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Myelin oligodendrocyte glycoprotein (MOG)-IgG-associated disease (MOGAD) is a relatively uncommon autoantibody demyelinating disorder of the central nervous system (CNS) with heterogeneous clinical manifestations and magnetic resonance imaging (MRI) findings. In recent years, a rare MOGAD subtype characterized by distinct clinical and MRI findings has been described. Seizures and cortical hyperintensities best seen on MRI T2-weighted fluid-attenuated inversion recovery (FLAIR) sequences, associated with headache and cerebral spine fluid (CSF) pleocytosis, are the most important characteristics of this MOGAD entity that is named FLAMES (FLAIR hyperintense cortical lesions in MOG-associated encephalitis with seizures). Because of its rarity and the peculiarities of the brain damage and clinical manifestations, it can be under-recognized and confused with focal viral encephalitis, meningitis, subarachnoid hemorrhage, CNS vasculitis, or mitochondrial cytopathy. We described the case of a 4-year-old previously healthy girl who was admitted for focal-onset, tonic-clonic seizures, fever, and headache, combined with optic neuritis. MRI was characterized by FLAIR imaging showing hyperintense cortical lesions, and a mild leukocytosis in the CSF was detected. Efficacy and rapid response to steroid therapy was observed, and no recurrences of neurological problems or further seizures were reported in the following 12 months. This case report can help in understanding FLAMES characteristics in pediatrics in order to favor early diagnosis and prompt therapy. [ABSTRACT FROM AUTHOR]

We describe a 64-year-old woman with relapsing encephalopathy. She initially presented with 5 days of psychomotor agitation, progressing to mania, psychosis and seizures that mimicked autoimmune limbic encephalitis. During her first hospital admission, extensive investigation failed to establish the underlying cause, and she improved with antiseizure medication alone. After a month at home, she relapsed with identical symptoms, and only then did we recognise that both episodes had been provoked by clarithromycin, prescribed for Helicobacter pylori eradication. Clarithromycin-induced neurotoxicity is rarely reported but likely to be under-recognised. It usually manifests within days of starting treatment, with delirium, mania, psychosis or visual hallucinations, sometimes termed 'antibiomania'. Seizures and status epilepticus appear to be less frequent. A full recovery is expected on stopping the medication. [ABSTRACT FROM AUTHOR]

Objective To report and analyze the clinical features of the first documented case in China of anti-neurofilament heavy chain (NfH) antibody-associated encephalitis. Methods and Results A 63-year-old female presented with cognitive impairment, recurrent epileptic episodes, and aphasia. Head MRI showed multiple abnormal signals in bilateral frontal and parietal lobes, and slight swelling in some gyri, dilatation of the supratentorial ventricular system and brain atrophy, especially in bilateral temporal lobes and hippocampus. 18F-DPA714 PET/MRI showed abnormal uptake in multiple brain regions in the frontal, temporal, parietal and occipital lobes, suggesting neuroinflammation in the brain. EEG showed frequent epileptiform discharges. Cerebrospinal fluid white cell count and glucose were elevated. Serum and cerebrospinal fluid antibodies related to autoimmune encephalitis were negative. Serum tissue-based indirect immunofluorescence assay showed cerebellar neurofilament-like fluorescence surrounding Purkinje cells, further detection of neurofilament protein-related antibodies, it suggested high titer of serum NfH-IgG (1:1000). The patient was diagnosed with anti-NfH antibody-associated encephalitis. After treatment with intravenous immunoglobulin and methylprednisolone, the prognosis was improved. Conclusions Anti-NfH antibody-associated encephalitis is a rare and clinically complex condition. The myriad of symptoms complicates early diagnosis and differential identification. Early initiation of immunomodulatory therapy may offer prognostic advantages. [ABSTRACT FROM AUTHOR]

Objective To summarize the clinical characteristics of patients with anti-contactin-associated protein 2 (CASPR2) antibody-associated encephalitis. Methods and Results Total 13 patients diagnosed and treated for anti-CASPR2 antibody-associated encephalitis at The Third Affiliated Hospital, Sun Yat-Sen University from August 2017 to August 2022 were collected. Initial manifestations: 9 patients exhibited limbic system symptoms, 2 patients presented with peripheral nerve symptoms (both with limb pain), one patient had cerebellar ataxia, and one patient had limb weakness. Anti-CASPR2 antibody detection: all the 13 patients underwent serum anti-CASPR2 antibody testing, which was positive in all cases; 10 patients underwent cerebrospinal fluid (CSF) anti-CASPR2 antibody testing, with 5 cases testing positive; 3 patients had other concomitant positive autoimmune antibodies. Head MRI examination: among the 13 patients, 2 patients head T2-FLAIR showed hyperintensity in the medial temporal lobes either unilaterally or bilaterally; 2 patients had cerebral microbleeds. Treatment: 2 patients were treated only with steroids, 4 patients received only intravenous immunoglobulin (IVIg), 5 patients were treated with steroids and IVIg, one patient received rituximab only, and one patient received a combination of IVIg, steroids, protein A immunoadsorption and rituximab. Follow-up: follow-up ranged from 1-5 years, with 2 patients lost to follow-up; one patient relapsed after self-discontinuation of medication and achieved a modified Rankin Scale (mRS) score of 0 after treatment; of the remaining 10 patients, one patient showed no symptom improvement, one patient had some symptoms preventing normal life, 6 patients had normal life, 2 patients died. Conclusions Anti-CASPR2 antibody-associated encephalitis has complex clinical characteristics, and serum antibody testing is crucial for diagnosis. [ABSTRACT FROM AUTHOR]

This paper aims to compare the performance of the classical machine learning (CML) model and the deep learning (DL) model, and to assess the effectiveness of utilizing fusion radiomics from both CML and DL in distinguishing encephalitis from glioma in atypical cases. We analysed the axial FLAIR images of preoperative MRI in 116 patients pathologically confirmed as gliomas and clinically diagnosed with encephalitis. The 3 CML models (logistic regression (LR), support vector machine (SVM) and multi-layer perceptron (MLP)), 3 DL models (DenseNet 121, ResNet 50 and ResNet 18) and a deep learning radiomic (DLR) model were established, respectively. The area under the receiver operating curve (AUC) and sensitivity, specificity, accuracy, negative predictive value (NPV) and positive predictive value (PPV) were calculated for the training and validation sets. In addition, a deep learning radiomic nomogram (DLRN) and a web calculator were designed as a tool to aid clinical decision-making. The best DL model (ResNet50) consistently outperformed the best CML model (LR). The DLR model had the best predictive performance, with AUC, sensitivity, specificity, accuracy, NPV and PPV of 0.879, 0.929, 0.800, 0.875, 0.867 and 0.889 in the validation sets, respectively. Calibration curve of DLR model shows good agreement between prediction and observation, and the decision curve analysis (DCA) indicated that the DLR model had higher overall net benefit than the other two models (ResNet50 and LR). Meanwhile, the DLRN and web calculator can provide dynamic assessments. Machine learning (ML) models have the potential to non-invasively differentiate between encephalitis and glioma in atypical cases. Furthermore, combining DL and CML techniques could enhance the performance of the ML models. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 56-year-old cardiac transplant recipient experiencing a collapse, revealing an intracranial mass mimicker, later diagnosed as Epstein-Barr virus (EBV) encephalitis. Topics discussed include the clinical presentation, diagnostic challenges, and treatment approach for EBV encephalitis in immunocompromised patients.

