BACKGROUND:The clinical understanding of limbic encephalitis associated with antibodies against adenylate kinase 5 (AK5) remains limited. Misinterpretation of antibody test results may lead to diagnostic errors and inappropriate management. We aim to assess the frequency of anti-AK5 encephalitis overdiagnosis and identify common diagnostic pitfalls. METHODS:Cases of confirmed and mimicking anti-AK5 limbic encephalitis from January 2021 to July 2024 using established criteria for autoimmune encephalitis (AE) were reviewed. AK5 mimics were defined as patients initially suspected of AE with a positive AK5 autoantibody result, but who ultimately received an alternative final diagnosis. RESULTS:A total of 21 patients were included (57.1% female; median age 34 years; range 14-82). Only 3 patients (14%) were diagnosed with definite anti-AK5 limbic encephalitis, while 18 patients (86%) were classified as AK5 mimics. Serum autoantibodies were predominantly of the IgG3 subclass, with titers ranging from 1:10 to 1:100. The mimics included primary psychiatric disorders (22%), central nervous system (CNS) infections (22%), other inflammatory disorders (28%), epilepsy (16%), neurodegenerative diseases (6%) and metabolic encephalopathy (6%). The most frequent confounding factor in misdiagnosis was the presence of prominent psychiatric and behavioral symptoms, seen in 50% (9 of 18) of AK5 mimics. The second most common confounder was the presence of low serum antibody titers or isolated serum positivity without corresponding cerebrospinal fluid (CSF) findings (< 1:100), observed in 94% (17 of 18) of mimics. CONCLUSION:Mimics of anti-AK5 encephalitis are common and that misdiagnosis is often driven by non-specific symptoms and clinically irrelevant antibody results.

Objective To investigate mitochondrial functional alterations in T lymphocytes of patients with anti-N-methyl-D-aspartate receptor encephalitis(anti-NMDAR encephalitis,NMDAR-E)and their association with immune exhaustion.Methods Twenty-five patients with NMDAR-E diagnosed in the Department of Neurology,West China Hospital,Sichuan University,between January and March 2025(14 mild cases and 11 severe cases)and 16 healthy controls were enrolled.Flow cytometry was performed to characterize immune-cell distributions in paired peripheral blood and cerebrospinal fluid(CSF),and to evaluate the percentage of cells with low mitochondrial membrane potential(MMP-Low%),mitochondrial mass(MM),and PD-1 expression.Results In severe patients,the proportions of CD3+and CD4+T cells in CSF,the MMP-Low%of CD3+,CD4+,and CD8+T cells(P<0.01),and the MM of CD3+(P<0.01)and CD8+T cells(P<0.05)were all significantly higher than those in matched peripheral blood.Compared with healthy controls,the MMP-Low%of naïve and central memory CD4+and CD8+T-cell subsets in peripheral blood was significantly decreased(P<0.05),whereas the proportion of PD-1-positive cells was significantly increased in CD4+naïve and central memory T-cell subsets(P<0.01).Conclusion CSF T cells in patients with anti-NMDAR encephalitis display a state of dysfunctional mitochondrial accumulation,suggesting a possible dysregulation of mitochondrial mass homeostasis under the central inflammatory milieu.Mitochondrial features of peripheral T cells indicate the presence of systemic immune exhaustion.The MMP-Low%of CSF CD8+T cells and the peripheral blood lymphocyte percentage may serve as potential immunometabolic biomarkers for distinguishing disease severity in NMDAR-E.

