SEZ6L2 Antibody-Associated Cerebellar Ataxia Responsive to Sequential Immunotherapy
We present a case report of a 73-year-old woman with progressive balance problems. Her condition had rapidly deteriorated in the 2 weeks before the admission to our hospital leading to repeated falls and eventually left her bed-ridden. Results She presented with severe trunk ataxia, bidirectional nystagmus, dysarthric speech, and persistent nausea. With the exception of cerebellar atrophy, extensive imaging studies revealed no pathology. SEZ6L2 antibodies were found in both CSF and serum. Over a period of 9 months, our patient received immunotherapy consisting of steroid pulse therapy, IV immunoglobulin infusions, rituxim...
Source: Neurology Neuroimmunology and Neuroinflammation - January 14, 2022 Category: Neurology Authors: Mehdiyeva, A., Hietaharju, A., Sipilä, J. Tags: All Immunology, Autoimmune diseases, Encephalitis, Gait disorders/ataxia, Class IV Clinical/Scientific Note Source Type: research

Assessing Neurofilaments as Biomarkers of Neuroprotection in Progressive Multiple Sclerosis: From the MS-STAT Randomized Controlled Trial
This study provides class I evidence that simvastatin treatment does not have a large impact on either serum NfL or NfH, as quantified in this study, in SPMS. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - January 14, 2022 Category: Neurology Authors: Williams, T. E., Holdsworth, K. P., Nicholas, J. M., Eshaghi, A., Katsanouli, T., Wellington, H., Heslegrave, A., Zetterberg, H., Frost, C., Chataway, J. Tags: Clinical trials Randomized controlled (CONSORT agreement), Class I, Multiple sclerosis Article Source Type: research

Increased Intrathecal B and Plasma Cells in Patients With Anti-IgLON5 Disease: A Case Series
Discussion Our findings indicate the importance of inflammatory mechanisms, in particular in early and acute anti-IgLON5 cases, which may support the use of immune-suppressive treatments in these cases. The main limitation of the study is the small number of cases due to the rarity of the disease. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - January 14, 2022 Category: Neurology Authors: Strippel, C., Heidbreder, A., Schulte-Mecklenbeck, A., Korn, L., Warnecke, T., Melzer, N., Wiendl, H., Pawlowski, M., Gross, C. C., Kovac, S. Tags: All Immunology, Autoimmune diseases Clinical/Scientific Note Source Type: research

Antineonatal Fc Receptor Antibody Treatment Ameliorates MOG-IgG-Associated Experimental Autoimmune Encephalomyelitis
Discussion We show preserved optomotor response and ameliorated course of disease after anti-FcRn treatment in an experimental model using a monoclonal MOG-IgG to mimic MOGAD. Selectively targeting FcRn might represent a promising therapeutic approach in MOGAD. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - January 13, 2022 Category: Neurology Authors: Remlinger, J., Madarasz, A., Guse, K., Hoepner, R., Bagnoud, M., Meli, I., Feil, M., Abegg, M., Linington, C., Shock, A., Boroojerdi, B., Kiessling, P., Smith, B., Enzmann, V., Chan, A., Salmen, A. Tags: Optic nerve, Retina Article Source Type: research

Central Vein Sign Profile of Newly Developing Lesions in Multiple Sclerosis: A 3-Year Longitudinal Study
This study provides Class III evidence that younger age and higher CVS+ percentage at baseline are associated with new CVS+ lesion development. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - January 13, 2022 Category: Neurology Authors: Al-Louzi, O., Letchuman, V., Manukyan, S., Beck, E. S., Roy, S., Ohayon, J., Pham, D. L., Cortese, I., Sati, P., Reich, D. S. Tags: MRI, Class III, Multiple sclerosis Article Source Type: research

Leveraging Visual Outcome Measures to Advance Therapy Development in Neuroimmunologic Disorders
The visual system offers unparalleled precision in the assessment of neuroaxonal damage. With the majority of patients with multiple sclerosis (MS) experiencing afferent and efferent visual dysfunction, outcome measures capturing these deficits provide insight into neuroaxonal injury, even in those with minimal disability. Ideal for use in clinical trials, visual measures are generally inexpensive, accessible, and reproducible. Quantification of visual acuity, visual fields, visual quality of life, and electrophysiologic parameters allows assessment of function, whereas optical coherence tomography (OCT) provides reliable ...
Source: Neurology Neuroimmunology and Neuroinflammation - December 26, 2021 Category: Neurology Authors: Graves, J. S., Oertel, F. C., Van der Walt, A., Collorone, S., Sotirchos, E. S., Pihl-Jensen, G., Albrecht, P., Yeh, E. A., Saidha, S., Frederiksen, J., Newsome, S. D., Paul, F., on behalf of IMSVISUAL, on behalf of IMSVISUAL Tags: MRI, All Neuro-ophthalmology, Multiple sclerosis, Devic's syndrome Views [amp ] Reviews Source Type: research

