anti-LGI1 Encephalitis Research Articles

Study of B Cell Repertoire in Patients With Anti-N-Methyl-D-Aspartate Receptor Encephalitis.

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the most common antibody-mediated encephalitis. There are several studies on B cell repertoire of anti-NMDAR encephalitis in Caucasians. Here, the cerebrospinal fluid (CSF) samples of 12 Chinese patients with first-episode anti-NMDAR encephalitis were collected to investigate the B cell receptor (BCR) binding to NMDAR by single cell amplification of BCR and Sanger sequencing. BCR data of healthy persons, and of patients with anti-leucine-rich glioma inactivated 1 (anti-LGI1) encephalitis, multiple sclerosis (MS), and neuromyelitis optica spectrum disorder (NMOSD) from the public databases were used as control. A heavy chain common clone IGHV1-18*04,IGHD1-26*01/ IGHD2-2*03/IGHD2-8*01, IGHJ3*02_(CDR3) ARVGSKYGFETFDI was found in 11 of 12 enrolled patients but not in the comparison data set. In addition, 4 shared clonotypes were found among these patients, and three of them contained the common clone. This study also revealed that the antibody gene family usage preference between patients and healthy controls were different, while they had similar antibody mutation rate. Our findings may have potential clinical implications for the diagnosis of anti-NMDAR encephalitis.

Clinical features and outcomes in relapsing and monophasic patients with anti-leucine-rich glioma-inactivated 1 encephalitis

Objective: To analyze the differences of clinical characteristics and outcomes between relapsing and monophasic patients with anti-leucine-rich glioma-inactivated 1 (anti-LGI1) encephalitis. Methods: Medical records of confirmed anti-LGI1 encephalitic patients who underwent immunotherapy were retrospectively collected from January 2015 to January 2019 in the first affiliated hospital of Zhengzhou University. Clinical data, treatment methods, duration of treatment and outcomes were analyzed between the relapsing and monophasic groups. Results: Among the 33 anti-LGI1 encephalitic patients, there were 12 and 21 cases in the relapsing and monophasic groups, respectively. No difference was found in age, sex, precipitating factors, intensive care unit (ICU) admission, symptoms and modified Rankin Scale (mRS) score in the acute phase (P>0.05). As to the lab test and image examination, no statistic difference was found in serum and cerebral spinal fluid (CSF) positive rate, hyponatremia, abnormal rate of electrocardiogram (ECG), electroencephalogram (EEG), CSF and magnetic resonance imaging (MRI) and lesion locations (P>0.05). No difference was found in time to diagnose the disease between the 2 groups (P>0.05). The median immunotherapy period was 102.5 days in relapsing group and 194.0 days in monophasic group, with a statistic difference (P=0.001). No patients had bad outcomes in the monophasic group at the last follow-up, while 6 patients had poor outcomes in the relapsing group (4 patients died). The patients in relapsing group had a worse prognosis compared to those in the monophasic group (P=0.007). Conclusions: Relapse is common in anti-LGI1 encephalitis. Patients in the relapsing group received a shorter term of immunotherapy and had worse outcomes than those in the monophasic group.

Polyradiculitis in autoimmune encephalitis: a case report and review

BackgroundLimbic encephalitis is a subacute progressive disorder characterized by disturbances in memory and behavior along with seizures. Antibodies against leucine-rich glioma-inactivated 1 (LGI1) are associated with a subtype of encephalitis which along with the abovementioned symptoms is also characterized by severe pain and autonomic dysfunction. The classical radiological presentation of LGI1 encephalitis is that of amygdala and hippocampal enlargement unilaterally or bilaterally with a T2 hyperintensity. Extratemporal involvement is considered a rare feature.Case descriptionWe present the only known case in our knowledge of anti-LGI1 encephalitis in a 47-year-old male presenting as dorsal root ganglia and spinal nerve enhancement on imaging.DiscussionClinicians should be aware of this atypical presentation and consider anti-LGI1 encephalitis as a possible diagnosis when presented with such a neuroradiological feature.