BACKGROUND:Neurosyphilis can trigger neuro-specific immune responses, but evidence remains scarce. This study investigates the prevalence and clinical significance of neural antibodies in neurosyphilis, with a focus on CV2/CRMP5 antibodies. METHODS:We retrospectively analyzed 1149 patients with syphilitic neurological symptoms. Of 247 diagnosed with neurosyphilis, 215 met inclusion criteria. Using CBA and line blot, 97 patients suspected of autoimmune encephalitis were tested for neural antibodies, with positives confirmed by in-house CBA. Complete medical records were collected. Homology between CV2/CRMP5 and Treponema pallidum was assessed via BLAST and structural modeling. RESULTS:Twenty neurosyphilis patients were positive for neural antibodies. Antibodies against neuronal intracellular antigens (NIA-abs) predominated (15/20, 75%), with CV2/CRMP5 being the most frequent (73.3% of NIA-abs). Critically, all CV2/CRMP5-positive cases (11/11, 100%) had parenchymal neurosyphilis (p-NS), and none were found in 86 asymptomatic neurosyphilis (a-NS) patients. In the prospective validation cohort of 115 p-NS patients, 8 CV2/CRMP5-positive cases were identified. Combining retrospective and prospective data, the serum positivity rate of CV2/CRMP5 antibodies in p-NS was 9.79%. These patients exhibited significantly higher serum Toluidine Red Unheated Serum Test (TRUST) titers (median 1:64 vs. 1:16, P < 0.001) and elevated cerebrospinal fluid Immunoglobulin G (CSF IgG) indices (median 2.90 vs. 2.06, P = 0.005) compared to CV2/CRMP5-negative p-NS patients. No homology between CV2/CRMP5 and Treponema pallidum proteins was found. CONCLUSION:Our study identifies a strong association between CV2/CRMP5 antibodies and p-NS. Screening for these antibodies is recommended for p-NS patients, especially those with high serum TRUST titers and intrathecal immunoglobulin synthesis, as they may represent a subgroup with distinct immunopathological mechanisms.

Unexplained seizures represent a substantial diagnostic and therapeutic challenge in clinical neurology. Immune-mediated mechanisms coupled with prior infections are increasingly recognized as pivotal etiological contributors, yet large-scale epidemiological data from western China remain scarce. This retrospective cohort study enrolled 471 patients with unexplained seizures from eight provinces in western China between January 2017 and June 2022. Serum and cerebrospinal fluid samples were tested for neural-specific autoantibodies, thyroid peroxidase antibody (TPO-Ab), thyroglobulin antibody (TgAb), antinuclear antibody (ANA), and immunoglobulin M/G antibodies against Toxoplasma gondii, rubella virus, cytomegalovirus, and herpes simplex virus. Patients were stratified into definite autoimmune-associated seizures (AAS), probable AAS, and non-AAS subgroups. This trial was registered with the Chinese Clinical Trial Registry (ChiCTR1900027074) on October 30, 2019, and was retrospectively registered as the first participant was enrolled in January 2017. Overall, 14.65

Anti-N-methyl-D-aspartate receptor encephalitis (NMDAR-E) is a prevalent autoimmune neurological disorder, yet T cell-driven immunopathology and its contributions to therapeutic resistance and fulminant neuroinflammation remain poorly defined. Integrative single-cell multi-omic profiling, combining single-cell RNA sequencing, single-cell T cell receptor (TCR) sequencing, and cellular indexing of transcriptomes and epitopes by sequencing, is applied to systematically map peripheral and cerebrospinal fluid (CSF) immune landscapes in treatment-naïve NMDAR-E patients, with orthogonal validation conducted across independent cohorts via multiparameter flow cytometry and ex vivo NR1 peptide stimulation. Clonally expanded CX3CR1-expressing CD4+ and CD8+ T cells are identified as a core disease-associated effector population, characterized by high predicted affinity for the pathogenic GluN1 N368/G369 epitope, robust proinflammatory and cytotoxic transcriptional programs, and enhanced intercellular crosstalk with activated B cells through major histocompatibility complex (MHC), CD99, and macrophage migration inhibitory factor signaling axes. Preferentially enriched in patient CSF, these cells produce elevated interferon-γ (IFN-γ) and tumor necrosis factor-α (TNF-α) upon antigen-specific stimulation, collectively highlighting a disease-associated inflammatory T cell state linked to peripheral immune activation and CNS immune remodeling, with potential translational relevance for future biomarker and therapeutic investigation.

OBJECTIVE:To investigate the clinical characteristics, immunotherapeutic responses, and long-term outcomes of glutamic acid decarboxylase-65 (GAD65)-associated neurological syndromes, and to identify potential factors linked to poor outcomes. METHODS:We conducted a retrospective cohort study of patients diagnosed with anti-GAD65-associated neurological syndromes at West China Hospital from August 2019 to March 2024. The clinical characteristics, laboratory and imaging findings, response to immunotherapy and prognosis of the patients were systematically analyzed. RESULTS:37 patients were included in this study. Predominant clinical phenotypes included epilepsy (Ep) (n = 12, 32.4 %), stiff-person spectrum disorder (SPSD) (n = 8, 21.6 %), limbic encephalitis (LE) (n = 7, 18.9 %), cerebellar ataxia (CA) (n = 3, 8.1 %). Among them, 26 (70.3 %) were female, with a median age of onset at 39 years (IQR 30; range 9-65). Concomitant systemic autoimmune diseases were observed in 10 patients (27.0 %), 15 (40.0 %) were positive for coexisting autoantibodies. Anxiety disorders were documented in 11 patients (29.7 %). 35 patients (94.6 %) received immunotherapy, with symptom remission observed in 30 patients. The follow-up data over a median duration of 16 months (IQR 31.0; range 12.0-67.0), anxiety disorders, elevated cerebrospinal fluid (CSF) protein, delayed treatment and disease relapse are associated with poor outcomes. CONCLUSIONS:Neurological syndromes related to GAD65 antibodies exhibited marked clinical heterogeneity. Although most patients responded to immunotherapy, early intervention was critical for improving outcomes. Anxiety disorders, elevated CSF protein, treatment delay, and disease relapse were associated with adverse prognosis.