Abnormal B-Cell and Tfh-Cell Profiles in Patients With Parkinson Disease: A Cross-sectional Study
Discussion Our findings define a novel signature of peripheral immune cells and implicate aberrant Tfh:B-cell interactions in patients with PD. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - December 26, 2021 Category: Neurology Authors: Li, R., Tropea, T. F., Baratta, L. R., Zuroff, L., Diaz-Ortiz, M. E., Zhang, B., Shinoda, K., Rezk, A., Alcalay, R. N., Chen-Plotkin, A., Bar-Or, A. Tags: All Immunology, Parkinson's disease/Parkinsonism Article Source Type: research

Retinal Optical Coherence Tomography in Neuromyelitis Optica
(Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - December 22, 2021 Category: Neurology Tags: CORRECTION Source Type: research

Isolated Memory Loss in Anti-NMDAR Encephalitis
Discussion This case shows that anti-NMDAR encephalitis can present with isolated memory loss. Neural antibody testing in these patients could play a pivotal role in early diagnosis and prompt treatment. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - December 22, 2021 Category: Neurology Authors: Iorio, R., Sabatelli, E., Campetella, L., Papi, C. Tags: Autoimmune diseases Clinical/Scientific Note Source Type: research

Anti-NMDAR Encephalitis in the Netherlands, Focusing on Late-Onset Patients and Antibody Test Accuracy
Background and Objectives To describe the clinical features of anti-NMDAR encephalitis, emphasizing on late-onset patients and antibody test characteristics in serum and CSF. Methods Nationwide observational Dutch cohort study, in patients diagnosed with anti-NMDAR encephalitis between 2007 and 2019. Results One hundred twenty-six patients with anti-NMDAR encephalitis were included with a median age of 24 years (range 1–86 years). The mean annual incidence was 1.00/million (95% CI 0.62–1.59). Patients ≥45 years of age at onset (19%) had fewer seizures (46% vs 71%, p = 0.021), fewer symptoms during disease ...
Source: Neurology Neuroimmunology and Neuroinflammation - December 22, 2021 Category: Neurology Authors: Bastiaansen, A. E. M., de Bruijn, M. A. A. M., Schuller, S. L., Martinez-Hernandez, E., Brenner, J., Paunovic, M., Crijnen, Y. S., Mulder, M. J. H. L., Schreurs, M. W. J., de Graaff, E., Smitt, P. A. E., Neuteboom, R. F., de Vries, J. M., Titulaer, M. J. Tags: Autoimmune diseases, Encephalitis Article Source Type: research

Population-Based Epidemiology Study of Paraneoplastic Neurologic Syndromes
Discussion PNSs are rare neurologic disorders but are associated with severe morbidity and mortality. The estimated number of prevalent PNS cases in the United States is 17,099, and predicted DALY for all US PNS cases is 292,393 years. Their apparent increasing rate of detection is attributable to increasing physician awareness and availability of serologic testing. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - December 22, 2021 Category: Neurology Authors: Shah, S., Flanagan, E. P., Paul, P., Smith, C. Y., Bryant, S. C., Devine, M. F., Lennon, V. A., McKeon, A., Pittock, S. J., Dubey, D. Tags: Autoimmune diseases, Paraneoplastic syndrome, Prevalence studies, Incidence studies Article Source Type: research

Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy
(Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - December 20, 2021 Category: Neurology Tags: Correction Source Type: research

Siponimod Inhibits the Formation of Meningeal Ectopic Lymphoid Tissue in Experimental Autoimmune Encephalomyelitis
Discussion Beneficial effects on the disease course correlated with a reduction in mELT, suggesting that inhibition of mELT may be an additional mechanism of action of siponimod in the treatment of EAE. Further studies are needed to establish causality and confirm this observation in MS. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - December 15, 2021 Category: Neurology Authors: Brand, R. M., Diddens, J., Friedrich, V., Pfaller, M., Radbruch, H., Hemmer, B., Steiger, K., Lehmann-Horn, K. Tags: Multiple sclerosis Article Source Type: research

Demystifying MOGAD and Double Seronegative NMOSD Further With IL-6 Blockade
(Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - December 15, 2021 Category: Neurology Authors: Yong, K. P., Kim, H. J. Tags: Autoimmune diseases, All Demyelinating disease (CNS) Editorial Source Type: research

CSF Neurofilament Light Chain Concentrations Predict Outcome in Bacterial Meningitis
The objective of this study was to determine the prognostic accuracy of NfL concentrations on unfavorable outcome in adults with community-acquired bacterial meningitis. Methods We measured NfL concentration CSF samples from a prospective cohort study of adults with community-acquired bacterial meningitis in The Netherlands and determined associations between NfL CSF concentrations, clinical characteristics, and outcome in multivariate analyses. We identified independent predictors of an unfavorable outcome (Glasgow Outcome Scale scores 1–4) by logistic regression. Results CSF NfL concentrations were evaluated in 4...
Source: Neurology Neuroimmunology and Neuroinflammation - December 13, 2021 Category: Neurology Authors: Chekrouni, N., van Soest, T. M., Brouwer, M. C., Willemse, E. A. J., Teunissen, C. E., van de Beek, D. Tags: Meningitis, Bacterial infections, Prognosis Article Source Type: research

