Timing of MOG-IgG Testing Is Key to 2023 MOGAD Diagnostic Criteria
Discussion The 2023 MOGAD diagnostic criteria were highly sensitive and specific and closely align with historically established cases of MOGAD. However, because additional supportive features are stipulated for patients with a low-positive MOG-IgG result, missed diagnoses may occur due to delayed testing or insufficient investigations. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - November 17, 2023 Category: Neurology Authors: Forcadela, M., Rocchi, C., San Martin, D., Gibbons, E. L., Wells, D., Woodhall, M. R., Waters, P. J., Huda, S., Hamid, S. Tags: All Demyelinating disease (CNS) Research Article Source Type: research

Antibody Investigations in 2,750 Children With Suspected Autoimmune Encephalitis
Discussion The repertoire of antibodies in children with AE is different from that of the adults. Except for NMDAR and MOG antibodies, many of the antibodies included in diagnostic panels are rarely positive and their up-front testing in children seems unneeded. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - November 15, 2023 Category: Neurology Authors: Chen, L.-W., Guasp, M., Olive-Cirera, G., Martinez-Hernandez, E., Ruiz Garcia, R., Naranjo, L., Saiz, A., Armangue, T., Dalmau, J. Tags: Autoimmune diseases, Encephalitis, All Pediatric Clinical/Scientific Notes Source Type: research

Glial Fibrillary Acidic Protein Autoimmunity After Aseptic Meningitis: A Report of 2 Cases
This study involved a retrospective chart review. Results Two female patients, 45 and 55 years of age, developed aseptic meningoencephalomyelitis or meningitis; in one patient, it was likely caused by herpes simplex virus 2. The patients were recovering from the infectious condition when they, 51 and 5 days after onset, had new symptoms with detection of GFAP antibodies in the CSF; CSF and serum samples from the initial lumbar punctures had been negative for GFAP antibodies. Both patients recovered with steroid treatment (in one case, plus rituximab; in the other, plus azathioprine) including resolution of MRI and CSF abn...
Source: Neurology Neuroimmunology and Neuroinflammation - November 10, 2023 Category: Neurology Authors: Bien, C. G., Büttner, T., Reichen, I. C., Thomas, A., Vlad, B., Woermann, F., Bien, C. I., Jelcic, I. Tags: Autoimmune diseases, Meningitis, All Clinical Neurology Clinical/Scientific Note Source Type: research

Neuropsychological Testing in Autoimmune Encephalitis: A Scoping Review
Discussion Given the dearth of literature examining NPT in AE in combination with small sample sizes and methodological differences, more research in this area is needed. However, we provide recommendations for a test battery to be used in future studies, with the aim of standardizing research in this area. Based on the available literature, we recommend the use of comprehensive NPT batteries, spanning all cognitive domains. The highest yield measures may include the tests of (1) visual and verbal learning/memory, (2) basic and sustained attention, (3) processing speed, and (4) executive functions. (Source: Neurology Neuro...
Source: Neurology Neuroimmunology and Neuroinflammation - November 10, 2023 Category: Neurology Authors: Galioto, R., Grezmak, T., Swetlik, C., Abbatemarco, J. R., Titulaer, M. J., Finke, C., Kunchok, A. Tags: Autoimmune diseases, Encephalitis, Neuropsychological assessment Research Article Source Type: research

Clinical Determinants of Longitudinal Disability in LGI-1-IgG Autoimmune Encephalitis
This study aims to evaluate longitudinal predictors of disability and disease severity in LGI-1-IgG AE. Methods This retrospective observational study of patients with LGI-1-IgG AE was conducted between 2013-2022. Disability and disease severity were defined by scores on the modified Rankin Scale (mRS) and the clinical assessment scale in AE (CASE), respectively. Demographic variables, clinical/paraclinical data, brain MRI, and Montreal Cognitive Assessment (MOCA) scores were examined as predictors of mRS and CASE scores in logistic and linear regression models, respectively. Results Thirty patients (60% male, median age...
Source: Neurology Neuroimmunology and Neuroinflammation - November 10, 2023 Category: Neurology Authors: Aboseif, A., Li, Y., Amin, M., Lapin, B., Milinovich, A., Abbatemarco, J. R., Cohen, J. A., Punia, V., Rae-Grant, A. D., Galioto, R., Kunchok, A. Tags: Autoimmune diseases, All Neuropsychology/Behavior, Assessment of cognitive disorders/dementia Research Article Source Type: research

