 year had passed from the date of diagnosis to the date of study enrollment. Eligible participants (if younger than 18 years, participants' legal guardians) were contacted by telephone and asked to consent to participation in a structured telephone interview for this study.Standard Protocol Approvals, Registrations, and Patient ConsentsThe study was approved by the institutional review boards at each site, and verbal informed consent was obtained and documented from all participants aged 18 years or older. For participants younger than 18 years, verbal informed consent was obtained and documented from their guardian. Verbal assent was also obtained and documented for children aged 8 years or older.Clinical Data CollectionAfter consent was obtained, the following information was extracted from participants' medical records: demographic details, symptoms and signs at initial presentation, diagnostic test results, immunotherapy administered, and clinical findings at hospital discharge and last follow-up with a neurologist.Assessment of Adaptive Behavior, Neurologic Disability, and Neuropsychiatric SymptomsThe Adaptive Behavior Assessment System, Third Edition (ABAS-3),11 was administered. The ABAS-3 is a standardized age-normalized neurobehavioral rating scale of over 200 items for individuals from early infancy to adulthood, which assesses development, behavior, and cognitive abilities. Scores on these subscales are summed and normalized to the standardization sample to generate a general adaptive composite (GAC) standard score, which comprises 3 domain standard scores (conceptual, social, and practical). The conceptual domain comprises the communication, functional academics, and self-direction subscales. The social domain comprises the leisure and social subscales. The practical domain comprises the community use, home living, health and safety, and self-care subscales. Child forms of the test include a stand-alone motor subscale, and adult forms of the test include a stand-alone work subscale. Neither the motor nor work subscales are included in the domain or composite standard scores.ABAS-3 GAC and domain standard scores are standardized to an average of 100 and SD of 15. Scores of 90–109 are classified average. Scores of 110–119 are classified above average, and 120 and above high. Scores of 80–89 are classified below average, 71–79 low, and 70 and below extremely low. For children (aged <18 years), parents or other caregivers completed the age-appropriate form for children (0–5 years or 5–21 years). For adults (aged ≥18 years), the individual themselves completed the form for adults (16–89 years).Scores for modified Rankin Scale (mRS), a motor disability scale with scores ranging from 0 for no symptoms to 6 for death,16 were assigned independently by 2 raters (A.Y. and E.G.-L.) based on documentation in medical records. In the event of a discrepancy, scores were adjudicated to consensus. Each participant was assigned a score at hospital admission, hospital discharge, and last neurology follow-up. A score at study enrollment was also determined based on the structured telephone interview. As has been done in other studies of autoimmune encephalitis, a good score was defined by mRS score 0–2 and a poor score by mRS score 3–6.4 Participants were also asked to respond yes or no to whether they were currently experiencing neuropsychiatric symptoms in multiple domains including fatigue, emotional lability, short-term memory, and concentration.Statistical AnalysesStatistical analyses were performed using STATA software version 14 (College Station, TX). The χ2 and Fisher exact tests were used to test for associations between categorical variables, and 2-sided t tests were used to evaluate differences in means for continuous variables. p Value < 0.05 was considered significant.The individual effects of participant and clinical factors on each outcome measure (ABAS-3 GAC standard score and mRS score at study enrollment) were tested with simple regression analyses, and the combined effects of multiple participant and clinical factors, adjusting for potential confounders, were tested in multiple regressions analysis. Examined participant and clinical factors included age at symptom onset, sex, percentage of White participants, seizure presence, tumor presence, requirement for ICU admission, immunotherapy administered (first-line treatment defined as steroids, plasma exchange, and/or IV immunoglobulin vs second-line treatment defined as rituximab and/or cyclophosphamide), and time interval from symptom onset to study enrollment. Given the cohort size, analyses for smaller subgroups were not performed.Age at symptom onset was initially evaluated as a continuous variable. Subsequently, based on prior literature and examination of the distribution of data within this cohort,4 age at symptom onset was subsequently categorized as the following: <12 years (presumed prepubertal), 12–30 years (presumed postpubertal age through young adulthood), and >30 years (older adulthood).Data AvailabilityThe data that support the findings of this study can be made available by the corresponding author on request.ResultsParticipant Characteristics and Clinical ProfilesA total of 41 participants with anti-NMDARE were enrolled in this study (eFigure 1, links.lww.com/NXI/A731). Thirty (73%) were female sex, and the mean age at the time of study enrollment was 23.4 years (SD 17.0 years). The mean time from symptom onset to study enrollment was 4.0 years (SD 2.4 years).Nearly all participants had an mRS score of 3–5 on admission (some dependence on others for age-expected tasks; 39/41, 95%), and 21/41 (51%) required ICU admission during their acute hospitalization. No individual had a prior history of herpes simplex virus encephalitis. All individuals received immunotherapy (24% first-line only; 76% first-line and second-line). Additional details regarding participant characteristics and clinical profiles are displayed in Table 1.View inline View popup Table 1 Participant Characteristics and Clinical ProfilesCategorization of Age at Symptom OnsetSeventeen participants were aged <12 years at symptom onset, 19 participants were aged 12–30 years, and 5 participants were aged >30 years (eTable 1, links.lww.com/NXI/A731). Comparing participants aged <12 years and those aged 12–30 years, there were no differences seen in sex, percentage of White participants, presence of seizures, presence of tumor, requirement for ICU admission, mRS score at admission, or time interval from symptom onset to study enrollment. However, there was a difference in immunotherapy administered: participants aged <12 years were more likely to receive first-line treatment only compared with those aged 12–30 years (8/17 vs 2/19, p = 0.02). Given the low representation of individuals aged >30 years, similar demographic comparisons with this age group were not performed.Adaptive Behavior, Neurologic Disability, and Neuropsychiatric Symptoms at Study EnrollmentOn the ABAS-3 completed at the time of study enrollment (Table 2), mean standard scores of the GAC for all participants in this study were in the average range (mean GAC standard score 92.5, SD 18.7). Twenty-five participants scored in average (n = 16), above average (n = 6), or high (n = 3) ranges. Fifteen participants scored in the below average (n = 6), low (n = 4), or extremely low (n = 5) ranges.View inline View popup Table 2 Adaptive Function, Neurologic Disability, and Neurobehavioral Features at Study EnrollmentThese findings were corroborated by assessment of neurologic disability, on which 35 participants (35/40, 88%) had a good outcome (mRS score of 0–2) at the time of study enrollment. This percentage was higher than at the time of initial hospital presentation (2/40, 5%; p < 0.0001), indicating that clinical improvement occurred in the time between initial presentation and study enrollment. At the time of study enrollment, 30 participants (30/39, 77%) endorsed at least one of the following persistent neuropsychiatric symptoms: fatigue (27%), emotional lability (46%), memory difficulties (41%), or concentration difficulties (39%).Factors Associated With Adaptive Behavior OutcomesIn individual comparative analyses, ABAS-3 GAC standard scores at study enrollment did not differ based on race (White vs non-White), presence of seizures, presence of tumor, requirement for ICU admission, immunotherapy administered, ABAS-3 rater (self vs parent/caregiver), or time interval from symptom onset to study enrollment (eTable 2, links.lww.com/NXI/A731). Of interest, male participants had lower scores in comparison to female participants (p < 0.01).The relationship between age at symptom onset and ABAS-3 GAC standard scores was examined (Figure 1, eFigure 2, links.lww.com/NXI/A731). However, given the low representation of individuals aged >30 years, they were excluded from these analyses. Among participants aged ≤30 years, those aged <12 years at symptom onset had lower ABAS-3 GAC standard scores and were in the below average range compared with those aged 12–30 years at symptom onset, who scored in the average range (87 vs 99, p < 0.05; Figure 2 and eTable 3, links.lww.com/NXI/A731). Similar differences were seen in 3 of the individual subscales (functional academics, health and safety, and self-care). Analysis was repeated after removing cases that were outliers across any of the domains, and this resulted in even more striking differences in scores between the age groups. Analyses coexamining the effect of age group and treatment received on outcomes were not performed because of sample size limitations.<img src="https://nn.neurology.org/content/nnn/9/5/e200013/F1.medium.gif"; class="highwire-fragment fragment-image" width="440" height="213" alt="Figure 1">Download figure Open in new tab Download powerpoint Figure 1 Age at Symptom Onset and Adaptive BehaviorNote: Adaptive Function as measured by the Adaptive Behavior Assessment System, Third Edition General Adaptive Composite standard score.<img src="https://nn.neurology.org/content/nnn/9/5/e200013/F2.medium.gif"; alt="Figure 2" height="300" class="highwire-fragment fragment-image" width="440">Download figure Open in new tab Download powerpoint Figure 2 Categorical Age at Symptom Onset and Adaptive Function (Total and Domain Scores)^Note: Adaptive Function as measured by the Adaptive Behavior Assessment System, Third Edition General Adaptive Composite and Conceptual, Social, and Practical Domain standard scores.Factors Associated With Neurologic Disability and Neuropsychiatric SymptomsAs noted in eTable 4, links.lww.com/NXI/A731, there was no association between age at symptom onset and mRS scores at study enrollment (p = 0.17), accounting for duration of follow-up, in participants aged 0–30 years. Likewise, there was no difference seen in mRS scores at study enrollment between participants aged <12 years and participants aged 12–30 years (p = 0.17). Furthermore, no associations were seen between age at symptom onset and current fatigue, short-term memory difficulties, and concentration difficulties at study enrollment. Individuals with emotional lability at study enrollment appear to have had an older age at symptom onset than those without emotional lability (p = 0.04); however, this is no longer the case when applying a correction for multiple comparisons (eTable 4, links.lww.com/NXI/A731).DiscussionThis study explores adaptive behavior outcomes after anti-NMDARE and demonstrates that although overall outcomes on this domain appear to be favorable, differences are seen by age such that younger children appear to have worse outcomes compared with adolescents and young adults. Long-term outcomes after anti-NMDARE appear to be favorable overall, as suggested by the fact that the average adaptive behavior score for participants in this study falls in the average range. Through use of a standardized age-normalized rating scale, adaptive behavior assesses an individual's ability to function independently and meet environmental demands. It has been demonstrated by our group13,17 and others18,19 that adaptive behavior and other outcomes following anti-NMDARE may be better than those following other forms of autoimmune encephalitis and infectious encephalitis. In this study, our findings regarding adaptive behavior are corroborated by examination of neurologic disability, for which we found that the majority of participants have good outcome (mRS score 0–2), akin to what has been found in other studies in this disease.4 Furthermore, less than 50% of participants reported ongoing difficulties with fatigue, emotional lability, memory difficulties, and concentration difficulties.Despite overall favorable adaptive behavior outcomes, we demonstrate that children with anti-NMDARE had worse scores compared with those who experienced anti-NMDARE onset in adolescence or young adulthood. Of note, younger age at onset did not lead to a higher risk of neurologic disability as assessed using the mRS. This finding emphasizes the importance of evaluating aspects of daily performance beyond traditional measures of motoric and fine motor functioning. Although children's outcomes were worse than those of adults, they did not, on average, fall into the ranges of low or extremely low. However, modest impairments in adaptive functioning can still affect quality of life at home, at school, and in the community.20 Our findings of worse adaptive behavior outcomes in younger children are supported by previous literature examining the effects of age at symptom onset on long-term neurologic disability, as measured by mRS scores. An early and pivotal study of treatment and prognostic factors for long-term outcomes demonstrated in analyses restricted to adolescents and children a relationship of improved odds of good outcome (defined as mRS score of 0–2) after 24 months of follow-up with increasing age.4 In a more recent meta-analysis of individual patient data of 1,550 cases, infants younger than 2 years (along with adults aged 65 years and older) were