Novel findings of HLA association with anti-LGI1 encephalitis: HLA-DRB1*03:01 and HLA-DQB1*02:01

This study was to investigate whether autoimmune encephalitis is associated with the human leukocyte antigen (HLA) genotypes in Chinese Han population. We compared and analyzed the HLA genotypes of 101 patients with autoimmune encephalitis (77 anti-NMDAR, 11 anti-LGI1 and 13 anti-GABABR antibody, respectively) to the 200 healthy control groups. Our results showed that the DRB1*03:01 or DQB1*02:01 allele and the extended DRB1*03:01 ~ DQB1*02:01 haplotype represented the strong susceptibility locus for anti-LGI1 encephalitis (OR = 18.84, 95% CI = 5.01–70.89, Pc = 0.004; OR = 18.84, 95% CI = 5.01–70.89, Pc = 0.004; OR = 18.84, 95% CI = 5.01–70.89, Pc = 0.001). Additionally, the DRB1*08:03 ~ DQB1*06:01 or B*08:01 ~ C*07:02 haplotype was likely to be associated with anti-LGI1 encephalitis (OR = 10.23, 95% CI = 2.87–36.42, Pc = 0.039; OR = 74.62, 95% CI = 6.97–799.06, Pc = 0.043). No statistically significant differences were found for HLA association between patients with anti-NMDAR or anti-GABABR encephalitis and healthy controls. These results indicated that HLA subtypes were only associated with anti-LGI1 encephalitis.

Functional and Structural Brain Alterations in Encephalitis With LGI1 Antibodies.

Objective: The purpose of this study was to examine the neural substrates and mechanisms that generate memory deficits, seizures and neuropsychiatric abnormalities in encephalitis with LGI1 antibodies using a data-driven, multimodal magnetic resonance imaging (MRI) approach.Methods: Functional MRI data were acquired from 14 anti-LGI1 encephalitis patients and 14 age and gender matched normal controls. Independent component analysis with hierarchical partner matching (HPM-ICA) was used to assess the whole-brain intrinsic functional connectivity. Granger causality (GC) was applied to investigate the effective connectivity among the brain regions that identified by HPM-ICA. Diffusion tensor imaging (DTI) was utilized to investigate white matter microstructural changes of the patients.Results: Participants with LGI1 antibodies encephalitis presented reduced functional connectivity in the brain areas associated with memory, cognition and motion circuits, while increased functional connectivity in putamen and caudate in comparison to the normal controls. Moreover, the effective connectivity in patients was decreased from the frontal cortex to supplementary motor area. Finally, patients had significant reductions in fractional anisotropy (FA) for the corpus callosum, internal capsule, corona radiata and superior longitudinal fasciculus, accompanied by increases in mean diffusivity (MD) for these regions as compared to controls.Conclusion: Our findings suggest that the neural disorder and behavioral deficits of anti-LGI1 encephalitis may be associated with extensive changes in brain connectivity and microstructure. These pathological alterations affect the basal ganglia and limbic system besides the temporal and frontal lobe.

The exploration of the spectrum of motor manifestations of anti-LGI1 encephalitis beyond FBDS

PurposeThe purpose of this study was to characterize the spectrum of motor events in patients with acute anti-leucine-rich glioma-inactivated protein 1 (anti-LGI1) encephalitis through video-electroencephalogram (VEEG) recordings. MethodWe collected data retrospectively from 16 patients diagnosed with anti-LGI1 encephalitis who had completed VEEG recording during hospitalization. ResultsVEEG monitoring lasted a median of 11.0 h (range 4.5∼20). Fourteen types of seizures were recorded in 9 patients (56.3 %). Eight of the 14 types of seizures demonstrated typical ictal EEG evolution (including 2 subclinical seizures), 3/14 demonstrated EEG electrodecremental events (EDE) at onset but without further evolution, and 3/14 could be only judged by analyzing semiology. FBDS was recorded in 6 patients (37.5 %), and all these attacks were followed by epileptic seizures. Simple hyperkinetic movements (HMs), such as jerk-like or twisting movements, were found in 8 (50 %) patients, and 6 of them had complex HMs, such as manipulating movements or mimics of daily activities, during sleep. Conclusions1. Atypical seizures, for instance, seizures without EEG evolution, are not rare but likely to be overlooked. 2. FBDS is closely linked with epileptic seizures, revealing FBDS to be a part of epileptic attacks. 3. HMs could expand the spectrum of motor manifestations, overlapping with sleep disorders. 4. The high prevalence of these motor events might be due to the disrupted cortical-subcortical network, which is critical in motor control and sleep.