Electroencephalography (EEG) is a useful adjunct to clinical neurological examination, particularly as it may detect subtle or subclinical disturbance of cerebral function and it allows monitoring of cerebral activity over time. Continuous EEG combined with quantitative analysis and machine learning may help identify changes in real time, before the emergence of clinical signs and response to interventions. EEG is rarely pathognomonic in encephalopathy/encephalitis but when interpreted correctly and within the clinical context, certain phenotypes may indicate a specific pathophysiology (eg, lateralised periodic discharges in HSV-1, generalised periodic discharges in sporadic Creutzfeldt-Jakob disease, and extreme delta brushes in anti-n-methyl-D-aspartate receptor autoimmune encephalitis). EEG is included in some specialist guidelines for disease assessment, monitoring and prognostication (ie, hepatic, cancer immunotherapy, viral, prion, autoimmune encephalitis and hypoxic ischaemic encephalopathy). EEG is invaluable for confirming or excluding non-convulsive seizures or status epilepticus, particularly in critically ill patients, and in understanding new concepts such as epileptic encephalopathy and the ictal-interictal continuum. [ABSTRACT FROM AUTHOR]

Autoimmune encephalitis (AE) is a group of inflammatory conditions that can associate with the presence of antibodies directed to neuronal intracellular, or cell surface antigens. These disorders are increasingly recognized as an important differential diagnosis of infectious encephalitis and of other common neuropsychiatric conditions. Autoantibody diagnostics plays a pivotal role for accurate diagnosis of AE, which is of utmost importance for the prompt recognition and early treatment. Several AE subgroups can be identified, either according to the prominent clinical phenotype, presence of a concomitant tumor, or type of neuronal autoantibody, and recent diagnostic criteria have provided important insights into AE classification. Antibodies to neuronal intracellular antigens typically associate with paraneoplastic neurological syndromes and poor prognosis, whereas antibodies to synaptic/neuronal cell surface antigens characterize many AE subtypes that associate with tumors less frequently, and that are often immunotherapy-responsive. In addition to the general features of AE, we review current knowledge on the pathogenic mechanisms underlying these disorders, focusing mainly on the potential role of neuronal antibodies in the most frequent conditions, and highlight current theories and controversies. Then, we dissect the crucial aspects of the laboratory diagnostics of neuronal antibodies, which represents an actual challenge for both pathologists and neurologists. Indeed, this diagnostics entails technical difficulties, along with particularly interesting novel features and pitfalls. The novelties especially apply to the wide range of assays used, including specific tissue-based and cell-based assays. These assays can be developed in-house, usually in specialized laboratories, or are commercially available. They are widely used in clinical immunology and in clinical chemistry laboratories, with relevant differences in analytic performance. Indeed, several data indicate that in-house assays could perform better than commercial kits, notwithstanding that the former are based on non-standardized protocols. Moreover, they need expertise and laboratory facilities usually unavailable in clinical chemistry laboratories. Together with the data of the literature, we critically evaluate the analytical performance of the in-house vs commercial kit-based approach. Finally, we propose an algorithm aimed at integrating the present strategies of the laboratory diagnostics in AE for the best clinical management of patients with these disorders. [ABSTRACT FROM AUTHOR]

Anti-leucine-rich, glioma-inactivated (LGI1) autoimmune encephalitis is one of the autoimmune encephalitides with a distinct movement abnormality - faciobrachial dystonic seizures (FBDS). FBDS, being the clinching sign towards a clinical diagnosis, is known to occur at disease onset, and if left untreated, can lead to disabling cognitive impairment. In this case series, we describe three cases of LGI1 encephalitis presenting with behavioral abnormalities at onset for a significant period, compelling an alternative diagnosis preceding the onset of FBDS. In addition, it highlights that imaging could be normal in the initial stages of the disease, further making the diagnosis a challenge at an initial stage. [ABSTRACT FROM AUTHOR]

Acute encephalitis syndrome (AES) is a serious disorder characterized by the sudden onset of inflammation in the brain, which may lead to life-threatening clinical situations. This syndrome encompasses a range of neurological disorders, including, but not limited to, viral and bacterial infections, toxins, and metabolic causes. It includes a wide array of spectrum of diseases, and diagnosis is based on clinical symptoms and a wide range of investigations. Treatment is usually symptomatic, and in some instances, definitive treatment is available. Long-term sequelae of AES can include cognitive deficits, behavioral changes, motor impairments, and epilepsy. Rehabilitation and supportive care are often necessary. [ABSTRACT FROM AUTHOR]

Background: The typical clinical and radiological presentation of Bickerstaff brainstem encephalitis (BBE) has been highlighted in this case report. Case presentation: Bickerstaff encephalitis is a rare autoimmune inflammatory disorder and is considered a subtype of Guillain–Barré syndrome (GBS) along with Miller Fisher syndrome. The diagnosis of BBE is largely clinical, though laboratory tests and imaging can be of supportive value. We report a case of a 5-year-old child who presented with a classical clinical triad of BBE with characteristic magnetic resonance imaging (MRI) findings. Conclusions: BBE is a rare disease with very few cases being reported with typical clinical and radiological findings. Hence, we reported a typical case of BBE to make an addition to the available literature. [ABSTRACT FROM AUTHOR]