likewise observed to have a more than 3 times increased odds of poor outcome, defined as mRS score of 3–6 after a median follow-up of 12.0 months (range 0.5–268.0 months).21 Conversely, in a separate literature review and meta-analysis of 80 previously reported cases of children with anti-NMDARE, no association was found between age at onset and rates of incomplete recovery, defined as mRS score of 2–6, after a median follow-up of 12 months (range 1.3–54 months).22 Future research is required to determine whether pediatric-onset anti-NMDARE portends a higher likelihood of need for school and social supports. It will also be of value to quantify the effect of anti-NMDARE on family functioning, not just the acute effect experienced during the acute illness, but the longer-term effect.Early-onset anti-NMDARE might have a greater effect in the context of the developing brain. It has been demonstrated that clinical recovery results from downregulation of B-cell production of anti-NMDAR antibodies, leading to restoration of NMDARs and subsequent reversal of impairment in NMDAR function.23 However, it is not clear that such restoration of NMDARs would lead to restoration of all activity of NMDAR-related networks in a developing brain. Furthermore, profound encephalopathy can occur for months in anti-NMDARE during periods of critical neural development in children. It is possible that the prolonged loss of environmental enrichment, such as missed school and socialization, during the acute periods of anti-NMDARE may in itself lead to impairment in neural networks underlying learning and development that would otherwise be normally developing. Such phenomena have been reported in studies of pediatric traumatic brain injury.24,25 Even subtle alterations of these developmental trajectories may lead to reductions in daily function and quality of life, which can have critical consequences in social and educational settings.Another potential explanation may be a difference in clinical factors by age that may play a role in outcomes. Prior studies have demonstrated that requirement for ICU admission and longer time between diagnosis and treatment initiation4,10 are associated with poorer long-term neurologic disability outcomes in anti-NMDARE. Although no differences were seen in this study in rates of ICU admission, delays in treatment initiation were not able to be evaluated, as it was difficult to accurately ascertain this information in many patients who had been transferred from outside hospitals. Furthermore, fewer children did receive second-line treatment (defined as rituximab and/or cyclophosphamide) compared with adults in this study. This may reflect physician discomfort with the use of these medications in children, despite several studies indicating safety and efficacy in the pediatric population, as they are not approved by the United States Food and Drug Administration for pediatric use.26 Correspondingly, prior studies have demonstrated a decreased risk of relapse in individuals receiving second-line treatment as well as improved neurologic disability outcomes in those who received second-line treatment after failing first-line treatment.10A final possibility is a difference in sociologic factors between children and adults. Although an adult's independence in activities of daily living is typically self-motivated, a child's independence occurs within the context of a family. Given the often severe and protracted course of anti-NMDARE, it is possible that parents remain guarded about the freedom and independence they afford to a child who is recovering from severe illness. Such imposed limitations would be reflected in many of the questions on the ABAS-3 (e.g., “makes simple meals that require no cooking” or “attends fun activities at another's home”). This may contribute to the observed lower scores in children in comparison to adolescents and young adults (notably, in the domains of health and safety, and self-care). Although this phenomenon may affect adaptive behavior in this patient population, it, nonetheless, may still represent an important psychosocial consequence of this disease and an important dynamic to consider when counseling families. Relatedly, because both children and adults were included in this study, respondents to the ABAS-3 included both participants themselves and their primary caregivers. Our analyses did not identify differences in ABAS-3 scores between those who responded themselves and those for which a caregiver responded. However, as seen in studies of other conditions,27,28 it is possible that individuals with anti-NMDARE themselves may answer differently than the parents/caregivers responding on their behalf.Adaptive behavior outcomes of older adults with anti-NMDARE were partially examined in this study. Five participants in this cohort were over the age of 30 years and, in fact, all 5 were over the age of 50 years. Given the small number of participants in this age group, they were excluded from subgroup analyses examining the role of age on outcomes. Although not specifically analyzed in this study, qualitatively, 2 participants scored in the above average range and 3 participants in the below average range on the ABAS-3. Of interest, the 2 participants who scored well both had a tumor discovered; however, there were no other apparent differences between the 2 groups in the clinical variables collected. Other comorbidities, including reasons for neurodegeneration, were not evaluated. Future studies should look at a larger cohort of older participants with anti-NMDARE with appropriate healthy controls and extraction of comprehensive medical information to determine if and how outcomes in this age group differ from those of children, adolescents, and young adults.This study across 3 subspecialty centers demonstrates that anti-NMDARE is associated with an overall favorable outcome, although younger age at onset associates with worse long-term adaptive behavior. Limitations include the retrospective identification of patients, which may have introduced the possibility of selection bias (e.g., the inclusion of a large children's hospital as one of the 3 cohorts led to a relative abundance of children and adolescents in this study.) An additional limitation was the cross-sectional assessment of participants, which led to variabilities in factors such as time from symptom onset to study enrollment. Future work through prospective and larger studies will evaluate behavioral recovery and quality of life after anti-NMDARE and identify additional factors associated with differential recovery. This will enable the examination of the role of factors that could not be examined in this study due to inconsistent data availability such as time from symptom onset to immunotherapy initiation and MRI brain findings. Although this study included a sizeable cohort because of its multisite nature, larger studies are needed to examine other variables such as potential differences between the outcomes of very young children and older adults compared with those of adolescents and young adults. Ultimately, improved understanding of outcomes may have implications for clinical management and the design of interventional (both pharmacologic and nonpharmacologic) studies in this patient population.Study FundingThe authors report no targeted funding.DisclosureA.K. Yeshokumar is a full-time employee at Bristol Myers Squibb but was not at the time that this work was completed. E. Gordon-Lipkin, A. Arenivas, and M. Rosenfeld report no disclosures relevant to the manuscript. K.R. Patterson is a full-time employee at Horizon but was not at the time that this work was completed. R.A. Blum, B. Banwell, and Arun Venkatesan report no disclosures relevant to the manuscript. E. Lancaster has consulted for Merck and receives patent money from Novartis. J. Panzer is deceased: disclosures are not included for this author. J. Probasco reports no disclosures relevant to the manuscript; he serves as Editor-in-Chief for NEJM Journal Watch Neurology. Go to Neurology.org/NN for full disclosures.AcknowledgmentDr. Panzer is deceased.Appendix Authors<img alt="Table" width="600" src="https://nn.neurology.org/content/nnn/9/5/e200013/T3.medium.gif"; height="1553" class="highwire-fragment fragment-image">FootnotesGo to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article.The Article Processing Charge was funded by the authors.Submitted and externally peer reviewed. The handling editor was Josep O. Dalmau, MD, PhD, FAAN.Received November 9, 2021.Accepted in final form May 17, 2022.© 2022 American Academy of NeurologyReferences1.↵Gable MS, Gavali S, Radner A. Anti-NMDA receptor encephalitis: report of ten cases and comparison with viral encephalitis. Eur J Clin Microbiol Infect Dis. 2009;28:1421-1429.OpenUrlCrossRefPubMed2.↵Gable MS, Sheriff H, Dalmau J, Tilley DH, Glaser CA. The frequency of autoimmune N-methyl-d-aspartate receptor encephalitis surpasses that of individual viral etiologies in young individuals enrolled in the California encephalitis project. Clin Infect Dis. 2012;54:899-904.OpenUrlCrossRefPubMed3.↵Dalmau J, Gleichman AJ, Hughes EG, et al. Anti-NMDA receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. 2008;7:1091-1098.OpenUrlCrossRefPubMed4.↵Titulaer MJ, McCracken L, Gabilondo I, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol. 2013;12:157-165.OpenUrlCrossRefPubMed5.↵Finke C, Kopp UA, Scheel M, et al. Functional and structural brain changes in anti-N-methyl-D-aspartate receptor encephalitis. Ann Neurol. 2013;74:284-296.OpenUrlCrossRefPubMed6.↵de Bruijn MAAM, Aarsen FK, van Oosterhout MP, et al. Long-term neuropsychological outcome following pediatric anti-NMDAR encephalitis. Neurology. 2018;90:e1997–e2005.OpenUrlAbstract/FREE Full Text7.↵Blum RA, Tomlinson AR, Jette N, Kwon CS, Easton A, Yeshokumar AK. Assessment of long-term psychosocial outcomes in anti-NMDA receptor encephalitis. Epilepsy Behav. 2020;108:107088.OpenUrl8.↵Tomlinson AR, Blum RA, Jette N, Kwon CS, Easton A, Yeshokumar AK. Assessment of care transitions and caregiver burden in anti-NMDA receptor encephalitis. Epilepsy Behav. 2020;108:107066.OpenUrl9.↵Heine J, Kopp UA, Klag J, Ploner CJ, Prüss H, Finke C. Long-term cognitive outcome in anti-NMDA receptor encephalitis. Ann Neurol. 2021;90:949–961.OpenUrl10.↵Balu R, McCracken L, Lancaster E, Graus F, Dalmau J, Titulaer MJ. A score that predicts 1-year functional status in patients with anti-NMDA receptor encephalitis. Neurology. 2019;92:e244–e252.OpenUrlAbstract/FREE Full Text11.↵Harrison P, Oakland T. Adaptive Behavior Assessment System (ABAS-3). 3rd ed. Pearson Education Inc; 2015.12.↵Balboni G, Incognito O, Belacchi C, Bonichini S, Cubelli R. Vineland-II adaptive behavior profile of children with attention-deficit/hyperactivity disorder or specific learning disorders. Res Dev Disabil. 2017;61:55-65.OpenUrl13.↵Yeshokumar AK, Gordon-Lipkin E, Arenivas A, et al. Neurobehavioral outcomes in autoimmune encephalitis. J Neuroimmunol. 2017;312:8-14.OpenUrl14.↵Gordon-Lipkin E, Yeshokumar AK, Saylor D, Arenivas A, Probasco JC. Comparative outcomes in children and adults with anti- N-Methyl-D-Aspartate (anti-NMDA) receptor encephalitis. J Child Neurol. 2017;32:930-935.OpenUrl15.↵Graus F, Titulaer MJ, Balu R, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 2016;15:391-404.OpenUrlCrossRefPubMed16.↵Banks JL, Marotta CA. Outcomes validity and reliability of the modified Rankin scale: implications for stroke clinical trials: a literature review and synthesis. Stroke. 2007;38:1091-1096.OpenUrlAbstract/FREE Full Text17.↵Diaz-Arias LA, Yeshokumar AK, Glassberg B, et al. Fatigue in survivors of autoimmune encephalitis. Neurol Neuroimmunol Neuroinflamm. 2021;8:e1064.OpenUrlAbstract/FREE Full Text18.↵Ilyas-Feldmann M, Prüß H, Holtkamp M. Long-term seizure outcome and antiseizure medication use in autoimmune encephalitis. Seizure. 2021;86:138-143.OpenUrlPubMed19.↵Zhang Y, Huang HJ, Chen WB, Liu G, Liu F, Su YY. Clinical efficacy of plasma exchange in patients with autoimmune encephalitis. Ann Clin Transl Neurol. 2021;8:763-773.OpenUrl20.↵Meert K, Slomine BS, Silverstein FS, et al. Therapeutic Hypothermia after Paediatric Cardiac Arrest (THAPCA) Trial Investigatorss. One-year cognitive and neurologic outcomes in survivors of paediatric extracorporeal cardiopulmonary resuscitation. Resuscitation. 2019;139:299-307.OpenUrl21.↵Nosadini M, Eyre M, Molteni E, et al. Use and safety of immunotherapeutic management of N-Methyl-d-Aspartate receptor antibody encephalitis: a meta-analysis. JAMA Neurol. 2021;78:1333-1344.OpenUrl22.↵Byrne S, Walsh C, Hacohen Y, et al. Earlier treatment of NMDAR antibody encephalitis in children results in a better outcome. Neurol Neuroimmunol Neuroinflamm. 2015;2:e130.OpenUrlAbstract/FREE Full Text23.↵Hughes EG, Peng X, Gleichman AJ, et al. Cellular and synaptic mechanisms of anti-NMDA receptor encephalitis. J Neurosci. 2010;30:5866-5875.OpenUrlAbstract/FREE Full Text24.↵Donders J, DeWit C. Parental ratings of daily behavior and child cognitive test performance after pediatric mild traumatic brain injury. Child Neuropsychol. 2017;23:554-570.OpenUrl25.↵Kriel RL, Krach LE, Sheehan M. Pediatric closed head injury: outcome following prolonged unconsciousness. Arch Phys Med Rehabil. 1988;69:678-681.OpenUrlPubMed26.↵Dale RC, Brilot F, Duffy LV, et al. Utility and safety of rituximab in pediatric autoimmune and inflammatory CNS disease. Neurology. 2014;83:142-150.OpenUrlAbstract/FREE Full Text27.↵Ferreira MC, Garcia NR, Prudente COM, Ribeiro MFM. Quality of life of adolescents with cerebral palsy: agreement between self-report and caregiver's report. Rev Lat Am Enfermagem. 2020;28:e3300.OpenUrl28.↵Colombatti R, Casale M, Russo G. Disease burden and quality of life of in children with sickle cell disease in Italy: time to be considered a priority. Ital J Pediatr. 2021;47:163.OpenUrl
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Josep Dalmau receives the “Scientific Breakthrough 2023” Award from the American Brain Foundation