Transdiagnostic hippocampal damage patterns in neuroimmunological disorders

Hippocampal damage and associated cognitive deficits are frequently observed in neuroimmunological disorders, but comparative analyses to identify shared hippocampal damage patterns are missing. Here, we adopted a transdiagnostic analytical approach and investigated hippocampal shape deformations and associated cognitive deficits in four neuroimmunological diseases.We studied 120 patients (n = 30 in each group), including patients with multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), anti-NMDAR and anti-LGI1 encephalitis. A control group was matched to each patient sample from a pool of 79 healthy participants. We performed an MRI-based vertex-wise hippocampal shape analysis, extracted hippocampal volume estimates and scalar projection values as a measure of surface displacement. Cognitive testing included assessment of verbal memory and semantic fluency performance.Our cross-sectional analyses revealed characteristic patterns of bilateral inward deformations covering up to 32% of the hippocampal surface in MS, anti-NMDAR encephalitis, and anti-LGI1 encephalitis, whereas NMOSD patients showed no deformations compared to controls. Significant inversions were noted mainly on the hippocampal head, were accompanied by volume loss, and correlated with semantic fluency scores and verbal episodic memory in autoimmune encephalitis and MS. A deformation overlap analysis across disorders revealed a convergence zone on the left anterior hippocampus that corresponds to the CA1 subfield.This convergence zone indicates a shared downstream substrate of immune-mediated damage that appears to be particularly vulnerable to neuroinflammatory processes. Our transdiagnostic morphological view sheds light on mutual pathophysiologic pathways of cognitive deficits in neuroimmunological diseases and stimulates further research into the mechanisms of increased susceptibility of the hippocampus to autoimmunity.

Telemedicine assessment of long-term cognitive and functional status in anti-leucine-rich, glioma-inactivated 1 encephalitis.

ObjectiveTo assess the feasibility of a structured telephone interview examining the long-term cognitive and functional status in anti–leucine-rich, glioma-inactivated 1 (LGI1) encephalitis.MethodsTelephone interviews were conducted with 37 patients after a median follow-up of 87 months from disease onset and 23 healthy controls matched for age and sex. Cognitive status was assessed with the telephone Mini-Mental State Examination (t-MMSE) and 3 tests exploring verbal memory, fluency, and executive function. Functional status was evaluated with the Functional Activities Questionnaire and the modified Rankin Scale (mRS). Patients were classified as normal, with mild cognitive impairment (MCI), or with dementia based on cognitive and functional status. Assessment of the cognitive reserve was performed with a structured questionnaire. Logistic regression analysis was applied to identify predictors of cognitive impairment.ResultsTelephone interviews were successful in 36/37 (97%) patients. Cognitive impairment was detected in 27 (75%) including 17 with MCI and 10 with dementia. Eight (29%) patients would have been misclassified using only the t-MMSE. Twenty-six (72%) patients were functionally independent according to the mRS, but only 9 (35%) were cognitively normal. Independent predictors for long-term cognitive impairment were a low cognitive reserve (OR = 1.36, 95% CI: 1.05–1.76; p = 0.02) and bilateral hippocampal hyperintensity at initial MRI (OR = 27.03, 95% CI: 1.87–390; p = 0.02).ConclusionsTelemedicine is a feasible tool to assess the cognitive and functional outcome in patients with anti-LGI1 encephalitis. Cognitive impairment is often missed if only functional scales are used. Premorbid cognitive reserve and MRI with bilateral hippocampal hyperintensity were predictors for long-term cognitive impairment.