Objective: Central nervous system (CNS) infections are a significant cause of mortality and morbidity all over the world. Currently, molecular methods with high sensitivity and specificity provide a diagnosis from a small amount of cerebrospinal fluid (CSF) sample in a short time. Our study aims to determine whether the meningitis/encephalitis panel test is used rationally and to make appropriate recommendations for practical use in the approach to CNS infections. Methods: Between January 2022 and 2023, 46 samples with meningitis/encephalitis panel requests were included in the study. Cerebrospinal fluid samples of the patients were studied with the QIAstat-Dx Analyzer (Qiagen, Hilden, Germany )and the QIAstat-Dx® Meningitis/Encephalitis (ME) Panel kiti (Qiagen, Hilden, Germany). Data about age, gender, CSF leukocyte, erythrocyte, glucose and protein, blood leukocytes and C-reactive protein (CRP) values, presence of fever, headache, neck stiffness, and altered consciousness in patients were obtained retrospectively from patient files and the hospital information system. Results: Of the 46 patients, 22 were female and 24 were male. Three Varicella-zoster virus, two Herpes simplex virus-1, two Cryptococcus neoformans/gatti, one Escherichia coli K1 and one Listeria monocytogenes positivity were detected in the ME panel of a total of nine samples, five from female and four male patients; the total positivity rate was 19.5%. CSF leukocyte and erythrocyte value, blurred consciousness, blood leukocyte count, and CRP values did not differ significantly between panel (-) and panel (+) groups. Fever, headache, nuchal rigidity, high CSF protein, and low CSF glucose were very different in the group with panel (+) (p<0.05). Conclusion: Requesting an ME panel with a rational algorithm after microbiological and biochemical evaluation of CSF samples taken with a strong clinical suspicion will be convenient and cost-effective, prevent unnecessary antibiotic or antiviral use, and reduce hospital stays. [ABSTRACT FROM AUTHOR]

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a relapsing–remitting neurological disorder that falls within the stiff person syndrome–spectrum disorders. We report a 16-year-old girl with PERM associated with an anti-glutamic acid decarboxylase (GAD) antibody. She had an aggressive initial presentation mimicking fulminant septic shock, followed by truncal and lower limb rigidity, stimulus-sensitive spasm, cognitive impairment, brainstem signs (hyperekplexia, nystagmus), and dysautonomia (urinary retention, constipation, facial flushing, blood pressure fluctuation). Cerebrospinal fluid, electroencephalography, and magnetic resonance imaging of the brain and spine showed features suggestive autoimmune encephalitis and myelitis. The serum anti-GAD antibody was positive, and the diagnosis of PERM was made. She had fluctuating clinical response despite intravenous immunoglobulin, steroids, plasmapheresis, and symptomatic medications. Eventually, in the fourth month since admission, she showed gradual and persistent clinical improvement after introducing rituximab. She was discharged after 6 months of hospitalization, and no relapse was observed in the first 3 years of follow-up. PERM is a rare and underrecognized condition in children. Contrary to previous reports, our case describes an aggressive and life-threatening presentation for PERM. Vague symptoms and the lack of gold diagnostic tests hinder a timely diagnosis. Our study also highlights the need for developing standardized diagnostic criteria and consensus in managing PERM. [ABSTRACT FROM AUTHOR]

BK polyoma virus (BKV) belongs to Polyomaviridae family. It is a double-stranded DNA virus. Only a few cases of BKV--associated neurological disease in renal transplant recipients have been reported. BKV related central nervous system (CNS) infection may often remain unrecognized in immunocompromised patients. Here, we are reporting a case of BKV encephalitis post renal transplantation for the awareness of all physicians regarding this entity. [ABSTRACT FROM AUTHOR]

Background: The Borna disease virus (BoDV-1) is an emerging zoonotic virus causing severe and mostly fatal encephalitis in humans. Methods and Results: A local cluster of fatal BoDV-1 encephalitis cases was detected in the same village three years apart affecting two children. While the first case was diagnosed late in the course of disease, a very early diagnosis and treatment attempt facilitated by heightened awareness was achieved in the second case. Therapy started as early as day 12 of disease. Antiviral therapy encompassed favipiravir and ribavirin, and, after bioinformatic modelling, also remdesivir. As the disease is immunopathogenetically mediated, an intensified anti-inflammatory therapy was administered. Following initial impressive clinical improvement, the course was also fatal, although clearly prolonged. Viral RNA was detected by qPCR in tear fluid and saliva, constituting a possible transmission risk for health care professionals. Highest viral loads were found post mortem in the olfactory nerve and the limbic system, possibly reflecting the portal of entry for BoDV-1. Whole exome sequencing in both patients yielded no hint for underlying immunodeficiency. Full virus genomes belonging to the same cluster were obtained in both cases by next-generation sequencing. Sequences were not identical, indicating viral diversity in natural reservoirs. Specific transmission events or a common source of infection were not found by structured interviews. Patients lived 750m apart from each other and on the fringe of the settlement, a recently shown relevant risk factor. Conclusion: Our report highlights the urgent necessity of effective treatment strategies, heightened awareness and early diagnosis. Gaps of knowledge regarding risk factors, transmission events, and tailored prevention methods become apparent. Whether this case cluster reflects endemicity or a geographical hot spot needs further investigation. [ABSTRACT FROM AUTHOR]

Encephalitis is a devastating neurologic disease often complicated by prolonged neurologic deficits. Best practices for the management of adult patients include universal testing for a core group of etiologies, including herpes simplex virus (HSV)-1, varicella zoster virus (VZV), enteroviruses, West Nile virus, and anti–N-methyl-D-aspartate receptor (anti-NMDAR) antibody encephalitis. Empiric acyclovir therapy should be started at presentation and in selected cases continued until a second HSV-1 polymerase chain reaction test is negative. Acyclovir dose can be increased for VZV encephalitis. Supportive care is necessary for other viral etiologies. Patients in whom no cause for encephalitis is identified represent a particular challenge. Management includes repeat brain magnetic resonance imaging, imaging for occult malignancy, and empiric immunomodulatory treatment for autoimmune conditions. Next-generation sequencing (NGS) or brain biopsy should be considered. The rapid pace of discovery regarding autoimmune encephalitis and the development of advanced molecular tests such as NGS have improved diagnosis and outcomes. Research priorities include development of novel therapeutics. [ABSTRACT FROM AUTHOR]

