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\"When twenty-four-year-old Susannah Cahalan woke up alone in a hospital room, strapped to her bed and unable to move or speak, she had no memory of how she'd gotten there. Days earlier, she had been on the threshold of a new, adult life: at the beginning of her first serious relationship and a promising career at a major New York newspaper. Now she was labeled violent, psychotic, a flight risk. What happened? In a swift and breathtaking narrative, [the author] tells the astonishing true story of her descent into madness, her family's inspiring faith in her, and the lifesaving diagnosis that nearly didnt happen\"--Amazon.com.

The rapidly expanding spectrum of autoimmune encephalitis in the last fifteen years is largely due to ongoing discovery of many neuronal autoantibodies. The diagnosis of autoimmune encephalitis can be challenging due to the wide spectrum of clinical presentations, prevalence of psychiatric features that mimic primary psychiatric illnesses, frequent absence of diagnostic abnormalities on conventional brain MR-imaging, non-specific findings on EEG testing, and the lack of identified IgG class neuronal autoantibodies in blood or CSF in a subgroup of patients. Early recognition and treatment are paramount to improve outcomes and achieve complete recovery from these debilitating, occasionally life threatening, disorders. This review is aimed to provide primary care physicians and hospitalists who, together with neurologist and psychiatrists, are often the first port of call for individuals presenting with new-onset neuropsychiatric symptoms, with up-to-date data and evidence-based approach to the diagnosis and management of individuals with neuropsychiatric disorders of suspected autoimmune origin.

Anti-leucine rich glioma inactivated 1 (LGI1) autoimmune encephalitis (AE) is characterized by cognitive impairment or rapid progressive dementia, psychiatric disorders, faciobrachial dystonic seizures (FBDS) and refractory hyponatremia. Since December 2020, millions of people worldwide have been vaccinated against COVID-19. Several soft neurological symptoms like pain, headache, dizziness, or muscle spasms are common and self-limited adverse effects after receiving the COVID-19 vaccine. However, several major neurological complications, despite the unproven causality, have been reported since the introduction of the COVID-19 vaccine. Herein, we describe a 48 years old man presenting with rapidly progressive cognitive decline and hyponatremia diagnosed with anti LGI1 AE, occurring shortly after the second dose of mRNA COVID -19 vaccine and possibly representing a severe adverse event related to the vaccination. Response to high dose steroid therapy was favorable. As millions of people worldwide are currently receiving COVID-19 vaccinations, this case should serve to increase the awareness for possible rare autoimmune reactions following this novel vaccination in general, and particularly of anti-LGI1 AE.

BackgroundThe neutrophil-to-lymphocyte ratio (NLR) and monocyte-to-lymphocyte ratio (MLR) are biomarkers that may reflect inflammatory status in some immune-related diseases. This study aims to investigate the association of NLR and MLR with the severity and prognosis of autoimmune encephalitis (AE).MethodsA total of 199 patients diagnosed with AE in the First Affiliated Hospital of Zhengzhou University from October 2015 to October 2021 were retrospectively analyzed. The Clinical Assessment Scale for Autoimmune Encephalitis (CASE) and the modified Rankin Scale (mRS) were used to evaluate the severity of the patients at admission, and the patients were divided into mild group (CASE ≤ 4) and severe group (CASE ≥ 5) according to the CASE score. Poor prognosis was described as an mRS of 3 or more at 12 months. Binary logistic regression analysis was performed to assess risk factors for the severity and prognosis of AE.ResultsNLR and MLR of severe group were significantly higher than that of mild group. NLR and MLR were positively correlated with the CASE score ( r = 0.659, P < 0.001; r = 0.533, P < 0.001) and the mRS score ( r = 0.609, P < 0.001; r = 0.478, P < 0.001) in AE patients. Multivariate logistic analysis showed that NLR (OR = 1.475, 95%CI: 1.211-1.796, P < 0.001) and MLR (OR = 15.228, 95%CI: 1.654-140.232, P = 0.016) were independent risk factors for the severity of AE. In addition, the CASE score and the mRS score were positively correlated ( r = 0.849, P < 0.001). Multivariate logistic analysis showed that the CASE at admission (OR = 1.133, 95%CI: 1.043-1.229, P = 0.003) and age (OR = 1.105, 95%CI: 1.062-1.150, P < 0.001) were independent risk factors for the poor prognosis of AE patients. The NLR and MLR at admission and whether they decreased after immunotherapy were not associated with the prognosis of AE patients ( P > 0.05).ConclusionsNLR and MLR, readily available and widespread inflammatory markers, were helpful for clinicians to monitor disease progression and identify potentially severe patients of AE early to optimize clinical treatment decisions.