Autoimmune encephalitis.

Autoimmune encephalitis is emerging as an important and relatively common cause of encephalitis in the developed world. Crucially, early recognition and prompt initiation of a range of immunotherapies is likely to improve the outcomes of patients with autoimmune encephalitis, particularly for those with identifiable antibodies against neuronal cell surface proteins. There are a rapidly growing number of specific autoantibodies and associated syndromes, but many of these remain very rare. The majority of cases comprise anti-N-methyl-D-aspartate (NMDA) receptor encephalitis or anti-leucine-rich glioma-inactivated protein 1 (LGI1) encephalitis with the remaining cases a mixture of over 10 other specific antibodies or being seronegative. The core anti-NMDA encephalitis phenotype is a distinct symptom complex involving psychiatric and neurological features and anti-LGI1 encephalitis presents with cognitive changes and distinct seizure types. Diagnosis can be delayed owing to limited access to specialised laboratory testing or in cases with atypical or limited features.

Coexistence of Autoimmune Encephalitis and Other Systemic Autoimmune Diseases.

Background: In recent years, the phenomenon of coexisting systemic autoimmune diseases (ADs) in patients with autoimmune encephalitis (AE) has been increasingly found, while its clinical significance remains unexplored. This study aimed to investigate the types and potential clinical associations of autoimmune comorbidities in patients with antibody-positive AE.Methods: A retrospective cohort study of patients with antibody-positive AE was conducted from 2011 to 2018. The demographics, clinical characteristics, and follow-up data were reviewed.Results: We enrolled 517 patients, among whom 45 were affected by one or more types of ADs, including Hashimoto's thyroiditis (HT) (n = 28), systemic lupus erythematosus (SLE) (n = 3), anaphylactoid purpura (n = 3), vitiligo (n = 3), Sjögren's syndrome (SS) (n = 2), chronic urticaria (n = 2), bullous pemphigoid (n = 1), uveitis (n = 1), myasthenia gravis (MG) (n = 1), and the coexistence of SLE and anaphylactoid purpura (n = 1). The proportion of patients with coexisting ADs was higher in those with anti–leucine-rich glioma-inactivated 1 (LGI1) encephalitis than in those with anti–N-methyl-d-aspartate receptor (NMDAR) encephalitis (13/111 vs. 16/307) (P = 0.021). In anti-NMDAR and anti-LGI1 encephalitis patients, there were no significant differences in the age at onset, sex ratio, proportion of patients with tumors, disease severity, or recurrence between the groups with and without ADs.Conclusions: One or more types of ADs developed in AE patients, and patients with anti-LGI1 encephalitis had a higher frequency of autoimmune comorbidities than those with anti-NMDAR encephalitis. And we found that autoimmune comorbidities did not affect the clinical course of AE.

CXCL13 Is A Biomarker Of Anti-Leucine-Rich Glioma-Inactivated Protein 1 Encephalitis Patients.

BackgroundAlthough antibody-mediated immune responses are considered pathogenic and responsible for neural injury in anti-leucine-rich glioma-inactivated protein 1 (anti-LGI1) encephalitis, previous studies have indicated that cytokines and chemokines might play roles in the pathogenic process by serving as B cell enhancers. In this study, we detected the profiles of cytokines and chemokines in the cerebral fluid (CSF) and serum of patients with anti-LGI1 encephalitis to identify potential biomarkers.MethodsSixteen patients diagnosed with anti-LGI1 encephalitis and nine patients diagnosed with noninflammatory neurologic disorders were included in the study. Cytokines and chemokines including IL-6, IL-10, IL-17, CXCL12, CXCL13, BAFF and HMGB1 in serum and CSF were measured.ResultsThe serum and CSF levels of CXCL13 were significantly higher in patients with anti-LGI1 encephalitis (36.32±34.71 pg/mL and 2.23±2.41 pg/mL, respectively) than in controls (10.84±5.02 pg/mL and 0.34±0.21 pg/mL, respectively). There was no significant difference in serum or CSF levels of IL-6, IL-10, IL-17, CXCL12, BAFF and HMGB1 between the two groups.ConclusionCXCL13 is a potential biomarker of active inflammation in anti-LGI1 encephalitis. The distinctive response of cytokines and chemokines might be closely linked to the mechanisms underlying this condition.