The accolade recognises the commitment of this Clínic Barcelona-IDIBAPS researcher to deepening our understanding of autoimmune neurological diseases such...
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IDIBAPS creates three multidisciplinary research programs to encourage collaboration among its groups

They are the Translational cancer research program, the Synaptic autoimmunity in neurology, psychiatry and cognitive neuroscience program and the Lymphoid...
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ExTINGUISH: A Beacon of Hope for NMDAR Encephalitis

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MR Imaging Findings in a Large Population of Autoimmune Encephalitis | American Journal of Neuroradiology

MR Imaging Findings in a Large Population of Autoimmune Encephalitis | American Journal of Neuroradiology | AntiNMDA | Scoop.it
Research ArticleAdult Brain MR Imaging Findings in a Large Population of Autoimmune Encephalitis S. Gillon, M. Chan, J. Chen, E.L. Guterman, X. Wu, C.M. Glastonbury and Y. Li American Journal of Neuroradiology July 2023, 44 (7) 799-806; DOI: https://doi.org/10.3174/ajnr.A7907 ArticleFigures & DataInfo & MetricsReferences PDF This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased. AbstractBACKGROUND AND PURPOSE: Autoimmune encephalitis is a rare condition in which autoantibodies attack neuronal tissue, causing neuropsychiatric disturbances. This study sought to evaluate MR imaging findings associated with subtypes and categories of autoimmune encephalitis.MATERIALS AND METHODS: Cases of autoimmune encephalitis with specific autoantibodies were identified from the medical record (2009–2019). Cases were excluded if no MR imaging of the brain was available, antibodies were associated with demyelinating disease, or >1 concurrent antibody was present. Demographics, CSF profile, antibody subtype and group (group 1 intracellular antigen or group 2 extracellular antigen), and MR imaging features at symptom onset were reviewed. Imaging and clinical features were compared across antibody groups using χ2 and Wilcoxon rank-sum tests.RESULTS: Eighty-five cases of autoimmune encephalitis constituting 16 distinct antibodies were reviewed. The most common antibodies were anti-N-methyl-D-aspartate (n = 41), anti-glutamic acid decarboxylase (n = 7), and anti-voltage-gated potassium channel (n = 6). Eighteen of 85 (21%) were group 1; and 67/85 (79%) were group 2. The median time between MR imaging and antibody diagnosis was 14 days (interquartile range, 4–26 days). MR imaging had normal findings in 33/85 (39%), and 20/33 (61%) patients with normal MRIs had anti-N-methyl-D-aspartate receptor antibodies. Signal abnormality was most common in the limbic system (28/85, 33%); 1/68 (1.5%) had susceptibility artifacts. Brainstem and cerebellar involvement were more common in group 1, while leptomeningeal enhancement was more common in group 2.CONCLUSIONS: Sixty-one percent of patients with autoimmune encephalitis had abnormal brain MR imaging findings at symptom onset, most commonly involving the limbic system. Susceptibility artifact is rare and makes autoimmune encephalitis less likely as a diagnosis. Brainstem and cerebellar involvement were more common in group 1, while leptomeningeal enhancement was more common in group 2.ABBREVIATIONS:AIEautoimmune encephalitisanti-Gq1banti-ganglioside Q1banti-LGI1anti-leucine-rich glioma inactivated 1CASPR2contactin-associated protein-like 2GABAgamma-aminobutyric acidGADglutamic acid decarboxylaseGFAPglial fibrillary acidic proteinNMDAN-methyl-D-aspartatePD-1programmed cell death protein 1VGCCvoltage gated calcium channelVGKCvoltage-gated potassium channel© 2023 by American Journal of NeuroradiologyView Full Text Log in using your username and password Username * Password * Forgot your user name or password? PreviousNext Back to top In this issue American Journal of Neuroradiology Vol. 44, Issue 7 1 Jul 2023 Table of ContentsIndex by authorComplete Issue (PDF) Print Download PDF Email Article Citation Tools Share Tweet WidgetFacebook LikeGoogle Plus One Purchase Related ArticlesNo related articles found.PubMedGoogle Scholar Cited By...No citing articles found.CrossrefGoogle Scholar More in this TOC Section Cost-Effectiveness Analysis of 68Ga-DOTATATE PET/MRI in Radiotherapy Planning in Patients with Intermediate-Risk Meningioma Choroid Plexus Calcification Correlates with Cortical Microglial Activation in Humans: A Multimodal PET, CT, MRI Study Show more ADULT BRAIN Similar Articles
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Elevated blood and cerebrospinal fluid biomarkers of microglial activation and blood‒brain barrier disruption in anti-NMDA receptor encephalitis | Journal of Neuroinflammation | Full Text