OBJECTIVE:Dentatorubral-pallidoluysian atrophy (DRPLA) is a rare, inherited neurodegenerative disorder caused by the expansion of cytosine-adenine-guanine repeats in ATN1. Most studies on DRPLA to date are limited to case reports. We aimed to provide a comprehensive summary of the clinical, genetic, biological, and magnetic resonance imaging characteristics of DRPLA using cross-sectional baseline data. METHODS:This is a cross-sectional observational cohort study. We used an extensive battery of assessments, included clinical phenotypes, genotypes, cognitive performance, biological markers, and magnetic resonance imaging characteristics. RESULTS:We enrolled 116 DRPLA patients, including 96 manifest patients and 20 prodromal patients. We identified a previously unreported ATN1 haplotype consisting of 8 single-nucleotide polymorphisms. Cognitive assessments revealed that 51 manifest patients (96%) and 4 prodromal patients (29%) scored <26 on the Montreal Cognitive Assessment. Manifest patients showed impairments across all cognitive domains, whereas prodromal patients showed deficits only in phonemic fluency. Biological analyses showed significantly elevated plasma neurofilament light levels in manifest patients compared with prodromal patients (P < 0.001) and healthy controls (P < 0.001). Magnetic resonance imaging findings revealed widespread gray matter loss across the whole brain in manifest patients, whereas prodromal patients showed gray matter loss localized to the bilateral cerebellar hemispheres. INTERPRETATION:This is the first DRPLA cohort study to comprehensively report clinical, genetic, cognitive, imaging, and plasma neurofilament light data. This study provides robust data to enhance our understanding of the overall features of DRPLA. We also propose clear definitions for the preclinical stage of DRPLA, and demonstrate the high diagnostic utility of plasma neurofilament light as a biomarker. ANN NEUROL 2025;98:750-761.

Case presentationIn the context of solid organ transplantation (SOT), patients are routinely administered immunosuppressive agents, rendering them highly vulnerable to opportunistic infections, particularly during the initial 1-12 month post-procedure.These infections are often attributed to a diverse array of pathogens, encompassing viruses, fungi, parasites, and bacteria 1 .Among the myriad of complications that can arise, respiratory infections and sepsis are the most frequently encountered, whereas central nervous system (CNS) infections, though less common, pose a substantial threat due to their association with poor prognostic outcomes and significant mortality rates among transplant recipients 2 .Epstein-Barr

BACKGROUND:N-methyl-d-aspartate receptor encephalitis (NMDAR-E) is a severe autoimmune disorder characterized by neuropsychiatric symptoms and immune dysregulation ，involves T-cell dysregulation, but specific T-cell subset roles remain unclear. This study analyzed peripheral blood T-cell subsets as biomarkers for monitoring and severity prediction. METHODS:Peripheral blood samples from 32 NMDAR-E patients, 31 antibody-mediated encephalitis, 26 viral encephalitis patients, and 23 healthy controls were analyzed using flow cytometry. Key markers of T-cell activation and co-stimulation were assessed. Clinical outcomes were correlated with immune profiles to develop a predictive model. RESULTS:NMDAR-E patients showed elevated CD4+ T-cell activation, with increased CD28, CD38, and HLA-DR expression versus controls, indicating immune hyperactivation with compensatory regulation. The T-cell-based model predicted severe cases with high accuracy (AUC = 0.91). CONCLUSION:CD4+ T-cell activation is central to NMDAR-E pathogenesis, highlighting diagnostic/therapeutic potential. Future studies must validate the model in larger cohorts and address peripheral blood analysis limitations.