Background: Anti-leucine-rich glioma-inactivated 1 (LGI-1) autoimmune encephalitis (AE), characterized by rapid decline of memory, seizures, and neuropsychiatric abnormalities, is a rare but devastating disorder. Early diagnosis and treatment are essential to prevent long-term sequelae. In this report, we provide a detailed description of clinical characteristics, laboratory test results, imaging, and electroencephalography (EEG) findings, as well as treatment responses of eight patients with anti-LGI-1 AE treated at our center. Case presentation: At the onset, all eight patients presented with confusion/memory deterioration, seizures (including faciobrachial dystonic seizures or other types of seizure), and behavioral changes such as hallucination, paranoia, and anxiety. Four patients were found with severe hyponatremia. Anti-LGI1 antibodies were detected in the cerebrospinal fluid and/or serum of all patients. For patients with faciobrachial dystonic seizures, no discernible scalp EEG change was detected, while EEG recording of patients experiencing other types of seizure showed focal slowing, focal epileptiform discharges, and focal onset seizures. All patients showed abnormal brain magnetic resonance imaging signals, mainly involving the mesial temporal lobe and the hippocampus. In addition, one patient also experienced fulminant cerebral edema during the acute phase of the illness. All patients received immunotherapy and anti-seizure medications and achieved good seizure control. Nevertheless, these patients continued to experience cognitive impairment during their long-term follow-ups. Conclusions: The care of anti-LGI1 AE patients requires rapid evaluation, prompt initiation of immunotherapy, and long-term follow-up. The long-term presence of neurocognitive complications observed in these patients underline the importance of developing reliable biomarkers that can distinguish between different subtypes of this disease with heterogeneous clinico-electrographico-radiological features. Further research is needed to understand the molecular mechanisms underlying the heterogeneity, in order to facilitate development of more effective treatments for anti-LGI1 AE. [ABSTRACT FROM AUTHOR]

Objectives : Anti-leucine glioma-inactivated protein 1 (anti-LGI1) autoimmune encephalitis (AE) presents as subacute memory loss, behavioral changes, and seizures. Diagnosis and treatment delays can result in long term sequelae, including cognitive impairment. 18F-FDG PET/CT may be more sensitive than MRI in patients with AE. Our objective was to determine if anti-LGI1 is associated with a distinct pattern of FDG uptake and whether this pattern persists following treatment. Methods: Nineteen18F-FDG PET/CT brain scans (13 pre-treatment, 6 convalescent phase) for 13 patients with anti-LGI1 were studied using NeuroQ™ and CortexID™. The sensitivity of the PET images was compared to MRI. The Z scores of 47 brain regions between the pre-treatment and next available follow-up images during convalescence were compared. Results: All 18F-FDG PET/CT scans demonstrated abnormal FDG uptake, while only 6 (42.9%) pre-treatment brain MRIs were abnormal. The pre-treatment scans demonstrated hypermetabolism in the bilateral medial temporal cortices, basal ganglia, brain stem, and cerebellum and hypometabolism in bilateral medial and mid frontal, cingulate, and parietotemporal cortices. Overall, the brain uptake during convalescence showed improvement of the Z scores towards 0 or normalization of previous hypometabolic activity in medial frontal cortex, inferior frontal cortex, Broca's region, parietotemporal cortex, and posterior cingulate cortex and previous hypermetabolic activity in medial temporal cortices, caudate, midbrain, pons and cerebellum. Conclusions: Brain FDG uptake was more commonly abnormal than MRI in the pre-treatment phase of anti-LGI1, and patterns of dysmetabolism differed in the pre-treatment and convalescent phases. These findings may expedite the diagnosis, treatment, and monitoring of anti-LGI1 patients. [ABSTRACT FROM AUTHOR]

Introduction: Breast cancer is the most frequent malignancy in women and is curable in ~70-80% of patients with early-stage detection and without complication. Paraneoplastic neurologic syndrome of breast cancer is one such complication which is often hard to diagnose, severely debilitating, and also challenging to treat. Materials and Methods: A 57-year-old woman with no known co-morbidity presented with sudden onset altered sensorium. She was in status epilepticus when brought to emergency and progressed to super refractory status epilepticus in-spite of our efforts. MRI of the brain with contrast done revealed hyper intense signal in bilateral frontal, parietal, occipital, bilateral thalami, periaqueductal region and dorsal midbrain. Cerebrospinal fluid (CSF) study revealed only increased protein. The comprehensive infective panel of CSF was negative. The vasculitis marker, anti NMO and anti MOG antibody were absent. So, we suspected a possibility of autoimmune aetiology and started methylprednisolone along with intravenous immunoglobulin. Results: Her epileptic discharges settled in an electroencephalogram, and we weaned her off ventilation. We detected a right breast mass on clinical examination. The PET CT scan revealed metastasis in the liver, bones, right axillary lymph nodes, and bone marrow. Conclusion: Our patient, who had the first-time presentation, was diagnosed to have paraneoplastic encephalopathy in the background of breast cancer. The importance of proper clinical examination and whole-body PET CT scan, followed by a trial of immunomodulator therapy, is necessary in all patients with non-infective encephalitis. [ABSTRACT FROM AUTHOR]

Coronavirus disease 2019 (COVID-19) results from infection by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Typical presentations include fever, shortness of breath, and cough though neurological manifestations have been rarely reported. Acute necrotizing encephalitis is a rare manifestation of COVID-19 and can be associated with devastating neurological outcomes. Difficulty in timely acquisition of neuroimaging and high rates of early mortality in these patients hinder timely diagnosis. In this clinicoradiological syndrome, patients suffer from rapidly worsening encephalopathy in first 2 weeks of illness and necrotizing parenchymal changes on neuroimaging. The pathophysiology is hypothesized to occur due to cytokine storm, blood–brain-barrier dysfunction, and viral-mediated immune dysregulation leading to endotheliopathy. Early immunomodulatory treatment with intravenous immunoglobulin and steroids is associated with a favorable outcome. Here, we report a one-and-half-year-old boy who presented with fever, seizures, and decreased activity since 3 days. He was noted to have hypertonia in all four limbs with exaggerated deep tendon reflexes. Nasopharyngeal reverse transcriptase polymerase chain reaction test for SARS-CoV-2 was positive. Magnetic resonance imaging brain was suggestive of acute necrotizing encephalopathy. Patient was treated with steroids. [ABSTRACT FROM AUTHOR]