Elevated blood and cerebrospinal fluid biomarkers of microglial activation and blood‒brain barrier disruption in anti-NMDA receptor encephalitis | Journal of Neuroinflammation | Full Text | AntiNMDA | Scoop.it
Background Anti-NMDA receptor (NMDAR) encephalitis is an autoimmune disease characterized by complex neuropsychiatric syndrome and cerebrospinal fluid (CSF) NMDAR antibodies. Triggering receptor expressed on myeloid cells 2 (TREM2) has been reported to be associated with inflammation of the...
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Anti-N-methyl-d-aspartate receptor encephalitis and positive human herpesvirus-7 deoxyribonucleic acid in cerebrospinal fluid: a case report | Journal of Medical Case Reports | Full Text

Anti-N-methyl-d-aspartate receptor encephalitis and positive human herpesvirus-7 deoxyribonucleic acid in cerebrospinal fluid: a case report | Journal of Medical Case Reports | Full Text | AntiNMDA | Scoop.it
Background Anti-N-methyl-d-aspartate receptor encephalitis is a neuroautoimmune syndrome typically presenting with seizures, psychiatric symptoms, and autonomic dysfunction. Human herpesvirus-7 is often found with human herpesvirus-6 and infects leukocytes such as T-cells, monocytes–macrophages,...
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We have a winner! - The Anti-NMDA Receptor Encephalitis Foundation Prize, 2023

We have a winner! - The Anti-NMDA Receptor Encephalitis Foundation Prize, 2023 | AntiNMDA | Scoop.it
It’s that time of year again, when the Foundation is delighted to offer its annual Anti-NMDA Receptor Encephalitis Foundation Prize to a promising neurology trainee ...Read More...
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Antibodies Associated With Autoimmune Encephalitis in Patients With Presumed Neurodegenerative Dementia | Neurology Neuroimmunology & Neuroinflammation