Pediatric Autoimmune Encephalitis: Case Series From Two Chinese Tertiary Pediatric Neurology Centers

Background and purpose: We retrospectively analyzed the clinical characteristics of children with autoimmune encephalitis (AE) in two Chinese tertiary pediatric neurology centers. We also compared anti-NMDAR encephalitis with and without co-positive MOG antibody, as well as specific autoantibody-positive AE and autoantibody-negative but probable AE.Methods: A retrospective study of children (0–18 years old) with AE in Peking University First Hospital and Children's Hospital Affiliated to Capital Institute of Pediatrics was carried out from May 2012 to January 2017. Demographics, clinical features, laboratory, and imaging findings, outcome, and co-positivity with MOG antibody were analyzed.Results: A total of 103 children had AE, 89 (86.4%) had anti-NMDAR encephalitis, 2 (1.9%) had anti-LGI1 encephalitis, 1 (0.9%) had anti-CASPR2 encephalitis, and 11 (10.7%) were diagnosed as autoantibody-negative but probable AE. Among the 89 children with anti-NMDAR encephalitis, 35 were males and 54 were females. The follow-up time was 1–3 years. A total of 15 cases (15/89, 16.9%) with anti-NMDAR encephalitis had co-positive MOG antibody (serum or cerebrospinal fluid or both). These patients were more likely to experience relapse later in life (P = 0.014). We had two cases with anti-LGI1 encephalitis, that is, one with sleep disorder onset, and the other one with seizure onset, both of whom recovered after treatment. One case with anti-CASPR2 encephalitis was treated with an antiepileptic drug and fully recovered. There were 11 cases diagnosed as autoantibody-negative but probable AE who had relatively poorer outcome than those with autoantibody-positive AE (15.2%, 14/89). However, the difference was not significant (P = 0.08). Only one 12-year-old girl with NMDAR-antibody AE had ovarian teratoma.Conclusion: Most subjects with AE in our Chinese cohort had anti-NMDAR AE, which had relatively good prognosis. Children with anti-LGI1 or anti-CASPR2 encephalitis were rare and showed good response on immunotherapy. Co-positive MOG antibody was relatively common in anti-NMDAR encephalitis, which was related to high relapse rate. In our study, the prognosis of autoantibody-negative but probable AE seemed worse than that of specific autoantibody-positive AE.

Clinical features and long-term outcomes of seizures associated with autoimmune encephalitis: A follow-up study in East China

PurposeTo evaluate the clinical characteristics, treatment outcomes, prognosis and potential risk factors of patients in East China with seizure secondary to autoimmune encephalitis. MethodsFrom February 2014 to June 2016, 113 patients diagnosed with autoimmune encephalitis in Huashan Hospital, Fudan University, were enrolled in our study. After at least two years of follow-up, we retrospectively analyzed the patients’ clinical details, electroencephalograph performance, brain MRI findings, and the therapeutic outcome. Patients underwent clinical evaluation every 3 months. We compared the clinical characteristics and epileptic prognosis of autoimmune encephalitis per antibody type. The association of the epileptic prognosis and EEG abnormalities was evaluated. GTE (Grand Total EEG) Score was used to evaluate EEG abnormalities. Statistic methods included ANOVA, Bonferroni correction test. ResultsTreatment outcomes were assessabled in 103 patients (10 patients died or withdrew), including anti-GABABR encephalitis (11), anti-LGI1 encephalitis (16), anti-NMDAR encephalitis (73), Caspr2 antibody encephalitis (3). 83 patients had seizures, who underwent both immunotherapy and anti-epileptic drugs therapy. In terms of seizure type, 57 (68.7%) patients exhibited focal to bilateral tonic-clonic seizure (FBTCS), 51 (61.4%) patients exhibited focal-impaired awareness seizure (FIAS) or focal aware seizure (FAS). 18 (21.7%) patients developed to status epilepticus. 30 (36%) patients had multiple types of seizures. 39 (47%) patients had daily seizures. 80.7% (67/83) of patients with epilepsy had seizure remission. During the 24 months of follow-up, 11 (11%) patients had clinical relapses. GTE scores were significantly different between the group with seizure reduction < 75% and the group with seizure remission (p = 0.009). Imaging abnormalities existed in 53% of the patients in our cohort, but lacked specificity during the acute phase. ConclusionAutoimmune encephalitis (AE) presents with large seizure burden with differing seizure semiology among different antibody types. Except for anti-GABAb receptor encephalitis, it may not be necessary for other AE types to apply long-term use of anti-epileptic drugs (AEDs). The GTE Score can be used to evaluate the EEG abnormalities and may be a predictor of seizure outcomes. MRI findings during the acute phase are non-specific. Long-term follow-up MRIs may be much more meaningful in evaluating prognosis.

