Receptor Encephalitis Research Articles

Regional cerebral blood flow in a patient with asystole episodes associated with anti-NMDA receptor encephalitis

ABSTRACT Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a rare and severe autoimmune encephalitis that displays neuropsychiatric symptoms and autonomic instability, e.g., hypoventilation and cardiac arrhythmia. Severe arrhythmia including asystole associated with this encephalitis is rare. Several causes have been suggested. Nevertheless, no report of the literature has described examination by functional brain imaging of a patient with asystole during anti-NMDA receptor encephalitis. This case is that of a 34-year-old woman diagnosed as having anti-NMDA receptor encephalitis. She repeatedly showed 10–20 s asystole episodes necessitating a temporary transvenous pacemaker. After resection of the bilateral ovarian cystic tumor, her symptoms improved. Regional cerebral blood flow (rCBF) was evaluated using single-photon emission computed tomography. The rCBF was increased in the amygdala, hypothalamus, anterior cingulate, hippocampus, and anterior temporal lobes, but decreased in the dorsolateral frontal lobes, parietal lobes, and occipital lobes. Findings in this case suggest that altered rCBF in the patient with asystole episodes associated with anti-NMDA receptor encephalitis was observed in several brain lesions. The rCBF increases in the central autonomic networks, i.e., the amygdala, hypothalamus, and anterior cingulate, might be associated with dysregulation of sympathetic and parasympathetic nervous systems leading to asystole.

Acute pediatric encephalitis: etiology, course, and outcome of a 12-year single-center immunocompetent cohort.

Encephalitis is an uncommon but severe disorder due to an inflammation of the brain parenchyma, usually diagnosed on clinical, laboratory, electroencephalographic, and neuroradiological features. New causes of encephalitis have been reported in recent years, so diagnostic criteria have changed over time. We report on a single-center experience of a pediatric Hospital, the hub of its region, over 12years (2008-2021), with the evaluation of all children managed for acute encephalitis. We retrospectively reviewed clinical, laboratory, neuroradiological, and EEG data from the acute phase and outcome of all immunocompetent patients diagnosed with acute encephalitis. According to the newly proposed criteria for pediatric autoimmune encephalitis, we divided patients into infectious, definite autoimmune, probable autoimmune, and possible autoimmune, and performed a comparison between the different groups. 48 patients (26 females, mean age 4.4years), 19 with infections, and 29 with autoimmune encephalitis, were included. Herpes simplex virus 1 encephalitis was the most frequently identified etiology followed by anti-NMDA receptor encephalitis. Movement disorders at onset and a longer hospital stay were observed more frequently in autoimmune compared to infectious encephalitis (p p < 0.001 and p = 0.001, respectively). Among the autoimmune group, children who started immunomodulatory treatment earlier (within 7days from onset) had more frequent complete functional recovery (p = 0.002). Herpes virus and anti-NMDAR encephalitis are the most frequent etiologies within our cohort. Clinical onset and course are extremely variable. Since early immunomodulatory treatment was associated with a better functional outcome, our data confirm that a timely diagnostic classification in definite, probable, or possible autoimmune encephalitis can help the clinician in a successful therapeutic approach.

Teratomas from past to the present: A scientometric analysis with global productivity and research trends between 1980 and 2022.