Mitoxantrone in NMO Spectrum Disorder in a Large Multicenter Cohort in French Caribbean
Discussion MiTX is an effective and safe treatment for most of our patients, drastically less expensive than new molecules, and could be allowed in NMOSD Afro-descendant patients in geographical areas where access to care is difficult. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - November 10, 2023 Category: Neurology Authors: Chaumont, H., Berard, N., Karam, J.-P., Lobjois, Q., Tressieres, B., Signate, A., Lannuzel, A., Cabre, P. Tags: Cost effectiveness/economic, All Demyelinating disease (CNS), Devic's syndrome, Transverse myelitis Research Article Source Type: research

Resident Memory-like CD8+ T Cells Are Involved in Chronic Inflammatory and Neurodegenerative Diseases in the CNS
Discussion Collectively, an increase in CD69+CD103+CD8+ Trm-like population in the CSF is related with both chronic neuroinflammatory and some neurodegenerative diseases in the CNS, suggesting a partially shared pathology in these diseases. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - November 10, 2023 Category: Neurology Authors: Kimura, K., Nishigori, R., Hamatani, M., Sawamura, M., Ashida, S., Fujii, C., Takata, M., Lin, Y., Sato, W., Okamoto, T., Kuzuya, A., Takahashi, R., Yamamura, T., Kondo, T. Tags: All Immunology, Parkinson disease/Parkinsonism, Alzheimer disease, Multiple sclerosis Research Article Source Type: research

Increased Intracranial Pressure in Pediatric Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease
The objectives of this study were to determine the frequency of increased ICP in MOGAD and its association with disease course and outcomes and to highlight cases requiring medical and/or surgical management of increased ICP. Methods In this retrospective, single-center cohort study, we examined the clinical and paraclinical data from the initial presentation and follow-up data of children diagnosed with MOGAD. In those with opening pressure (OP) measurements, univariate analyses were used to evaluate factors associated with increased ICP, which was defined as OP > 28 cm H2O. We also present a case series of patients w...
Source: Neurology Neuroimmunology and Neuroinflammation - November 2, 2023 Category: Neurology Authors: Nguyen, L., Miles, D. K., Harder, L., Singh, S., Whittemore, B. A., Greenberg, B. M., Wang, C. X. Tags: All Demyelinating disease (CNS), Acute disseminated encephalomyelitis Research Article Source Type: research

Early Neuroaxonal Damage in Neurologic Disorders Associated With GAD65 Antibodies
In this study, we investigate surrogates of neuroaxonal damage in relation to disease duration and clinical presentation. Methods In a multicentric cohort of 50 patients, we measured serum neurofilament light chain (sNfL) in relation to disease duration and disease phenotypes, applied automated MRI volumetry, and analyzed clinical characteristics. Results In patients with neurologic disorders associated with GAD65 antibodies, we detected elevated sNfL levels early in the disease course. By contrast, this elevation of sNfL levels was less pronounced in patients with long-standing disease. Increased sNfL levels were observ...
Source: Neurology Neuroimmunology and Neuroinflammation - November 1, 2023 Category: Neurology Authors: Eisenhut, K., Faber, J., Engels, D., Gerhards, R., Lewerenz, J., Doppler, K., Sommer, C., Markewitz, R., Falk, K. K., Rössling, R., Pruess, H., Finke, C., Wickel, J., Geis, C., Ratuszny, D., Pfeffer, L. K., Bittner, S., Piepgras, J., Kraft, A., Kl Tags: Volumetric MRI, Gait disorders/ataxia, Stiff person syndrome Clinical/Scientific Note Source Type: research

Immune Checkpoint Inhibition-Related Myasthenia-Myositis-Myocarditis Responsive to Complement Blockade
We present a case of severe myasthenia-myositis-myocarditis overlap in a patient receiving ICIs for breast cancer. Clinical findings were recorded. Results A 47-year-old woman developed tetraparesis, dysphagia, and muscle pain during ICI treatment. MG with a thymoma had been diagnosed earlier. Neuromuscular overlap irAEs with cardiac affection was confirmed, and ICI treatment was discontinued. Given a lack of clinical response to standard therapies, a muscle biopsy was performed demonstrating complement deposition. Eculizumab treatment led to rapid improvement in muscle strength and cardiac function. Discussion Neuromusc...
Source: Neurology Neuroimmunology and Neuroinflammation - October 26, 2023 Category: Neurology Authors: Nelke, C., Pawlitzki, M., Kerkhoff, R., Schroeter, C. B., Aktas, O., Neuen-Jacob, E., Polzin, A., Meuth, S. G., Ruck, T. Tags: Autoimmune diseases, All Neuromuscular Disease, Myasthenia Clinical/Scientific Note Source Type: research