Antibodies Associated With Autoimmune Encephalitis in Patients With Presumed Neurodegenerative Dementia | Neurology Neuroimmunology & Neuroinflammation | AntiNMDA | Scoop.it
AbstractBackground & Objectives Autoimmune encephalitis (AIE) may present with prominent cognitive disturbances without overt inflammatory changes in MRI and CSF. Identification of these neurodegenerative dementia diagnosis mimics is important because patients generally respond to immunotherapy. The objective of this study was to determine the frequency of neuronal antibodies in patients with presumed neurodegenerative dementia and describe the clinical characteristics of the patients with neuronal antibodies.Methods In this retrospective cohort study, 920 patients were included with neurodegenerative dementia diagnosis from established cohorts at 2 large Dutch academic memory clinics. In total, 1,398 samples were tested (both CSF and serum in 478 patients) using immunohistochemistry (IHC), cell-based assays (CBA), and live hippocampal cell cultures (LN). To ascertain specificity and prevent false positive results, samples had to test positive by at least 2 different research techniques. Clinical data were retrieved from patient files.Results Neuronal antibodies were detected in 7 patients (0.8%), including anti-IgLON5 (n = 3), anti-LGI1 (n = 2), anti-DPPX, and anti-NMDAR. Clinical symptoms atypical for neurodegenerative diseases were identified in all 7 and included subacute deterioration (n = 3), myoclonus (n = 2), a history of autoimmune disease (n = 2), a fluctuating disease course (n = 1), and epileptic seizures (n = 1). In this cohort, no patients with antibodies fulfilled the criteria for rapidly progressive dementia (RPD), yet a subacute deterioration was reported in 3 patients later in the disease course. Brain MRI of none of the patients demonstrated abnormalities suggestive for AIE. CSF pleocytosis was found in 1 patient, considered as an atypical sign for neurodegenerative diseases. Compared with patients without neuronal antibodies (4 per antibody-positive patient), atypical clinical signs for neurodegenerative diseases were seen more frequently among the patients with antibodies (100% vs 21%, p = 0.0003), especially a subacute deterioration or fluctuating course (57% vs 7%, p = 0.009).Discussion A small, but clinically relevant proportion of patients suspected to have neurodegenerative dementias have neuronal antibodies indicative of AIE and might benefit from immunotherapy. In patients with atypical signs for neurodegenerative diseases, clinicians should consider neuronal antibody testing. Physicians should keep in mind the clinical phenotype and confirmation of positive test results to avoid false positive results and administration of potential harmful therapy for the wrong indication.GlossaryAD=Alzheimer dementia; AIE=autoimmune encephalitis; CBA=cell-based assays; DLB=dementia with Lewy bodies; IHC=immunohistochemistry; LN=live hippocampal cell cultures; PPA=primary progressive aphasia; PSP=progressive supranuclear palsy; RPD=rapidly progressive dementia; VGCC=voltage-gated calcium channelCognitive dysfunction can be the presenting and most prominent symptom in patients with autoimmune encephalitis (AIE).1,2 In contrast to neurodegenerative diseases, patients with antibody-mediated encephalitis might benefit from immunotherapy and improve considerably.3,4 The presence of neuronal antibodies has been reported predominantly in rapidly progressive dementia (RPD).5,6 However, AIE can present less fulminantly and is therefore potentially missed, resulting in diagnosis and treatment delay or even misdiagnosis.7,8 We hypothesized that a small—but not insignificant—part of dementia syndromes is indeed caused by antibody-mediated encephalitis and underdiagnosed, withholding these patients' available treatments. The wish to diagnose every single patient with autoimmune encephalitis is in opposition with the risk for false positive tests.9 Therefore, we strictly adhere to confirmation of positive test results with 2 different test techniques. In this study, we describe the frequency of neuronal antibodies in a cohort of patients diagnosed with various dementia syndromes in a memory clinic. In addition, we present clues to improve clinical recognition of AIE in dementia syndromes.MethodsPatients and Laboratory StudiesIn this retrospective multicenter study, we tested for the presence of neuronal antibodies in serum and CSF samples from patients diagnosed with neurodegenerative dementia diagnosis, included earlier prospectively in established cohorts at 2 large Dutch academic memory clinics (Erasmus University Medical Center, Amsterdam University Medical Centers, location VUmc)10 between 1998 and 2016 (84% last 10 years). All patients fulfilled the core clinical criteria for dementia, as defined by the National Institutes of Aging-Alzheimer Association workgroups.11 Patients were classified into 4 subgroups (based on diagnostic criteria): Alzheimer dementia (AD), frontotemporal dementia (FTD; both behavioral variant and primary progressive aphasia [PPA]), dementia with Lewy bodies (DLB), and other dementia syndromes.11,-,14 Rapidly progressive dementia was defined as dementia within 12 months or death within 2 years after the appearance of the first cognitive symptoms.15 Patients with vascular dementia were not included. Clinic information was retrieved from the prospectively collected data. A subacute deterioration was defined as a marked progression of symptoms in 3 months and a fluctuating course as a disease course fluctuating over a longer period (e.g., weeks to months; different from the fluctuations within a day as seen in some patients with DLB). Dementia markers were scored according to the reference values (per year and per center; included in Table 1).View inline View popup Table 1 Patient Characteristics of Auto-antibody Positive PatientsAll samples, stored in both cohorts' biobanks, were screened for immunoreactivity with immunohistochemistry (IHC), as previously described.16 Preferably, paired serum and CSF were tested for optimal sensitivity and specificity. Samples that were showing a positive or questionable staining pattern were tested more extensively using validated commercial cell-based assays (CBA) and in-house CBA (eTable 1, links.lww.com/NXI/A869). In addition, these samples were tested with live hippocampal cell cultures (LN).16,17 To ascertain specificity, only samples that could be confirmed by CBA or LN were scored as positive because there is a higher risk for false-positive test results in this population with a low a priori chance to have encephalitis.9,18 If IHC was suggestive for antibodies against intracellular (paraneoplastic) targets, this was explored by a different IHC technique.19 Anti-thyroid peroxidase (TPO), voltage-gated calcium channel (VGCC), or low titer glutamic acid decarboxylase antibodies were not tested for because these are generally nonspecific at these ages and are not associated with dementia syndromes.Antibody-positive patients were described exploratory and compared with a randomly selected antibody-negative group (ratio 1:4) matched for memory clinic, dementia subtype, sex, and age (±5 years). For these comparisons, medical records were additionally assessed for both the antibody-positive and antibody-negative patients. All antibody-positive patients were reviewed by a panel consisting of neurologists specialized in neurodegenerative (F.J., H.S., J.S.) or autoimmune diseases (J.V., P.S.S., M.T.), and a consensus classification of AIE vs AIE with a neurodegenerative dementia comorbidity was reached.Statistical AnalysisWe used IBM SPSS 25.0 (SPSS Inc) and Prism 8.4.3 (GraphPad) for statistical analysis. Baseline characteristics were analyzed using the Fisher exact test, the Fisher-Freeman-Halton test, or the Kruskal-Wallis test, when appropriate. For group comparisons, encompassing categorical data, we used the Pearson χ2 test or the Fisher-Freeman-Halton test, when appropriate. Continuous data were analyzed using the Mann-Whitney U test. All p-values were two-sided and considered statistically significant when below 0.05. We applied no correction for multiple testing, and therefore, p values between 0.05 and 0.005 should be interpreted carefully.Standard Protocol Approvals, Registrations, and Patient ConsentsThe study was approved by The Institutional Review Boards of Erasmus University Medical Center Rotterdam and Amsterdam University Medical Center, location VUmc. Written informed consent was obtained from all patients.Data AvailabilityAny data not published within this article are available at the Erasmus MC University Medical Center. Patient-related data will be shared on reasonable request from any qualified investigator, maintaining anonymization of the individual patients.ResultsIn total, 1,398 samples from 920 patients were tested (Figure; in 478, both CSF and serum [52%]). Three-hundred fifty-eight patients were classified as AD (39%), 283 FTD (31%), and 161 DLB (17%). The fourth subgroup with other dementia syndromes consisted of 118 patients (13%), including progressive supranuclear palsy (n = 48, 5%) and corticobasal syndrome (n = 29, 3%). The median age at disease onset was 62 years (range 16–90 years). Male patients were overrepresented (n = 542, 59%), and 60 patients (7%) fulfilled the criteria for rapidly progressive dementia (RPD; eTable 2, links.lww.com/NXI/A869).