There is currently no bibliometric study on teratomas in the literature. This study aims to analyze the published articles on teratomas to provide an overview of the subject, determine global productivity, and identify current research trends. Additionally, data on different components of scientific output (countries, journals, institutions, authors) were analyzed. A total of 4209 articles published on teratomas between 1980 and 2022 were analyzed using various bibliometric and statistical methods. Bibliometric network visualization maps were used to determine trending topics, citation analyses, and international collaborations. Spearman correlation coefficient was used for correlation analysis. The top 3 countries that made the most contributions to the literature were the USA (1041, 24.7%), Japan (501, 11.9%), and India (310, 7.3%). The top 3 active institutions were the University of California System (n = 78), University of London (64), and Harvard University (62). The top 3 productive journals were the Journal of Pediatric Surgery (n = 141), Pediatric Surgery International (n = 70), and Journal of Pediatric Surgery Case Reports (69). The most productive author was Ulbright TM. (n = 18). The most studied topics from past to present were ovarian cancer/ovarian teratoma/ovarian torsion, mature cystic teratoma/dermoid cyst, sacrococcygeal teratoma, germ cell tumors, immature teratoma, malignant transformation, mediastinal teratoma/mediastinum, neonate/newborn/infant, prenatal diagnosis, testis/testicular cancer/teratoma, ultrasonography/ultrasound, magnetic resonance imaging, chemotherapy, growing teratoma syndrome, surgery, retroperitoneal teratoma/retroperitoneum, laparoscopic surgery/laparoscopy, children/child, and fetal surgery/fetus. We identified trend research topics in the field of teratomas in recent years, including mature cystic teratoma, ovarian teratoma/neoplasm, ovarian cancer, ovarian torsion, growing teratoma syndrome, recurrence, pediatric, testicular cancer, anti-n-methyl-d-aspartate receptor encephalitis, immature teratoma, retroperitoneal, struma ovarii, and carcinoid. The research leadership in the development of teratoma literature was determined by countries with major economies such as the USA, Japan, India, the UK, China, Turkey, South Korea, and other European countries (France, Germany, Italy).

Anti-N-methyl-d-aspartate receptor encephalitis and positive human herpesvirus-7 deoxyribonucleic acid in cerebrospinal fluid: a case report

BackgroundAnti-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, epithelial cells, and central nervous system cells. The pathogenicity of human herpesvirus-7 is unclear. Cases of anti-N-methyl-d-aspartate receptor encephalitis with human herpesvirus-7 present in cerebrospinal fluid have been documented, but the clinical significance of this finding remains unclear.Case presentationAn 11-year-old Caucasian boy was admitted to hospital after a generalized tonic–clonic seizure. Generalized tonic seizures repeated three more times during the day of hospitalization. Blood tests showed minor ongoing inflammation, while brain computed tomography yielded normal results. Brain magnetic resonance imaging showed hyperintense focal alterations in both temporal lobes, hippocampi, and at the base of the right frontal lobe. Positive anti-N-methyl-d-aspartate receptor antibodies were found in both serum and cerebrospinal fluid. Positive novel coronavirus 2 (severe acute respiratory syndrome coronavirus 2) immunoglobulin G antibodies were found in serum. Polymerase chain reaction test for severe acute respiratory syndrome coronavirus 2 was negative. Furthermore, positive human herpesvirus-7 deoxyribonucleic acid was found in cerebrospinal fluid. The patient was treated with acyclovir, human immunoglobulin, and methylprednisolone. The seizures did not repeat, and no psychiatric symptoms were present. The patient made a full recovery.ConclusionsWe present a pediatric case of anti-N-methyl-d-aspartate receptor encephalitis with atypical clinical presentation. The role of human herpesvirus-7 in neurological disorders remains unclear in immunocompetent patients.

The importance of ruling out herpes simplex virus encephalitis in N-methyl-ᴅ-aspartate receptor encephalitis studies.

The importance of ruling out herpes simplex virus encephalitis in N-methyl-ᴅ-aspartate receptor encephalitis studies.