Dystrophic epidermolysis bullosa pruriginosa (DEBP) is characterized by intense pruritus and lichenoid, nodular prurigo-like lesions. The intractable itching often decreases the life quality of DEBP patients. Current effective treatments for DEBP are limited. Here, we reported a Chinese recessive DEBP (RDEBP) patient who carried novel COL7A1 compound heterozygous mutations with digestive symptoms improved in pruritus and mucocutaneous findings after treatment with tofacitinib. A 27-year-old Chinese man had developed erythema, with mechanically induced blisters all over the body since birth. He also had intense itching from childhood. Odynophagia and dysphagia (grade I)1 were present at 7 years old. He visited the gastroenterology department due to worsening dysphagia (grade III) 1 year ago. Upper gastrointestinal endoscopy showed esophageal stenosis, and endoscopic esophageal dilatation was performed (Supporting Information Figure S1a–c). Dysphagia was relieved postoperatively (grade I). Mutation analysis revealed the patient carried compound heterozygous mutations in the COL7A1 gene: two splicing mutations, c.4980+2T>G (father was a heterozygous carrier) and c.4198-1G>A (mother was a heterozygous carrier) (Supporting Information Figure S2a,b). The patient was diagnosed as RDEBP. Five months ago, he came to our department again since itching was preventing him from falling asleep. His eating status had deteriorated (dysphagia of grade II) accompanied with obvious odynophagia. Nasopharyngoscopy showed a hypopharynx ulcer (Supporting Information Figure S3a,b). As the itching seriously affected him, we treated him with tofacitinib 5 mg twice daily. Within 4 weeks of therapy, the patient reported a marked improvement not only in pruritus, but also in skin lesions. During the subsequent 8 weeks, his condition continued to improve. On his latest visit at week 28 (March 2, 2023), he showed a stabilized general condition (Figure 1a–d). No erosions were observed on the esophagus (Supporting Information Figure S1d–f) or throat (Supporting Information Figure S3c,d), and no adverse events were reported during the treatment. RDEBP is a rare form of dystrophic epidermolysis bullosa (DEB) caused by COL7A1 gene mutation. The c.4980+2T>G and c.4198-1G>A identified in this patient affected splicing, which is predicted to cause frameshift results (Supporting Information Figure S4). Both variants were novel, absent in the gnomAD database (v2.1.1), and classified as pathogenic. The patient experienced significant relief of pruritus and also did not experience odynophagia (experienced two or three times a month before treatment) during the 28-week treatment period of tofacitinib. Taken together, Janus kinase inhibitors (JAKi)–tofacitinib may be an effective clinical management for RDEBP-related symptoms. Dysregulation of itch-related mediators interleukin 13 receptor subunit ajpha 1 IL13RA and interleukin 4 receptor, and upregulation of helper T cell (Th) 1, Th2 and Th17 cytokines, including interleukin (IL)-1b, -2, -6, tumor necrosis factor (TNF)-β, and interferon (IFN)-γ, have been reported in DEB patients.2-4 Tofacitinib could block out the downstream signal transduction for IL-2, -4, -6, -7, -9, -15, and IFN-γ, and thus may alleviate pruritus and inflammation of DEB patients. Moreover, JAKi could also inhibit the direct connection between pruritus-related factors like IL-31, TNF-α, and skin sensory neurons,5 and thereby also alleviate itching. Since there are no effective drugs for epidermolysis bullosa at present, the rapid development in small molecule drugs may provide an option for patients with epidermolysis bullosa to get maximum clinical benefits. More studies should be performed to verify this finding. This work was supported by the 1·3·5 project for disciplines of excellence, West China Hospital, Sichuan University (No. ZYJC18003) and the postdoctoral fund of Sichuan University (No. 2023SCU12065). None declared. Consent for the publication of recognizable patient photographs or other identifiable material was obtained by the authors and included at the time of article submission to the journal, stating that the patient gave consent with the understanding that this information may be publicly available. Figures S1–S4. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