Simple Summary: The median age of diagnosis of dementia, as well as for many cancers, is above 60 years. Dementia should not exclude even frail, elderly patients from cancer treatment. Before beginning any treatment, the effects of the patient's diet, medications and caregiver situation on neurological symptoms should be determined. Malnutrition, dehydration, alcohol consumption, and even loneliness can all accentuate or cause the "3Ds" of dementia, delirium and depression. Common drugs, especially if taken together, can cause cognitive difficulties resembling neurodegenerative diseases such as Alzheimer or Parkinson. These syndromes may be reversed by diet, social and caregiver changes, and stopping or lowering the dose of potentially inappropriate medications (PIMs). Brain scans and genetic analysis can be used to guide immunotherapies and tumor-antigen directed treatments. Discussion among the patient, caregivers and medical team is needed to assess cognition state and ability to accept diagnostic and therapeutic procedures. While surgery may be too dangerous for frail patients, radiation and many oral or infusion therapies, some discussed in this review, may be better tolerated. The likelihood of a diagnosis of dementia increases with a person's age, as is also the case for many cancers, including melanoma and multiple myeloma, where the median age of diagnosis is above 60 years. However, patients diagnosed with dementia are less likely to be offered invasive curative therapies for cancer. Together with analysis of diet and medication history, advanced imaging methods and genetic profiling can now indicate more about syndromes causing the neurological symptoms. Cachexia, malnutrition, dehydration, alcohol consumption, and even loneliness can all accentuate or cause the "3Ds" of dementia, delirium and depression. Many common drugs, especially in the context of polypharmacy, can cause cognitive difficulties resembling neurodegenerative disease. These syndromes may be reversed by diet, social and caregiver changes, and stopping potentially inappropriate medications (PIMs). More insidious are immune reactions to many different autoantigens, some of which are related to cancers and tumors. These can induce movement and cognitive difficulties that mimic Alzheimer's and Parkinson's diseases and other ataxias associated with aging. Paraneoplastic neurological syndromes may be reversed by directed immunotherapies if detected in their early stages but are best treated by removal of the causative tumor. A full genetic workup should be done for all individuals as soon as possible after diagnosis, to guide less invasive treatments suitable for frail individuals. While surgical interventions may be contraindicated, genetic profile guided immunotherapies, oral treatments, and radiation may be equally curative in a significant number of cancers. [ABSTRACT FROM AUTHOR]

Seizures and epilepsy can result from various aetiologies, yet the underlying cause of several epileptic syndromes remains unclear. In that regard, autoimmune-mediated pathophysiological mechanisms have been gaining attention in the past years and were included as one of the six aetiologies of seizures in the most recent classification of the International League Against Epilepsy. The increasing number of anti-neuronal antibodies identified in patients with encephalitic disorders has contributed to the establishment of an immune-mediated pathophysiology in many cases of unclear aetiology of epileptic syndromes. Yet only a small number of patients with autoimmune encephalitis develop epilepsy in the proper sense where the brain transforms into a state where it will acquire the enduring propensity to produce seizures if it is not hindered by interventions. Hence, the term autoimmune epilepsy is often wrongfully used in the context of autoimmune encephalitis since most of the seizures are acute encephalitis-associated and will abate as soon as the encephalitis is in remission. Given the overlapping clinical presentation of immune-mediated seizures originating from different aetiologies, a clear distinction among the aetiological entities is crucial when it comes to discussing pathophysiological mechanisms, therapeutic options, and long-term prognosis of patients. Moreover, a rapid and accurate identification of patients with immune-mediated epilepsy syndromes is required to ensure an early targeted treatment and, thereby, improve clinical outcome. In this article, we review our current understanding of pathogenesis and critically discuss current and potential novel treatment options for seizures and epilepsy syndromes of underlying or suspected immune-mediated origin. We further outline the challenges in proper terminology. [ABSTRACT FROM AUTHOR]

Background Encephalitis represents a challenging condition to diagnose and treat. To assist physicians in considering autoimmune encephalitis (AE) sooner, we developed and validated a risk score. Methods The study was conducted as a retrospective cohort of patients with a diagnosis of definite viral encephalitis (VE) and AE from​​ February 2005 to December 2019. Clinically relevant and statistically significant features between cases of AE and VE were explored in a bivariate logistic regression model and results were used to identify variables for inclusion in the risk score. A multivariable logistic model was used to generate risk score values and predict risk for AE. Results were externally validated. Results A total of 1310 patients were screened. Of the 279 enrolled, 36 patients met criteria for definite AE and 88 criteria for definite VE. Patients with AE compared with VE were more likely to have a subacute to chronic presentation (odds ratio [OR] = 22.36; 95% confidence interval [CI], 2.05–243.7), Charlson comorbidity index <2 (OR = 6.62; 95% CI, 1.05–41.4), psychiatric and/or memory complaints (OR = 203.0; 95% CI, 7.57–5445), and absence of robust inflammation in the cerebrospinal fluid defined as <50 white blood cells/µL and protein <50 mg/dL (OR = 0.06; 95% CI,.005–0.50). Using these 4 variables, patients were classified into 3 risk categories for AE: low (0–1), intermediate (2–3), and high (4). Results were externally validated and the performance of the score achieved an area under the curve of 0.918 (95% CI,.871–.966). Discussion This risk score allows clinicians to estimate the probability of AE in patients presenting with encephalitis and may assist with earlier diagnosis and treatment. [ABSTRACT FROM AUTHOR]

The article focuses on the misdiagnosis of Autoimmune encephalitis (AE), a rare neurological disease. It mentions AE's clinical manifestations, such as behavioral and psychiatric symptoms, autonomic changes, and movement disorder. It also mentions reasons for misdiagnosis, including the insidious onset of symptoms, functional neurologic disorder, mitochondrial disease, normal MRI, and progressive atrophy without signal abnormalities or enhancement.

New onset refractory status epilepticus (NORSE) is a clinical presentation in patients, not a specific diagnosis characterized by the occurrence of a prolonged period of refractory seizures. NORSE is the presenting clinical feature in some patients with paraneoplastic etiology. Paraneoplastic limbic encephalitis (PLE) is a rare disorder of the nervous system associated with malignant disease. It presents as a subacute disease with symptoms like seizures, cognitive dysfunction, irritability, hallucination, and short-term memory loss. In our study, we reported a case of a 30- year-old woman with PLE associated with craniopharyngioma and serum anti-Hu antibodies. The patient presented with NORSE. Initial therapy with antiepileptic drugs failed to control seizures. The patient presented with short-term or recent memory loss, cognitive dysfunction, and behavioral changes (anger/apathy) for the last three months, suggesting a rapidly progressing dementia. Baseline investigations (blood counts, renal function, thyroid function, liver function, serum B12/folate level, serum cortisol, and urinalysis) were normal, and she had no background comorbidities. Computerized tomography (CT) scan and magnetic resonance imaging (MRI) revealed a sellar/suprasellar lesion suggestive of craniopharyngioma. The tumor was successfully removed and repeat imaging after surgery was clear and did not result in further seizures. Now the patient is on follow-up with tapering doses of prednisolone. This is a rare condition; we treated our patient successfully via surgical resection and postoperative chemotherapy. Also, we considered that her new onset refractory epilepsy was associated with her craniopharyngioma. Hence a high degree of suspicion is required, which can be guided by clinical findings and imaging. [ABSTRACT FROM AUTHOR]

The article discusses the specialization of Aedes aegypti mosquitoes in feeding on humans as opposed to other vertebrate animals. It explores how changes in climate, urbanization, and historical events like the African slave trade have influenced the mosquito's preference for humans, increasing the risk of diseases like yellow fever, dengue, chikungunya, and Zika virus in urban areas of Africa and beyond.