Anti-NMDAR encephalitis in Southeast Asia – A single-centre, longitudinal study

AimsTo describe the clinical features and outcomes of anti-NMDA receptor encephalitis (ANMDARE) in Southeast Asian (SEA) patients. MethodSEA patients diagnosed and treated for ANMDARE at Singapore General Hospital between January 2010 and June 2020 were included in this observational study, in which their clinical features and outcomes were retrospectively analysed. ResultsWe studied 20 patients: 11 Chinese, 3 Tagalogs, 2 Malays, 2 Indians, 1 Eurasian and 1 Javanese. Their median age was 28 years. 15 were females, amongst whom teratomas were demonstrated in 13 (12 ovarian, 1 mediastinal). Delirium and seizures were the two commonest events leading to their presentation at our facility. 1 male had biliary neuroendocrine tumour. Comparison between genders revealed a strong male predilection for early seizures and insomnia; females were four times likelier than males to develop movement disorders or have underlying neoplasms. Patients with dysautonomia required longer ICU stay beyond 14 days, but their outcomes at 1 year did not differ. When reviewed at 1 year, none had clinical relapses, and outcomes were favourable (mRS 0–2) in nearly two-thirds. ConclusionsSEA patients with ANMDARE frequently present with delirium and seizures. Underlying neoplasms are very common in females. Differences in clinical characteristics may exist between the two genders. Recognition of these can facilitate diagnosis, and permit earlier initiation of appropriate treatment strategies, and thus improve outcomes of SEA patients.

Infectious profiles in pediatric anti-N-methyl-d-aspartate receptor encephalitis

Anti-N-methyl-d-aspartate receptor autoimmune encephalitis (NMDAR AE) is an antibody-mediated neurological disorder that may be caused by post-herpes simplex virus-1 meningoencephalitis (HSV ME) and ovarian teratomas, although most pediatric cases are idiopathic. We sought to evaluate if other infections precede NMDAR AE by conducting a single-center, retrospective, case-control study of 86 pediatric cases presenting to Texas Children's Hospital between 2006 and 2022. HSV ME (HSV-1 and HSV-2) was a significantly more common preceding infection in the experimental group compared to control patients with idiopathic intracranial hypertension, while there was no difference in remote HSV infection between the two groups. Recent Epstein-Barr virus infection was evident in 8/42 (19%) tested experimental patients in comparison to 1/25 (4%) tested control patients which provided evidence for a genuine measure of effect but was not statistically significant due to small sample size (p = 0.07). The other 25 infectious etiologies were not different among the two groups and not all variables were clinically indicated or obtained in every subject, highlighting the need for future standardized, multi-institutional studies on underlying infectious precursors of autoimmune encephalitis.

Takotsubo cardiomyopathy associated with Herpes simplex encephalitis: A case report and literature review

Takotsubo cardiomyopathy (TCM) is a stress-induced cardiomyopathy characterized by transient left ventricular dysfunction. It can be triggered by various central nervous system pathologies including status epilepticus (SE) and N-methyl-d-aspartate receptor (NMDAr) encephalitis. Herpex simplex encephalitis (HSE) is a life-threatening, sporadic, encephalitis associated with focal or global cerebral dysfunction caused by herpes simplex viruses type 1(HSV-1), or less commonly, type 2(HSV-2). While approximately 20% of patients with HSE develop NMDAr antibodies, not all manifest with encephalitis clinically. We present here a 77-year-old woman admitted with HSV-1 encephalitis who presented with acute encephalopathy and seizure-like activity. Continuous EEG monitoring (cEEG) showed periodic lateralized epileptiform discharges (PLEDs) involving the left parietotemporal region but no evidence of electrographic seizures. Her early hospital course was complicated by TCM which subsequently resolved on repeat TTE. She demonstrated initial neurological improvement. However, five weeks later her mental status declined. Again, no seizures were appreciated on cEEG. Unfortunately, repeat studies including lumbar puncture and magnetic resonance imaging (MRI) of the brain were consistent with NMDAr encephalitis. She was treated with immunosuppression and immunomodulation therapies. To our knowledge we report the first case of TCM secondary to HSE without comorbid status epilepticus. However, further studies are needed to better understand the correlation between, and underlying pathophysiology of HSE and TCM, as well as any potential association with this presentation and subsequent development of NMDAr encephalitis.