Congenital nephrogenic diabetes insipidus (NDI) primarily arises from an X-linked recessive inheritance caused by mutations in the AVPR2 gene, which is responsible for approximately 90% of cases. This condition has an incidence rate of 4–8 per million male live births, with females being much less frequently affected. Symptoms typically manifest shortly after birth, predominantly in males. The key clinical features of NDI include excessive urination (polyuria), compensatory excessive thirst (polydipsia), cognitive impairment, consistently low urine specific gravity, dehydration, and imbalances in electrolyte levels. This case study highlights an unusual occurrence of NDI in a 50-year-old Chinese woman attributed to a mutation in the AVPR2 gene. For more than a year, she had been suffering from excessive urination and severe thirst. The patient, who had undergone surgery for cervical cancer, developed polyuria and hypernatremia postoperatively. Initial laboratory analyses revealed normal blood sodium and chloride levels but reduced urine osmolality and specific gravity. Imaging assessments revealed no irregularities. To validate the diagnosis of NDI, she participated in a water deprivation and vasopressin test. Subsequent genetic tests revealed a thymine (T) to adenine (A) mutation, leading to a missense mutation in the AVPR2 gene. As part of her treatment, she was placed on a low-sodium diet and prescribed oral hydrochlorothiazide and indomethacin for 1 month, resulting in a marked improvement in her symptoms. To the best of our knowledge, this is the first documented case of NDI diagnosed postoperatively in an older female patient with AVPR2 heterozygosity. This case highlights an unusual instance of an X-linked recessive clinical presentation of NDI in an elderly female patient. This study also underscores the importance of conducting water deprivation, vasopressin tests, and genetic testing in establishing the underlying cause for individuals diagnosed with NDI.

Lipid-accumulated reactive astrocytes (LARAs) have recently been confirmed to be a pivotal cell type present in temporal lobe epilepsy (TLE) lesions. These cells not only induce anomalous lipid accumulation within the epileptic foci but also decrease the seizure threshold by employing upregulated activation of the adenosine A2A receptor (A2AR). Furthermore, disturbances in mitochondrial oxidative phosphorylation (OxPhos) have been noted as significant drivers of lipid accumulation in astrocytes. Moreover, the deficiency of OxPhos in astrocytes can induce severe neuroinflammation, which can worsen the progression of TLE. Accordingly, further exploration of the correlation between mitochondrial dysfunction, LARAs-mediated lipid accumulation, and A2AR activation within epilepsy lesions is warranted. It could potentially elucidate the vital role of mitochondrial dysfunction in the pathogenesis of TLE.

OBJECTIVES:To investigate the immunopathogenic mechanisms of anti-N-methyl-D-aspartate receptor encephalitis (NMDAR-E) by characterizing the changes of immune cells in both peripheral blood (PB) and cerebrospinal fluid (CSF) of patients with NMDAR-E.METHODS:Cytology and flow cytometry were used to explore and compare different immunological parameters in PB and CSF of patients with NMDAR-E, viral encephalitis (VE) and healthy volunteers. Moreover, different models were established to assess the possibility of identifying NMDAR-E patients based on PB and CSF parameters.RESULTS:The neutrophil counts and monocyte-to-lymphocyte ratios (MLR) in PB are higher in NMDAR-E patients than in both VEs and controls (P < 0.001, respectively), while the percentages of CD3 + T, CD4 + T lymphocytes, and the leukocytes count in CSF were lower in NMDAR-Es than in VEs (P < 0.01, respectively). The higher percentages of CD8 + T cells in blood and CSF were both correlated with more severe NMDAR-E (P < 0.05, respectively). The poor neurological status group had significantly higher PB leukocytes but lower CSF leukocyte count (P < 0.05). Longitudinal observations in patients with NMDAR-E showed a decreasing trend of leukocyte count, neutrophils count, neutrophil-to-monocyte ratios (NMR), and neutrophil-to-lymphocyte ratios (NLR) with the gradual recovery of neurological function.CONCLUSIONS:The expression patterns of T lymphocyte subsets were different in patients with NMDAR-E and viral encephalitis. The changing trends of leukocyte and lymphocyte populations in peripheral blood and cerebrospinal fluid may provide clues for the diagnosis of different types of encephalitides, including NMDARE, and can be used as immunological markers to assess and predict the prognosis.