Antibody-Negative Autoimmune Encephalitis: A Single-Center Retrospective Analysis
Discussion Antibody-negative AE should be diagnosed only after comprehensive testing. Diagnostic effort is important because many patients benefit from immunotherapy and some have malignancies. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - October 25, 2023 Category: Neurology Authors: Mojzisova, H., Krysl, D., Hanzalova, J., Dargvainiene, J., Wandinger, K.-P., Leypoldt, F., Elisak, M., Marusic, P. Tags: Autoimmune diseases, Cohort studies Research Article Source Type: research

Long-term Effects of IL-6 Receptor Blockade Therapy on Regulatory Lymphocytes and Neutrophils in Neuromyelitis Optica Spectrum Disorder
In this study, we examined the long-term effects of the anti–IL-6R monoclonal antibody tocilizumab (TCZ) on immune cell profiles in patients with NMOSD. Methods Monthly IV injections of TCZ (8 mg/kg) were administered as an add-on therapy to 19 anti–AQP4-IgG-positive patients, who had been refractory to corticosteroids and immunosuppressive drugs. Peripheral blood was collected before infusion of TCZ for flow cytometry analysis of lymphocyte subsets. Seven patients provided whole blood samples for gene expression profiles. Results Patients with NMOSD had reduced numbers of lymphocyte subsets with regulatory f...
Source: Neurology Neuroimmunology and Neuroinflammation - October 20, 2023 Category: Neurology Authors: Matsuoka, T., Araki, M., Lin, Y., Okamoto, T., Gold, R., Chihara, N., Sato, W., Kimura, A., Tachimori, H., Miyamoto, K., Kusunoki, S., Yamamura, T. Tags: Autoimmune diseases, All Clinical Neurology Research Article Source Type: research

Electrolyte Imbalance in Anti LGI1 Encephalitis: It Is Not All in Your Head
In the Research Article "Electrolyte Imbalance in Anti LGI1 Encephalitis: It Is Not All in Your Head" by Gadoth et al.,1 the second author's name should be listed as "Michal Nisnboym." The article has been replaced by a corrected version. The authors regret the error. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - October 10, 2023 Category: Neurology Authors: Gadoth, A. Tags: Correction [amp ] Replacement Source Type: research

Chronic Enterovirus Meningoencephalitis in Prolonged B-Cell Depletion After Rituximab Therapy: Case Report
In this study, we report a case of chronic enterovirus meningoencephalitis in prolonged B-cell depletion years after rituximab therapy. Methods This is a case report from a German academic hospital. In addition to repeated clinical examinations, repeated brain MRI and extended CSF and laboratory diagnostics were performed. We used the CARE checklist when writing our report. Results A 38-year-old man presented with high fever (>40°C), severe headache, and progressive neurologic and cognitive deficits. As result of previous lymphoma therapy with rituximab years ago, prolonged B-cell aplasia was detected. To restore ...
Source: Neurology Neuroimmunology and Neuroinflammation - October 9, 2023 Category: Neurology Authors: Jacksch, C., Dargvainiene, J., Böttcher, S., Diedrich, S., Leypoldt, F., Stürner, K., Berg, D., Schäffer, E. Tags: All Immunology, Encephalitis Clinical/Scientific Note Source Type: research

The OSCAR-MP Consensus Criteria for Quality Assessment of Retinal Optical Coherence Tomography Angiography
Discussion We propose a 3-step approach for standardized quality control: (1) To define a specific ROI, (2) to assess the occurrence of OCTA artifacts according to the OSCAR-MP criteria, and (3) to evaluate OCTA quality based on the occurrence of different artifacts within the ROI. OSCAR-MP OCTA QC criteria achieved high interrater agreement in an international multicenter study and is a promising QC protocol for application in the context of future clinical trials and studies. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - October 9, 2023 Category: Neurology Authors: Wicklein, R., Yam, C., Noll, C., Aly, L., Banze, N., Romahn, E. F., Wolf, E., Hemmer, B., Oertel, F. C., Zimmermann, H., Albrecht, P., Ringelstein, M., Baumann, C., Feucht, N., Penkava, J., Havla, J., Gernert, J. A., Mardin, C., Vasileiou, E. S., Van Der Tags: All Imaging, Retina, Multiple sclerosis Research Article Source Type: research