<img class="highwire-fragment fragment-image" alt="Figure" width="440" height="305" src="https://nn.neurology.org/content/nnn/10/5/e200137/F1.medium.gif">Download figure Open in new tab Download powerpoint Figure Flowchart of Patient Inclusion With Antibody ResultsIn total, 920 patients (1,398 samples) with a presumed neurodegenerative dementia syndrome were tested for the presence of neuronal antibodies in serum and CSF. Neuronal antibodies were detected in 7 patients (0.8%, 95% CI 0.2–1.3); five among the 358 Alzheimer disease patients. Subclassification of the ‘other’ group is provided in supplementary table eTable 2 (links.lww.com/NXI/A869). AD = Alzheimer disease; DLB = diffuse Lewy body dementia; DPPX = dipeptidyl aminopeptidase-like protein 6; FTD = frontotemporal dementia; IgLON5 = Ig-like domain-containing protein family member 5; LGI1 = leucin-rich glioma inactivated protein 1; NMDAR = N-methyl-d-aspartate receptor; S = serum.Neuronal antibodies were detected in 7 patients (0.8%; 5 in the AD group: 1.4%; Figure), including anti-IgLON5 (n = 3), anti-LGI1 (n = 2), anti-DPPX (n = 1), and anti-NMDAR antibodies (n = 1; Table 1). Among these 7, 4 patients were diagnosed retrospectively with an exclusive diagnosis of AIE, while 3 patients were classified to have AIE (anti-IgLON5 [n = 2] and anti-NMDAR antibodies [n = 1]) with a neurodegenerative dementia comorbidity. No patients with antibodies fulfilled the criteria for RPD, yet a subacute deterioration later in the disease was reported in 3 patients. Atypical clinical signs for neurodegenerative diseases were present in 7 of 7 antibody-positive patients (100% vs 21% in antibody-negative patients, p = 0.0003; Table 2). These included a subacute deterioration (n = 3), myoclonus (n = 2), a fluctuating disease course over months (n = 1), a history of autoimmune disease (n = 2), and epileptic seizures (n = 1; Table 1). Brain MRI of none of the patients demonstrated abnormalities suggestive for active AIE, in particular no hippocampal swelling nor increased T2-signal intensity. CSF pleocytosis was found in 1 patient. CSF biomarkers (t-tau, p-tau, and Aβ42) were tested in 5 of 7 patients, and t-tau and p-tau were increased in 4, while a low Aβ42 was seen in 2. Of note, only 1 patient had the combination of reduced Aβ42 and increased p-tau/t-tau, and was diagnosed with a comorbid AD. No patient received immunotherapy. Two patients still alive (1 anti-LG1, 1 anti-DPPX positive) were contacted but refused to visit our clinic to try very delayed immunotherapy trials. It is of interest that the patient with anti-DPPX antibodies showed spontaneous improvement of cognitive disturbances, atypical for a pure neurodegenerative disease.View inline View popup Table 2 Comparisons Between Patients With Neuronal Auto-antibodies and Antibody-Negative PatientsCompared with the patients without neuronal antibodies, subacute cognitive deterioration or fluctuating course was present more frequently (4/7 [57%] vs 2/28 [7%], p = 0.009). Although movement disorders (myoclonus) and autoimmune disorders were present in 2 of 7 patients each, this did not reach significance (Table 2).DiscussionIn this large, multicenter, cohort study consisting of patients with a presumed neurodegenerative dementia diagnosis, we show that a small, but clinically relevant proportion (0.8%) have neuronal antibodies. In this particular group, 4 of 7 antibody-positive patients presented with an atypical clinical course (subacute deterioration or fluctuating disease course), which is considered as a clinical clue (‘red flag’) for an antibody-mediated etiology of dementia.4 It is important that a fluctuating disease course was observed over a longer period (e.g., weeks or months) in AIE and should not be confused with shorter fluctuations of cognition or alertness (over the day) in DLB. Other known red flags, which we observed in these 7 patients, were myoclonus, epilepsy, pleocytosis, or a history of autoimmune disorders, as described earlier.1,4,-,6 Compared with antibody-negative patients, no significant difference was found related to these symptoms alone, probably due to the low number of positive patients and related low power. However, atypical clinical signs for neurodegenerative diseases together were seen significantly more frequently in the antibody-positive group. Within this cohort mostly devoid of patients with RPD, none of the antibody-positive patients fulfilled the criteria for RPD, nor ancillary testing showed specific signs for AIE in most patients. This implicates that AIE can resemble more protracted, progressive neurodegenerative dementia syndromes, as we reported earlier.1Three antibody-positive patients had IgLON5 antibodies, which is a very rare and known to have heterogeneous (chronic) clinical manifestations, including pronounced sleep problems, cognitive dysfunction, and movement disorders.20,21 Misdiagnosis with progressive supranuclear palsy (PSP) is reported, mainly associated with the preceding movement disorders. In addition, half of the patients have cognitive impairment of whom 20% fulfilled clinical criteria for dementia.21 It is of interest that IgLON5 disease shares features with neurodegeneration because autopsy studies showed tau deposits.22 However, there is a strong HLA association,20 and studies show that antibodies directly bind to surface IgLON5 on neurons and directly alter neuronal function and structure,23 suggesting a primary inflammatory disease.In previous research, a notably higher frequency (14%) of neuronal antibodies in patients with dementia was reported by Giannocaro et al.24 The discrepancy with our test results is probably explained by differences in patient selection and antibody testing methodology. First, 30% of the patients in the cohort described by Giannocaro et al. demonstrated CSF inflammatory abnormalities, indicating a relatively high pretest probability of antibody-positivity compared with our study.24 A lack of CSF pleocytosis probably better represents the population of memory clinics. Second, the previous study exclusively tested serum by cell-based assay without confirmatory tests nor testing antibodies in CSF.24 We only considered antibody test results positive when confirmed by additional techniques to avoid suboptimal specificity and false-positive test results.9Previous studies, including our own, suggested RPD as a relevant red flag for AIE,1,4,9,25 but we cannot determine this from our study based on the design of our study. We included patients at tertiary memory clinics without overt signs or symptoms suggestive for encephalitis. Therefore, the amount of patients with RPD included was very limited (7%), comparable with other large dementia cohort studies, as was the amount of patients with abnormal ancillary testing suggestive for AIE because this would have prompted a different approach than referral to a tertiary memory clinic. These patients with RPD and ancillary testing suggestive of AIE were not included in our study. Inclusion of those patients would have likely increased our rate of positivity.The strength of our study is the large number of paired samples (serum and CSF combined) from a cohort with various presumed neurodegenerative diseases without AIE suspicion, representative for academic memory clinics. A limitation is the lack of neuropathologic data to support our findings and make diagnoses of neurodegeneration or inflammation definite. To confirm if the symptoms are related to the presence of antibodies, we tried to overcome this concern in different ways. First, the presence of antibodies in serum and CSF was confirmed by different techniques (cell-based assay, tissue immunohistochemistry, and cultured live neurons), indicating optimal test specificity. Second, afterward patients were thoroughly reviewed by a panel of neurologists specialized in neurodegenerative or autoimmune disease to detect atypical signs and symptoms related to AIE. This is a very large cohort of patients with dementia examined for the presence of neuronal antibodies. Nevertheless, an important limitation of this study is the small number of antibody-positive patients, underpowering the probability to identify significant differences between antibody-positive and antibody-negative patients. The low number of patients with RPD has probably added to this small number, and a prospective study including patients with RPD is recommended. Nevertheless, several probable red flags could be identified. Diagnosing AIE in patients with dementia is highly relevant because these patients might respond to immunotherapy. Therefore, clinicians should test for neuronal antibody in patients demonstrating red flags suggestive for an autoimmune etiology, if possible early in disease course. When profound temporal lobe atrophy already has developed, little effect is to be expected. Red flags identified in this study are subacute deterioration or fluctuating course. Other red flags described previously, we also see reflected in our study, are autoimmune disorders, myoclonus, seizures, and pleocytosis,1,4,-,6 Preferably, both serum and CSF should be tested and confirmed by additional techniques. Always consider the possibility of a false positive test result, especially when only using a single technique (like the commercial cell-based assay). If the clinical phenotype is atypical, confirmation in a research laboratory should be mandatory. The use of antibody panels is discouraged, especially including the paraneoplastic blots, because these are associated with higher risks of lack of clinical relevance.26 This caution is even more warranted for tests not associated with neurodegenerative syndromes, but with a history of nonspecificity, including VGKC (in the absence of LGI1 or CASPR2), VGCC, anti-TPO, and low-titer anti-GAD65.27,-,30 Further research should focus on improving clinical recognition of AIE in patients with dementia determining the effect of immunotherapy in this specific patient category and assessing the frequency of AIE in RPD.In conclusion, we have shown that a clinically relevant, albeit small proportion of patients with a suspected neurodegenerative disease and nonrapidly progressive course have neuronal antibodies indicative of AIE.Study FundingM.J. Titulaer was supported by an Erasmus MC fellowship and has received funding from the Netherlands Organization for Scientific Research (NWO, Veni incentive), ZonMw (Memorabel program), the Dutch Epilepsy Foundation (NEF 14-19 & 19-08), Dioraphte (2001 0403), and E-RARE JTC 2018 (UltraAIE, 90030376505). F. Leypoldt has received funding from the German Ministry of Education and Research (01GM1908A) and the Era-Net funding program (LE3064/2-1).DisclosureA.E.M. Bastiaansen reports no disclosures. R.W. van Steenhoven reports no disclosures. Research programs of Wiesje van der Flier have been funded by ZonMW, now, EUFP7, EU-JPND, Alzheimer Nederland, Hersenstichting CardioVascular Onderzoek Nederland, Health∼Holland, Topsector Life Sciences & Health, stichting Dioraphte, Gieskes-Strijbis fonds, stichting Equilibrio, Edwin Bouw fonds, Pasman stichting, stichting Alzheimer & Neuropsychiatrie Foundation, Philips, Biogen MA Inc, Novartis-NL, Life-MI, AVID, Roche BV, Fujifilm, and Combinostics. W.M. van der Flier holds the Pasman chair. W.M. van der Flier is recipient of ABOARD, which is a public-private partnership receiving funding from ZonMW (#73305095007) and Health Holland, Topsector Life Sciences & Health (PPP-allowance; #LSHM20106). All funding is paid to her institution. WF has performed contract research for Biogen MA Inc and Boehringer Ingelheim. All funding is paid to her