Core Cerebrospinal Fluid Biomarker Profile in Anti-Leucine Rich Glioma Inactivated 1 (Anti-LGI1) Encephalitis

Core Cerebrospinal Fluid Biomarker Profile in Anti-Leucine Rich Glioma Inactivated 1 (Anti-LGI1) Encephalitis

Anti-LGI1 Encephalitis: A Case Report of a Recently Described Disease Entity

Anti-LGI1 Encephalitis: A Case Report of a Recently Described Disease Entity

Immune-mediated encephalitis for the infectious disease specialist.

Autoimmune encephalitis is increasingly recognized and must be distinguished from infectious forms of encephalitis. Moreover, physicians should be aware of infectious triggers of autoimmune encephalitis and of infectious complications associated with treatment. Recent epidemiological studies suggest that the incidence of autoimmune encephalitis may rival that of infectious encephalitis. Although distinguishing autoimmune from infectious forms of encephalitis on clinical grounds can be challenging, recently proposed diagnostic criteria can provide some assistance. There has been an explosion in our knowledge of autoimmune encephalitis associated with antibodies to neuronal cell surface antigens, and two of the most common forms, anti-NMDA receptor encephalitis and anti-LGI1 encephalitis, are typically associated with distinctive clinical features. Although tumors have long been known to trigger autoimmune encephalitis, it has been recently recognized that herpes simplex encephalitis may trigger the generation of antineuronal autoantibodies resulting in an autoimmune neurologic relapse. Both first and second-line therapies for autoimmune encephalitis are associated with infectious complications, whereas emerging treatments, including anakinra and tocilizumab, may also result in increased susceptibility to certain infections. The diagnosis and management of autoimmune encephalitis is complex, and awareness of diagnostic criteria and modalities, typical clinical syndromes, infectious triggers of disease, and infectious complications of therapies is critical in optimizing care for affected patients.

Evaluation of seizure treatment in anti-LGI1, anti-NMDAR, and anti-GABABR encephalitis.

ObjectiveThis nationwide cohort study evaluates seizure responses to immunotherapy and antiepileptic drugs (AEDs) in patients with anti-leucine-rich glioma-inactivated 1 (LGI1), anti-NMDA receptor (NMDAR), and anti-gamma-aminobutyric-acid B receptor (GABABR) encephalitis.MethodsAnti-LGI1, anti-NMDAR, and anti-GABABR encephalitis patients with new-onset seizures were included. Medical information about disease course, AEDs and immunotherapies used, effects, and side effects were collected. Outcome measures were (1) seizure freedom while using AEDs or immunotherapy, (2) days to seizure freedom from start of AEDs or immunotherapy, and (3) side effects.ResultsOf 153 patients with autoimmune encephalitis (AIE) (53 LGI1, 75 NMDAR, 25 GABABR), 72% (n = 110) had epileptic seizures, and 89% reached seizure freedom. At least 53% achieved seizure freedom shortly after immunotherapy, and 14% achieved seizure freedom while using only AEDs (p < 0.0001). This effect was similar in all types (p = 0.0001; p = 0.0005; p = 0.013, respectively). Median time to seizure freedom from AEDs start was 59 days (interquartile range [IQR] 27–160), and 28 days from start of immunotherapy (IQR 9–71, p < 0.0001). Side effects were psychotic behavior and suicidal thoughts by the use of levetiracetam, and rash by the use of carbamazepine. Carbamazepine was more effective than levetiracetam in reducing seizures in anti-LGI1 encephalitis (p = 0.031). Only 1 patient, of 86 surviving patients, developed epilepsy after resolved encephalitis.ConclusionEpilepsy after resolved encephalitis was rare in our cohort of patients with AIE treated with immunotherapy. In addition, seizure freedom is achieved faster and more frequently after immunotherapy. Therefore, AEDs should be considered as add-on treatment, and similar to treatment of other encephalitis symptoms, immunotherapy is crucial.