Background and objectives Antibody-negative autoimmune encephalitis (AE) is a form of encephalitis characterized by the absence of detectable autoimmune antibodies, despite immunological evidence. However, data on management of patients with antibody-negative AE in the intensive care unit (ICU) are limited. This study aimed to explore the characteristics and subtypes of antibody-negative AE, assess the effects of immunotherapy, and identify factors independently associated with poor functional outcomes in patients requiring intensive care. Methods This retrospective, single-center study analyzed consecutive adult patients diagnosed with antibody-negative AE and admitted to the ICU of a large tertiary hospital between 2019 and 2023. Multivariate regression analysis was used to identify factors linked to poor functional outcomes six months after ICU admission, as defined by a modified Rankin Scale score of 3–6. Generalized linear mixed models were applied to evaluate the effect of immunotherapy on longitudinal changes in the Clinical Assessment Scale in Autoimmune Encephalitis and modified Rankin Scale scores. Results Of the 1220 patients with severe encephalitis admitted to the ICU, 107 were diagnosed with antibody-negative AE and included in the analysis. Six months after ICU admission, 67 patients (62.6%) had poor functional outcomes, including 28 deaths (26.2%). Factors independently associated with poor outcomes were high-dose corticosteroid therapy (odds ratio [OR] 8.734, 95% confidence interval [CI] 2.483–30.717), older age at onset (OR 1.063, 95% CI 1.028–1.099), acute respiratory failure at ICU admission (OR 10.931, 95% CI 2.062–57.751), and dyskinesia/dystonia (OR 14.109, 95% CI 1.336–148.957). The generalized linear mixed model also indicated that high-dose corticosteroid therapy was associated with poorer longitudinal outcomes. Conclusions While high-dose corticosteroids are frequently used to treat AE, their risks may outweigh their benefits in severe antibody-negative AE cases. Older patients and those with dyskinesia/dystonia or respiratory failure, may require more careful monitoring and timely intervention for improved outcomes. However, prospective validation of these findings is necessary to confirm their applicability and guide future treatment strategies.

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. Mimics of anti‐AK5 encephalitis are common and that misdiagnosis is often driven by non‐specific symptoms and clinically irrelevant antibody results. The absence of cognitive impairment as a major symptom, prominence of psychiatric and behavioral symptoms, isolated from serum positivity or low CSF antibody titers (< 1:100), and objective clinical improvement following immunotherapy should prompt reconsideration of the diagnosis. The figure was drawn by Figdraw.

Despite the increasing incidence of autoimmune encephalitis and the incomplete recovery observed in patients post-affliction, the issue of timely diagnosis remains unresolved. The primary objective of this study is identification the distinctive clinical presentation features evaluation the management strategies, and assess the outcomes of the disease in patients with various forms of autoimmune encephalitis. The research aims to contribute in a better understanding of the disease progression and facilitate the selection of optimal therapeutic interventions. A retrospective observational study enrolled 68 patients aged 18 years and older with verified autoimmune encephalitis who underwent treatment in state hospitals in Sofia, Bulgaria, from the beginning of 2014 to the end of 2022. The number of patients with pathology linked to antibodies against glycine receptors (Gly-R) was half as much, with 32 and 17 patients, respectively. The primary manifestations of autoimmune encephalitis included cognitive impairments observed in 51 patients, seizures occurring in 44 patients, and mood disorders observed in 22 patients. While the findings of imaging studies were nonspecific, hospitalizations for patients with this pathology, especially those with antibodies to CASPR2 and DPPX, were prolonged (114 and 232 days, respectively). In the vast majority of cases, incomplete recovery with residual symptoms was noted. Among the diverse forms of autoimmune encephalitis, the most prevalent is NMDA-R. Cognitive impairments predominate in the autoimmune encephalitis clinical presentation. Prolonged hospitalization periods and incomplete recovery of patients are characteristic features of autoimmune encephalitis, despite combined therapy involving intravenous administration of methylprednisolone and immunoglobulins.