The article focuses on assessing the utility of video-electroencephalography monitoring (VEM) for patients with autoimmune encephalitis to improve seizure control and diagnostic accuracy. It is reported that the study, conducted across three tertiary hospitals in Australia, found that VEM admissions led to updated diagnoses in 50 percent of cases and improved seizure frequency in half of the patients, highlighting the potential benefits of VEM in this specific patient population.

We report the first case of Balamuthia mandrillaris granulomatous amoebic encephalitis definitively acquired in Africa. Our case emphasizes initial nonspecific dermatological features, delays in confirmation of the diagnosis, difficulties accessing recommended medication, and uncertainty about optimal treatment of a disease with a frequently fatal outcome. [ABSTRACT FROM AUTHOR]

Background: Fluorodeoxyglucose positron emission tomography (FDG PET) in autoimmune encephalitis (AE) as an adjunctive investigation helps in characterizing the type of AE based on characteristic metabolic patterns. Objectives: We aimed to study the following: (i) the sensitivity of FDG PET in the diagnosis of AE, (ii) describe abnormal patterns of metabolism of various subtypes of AE, and (iii) correlate serum serology with FDG PET abnormalities. Materials and Methods: This study was conducted at a tertiary university hospital in South India. The demographic profile, clinical features, and investigations (FDG PET, magnetic resonance imaging (MRI) brain, electroencephalography (EEG), cerebrospinal fluid (CSF)) were reviewed. The nuclear medicine physician performed blinded qualitative visual and semi quantitative analysis of the 18 FDG PET (fluorine 18 FDG PET) findings of these patients. Results: Twenty nine (M:F: 11:18) patients were recruited; among them, 22 (75.8%) patients had autoimmune antibodies; the rest seven (24.1%) patients were seronegative. Among the 22 seropositive patients, 9 (31%) patients were positive for anti N methyl D aspartate receptor (NMDAR), 8 (28%) for anti leucine rich glioma inactivated 1 (LGI 1), 4 (14%) for anti contactin associated protein 2 (CASPR2), 1 (3%) for anti glutamic acid decarboxylase (GAD) 65, and rest 7 (24%) patients were seronegative. The patterns most commonly observed were isolated hypermetabolism (41%), isolated hypometabolism (41%), and combined hypermetabolism with hypometabolism (18%). The fraction of abnormalities was lower for MRI (17/22; 73.9%) than for FDG PET (27/29; 93.1%). FDG PET correlated with serology in 10 (34%) cases [NMDAR: 6 (60%) and LGI 1: 4 (40%)]. The sensitivity of FDG PET was 94.1% when compared with MRI. Discussion and Conclusion: FDG PET correlated with serology in only one third of patients. The most consistent pattern in both seropositive and seronegative AE is characterized by parieto occipital hypometabolism and fronto temporal with basal ganglia hypermetabolism. [ABSTRACT FROM AUTHOR]

Background: Acute pediatric encephalitis is a debilitating neurological disorder that develops as a rapidly progressive encephalopathy (usually in less than 6 weeks) due to brain inflammation, autoimmune encephalitis (AE) is a heterogeneous group of recently identified disorders. Despite severe and even prolonged neurologic deficits, dramatic improvements may occur with proper immunotherapy in some patients with AE. Anti-neuronal antibodies have been discovered in cerebrospinal fluid (CSF) of patients, Aim and Objectives: The current study aimed to test the relation of early diagnosis with the treatment of N-methyl D- Aspartate Receptor autoimmune encephalitis. Methods: This is a Prospective observational cohort study that was carried out at the pediatric department, Faculty of Medicine, Zagazig University, the study was conducted on 36 cases. Results: There was no statistically significant difference between the studied groups with and without autoimmune encephalitis regarding results of CSF analysis (a larger percentage without autoimmune encephalitis had abnormal CSF analysis). There is a statistically significant difference between the studied groups with and without autoimmune encephalitis regarding prognosis. Conclusion: CSF NMDAR antibodies are highly sensitive biomarkers in the prediction of mortality and poor prognosis of encephalitis in children. [ABSTRACT FROM AUTHOR]

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Purpose: In infections of the Central Nervous System (iCNS), rapid identification of causing pathogens is crucial for survival and to avoid long-term sequelae. Targeted therapy may reduce side effects and development of antibiotic resistance. New molecular-based syndromic tests such as the "meningitis/encephalitis panel" (MEP) allow accelerated pathogen identification from cerebrospinal fluid. We conducted a clinical study to evaluate the MEP's efficacy in paediatric patients. Methods: Cohort study in a unique clinical setting by comparing the outcome data of two neighbouring Children's Hospitals in Germany which are comparable in size, catchment area and equipment but differ regarding availability of the MEP: study centre 1 (SC1): yes; SC2: no. The study population included 213 paediatric patients with a suspected iCNS (SC1: 106; SC2: 107), with comparable age, CRP at admission and frequency of intensive care. The primary outcome was total use of antibiotics. Results: Total antibiotic use per patient was numerically lower in SC1 than in SC2 (SC1: median 2.83 days; SC2 3.67 days; p = 0.671). Multiple linear regression analysis did not show a relevant association between MEP-availability and total antibiotic use (ß = 0.1, 95% confidence interval [−1.46; +1.67], p = 0.897). In the subcohort with suspected meningoencephalitis (SC1: 18, SC2: 17), duration of acyclovir treatment was shorter in SC1 than in SC2 (median 1.3 days vs. 2.7 days, descriptive p = 0.0397). Conclusions: The add-on use of the MEP in paediatric patients with suspected iCNS was associated with a non-significant reduction in total antibiotic use, and with a reduced exposure to acyclovir in treated patients. [ABSTRACT FROM AUTHOR]