Inhibiting PI3K/Akt-Signaling Pathway Improves Neurobehavior Changes in Anti-NMDAR Encephalitis Mice by Ameliorating Blood–Brain Barrier Disruption and Neuronal Damage

The disruption of the blood–brain barrier (BBB) is hypothesized to be involved in the progression of anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis, but its mechanism is still unclear. Recently, the phosphatidylinositol 3-kinase (PI3K)/threonine kinase (Akt) pathway is involved in the regulation of the BBB in various diseases. This study is aimed to investigate the mechanism of BBB damage and neurobehavior changes in anti-NMDAR encephalitis mice. Female C57BL/6J mice were actively immunized to establish an anti-NMDAR encephalitis mouse model and evaluate the neurobehavior changes of mice. To study its potential mechanism, LY294002 (PI3K inhibitor, 8 mg/kg) and Recilisib (PI3K agonist, 10 mg/kg) were treated by intraperitoneal injection, respectively. Anti-NMDAR encephalitis mice showed neurological deficits, increased BBB permeability, open endothelial tight junctions (TJs), and decreased expression of TJ-related proteins zonula occludens (ZO)-1 and Claudin-5. However, administration of PI3K inhibitor significantly reduced the expression of p-PI3K and p-Akt, improved neurobehavior function, decreased BBB permeability, and upregulated the expressions of ZO-1 and Claudin-5. Furthermore, PI3K inhibition reversed the decline of NMDAR NR1 in the membranes of hippocampal neurons, which reduced the loss of neuron-specific nucleoprotein (NeuN) and microtubule-associated protein 2 (MAP2). In contrast, administration of the PI3K agonist Recilisib showed a tendency to exacerbate BBB breakdown and neurological deficits. Our results showed that the activation of PI3K/Akt, along with the changes in TJ-related proteins ZO-1 and Claudin-5, may be closely related to BBB damage and neurobehavior changes in anti-NMDAR encephalitis mice. PI3K inhibition attenuates BBB disruption and neuronal damage in mice, thereby improving neurobehavior.

Elevated cerebrospinal fluid YKL-40 levels in patients with anti-gamma-aminobutyric- acid-B receptor encephalitis

ObjectiveAnti-gamma-aminobutyric-acid-B receptor (GABAbR) encephalitis is a rare form of autoimmune encephalitis. Until now, there are few biomarkers that can indicate the severity and prognosis of patients with anti-GABAbR encephalitis. The objective of this study was to exam the changes of chitinase-3-like protein 1 (YKL-40) in patients with anti-GABAbR encephalitis. In addition, whether YKL-40 could indicate the disease severity was also evaluated. MethodsThe clinical features of 14 patients with anti-GABAbR encephalitis and 21 patients with anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis were retrospectively studied. YKL-40 levels in serum and cerebral fluid (CSF) of patients were detected by enzyme-linked immunosorbent assay. The correlation of modified Rankin Scale (mRS) score of encephalitis patients and YKL40 levels were analyzed. ResultsYKL-40 levels in CSF were significantly higher in patients with anti-GABAbR encephalitis or anti-NMDAR encephalitis than those in controls. YKL-40 levels between these two encephalitis groups were not different. Moreover, YKL-40 levels in CSF from patients with anti-GABAbR encephalitis were positively correlated with the mRS score at admission and at 6-month follow-up. ConclusionYKL-40 level is elevated in CSF from patients with anti-GABAbR encephalitis at early disease stage. YKL-40 may be a potential biomarker indicating the prognosis of patients with anti-GABAbR encephalitis.

Risk Factors and Prognosis in Anti-NMDA Receptor Encephalitis Patients with Disturbance of Consciousness.