institution. W.M. van der Flier has been an invited speaker at Boehringer Ingelheim, Biogen MA Inc, Danone, Eisai, WebMD Neurology (Medscape), and Springer Healthcare. All funding is paid to her institution. W.M. van der Flier is consultant to Oxford Health Policy Forum CIC, Roche, and Biogen MA Inc. All funding is paid to her institution. W.M. van der Flier participated in advisory boards of Biogen MA Inc and Roche. All funding is paid to her institution. W.M. van der Flier is a member of the steering committee of PAVE and Think Brain Health. W.M. van der Flier was an associate editor of Alzheimer, Research & Therapy in 2020/2021. W.M. van der Flier is an associate editor at Brain. Research of C. Teunissen was supported by the European Commission (Marie Curie International Training Network, Grant Agreement No. 860197 (MIRIADE)), Innovative Medicines Initiatives 3TR (Horizon 2020, Grant No. 831434), EPND (IMI 2 Joint Undertaking (JU) under Grant Agreement No. 101034344) and JPND (bPRIDE), National MS Society (Progressive MS alliance) and Health Holland, the Dutch Research Council (ZonMW), Alzheimer Drug Discovery Foundation, The Selfridges Group Foundation, Alzheimer Netherlands, and Alzheimer Association. C. Teunissen is recipient of ABOARD, which is a public-private partnership receiving funding from ZonMW (#73305095007) and Health∼Holland, Topsector Life Sciences & Health (PPP-allowance, #LSHM20106). ABOARD also receives funding from Edwin Bouw Fonds and Gieskes-Strijbisfonds. C. Teunissen has a collaboration contract with ADx Neurosciences, Quanterix, and Eli Lilly, performed contract research or received grants from AC-Immune, Axon Neurosciences, Bioconnect, Bioorchestra, Brainstorm Therapeutics, Celgene, EIP Pharma, Eisai, Grifols, Novo Nordisk, PeopleBio, Roche, Toyama, and Vivoryon. She serves on editorial boards of Medidact Neurologie/Springer, Alzheimer Research and Therapy, and Neurology: Neuroimmunology & Neuroinflammation and is an editor of a Neuromethods book Springer. She had speaker contracts for Roche, Grifols, and Novo Nordisk. E. de Graaff holds a patent for the detection of anti-DNER antibodies. M.M.P. Nagtzaam reports no disclosures. M. Paunovic reports no disclosures. S. Franken reports no disclosures. M.W.J. Schreurs reports no disclosures. F. Leypoldt has received speakers honoraria from Grifols, Roche, Novartis, Alexion, and Biogen and serves on an advisory board for Roche and Biogen. He works for an academic institution (University Hospital Schleswig-Holstein) which offers commercial autoantibody testing. P.A.E. Sillevis Smitt holds a patent for the detection of anti-DNER and received research support from Euroimmun. J.M. de Vries reports no disclosures. H. Seelaar reports no disclosures. J.C. van Swieten reports no disclosures. F.J. de Jong reports no disclosures. Y.A.L. Pijnenburg Research of Alzheimer center Amsterdam is part of the neurodegeneration research program of Amsterdam Neuroscience. Alzheimer Center Amsterdam is supported by Stichting Alzheimer Nederland and Stichting VUmc fonds. The chair of Wiesje van der Flier is supported by the Pasman stichting. M.J. Titulaer has filed a patent, on behalf of the Erasmus MC, for methods for typing neurologic disorders and cancer, and devices for use therein, and has received research funds for serving on a scientific advisory board of Horizon Therapeutics, for consultation at Guidepoint Global LLC, for consultation at UCB, for teaching colleagues by Novartis. MT has received an unrestricted research grant from Euroimmun AG and from CSL Behring. Go to Neurology.org/NN for full disclosure.AcknowledgmentThe authors thank all patients for their participation. The authors also thank Esther Hulsenboom and Ashraf Jozefzoon-Aghai for their technical assistance. M.W.J. Schreurs, F. Leypoldt, P.A.E. Sillevis Smitt, J.M. de Vries, and M.J. Titulaer of this publication are members of the European Reference Network for Rare Immunodeficiency, Autoinflammatory, and Autoimmune Diseases—Project ID No. 739543 (ERN-RITA; HCP Erasmus MC and University Hospital Schleswig-Holstein). H. Seelaar, J.C. van Swieten, and F.J. de Jong of this publication are members of the European Reference Network for Rare Neurological Diseases—Project ID 73910. Research of the VUmc Alzheimer center is part of the neurodegeneration research program of Amsterdam Neuroscience. The Alzheimer Center VUmc is supported by Alzheimer Nederland and Stichting VUmc Fonds. The clinical database structure was developed with funding from Stichting Dioraphte.Appendix Authors<img class="highwire-fragment fragment-image" alt="Table" src="https://nn.neurology.org/content/nnn/10/5/e200137/T3.medium.gif"; width="599" height="2531">FootnotesGo to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article.The Article Processing Charge was funded the authors.Submitted and externally peer reviewed. The handling editor was Editor Josep O. Dalmau, MD, PhD, FAAN.Received December 8, 2022.Accepted in final form May 8, 2023.Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.References1.↵Bastiaansen AEM, van Steenhoven RW, de Bruijn M, et al. Autoimmune encephalitis resembling dementia syndromes. Neurol Neuroimmunol Neuroinflamm. 2021;8(5):e1039.OpenUrlAbstract/FREE Full Text2.↵Lancaster E, Lai M, Peng X, et al. Antibodies to the GABA(B) receptor in limbic encephalitis with seizures: case series and characterisation of the antigen. Lancet Neurol. 2010;9(1):67-76.OpenUrlCrossRefPubMed3.↵Titulaer MJ, McCracken L, Gabilondo I, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol 2013;12(2):157-165.OpenUrlCrossRefPubMed4.↵Flanagan EP, McKeon A, Lennon VA, et al. Autoimmune dementia: clinical course and predictors of immunotherapy response. Mayo Clin Proc. 2010;85(10):881-897.OpenUrlCrossRefPubMed5.↵Geschwind MD, Tan KM, Lennon VA, et al. Voltage-gated potassium channel autoimmunity mimicking creutzfeldt-jakob disease. Arch Neurol. 2008;65(10):1341-1346.OpenUrlCrossRefPubMed6.↵Grau-Rivera O, Sanchez-Valle R, Saiz A, et al. Determination of neuronal antibodies in suspected and definite Creutzfeldt-Jakob disease. JAMA Neurol. 2014;71(1):74-78.OpenUrl7.↵Titulaer MJ, McCracken L, Gabilondo I, et al. Late-onset anti-NMDA receptor encephalitis. Neurology. 2013;81(12):1058-1063.OpenUrlAbstract/FREE Full Text8.↵Gaig C, Graus F, Compta Y, et al. Clinical manifestations of the anti-IgLON5 disease. Neurology. 2017;88(18):1736-1743.OpenUrlAbstract/FREE Full Text9.↵Bastiaansen AEM, de Bruijn M, Schuller SL, et al. Anti-NMDAR encephalitis in The Netherlands, focusing on late-onset patients and antibody test accuracy. Neurol Neuroimmunol Neuroinflamm. 2022;9(2):e1127.OpenUrl10.↵van der Flier WM, Scheltens P. Amsterdam dementia cohort: performing research to optimize care. J Alzheimers Dis. 2018;62(3):1091-1111.OpenUrl11.↵McKhann GM, Knopman DS, Chertkow H, et al. The diagnosis of dementia due to Alzheimer's disease: recommendations from the National Institute on Aging-Alzheimer's Association workgroups on diagnostic guidelines for Alzheimer's disease. Alzheimers Dement. 2011;7(3):263-269.OpenUrlCrossRefPubMed12.↵Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. 2011;134(Pt 9):2456-2477.OpenUrlCrossRefPubMed13.↵Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology. 2011;76(11):1006-1014.OpenUrlAbstract/FREE Full Text14.↵McKeith IG, Boeve BF, Dickson DW, et al. Diagnosis and management of dementia with Lewy bodies: fourth consensus report of the DLB Consortium. Neurology. 2017;89(1):88-100.OpenUrlAbstract/FREE Full Text15.↵Geschwind MD. Rapidly progressive dementia. Continuum (Minneap Minn). 2016;22(2 Dementia):510-537.OpenUrl16.↵Ances BM, Vitaliani R, Taylor RA, et al. Treatment-responsive limbic encephalitis identified by neuropil antibodies: MRI and PET correlates. Brain. 2005;128(Pt 8):1764-1777.OpenUrlCrossRefPubMed17.↵Gresa-Arribas N, Titulaer MJ, Torrents A, et al. Antibody titres at diagnosis and during follow-up of anti-NMDA receptor encephalitis: a retrospective study. Lancet Neurol. 2014;13(2):167-177.OpenUrlCrossRefPubMed18.↵Martinez-Martinez P, Titulaer MJ. Autoimmune psychosis. Lancet Psychiatry. 2020;7(2):122-123.OpenUrl19.↵van Coevorden-Hameete MH, Titulaer MJ, Schreurs MW, et al. Detection and characterization of autoantibodies to neuronal cell-surface antigens in the central nervous system. Front Mol Neurosci. 2016;9:37.OpenUrl20.↵Sabater L, Gaig C, Gelpi E, et al. A novel non-rapid-eye movement and rapid-eye-movement parasomnia with sleep breathing disorder associated with antibodies to IgLON5: a case series, characterisation of the antigen, and post-mortem study. Lancet Neurol. 2014;13(6):575-586.OpenUrlCrossRefPubMed21.↵Gaig C, Compta Y, Heidbreder A, et al. Frequency and characterization of movement disorders in anti-IgLON5 disease. Neurology. 2021;97(14):e1367–e1381.OpenUrlAbstract/FREE Full Text22.↵Gelpi E, Hoftberger R, Graus F, et al. Neuropathological criteria of anti-IgLON5-related tauopathy. Acta Neuropathol. 2016;132(4):531-543.OpenUrlCrossRefPubMed23.↵Landa J, Gaig C, Plaguma J, et al. Effects of IgLON5 antibodies on neuronal cytoskeleton: a link between autoimmunity and neurodegeneration. Ann Neurol. 2020;88(5):1023-1027.OpenUrlCrossRefPubMed24.↵Giannoccaro MP, Gastaldi M, Rizzo G, et al. Antibodies to neuronal surface antigens in patients with a clinical diagnosis of neurodegenerative disorder. Brain Behav Immun. 2021;96:106-112.OpenUrl25.↵Hermann P, Zerr I. Rapidly progressive dementias - aetiologies, diagnosis and management. Nat Rev Neurol. 2022;18(6):363-376.OpenUrl26.↵Dechelotte B, Muniz-Castrillo S, Joubert B, et al. Diagnostic yield of commercial immunodots to diagnose paraneoplastic neurologic syndromes. Neurol Neuroimmunol Neuroinflamm. 2020;7(3):e701.OpenUrlAbstract/FREE Full Text27.↵van Sonderen A, Schreurs MW, de Bruijn MA, et al. The relevance of VGKC positivity in the absence of LGI1 and Caspr2 antibodies. Neurology. 2016;86(18):1692-1699.OpenUrlCrossRefPubMed28.↵Muñoz Lopetegi A, Boukhrissi S, Bastiaansen A, et al. Neurological syndromes related to anti-GAD65: clinical and serological response to treatment. Neurol Neuroimmunol Neuroinflamm. 2020;7(3):e696.OpenUrlAbstract/FREE Full Text29.↵Mattozzi S, Sabater L, Escudero D, et al. Hashimoto encephalopathy in the 21st century. Neurology. 2020;94(2):e217-e224.OpenUrlAbstract/FREE Full Text30.↵Flanagan EP, Geschwind MD, Lopez-Chiriboga AS, et al. Autoimmune encephalitis misdiagnosis in adults. JAMA Neurol. 2023;80(1):30-39.OpenUrl
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Research study - can you help?