The presence of oligoclonal bands (OCBs) in cerebrospinal fluid (CSF) is a pivotal diagnostic marker for multiple sclerosis (MS). These bands play a crucial role in the diagnosis and understanding of a wide array of immune diseases. In this study, we explore the relationship between the cognitive profile of autoimmune encephalitis (AIE) and the presence of OCBs in CSF, with a particular emphasis on NMDA receptor antibodies. We studied a cohort of 21 patients across five tertiary centers, segregated into two distinct categories. One group comprised individuals who tested positive only for autoimmune encephalitis antibodies indicative of encephalitis, while the other group included patients whose CSF was positive for both autoimmune encephalitis antibodies and OCBs. Our investigation focused primarily on cognitive functions and behavioral alterations, supplemented by auxiliary diagnostic assessments such as CSF cell count, magnetic resonance imaging (MRI), and electroencephalogram (EEG) results, evaluated for the two patient groups. To validate our findings, we employed statistical analyses such as Fisher's exact test with Benjamini-Hochberg correction. Our study included 21 patients, comprising 14 who were presented with only autoimmune encephalitis antibodies, and 7 who were dual-positive. Among these patients, we focused on those with NMDA receptor antibodies. Of these, five were dual positive, and nine were positive only for NMDA receptor antibodies. The dual-positive NMDA group, with an average age of 27 ± 16.47 years, exhibited significantly higher CSF cell counts (p=0.0487) and more pronounced language and attention deficits (p= 0.0264). MRI and EEG results did not differ significantly between the groups. Our results point to OCBs as an additional marker of disease severity in AIE, especially in NMDA receptor-antibody positive patients, possibly indicating a broader inflammatory process, as reflected in elevated CSF lymphocytes. Regular testing for OCBs in cases of suspected AIE may aid in disease prognosis and identification of patients more prone to language and attention disorders, improving diagnosis and targeting treatment for these cognitive aspects.

We summarized the clinical manifestations, auxiliary examinations, treatment, and prognostic characteristics of a patient with neurexin-3a IgG-mediated autoimmune encephalitis. On March 2, 2024, a 43-year-old male patient was admitted to the Second Hospital of Shandong University and had prodromal symptoms of infection before the onset of encephalitis. The main manifestations were episodic loss of consciousness, eyes turned upward to the right, clenched teeth, bleeding from tongue bite, and limb twitching. Imaging results showed that the left frontal lobe was characterized by a patchy, slightly longer T1 and T2 signal foci, with a slightly higher signal in the pressurized water image. The CSF virus test was normal; both the serum and CSF were positive for neurexin-3a antibodies using CBA, which were confirmed by TBA. The patient’s symptoms improved after glucocorticosteroid therapy. Neurexin-3a IgG-mediated autoimmune encephalitis is a new type of autoimmune encephalitis, and suspicion of associated disease requires further testing for neurexin-3a IgG for a definitive diagnosis.

Our study aimed to explore the association of systemic inflammatory factors in relations to disease severity of the cell surface antibody-mediated autoimmune encephalitis (AE) across various stages. We retrospectively analyzed patients with AE from two hospitals between October 2016 and December 2023. Systemic inflammatory factors were measured at admission and discharge. Disease severity and prognosis were assessed using the clinical assessment scale for autoimmune encephalitis (CASE), and multivariate logistic regression analysis was used to identify associated risk factors. A total 83 patients were enrolled. The CASE score and the modified Rankin Scale score were positively correlated at admission, discharge and follow-up (r=0.937, P < 0.001; r=0.910, P < 0.001; r=0.972, P < 0.001). Multivariate logistic regression analysis revealed that a higher systemic immune-inflammation index (SII) at admission (OR=27.617, 95% CI: 1.060-719.699, P=0.046) and an elevated platelet-to-lymphocyte ratio (PLR) at discharge (OR=11.373, 95% CI: 1.166-110.893, P=0.036) were independent risk factors for severe disease at admission and discharge, respectively. Additionally, a high neutrophil-to-platelet ratio (NPR) at either admission (OR=10.384, 95% CI: 2.036-52.958, P=0.005) or discharge (OR=5.714, 95% CI: 1.189-27.455, P=0.036) was associated with poor prognosis. SII and PLR were associated with disease severity, while NPR was a consistent predictor of poor long-term outcomes. These findings highlight the value of systemic inflammatory factors in monitoring disease progression and guiding treatment decisions in patients with AE mediated by cell surface antibody.