Disturbance of consciousness is common in patients with severe anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. However, little is known about it. This study aimed to analyze the clinical manifestations and prognostic factors of anti-NMDAR encephalitis with disturbance of consciousness. In this retrospective study, the clinical features, treatment results, and long-term outcomes of anti-NMDAR encephalitis patients with disturbance of consciousness were analyzed, and multivariate logistic regression was used to analyze the factors affecting their prognosis. In the group with disturbance of consciousness, the incidences of seizures, involuntary movements, pulmonary infection, mechanical ventilation, intensive care unit (ICU) admission, neutrophil-lymphocyte ratio (NLR), abnormal cerebrospinal fluid index, plasma exchange, and immunosuppressive therapy were higher than those in the group without disturbance of consciousness (all P<0.05). During the follow-up period (median: 36 months, range: 12-78 months), the modified Rankin scale (mRS) score, the maximum mRS score during hospitalization, the mRS score at discharge, and the mRS score at 12 months after discharge were higher in the disturbance of consciousness group (all P < 0.001). However, there was no significant difference in long-term outcomes and recurrence between the two groups. Multivariate logistic regression analysis showed that mechanical ventilation, elevated IgG index, and delayed immunotherapy were independent risk factors for poor outcomes in patients with anti-NMDAR encephalitis with disturbance of consciousness at 12 months (odds ratio: 22.591, 39.868, 1.195). The receiver operating characteristics (ROC) curve analysis showed that the area under the curve (AUC) of mechanical ventilation, elevated IgG index, and delayed immunotherapy was 0.971 (95% CI=0.934-1.000, P<0.001). Mechanical ventilation, elevated IgG index, and delayed immunotherapy may be the influencing factors of poor prognosis of anti-NMDAR encephalitis patients with disturbance of consciousness. Although their condition is relatively serious, most patients with anti-NMDAR encephalitis with disturbance of consciousness will achieve favorable long-term outcomes after long-term treatment.

CNS4 causes subtype-specific changes in agonist efficacy and reversal potential of permeant cations in NMDA receptors.

The NMDA subtype of glutamate receptor serves as an attractive drug target for the treatment of disorders evolving from hyper- or hypoglutamatergic conditions. Compounds that optimize the function of NMDA receptors are of great clinical significance. Here, we present the pharmacological characterization of a biased allosteric modulator, CNS4. Results indicate that CNS4 sensitizes ambient levels of agonists and reduces higher-concentration glycine & glutamate efficacy in 1/2AB receptors, but minimally alters these parameters in diheteromeric 1/2A or 1/2B receptors. Glycine efficacy is increased in both 1/2C and 1/2D, while glutamate efficacy is decreased in 1/2C and unaltered in 1/2D. CNS4 does not affect the activity of competitive antagonist binding at glycine (DCKA) and glutamate (DL-AP5) sites; however, it decreases memantine potency in 1/2A receptors but not in 1/2D receptors. Current-voltage (I-V) relationship studies indicate that CNS4 potentiates 1/2A inward currents, a phenomenon that was reversed in the absence of permeable Na+ ions. In 1/2D receptors, CNS4 blocks inward currents based on extracellular Ca2+ concentration. Further, CNS4 positively modulates glutamate potency on E781A_1/2A mutant receptors, indicating its role at the distal end of the 1/2A agonist binding domain interface. Together, these findings reveal that CNS4 sensitizes ambient agonists and allosterically modulates agonist efficacy by altering Na+ permeability based on the GluN2 subunit composition. Overall, the pharmacology of CNS4 aligns with the need for drug candidates to treat hypoglutamatergic neuropsychiatric conditions such as loss function GRIN disorders and anti-NMDA receptor encephalitis.

HSV-1 Encephalitis Presenting with Diplopia: Effects of Infection or Autoimmunity?

This report describes a case in which diplopia was developed as a finding of postinfectious anti- N -methyl- d -aspartate receptor encephalitis. Infectious encephalitis, especially herpes simplex virus, is essential as it is one of the triggers of autoimmune encephalitis. Even if the cases present unexpected clinical findings, we should be vigilant in terms of autoimmune processes, such as diplopia seen in our case.

Anti-N-Methyl-D-Aspartate Receptor Encephalitis Presenting Atypically as a Mimic of Rapidly Progressive Neurodegenerative Disorder in a Toddler: A Case Report

Anti-N-Methyl-D-Aspartate Receptor Encephalitis Presenting Atypically as a Mimic of Rapidly Progressive Neurodegenerative Disorder in a Toddler: A Case Report

Long-term effects of anti-N-methyl-d-aspartate receptor encephalitis on quality of life.