Objective To summarize the clinical manifestations, laboratory examination, imaging examination, genetic test, treatment and prognosis of scrub typhus combined with central nervous system (CNS) infection. Methods and Results The clinical data of 4 cases of scrub typhus encephalitis diagnosed and treated from October to November in Tianjin Huanhu Hospital were analyzed. The main clinical manifestations were eschar and lymphadenopathy (4 cases), headache (4 cases), nausea and vomiting (3 cases), fever (3 cases), rash (3 cases), and mental behavior disorder (3 cases); laboratory examination of hepatic in 3 cases were abnormality. Three patients underwent lumbar puncture of cerebral spinal fluid (CSF), including 3 cases with elevated pressure, 2 with elevated white blood cell count, 3 with elevated protein quantification, and 3 with abnormal immune function. Head MRI examination in 3 cases were normal, and one case showed a slightly linear hyperintensity of FLAIR in the bilateral occipital lobes. Metagenomic next-generation sequencing (mNGS) of CSF was completed in 3 patients, and Orientia tsutsugamushi was detected in all of them. All 4 cases were diagnosed as scrub typhus encephalitis and recovered after effective antibiotic treatment. Conclusions The clinical manifestations of scrub typhus combined with CNS infection are not specific, so it is necessary to be vigilant against the occurrence of scrub typhus in patients with CNS infection of unknown etiologys. In particular, scrub typhus should be considered during the epidemic season, if there is a history of farm work or suburban activities. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 45-year-old male with dyspnea, chronic cough, weight loss, and episodes of massive hemoptysis. The patient was diagnosed with Rasmussen aneurysm due to pulmonary tuberculosis. The study highlights the rarity of this condition, its association with tuberculosis, and the need for prompt diagnosis and management to prevent life-threatening complications.

Background and Objectives: The 2023 criteria for myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) perform well in adults but have not been assessed in children., Methods: This prospective observational nationwide study includes children and adults with demyelinating syndromes or encephalitis, whose serum or CSF was found MOG-immunoglobulin G (IgG) positive at Institut d'Investigacions Biomèdiques August Pi i Sunyer-Hospital Clínic of Barcelona (Spain). Exclusion criteria were lack of clinical information and follow-up <1 year, and serum unavailable for antibody testing. The primary outcome was to assess the accuracy of the 2023 MOGAD criteria, using as gold standard the most plausible diagnosis after a follow-up >1 year. MOGAD criteria were retrospectively applied assessing core syndromes, supportive clinical-radiological features, and MOG-IgG titers. Patients tested ≤3 months of a disease attack (acute phase) or afterward (remission) were considered separately. The positive predictive value (PPV) of the criteria (true-positive [patients classified as MOGAD and MOGAD diagnosis last follow-up] divided by total positive [all patients classified as MOGAD]), and its 95% CI, was calculated with the Wilson procedure., Results: A total of 257 patients (133 children) were included in the study (median age 15 years [interquartile range 6-38], 54% female). Among 202 patients assessed during a disease attack, 158 (78%) had high MOG-IgG serum titers, 36 (18%) low titers, and 8 (4%) antibodies only in CSF. No differences were identified between patients with high and low titers, but those with low titers were more likely to have an alternative diagnosis at last follow-up (2/36 [6%] vs 0/158, p = 0.012). Supportive features were present in 230 of 257 (89%) patients, regardless of age, MOG-IgG titers, and core syndromes except for optic neuritis in adults whose assessment with orbital MRI was not systematic. Overall, 240 of 257 (94%) patients were well classified by the MOGAD criteria (e.g., 236 eventually having MOGAD and 4 alternative diagnoses), and 17 were wrongly classified (e.g., 11 eventually having MOGAD and 6 alternative diagnoses). Although the criteria classified better during disease attacks than during remissions (187 [96%] vs 49 [89%] serum MOG-IgG-positive patients were well-classified, p = 0.038), the PPV was high in both settings (99% [95% CI 97-100] vs 98% [95% CI 89-100])., Discussion: The 2023 MOGAD criteria correctly identified most children and adults with MOGAD. The highest accuracy occurred when they were applied during disease attacks., Classification of Evidence: This study provides Class IV evidence that the 2023 MOGAD criteria accurately identify adults and children with MOGAD.

Kleine-Levin syndrome (KLS) is a rare, recurring sleep disorder that easily ignored. Episodic upward-gaze palsy is an uncommon manifestation observed in patients of KLS, which further complicates this disorder. Although peripheral microbial infection have been recognized as most common triggers for KLS, the underlying pathophysiology of this disorder remains unclear. We reported a unique case of KLS elicited by acute encephalitis, which was confirmed by pleocytosis of cerebrospinal fluid at the early stage. The cerebrospinal fluid returned to normal over time while the attacks continued to recur frequently. Episodic upward-gaze palsy was observed during attacks and clinical symptoms were exacerbated following a subsequent COVID-19 infection. This report presents a classic KLS case with distinctive characteristics, which should facilitate more accurate and earlier diagnosis for clinicians. Furthermore, it provides a new perspective for understanding the pathogenesis of this rare disease., Citation: Lv H, Long X, Lv Y, Zhou J. Acute encephalitis induced Kleine-Levin syndrome with episodic vertical gaze dysfunction during hypersomnia episodes. J Clin Sleep Med . 2024;20(9):1555-1556., (© 2024 American Academy of Sleep Medicine.)

Objectives: Pathogenic variants in presenilin 1 (PSEN1) are related to early-onset Alzheimer disease (AD) and may occur as de novo variants. In comparison with sporadic forms, it can present with psychiatric manifestations, seizures, myoclonus, and focal presentation. Because PSEN1 can occur in young patients who lack a family history of neurologic disorders and because these symptoms are also frequent in autoimmune encephalitis (AE), diagnosis may be overlooked. Our aim was to demonstrate the challenge in diagnosing young patients with neurodegenerative diseases that simulate AE., Methods: We describe a case of a young patient with insidious progressive dementia, myoclonus, seizures, and aphasia, with no family history of dementia, along with signs suggestive of neuroinflammation on brain MRI and CSF examination., Results: She was initially misdiagnosed as having AE. Further investigation was performed, leading to the discovery of a novel and de novo pathogenic variant in PSEN1., Discussion: This case demonstrates the importance of considering PSEN1 in young patients with insidious progressive dementia with atypical clinical and neuroimaging features, even in patients without a family history of neurologic disorders. Not adhering to published criteria of possible and probable AE and overinterpretation of subtle inflammatory findings in CSF and MRI contribute to misdiagnosis.