Neuro-COVID, a condition marked by persistent symptoms post-COVID-19 infection, notably affects various organs, with a particular focus on the central nervous system (CNS). Despite scant evidence of SARS-CoV-2 invasion in the CNS, the increasing incidence of Neuro-COVID cases indicates the onset of acute neurological symptoms early in infection. The Omicron variant, distinguished by heightened neurotropism, penetrates the CNS via the olfactory bulb. This direct invasion induces inflammation and neuronal damage, emphasizing the need for vigilance regarding potential neurological complications. Our multicenter study represents a groundbreaking revelation, documenting the definite presence of SARS-CoV-2 in the cerebrospinal fluid (CSF) of a significant proportion of Neuro-COVID patients. Furthermore, notable differences emerged between RNA-CSF-positive and negative patients, encompassing aspects such as blood-brain barrier integrity, extent of neuronal damage, and the activation status of inflammation. Despite inherent limitations, this research provides pivotal insights into the intricate interplay between SARS-CoV-2 and the CNS, underscoring the necessity for ongoing research to fully comprehend the virus's enduring effects on the CNS. The findings underscore the urgency of continuous investigation Neuro-COVID to unravel the complexities of this relationship, and pivotal in addressing the long-term consequences of COVID-19 on neurological health.

This study aims to explore the epidemiological characteristics of neuro-specific antibodies (ns-Ab) induced by different viral infections within the central nervous system (CNS). Additionally, it seeks to compare the autoimmune effects following several typical viral infections in CNS. We conduct a retrospective study to compare and analyze the prevalence trends of ns-Ab in patients with different viral infections. Additionally, evaluate the intensity of CNS inflammatory responses postviral infection by correlating clinical characteristics and laboratory findings, and briefly demonstrate the immune effects in CNS following various viral infections. This study retrospectively collected data from 1037 patients hospitalized with suspected CNS infections. A total of 654 patients (63.1%) were included in the final analysis. A higher proportion of patients with pathogens present in their cerebrospinal fluid (CSF) (114 out of 332, 34.3%) tested positive for ns-Ab compared to those without pathogens (70 out of 322, 21.7%) (p = 0.0004). Specifically, the screening rate for ns-Ab in patients with CNS viral infections (83 out of 165, 50.3%) and the prevalence of ns-Ab (27 out of 83, 32.5%) were significantly higher than in those with other pathogen infections (p < 0.0001 and p = 0.016, respectively). Among these, human herpesvirus 7 (HHV7) patients had the highest detection rate of ns-Ab during the disease course (11 out of 26, 42.3%), but exhibited infection characteristics distinctly different from those of herpes simplex virus 1 (HSV1). Viral infections significantly promote the development of autoimmune responses in CNS. The production of ns-Ab and the subsequent autoimmune response vary across different viral infections. There is a strong statistical correlation between HHV7 and the presence of ns-Ab, suggesting that HHV7 may serve as an early indicator of secondary autoimmune response following CNS infections.

BACKGROUND:The identification of patients with seizures of unknown etiology who would benefit from neural antibody testing necessitates effective assessment tools. The study aimed to compare the performance of the Antibody Prevalence in Epilepsy and Encephalopathy (APE2) score and the "Obvious" Indications for Neural Antibody Testing in Epilepsy or Seizures (ONES) checklist. We also intended to evaluate whether the performance of the tools varied by types of antibody.METHODS:Patients diagnosed with epilepsy, seizures, or status epilepticus of unknown etiology at West China Hospital from January 2019 to December 2021 were included. Paired serum/cerebrospinal fluid samples were analyzed for antineuronal and antiglial antibodies. The APE2 score and ONES checklist were applied, and their outcomes were compared to laboratory antibody test results. Possible false positive neuronal antibody results were excluded in sensitivity/specificity analysis reasonably.RESULTS:A total of 113 antibody-positive and 159 antibody-negative patients were enrolled in sensitivity/specificity analysis. The ONES checklist showed superior sensitivity than APE2 score (95.6 % vs.79.6 %, P < 0.001). Specificity was not statistically different (60.4 % vs. 57.9 %, P = 0.557). The negative predictive value (NPV) of ONES checklist was higher than that of APE2 score (94.8 % vs 80.7 %, P < 0.001). The positive predictive value of them was not statistically different (61.7 % vs 58.8 %, P = 0.557). APE2 score exhibited lower sensitivity for predicting LGI-Abs (52.9 % vs. 80.3 %, P = 0.022) compared to NMDAR-Abs. Similarly, ONES checklist showed lower sensitivity for LGI1-Abs than NMDAR-Abs (82.4 % vs. 100.0 %, P = 0.009).CONCLUSIONS:The ONES checklist demonstrates superior sensitivity for neural antibody positivity than APE2 score. Specificity of the two assessment tools was similar. ONES checklist performed better NPV than the APE2 score. Both assessment tools performed less well in predicting the presence of LGI1- Abs when compared to NMDAR-Abs.