Patients with anti-N-methyl-d-aspartate receptor encephalitis (NMDARE) usually achieve neurologically favorable outcomes in the post-acute-phase. Even when motor function recovers, many patients experience numerous non-motor sequelae and cannot resume their pre-NMDARE lives even years later. Additionally, the needs of patients with NMDARE may impose a severe caregiver burden. Unfortunately, few studies have comprehensively examined patients recovering from NMDARE. We investigated the long-term effects of NMDARE on patients' quality of life (QOL). Data collected via structured self-reported questionnaires included clinical features, long-term outcomes, and QOL. These questionnaires were administered to adult members of the Japanese Anti-NMDARE Patients' Association. We used the NeuroQOL battery to assess QOL in physical, mental, and social domains. Raw NeuroQOL scores were converted to T-scores for comparison with controls. Twenty-two patients completed the questionnaire. The median interval between disease onset and questionnaire response was 78 months. Forty-six percent of patients reported persistent sequelae, with only 73% able to resume prior work/school activities. Although patients' Global QOL was similar to controls, patients with NMDARE had significantly worse social QOL. Patients with worse social QOL had more frequent sequelae than those with better social QOL. Furthermore, patients with persistent sequelae had significantly worse Global QOL than those without sequelae and controls. Patients with NMDARE had worse social QOL than controls. Given the adverse effects of disease sequelae on QOL, treatment strategies that minimize sequelae during the acute-phase may improve patients' QOL, even years post-disease onset.

Anti-GABA-A Receptor Antibody-Mediated Epilepsia Partialis Continua After Treatment With Alemtuzumab: A Case Report.

Patients with anti-GABA-A receptor encephalitis characteristically experience therapy-refractory epileptic seizures. General anesthesia is often required to terminate refractory status epilepticus. The immunologic mechanisms leading to antibody formation remain to be elucidated. Described triggers of anti-GABA-A autoimmunity are tumors, mainly thymomas, and herpes simplex encephalitis. We present a young woman with prediagnosis of relapse remitting multiple sclerosis (MS), treated with interferons, natalizumab, and alemtuzumab. Six months after one and only cycle of alemtuzumab, speech arrest and behavioral changes with aggressive and anxious traits appeared. She showed increasing motor convulsions resulting in focal status epilepticus. Anti-GABA-A receptor antibodies in CSF and serum were confirmed in different external laboratories, in a more extensive analysis after antibodies against NMDAR, CASPR2, LGI1, GABABR, and AMPAR were ruled out during in-house examination. Clinical condition improved temporarily with cortisone therapy, plasmapheresis, and IVIG but deteriorated rapidly after steroid discontinuation, resulting in brain biopsy. On histopathologic confirmation consistent with anti-GABA-A receptor antibody-associated CNS inflammation, completing the first rituximab cycle, continuing oral corticosteroids and supplementing immunosuppression with cyclosporine A led to quick recovery. Our case describes a severe autoantibody-induced encephalitis in a young patient with MS, with alemtuzumab as a potential trigger for anti-GABA-A receptor encephalitis.

Anti-N-methyl-D-aspartate receptor encephalitis after resection of cerebral astrocytoma

Anti-N-methyl-D-aspartate receptor encephalitis after resection of cerebral astrocytoma

Suicidal Thoughts and Behaviors in Anti-NMDA Receptor Encephalitis: Psychopathological Features and Clinical Outcomes.

A wide variety of neuropsychiatric symptoms are described during the acute phase of anti-N-methyl-d-aspartate receptor encephalitis (ANMDARE), including psychosis, mania, depression, and catatonia, but there are few reports on suicidal thought and behaviors in ANMDARE. To address this gap in the literature, the authors measured the presence of suicidal thoughts and behaviors among a large cohort of Mexican patients diagnosed with definite ANMDARE. This observational and longitudinal study included patients with definite ANMDARE hospitalized at the National Institute of Neurology and Neurosurgery of Mexico between 2014 and 2021. Suicidal thoughts and behaviors were assessed before and after treatment by means of a clinical interview with relatives and a direct clinical assessment with each patient. Thoughts of engaging in suicide-related behavior and acts of suicidal and nonsuicidal self-directed violence before and during hospitalization were recorded. From a total sample of 120 patients who fulfilled the diagnostic criteria for definite ANMDARE, 15 patients (13%) had suicidal thoughts and behaviors during the acute phase of the disease. All 15 of these patients experienced psychosis and had suicidal ideation with intention. Three patients engaged in preparatory behaviors and seven carried out suicidal self-directed violence. Psychotic depression and impulsivity were more frequent among those patients with suicidal thoughts and behaviors than among those without any form of suicidality. Four patients engaged in self-directed violence during hospitalization. Remission was sustained in 14 of 15 patients, with suicidal ideation and self-directed violence persisting during follow-up in only one patient. Suicidal thoughts and behaviors are not uncommon during the acute phase of ANMDARE. On the basis of our sample, the persistence of these features after immunotherapy is rare but may be observed. A targeted assessment of suicidal risk should be strongly considered in this population.