Evaluation of seizure treatment in anti-LGI1, anti-NMDAR, and anti-GABABbR encephalitis (S11.008)

Evaluation of seizure treatment in anti-LGI1, anti-NMDAR, and anti-GABABbR encephalitis (S11.008)

Clinical characteristics and factors associated with short-term prognosis in adult patients with autoimmune encephalitis of non-neoplastic etiology.

Reports that autoimmune encephalitis (AE) is associated with antibodies have increased; however, little is known about the distribution of clinical symptoms, imaging changes, and prognostic factors in patients with AE of non-neoplastic etiology. Accordingly, we evaluated the clinical characteristics and factors associated with short-term prognosis. From January 2016 to June 2018, 31 adult patients were diagnosed with AE of non-neoplastic etiology at Shengjing Hospital of China Medical University and their demographic and clinical characteristics were abstracted. Factors affecting disease severity and predictors of prognosis were analyzed. Among 31 patients, 19 had anti-NMDAR, 5 had anti-GABABR, and 7 had anti-LGI1 antibody encephalitis. Status epilepticus, ataxia, and cognitive dysfunction were the most common neurological symptoms. Deep white matter (DWM) abnormalities were the most common changes observed on MRI. Logistic regression analysis indicated that conscious disturbance (odds ratio = 11.67, 95%, confidence interval 2.13-64.04; p = 0.005) is an independent factor associated with poor prognosis in AE. The clinical manifestations of AE are diverse; status epilepticus, ataxia, and cognitive dysfunction are most common. The DWM of the brain, rather than the limbic lobe system, was most prone to MR signal abnormalities. Conscious disturbance may be an important predictor of poor short-term prognosis in patients with AE of non-neoplastic etiology.

Increased adverse events associated with antiepileptic drugs in anti-leucine-rich glioma-inactivated protein 1 encephalitis.

Anti-leucine-rich glioma-inactivated protein 1 (LGI1) encephalitis is a rare autoimmune condition presenting mainly as altered mental state, cognitive dysfunction, and seizure. Antiepileptic drugs (AEDs) are usually initiated to control seizures despite their limited efficacy; however, accumulating clinical experience suggests a high incidence of adverse reactions to AEDs in anti-LGI1 encephalitis. We reviewed the medical records of patients who were diagnosed with anti-LGI1 encephalitis to analyze the adverse effects of AEDs in these patients. Among the 20 patients who were treated with AEDs, 10 (50%) changed their AEDs due to adverse cutaneous drug reaction. Eight of them presented with maculopapular eruption, one with drug rash with eosinophilia and systemic symptoms syndrome, and one with eczema. Causative agents mostly consisted of aromatic AEDs. Oxcarbazepine was discontinued in two additional patients due to hyponatremia. Six patients (30%) discontinued their dose of levetiracetam because of psychiatric manifestations including irritability/aggressive behavior (four patients), insomnia (one patient), and depressive mood (one patient). Clinicians should consider adverse cutaneous drug reaction, psychiatric adverse events, and hyponatremia when selecting AEDs for the treatment of anti-LGI1 encephalitis.