Mycoplasma pneumoniae is a common cause of community-acquired pneumonia in children and young adults. It is responsible of a broad array of extrapulmonary manifestations, the most severe affecting the central nervous system. We report a challenging diagnosis of macrolide-resistant M. pneumoniae-induced Bickerstaff encephalitis in a 16-year-old man., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Contactin-associated protein-2(CASPR2) antibody-associated autoimmune encephalitis(AE) is rare in children. This study aimed to report the clinical characteristics and long-term outcome of CASPR2 autoimmunity in children to expand the disease spectrum., Methods: Children who were hospitalized in our hospital with clinically suspected AE from May 2015 to April 2022 and underwent neuronal surface antibodies detections were retrospectively analyzed. Clinical data of patients with CASPR2 autoimmunity were collected., Results: Patients who were positive for NMDAR-IgG, CASPR2-IgG, LGI1-IgG and IgLON5-IgG occupied 95.2%(119/125),3.2%(4/125),0.8%(1/125) and 0.8%(1/125), respectively.The median onset age of the 4 patients with CASPR2-IgG was 5.6 years. The most common symptoms were psychiatric symptoms/abnormal behavior(3/4) and sleep dysfunction(3/4). One patient developed a phenotype of Rasmussen encephalitis(RE). Tumor was absent in our patients. Two patients showed abnormal findings on initial brain magnetic resonance imaging(MRI) scans. All the patients showed favorable response to immunotherapy except the patient with RE experienced recurrent symptoms who finally achieved remission after surgery. All the patients had a favorable long-term outcome at the last follow-up(33-58months)., Conclusions: CASPR2 autoimmunity may be the second most common anti-neuronal surface antibodies associated neurological disease in children. Psychiatric symptoms/abnormal behavior and sleep disorder were common in children with CASPR2-associated AE. Tumor was rare in those patients. Most pediatric patients had a favorable long-term outcome., (© 2024. The Author(s).)

Objective: Autoimmune encephalitis includes a heterogeneous group of rare and complex diseases, usually presenting with severe and disabling symptoms, such as behavioral changes, cognitive deficits, and seizures., Method: This report presents the case of a 26-year-old man who was diagnosed with autoimmune encephalitis following SARS-CoV-2 vaccination (<40 days). Symptoms first appeared in February 2022 with a temporal seizure, associated with confusion and memory loss. Psychiatric manifestations such as disorientation and altered thought contents emerged soon after., Results: Neuroimaging testing showed signs of hypometabolism in occipital, prefrontal, and temporal regions, whereas an extensive neuropsychological assessment revealed the presence of multiple alterations in memory, executive, and visuoconstructive processes., Conclusions: In this case, a combination of neuroimaging testing, psychiatric evaluation, and neuropsychological assessment provided evidence for a diagnosis of autoimmune encephalitis post-vaccination. Early recognition is essential in order to prevent clinical progression; avoid intractable epilepsy, brain atrophy, and cognitive impairment; and improve prognosis., (© The Author(s) 2024. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: Cognitive disorders usually develop slowly over years and are mainly caused by untreatable neurodegenerative disorders. Rapidly progressive cognitive disorders should raise suspicion of an underlying and treatable psychiatric, internal or neurological condition. Timely recognition of these conditions is important., Case: We present the case of a 68-year old man, presenting on the emergency department with a history of progressive cognitive impairment since several weeks. Cerebral MRI showed T2-hyperintensities in the left hippocampal, mesotemporal and insular regions; lesser so in the right mesotemporal region. After initial treatment for herpesencephalitis and autoimmune encephalitis, we diagnosed neurolues and started treatment with benzylpenicillin., Conclusion: It may be difficult to diagnose neurolues because the vast variety of clinical symptoms and radiological signs. This case shows that neurolues should be considered in a patient with rapidly progressive cognitive disorders and that neurolues can mimic a herpesencephalitis or an autoimmune encephalitis. Timely recognition is important to prevent irreversible damage.

Rationale: Anti-Myelin oligodendrocyte glycoprotein (MOG) and anti-metabotropic glutamate receptor 5 (mGluR5) double antibody positive encephalitis characterized by optic neuritis is extremely rare. We present a case of overlapping syndrome of MOG-IgG-associated disease and anti-mGluR5 encephalitis manifested as optic neuritis., Patient Concerns: A 60-year-old Chinses woman presented to the hospital with progressive vision loss and headache for 1 week. The cerebrospinal fluid examination was within the normal range. Visual evoked potentials study disclosed prolonged latency of P100 bilaterally. Fundus examination revealed indistinct boundaries of both optic discs. Her brain magnetic resonance imaging showed patchy hyperintensity in the posterior horn of the left ventricle and the left optic nerve. Her serum was positive for anti-MOG and anti-mGluR5 antibodies., Diagnosis: The patient was diagnosed with overlapping syndrome of anti-MOG antibody-associated disease and anti-mGluR5 encephalitis mainly based on the clinical symptoms and further test of the antibody in serum., Interventions and Outcomes: She was subsequently subjected to empirical treatment with intravenous methylprednisolone. After discharge, she was given a tapering dose of oral prednisone, alongside mycophenolate mofetil. On outpatient follow-up, her symptoms showed no relapse after 1 month, and her condition remained stable., Lessons: Early recognition of autoimmune encephalitis is crucial. The detection of cerebrospinal fluid and serum of autoimmune encephalitis and demyelinating diseases of the CNS, including MOG-IgG and mGluR5-IgG, should be strengthened in order to make a precise diagnosis and develop a comprehensive treatment plan in a timely manner., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: The presence of inflammatory changes in the cerebrospinal fluid (CSF), including immunoglobulin intrathecal synthesis (IS), can support the diagnosis of autoimmune encephalitis (AE) and allow prompt treatment. The main aim of our study was to calculate the Kappa index as a marker of IS, in patients with AE., Methods: Charts of patients undergoing a diagnostic work-up for suspected AE between 2009 and 2023 were reviewed and the Graus criteria applied. CSF and serum kappa free light chains were determined using the Freelite assay (The Binding Site Group) and the turbidimetric Optilite analyzer., Results: We identified 34 patients with "definite" AE (9 anti-NMDAR AE and 25 limbic AE) and nine patients with "possible" AE. Five patients (15%) with definite AE had pleocytosis and twelve (34%) showed CSF-restricted oligoclonal bands (OCB) at isoelectric focusing. The Kappa index was >6 in 29.4% and > 3 in 50% of the definite AE patients. It was elevated (>3) in 36.4% of patients with definite AE who resulted negative to OCB testing and was the only altered parameter suggestive of an ongoing inflammatory process in the CNS in three definite AE patients with otherwise normal CSF findings (i.e. normal cell count and protein levels, no OCBs). In the possible AE group, one patient had a Kappa index >3 in the absence of OCB., Conclusions: The Kappa index could be useful, as a more sensitive marker of IS and as a supportive marker of neuroinflammation, in the diagnostic work-up of suspected AE., Competing Interests: Declaration of competing interest DF's institution has received kappa free light chain assays (Freelite) for research purposes from Binding Site (Birmingham). GDN, MG, PN, RB, SM, AMS, MS, FV, KS, MC and SM have nothing to disclose., (Copyright © 2024. Published by Elsevier B.V.)