Nonparaneoplastic anti-NMDA receptor encephalitis in an adolescent girl: a case report.

A 17-year-old female presented to the hospital with an 11-day history of fever, altered behavior, abnormal body movements, and altered sensorium. Upon examination, she was found to be febrile, tachycardic, and tachypneic, with a Glasgow Coma Scale score of 8. The diagnosis of anti-NMDA receptor encephalitis is usually confirmed by the presence of anti-NMDA receptor antibodies in the cerebrospinal fluid. The first-line treatment options include steroids, intravenous immunoglobulin, and plasmapheresis, while second-line therapies such as rituximab and cyclophosphamide may be necessary for some patients. While most patients respond well to treatment, complications can arise, and as in this case, death can occur. New onset symptoms like alteration in behavior, abnormal body movement, altered sensorium, and psychiatric symptoms in a young female should raise suspicion of this disease. Immunotherapy is promising; however, anticipation and management of complication are essential in reducing mortality.

PO-04-103 WIDE COMPLEX TACHYCARDIA IN ACUTE ANTI-NMDA RECEPTOR ENCEPHALITIS

NA NA NA A 20-year-old male with no known past medical history presented with two months of episodic right-sided weakness. An extensive workup was notable for a lumbar puncture that was positive for antibodies to the N-methyl-D-aspartate (NMDA) receptor, confirming anti-NMDA receptor (NMDAR) encephalitis. Baseline cardiac workup included a transthoracic echocardiogram (TTE) that showed left ventricular (LV) hypokinesis with borderline ejection fraction (EF 52%), and an ECG showing sinus tachycardia (Figure-A). While admitted, the patient developed shock, necessitating intubation and vasopressors. Serial ECGs while in shock showed sinus tachycardia, a prolonged QTc, and broad sail-like T wave in V1 (similar to type 1 Brugada pattern), with ST elevations in aVR and diffuse ST depressions inferolaterally (Figure-B). Serial troponins were minimally elevated (0.11 ug/L) and TTE showed no regional wall motion abnormalities, so invasive coronary angiography was not pursued. During progressive shock, the patient developed a wide complex tachycardia (WCT, Figure-C), that was thought to most likely be LV outflow tract ventricular tachycardia. The patient became hemodynamically unstable with the WCT, necessitating amiodarone, lidocaine, and ultimately five cardioversions. Repeat TTE showed severe biventricular dysfunction, and the patient needed veno-arterial extracorporeal membrane oxygenation (VA-ECMO). Ultimately, the patient’s biventricular function recovered, and he was able to be decannulated from VA-ECMO. He was managed with intravenous immunoglobulin, steroids and rituximab for his anti-NMDAR encephalitis. Cardiac arrhythmias are a known complication of anti-NMDAR encephalitis in up to one-third of patients. To the best of our knowledge, this is the first case of anti-NDMAR encephalitis associated with diffuse ST elevations suggestive of ischemia. Models suggest NMDA receptor-mediated signaling in the nervous system can alter the vagal outflow to the heart, leading to sinus brady/tachycardia. Other explanations for ECG changes in our patient include focal myocarditis/vasospasm. This is an unusual case of marked ST elevation and sustained VT in anti-NMDAR encephalitis. Pathogenesis and management of arrhythmias related to this condition warrant further study.