Research study - can you help? | AntiNMDA | Scoop.it
Researchers at Kings College London are looking for young people to travel to London and help with an encephalitis study...
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Sociocultural Influences in Autoimmune Encephalitis Without Neurologic Symptoms

Sociocultural Influences in Autoimmune Encephalitis Without Neurologic Symptoms | AntiNMDA | Scoop.it
This complex case highlights barriers to identifying autoimmune encephalitis when no neurologic symptoms are present, which are normally central to disease detection.
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Anti N-Methyl-D-Aspartate receptor antibody associated Acute Demyelinating Encephalomyelitis in a patient with COVID-19: a case report | Journal of Medical Case Reports | Full Text

Anti N-Methyl-D-Aspartate receptor antibody associated Acute Demyelinating Encephalomyelitis in a patient with COVID-19: a case report | Journal of Medical Case Reports | Full Text | AntiNMDA | Scoop.it
Background Anti N-Methyl-D-Aspartate (NMDA) receptor antibody associated ADEM is a diagnosis that was first described relatively recently in 2007 by Dalmau et al. The recent COVID-19 pandemic has resulted in multiple neurological complications being reported.
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Autoimmune Encephalitis Consensus Criteria | Neurology Clinical Practice

Autoimmune Encephalitis Consensus Criteria | Neurology Clinical Practice | AntiNMDA | Scoop.it
June 2023; 13 (3) Editorial Autoimmune Encephalitis Consensus CriteriaLessons Learned From Real-World Practice View ORCID ProfileJeffrey M. Gelfand, Chu-Yueh Guo First published April 25, 2023, DOI: https://doi.org/10.1212/CPJ.0000000000200155 Full PDF Citation Permissions Make Comment See Comments Downloads133 Share Article Info & Disclosures This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased. Autoimmune encephalitis (AE) encompasses a spectrum of neurologic disorders caused by brain inflammation, a subset of which is associated with autoantibodies to neuronal cell-surface antigens such as anti-N-methyl-d-aspartate (NMDA) receptor AE or anti-leucine-rich glioma-inactivated 1 (LGI1) AE.1 Up to half of patients with AE, however, do not have abnormal neuronal or glial autoantibodies identified and are classified as having “seronegative” AE.2 Clinical antibody testing can take several days to result, a time in which clinicians caring for patients with suspected AE may wish to initiate empiric immunosuppressive therapy. Antibody testing is also not readily accessible in some health care settings and, even when technically available, may require time-consuming advocacy with local clinical laboratories to justify relatively costly send-out testing. To add further complexity, some patients with immunoreactive (e.g., laboratory true-positive) antibodies do not have clinical AE, and over-reliance and misapplication of antibody testing were identified as important contributors to AE misdiagnosis in a 2023 multicenter analysis.3FootnotesFunding information and disclosures are provided at the end of the article. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/cp.See page e200151© 2023 American Academy of NeurologyView Full Text AAN Members We have changed the login procedure to improve access between AAN.com and the Neurology journals. If you are experiencing issues, please log out of AAN.com and clear history and cookies. (For instructions by browser, please click the instruction pages below). After clearing, choose preferred Journal and select login for AAN Members. You will be redirected to a login page where you can log in with your AAN ID number and password. When you are returned to the Journal, your name should appear at the top right of the page. Google Safari Microsoft Edge Firefox Click here to login AAN Non-Member Subscribers Click here to login Purchase access For assistance, please contact: AAN Members (800) 879-1960 or (612) 928-6000 (International) Non-AAN Member subscribers (800) 638-3030 or (301) 223-2300 option 3, select 1 (international) Sign Up Information on how to subscribe to Neurology and Neurology: Clinical Practice can be found here Purchase Individual access to articles is available through the Add to Cart option on the article page. Access for 1 day (from the computer you are currently using) is US$ 39.00. Pay-per-view content is for the use of the payee only, and content may not be further distributed by print or electronic means. The payee may view, download, and/or print the article for his/her personal, scholarly, research, and educational use. Distributing copies (electronic or otherwise) of the article is not allowed. You May Also be Interested in Back to top Safety and Efficacy of Tenecteplase and Alteplase in Patients With Tandem Lesion Stroke: A Post Hoc Analysis of the EXTEND-IA TNK Trials Dr. Nicole Sur and Dr. Mausaminben Hathidara ► Watch Related Articles Autoimmune Encephalitis Criteria in Clinical Practice Topics Discussed All Clinical Neurology Autoimmune diseases Encephalitis Alert Me Alert me when eletters are published
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Predictive Value of Serum Neurofilament Light Chain Levels in Anti-NMDA Receptor Encephalitis

Predictive Value of Serum Neurofilament Light Chain Levels in Anti-NMDA Receptor Encephalitis | AntiNMDA | Scoop.it
Increased serum NfL levels reflect neuroaxonal damage in anti-NMDAR encephalitis. No relationship was identified with disease severity, whereas the association with outcome was confounded by age.The implied role of sampling timing on NfL levels also limits the applicability of NfL as a prognostic...
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Frontiers | The MOG antibody associated encephalitis preceded by COVID-19 infection; a case study and systematic review of the literature

Frontiers | The MOG antibody associated encephalitis preceded by COVID-19 infection; a case study and systematic review of the literature | AntiNMDA | Scoop.it
BackgroundNew neurological complications of COVID-19 infection have been reported in recent research. Among them, the spectrum of anti-MOG positive diseases, defined as anti-MOG antibody associated disease (MOGAD), is distinguished, which can manifest as optic neuritis, myelitis, or various forms...
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Enceph-IG Study - Institute of Infection, Veterinary and Ecological Sciences - University of Liverpool

Enceph-IG Study - Institute of Infection, Veterinary and Ecological Sciences - University of Liverpool | AntiNMDA | Scoop.it
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A Rare Presentation of Steroid-responsive Encephalopathy Associated with Autoimmune Thyroiditis with Neuropsychiatric Symptoms: A Case Report

A Rare Presentation of Steroid-responsive Encephalopathy Associated with Autoimmune Thyroiditis with Neuropsychiatric Symptoms: A Case Report | AntiNMDA | Scoop.it
A 42-year-old woman presented in the emergency department with acute onset whole-body myoclonic jerks for 1 day.On enquiry, the patient’s parents advised...
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Pioneering Research in Autoimmune Neurology: Vanda Lennon, M.D., Ph.D.

Pioneering Research in Autoimmune Neurology: Vanda Lennon, M.D., Ph.D. | AntiNMDA | Scoop.it
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New center to spotlight neurological autoimmune disorders

New center to spotlight neurological autoimmune disorders | AntiNMDA | Scoop.it
How do neurological disorders arise that are caused, triggered, or influenced by antibodies? What better possibilities are there for diagnosis – and above all for treatment? These are the questions addressed by the new Clinical Research Unit “BecauseY” headed by Charité – Universitätsmedizin Berlin.
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Progressive alliance advances science through patient-powered research

Progressive alliance advances science through patient-powered research | AntiNMDA | Scoop.it
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ENCEPH-IG Trial: The Challenges Of Running A Rare Disease Trial - Centre for Trials Research

ENCEPH-IG Trial: The Challenges Of Running A Rare Disease Trial - Centre for Trials Research | AntiNMDA | Scoop.it
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30 neurological disorders every doctor should know about –

30 neurological disorders every doctor should know about – | AntiNMDA | Scoop.it
Neurology is a jungle of disorders and syndromes. This creates a challenge for doctors and medical students... What to prioritise for learning and practice? *** To solve this conundrum... We combed the extensive database of Neurochecklists...
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A score that predicts 1-year functional status in patients with anti-NMDA receptor encephalitis

A score that predicts 1-year functional status in patients with anti-NMDA receptor encephalitis | AntiNMDA | Scoop.it
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.
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Canadian Blood Services needs thousands more donors to roll up their sleeves | CBC News

Canadian Blood Services needs thousands more donors to roll up their sleeves | CBC News | AntiNMDA | Scoop.it
Canadian Blood Services is looking to fill 150,000 appointments for people willing to donate their blood or plasma to tackle a shortage.
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A catatonic woman awakened after 20 years. Her story may change psychiatry – My Health CRM

A catatonic woman awakened after 20 years. Her story may change psychiatry – My Health CRM | AntiNMDA | Scoop.it
New research suggests that a subset of patients with psychiatric conditions such as schizophrenia may actually have autoimmune disease that attacks the brain...
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Case Report: Paroxysmal weakness of unilateral limb as an initial symptom in anti-LGI1 encephalitis: a report of five cases

Case Report: Paroxysmal weakness of unilateral limb as an initial symptom in anti-LGI1 encephalitis: a report of five cases | AntiNMDA | Scoop.it
Anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis is the second most common kind of autoimmune encephalitis following anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis.Anti-LGI1 encephalitis is characterized by cognitive impairment or rapid progressive dementia, psychiatric disorders...
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Medical Moment: The signs of ‘brain-on-fire’ disease

Medical Moment: The signs of ‘brain-on-fire’ disease | AntiNMDA | Scoop.it
(WNDU) - Imagine being totally fine one day, then the next, you’re having hallucinations, seizures, memory loss, and even trouble talking.It’s called “brain-on-fire” disease or anti-NMDA receptor encephalitis. It’s a rare neurological disorder that can cause inflammation in the brain.It occurs when the body’s immune system mistakenly attacks the NMDA receptors in the brain, which are responsible for regulating communication between nerve cells. Brain-on-fire disease is often misdiagnosed as other neurological disorders or psychiatric illnesses because its symptoms are similar to those of many other conditions.However, a blood or cerebrospinal fluid test can help diagnose the disease by detecting the presence of antibodies that attack the NMDA receptors in the brain. The disease is rare as it affects one in 1.5 million people a year.Katie Miller would be one of those people.Hunting, mountain biking, horseback riding - you name it, Katie Miler would do it... until she couldn’t.“I just didn’t feel like myself, like normal,” Katie recalled.“Katie said, ‘Mom, I feel like my brain snapped,’” said Colleen Miller, Katie’s mother.Local doctors admitted Katie into a psychiatric ward, but what was happening to Katie wasn’t mental; it was physical.“What happens is you’re perfectly normal one day, and suddenly overnight, this person can become paranoid, can start having visual hallucinations, auditory hallucinations,” explained Stacy Clardy, MD, PhD, an autoimmune neurologist at the University of Utah.Anti-NMDA receptor encephalitis is misdiagnosed as a psychiatric disorder in up to 40% of patients.“So, for many of the females, especially after puberty, they can develop what’s called an ovarian dermoid cyst or an ovarian teratoma,” Dr. Clardy said.These cysts often have hair and teeth in them. The immune system sees it as foreign and attacks it, but...“In these cysts, there is a component of tissue that really is brain tissue,” Dr. Clardy continued.Within four days, Katie was catatonic and needed a ventilator to breathe. There is no single approved treatment. That’s why a five-year, nationwide clinical trial is testing whether a drug called Inebilizumab will stop the assault on the brain. It has the potential to improve outcomes for patients who are not responding to other treatments and may also lead to fewer long-term neurological effects.Katie had her cyst removed; she can’t remember three months of her life. But now, with various medications, Katie is on her way to recovery.Up to 50% of patients can suffer long-term consequences, especially cognitive and mood symptoms.Copyright 2023 WNDU. All rights reserved.
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