Allosteric Modulation of NMDARs Reverses Patients' Autoantibody Effects in Mice
Discussion An oxysterol biology-based PAM of NMDARs is able to reverse the synaptic and memory deficits caused by CSF from patients with anti-NMDAR encephalitis. These findings suggest a novel adjuvant treatment approach that deserves future clinical evaluation. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - December 13, 2021 Category: Neurology Authors: Radosevic, M., Planaguma, J., Mannara, F., Mellado, A., Aguilar, E., Sabater, L., Landa, J., Garcia-Serra, A., Maudes, E., Gasull, X., Lewis, M., Dalmau, J. Tags: Autoimmune diseases Article Source Type: research

Stress Signal ULBP4, an NKG2D Ligand, Is Upregulated in Multiple Sclerosis and Shapes CD8+ T-Cell Behaviors
Discussion Our study provides new evidence for the involvement of NKG2D and its ligand ULBP4 in MS pathology. Our results point to ULBP4 as a viable target to specifically block 1 component of the NKG2D pathway without altering immune surveillance involving other NKG2DL. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - December 6, 2021 Category: Neurology Authors: Carmena Moratalla, A., Carpentier Solorio, Y., Lemaitre, F., Farzam-kia, N., Levert, A., Zandee, S. E. J., Lahav, B., Guimond, J. V., Haddad, E., Girard, M., Duquette, P., Larochelle, C., Prat, A., Arbour, N. Tags: All Immunology, Autoimmune diseases, Multiple sclerosis Article Source Type: research

Recovery From COVID-19 in Multiple Sclerosis: A Prospective and Longitudinal Cohort Study of the United Kingdom Multiple Sclerosis Register
Discussion Patients with MS are affected by postacute sequelae of COVID-19. Preexisting severe neurologic impairment or mental health problems appear to increase this risk. These findings can have implications in tailoring their post–COVID-19 rehabilitation. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - November 30, 2021 Category: Neurology Authors: Garjani, A., Middleton, R. M., Nicholas, R., Evangelou, N. Tags: All Psychiatric disorders, Disability, Multiple sclerosis Article Source Type: research

CSF-Derived CD4+ T-Cell Diversity Is Reduced in Patients With Alzheimer Clinical Syndrome
Discussion Examination of CSF indicates that CD4+ T cell–mediated adaptive immune responses are altered in patients with ACS. Understanding the underlying mechanisms affecting adaptive immunity will help move us toward the goal of slowing cognitive decline. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - November 30, 2021 Category: Neurology Authors: Joshi, C., Sivaprakasam, K., Christley, S., Ireland, S., Rivas, J., Zhang, W., Sader, D., Logan, R., Lambracht-Washington, D., Rosenberg, R., Cullum, M., Hitt, B., Li, Q.-Z., Barber, R., Greenberg, B., Cowell, L., Zhang, R., Stowe, A., Huebinger, R., Kell Tags: All Immunology, Alzheimer's disease, Gene expression studies Article Source Type: research

COVID-19 Infection in Fingolimod- or Siponimod-Treated Patients: Case Series
Discussion Based on a review of available information, the risk of more severe COVID-19 in patients receiving fingolimod or siponimod seems to be similar to that reported in the general population and the MS population with COVID-19. However, limitations of spontaneous reporting, especially missing data, should be considered in the interpretation of these observations. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - November 30, 2021 Category: Neurology Authors: Sullivan, R., Kilaru, A., Hemmer, B., Campbell Cree, B. A., Greenberg, B. M., Kundu, U., Hach, T., DeLasHeras, V., Ward, B. J., Berger, J. Tags: COVID-19, Multiple sclerosis Article Source Type: research

IgG4-Mediated Neurologic Autoimmunities: Understanding the Pathogenicity of IgG4, Ineffectiveness of IVIg, and Long-Lasting Benefits of Anti-B Cell Therapies
Discussion Rituximab is the preferred treatment in IgG4-ND patients with severe disease by effectively targeting the production of pathogenic IgG-4 antibodies. In contrast, IVIG is ineffective because it inhibits immunoinflammatory functions irrelevant to the mechanistic effects of IgG4 and contains IgG-1 idiotypes that cannot sufficiently neutralize or possibly catabolize IgG4. Controlled studies with anti-CD19/20 monoclonals that also activate FcRIIb may be more promising in treating IgG4-ND. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - November 29, 2021 Category: Neurology Authors: Dalakas, M. C. Tags: Autoimmune diseases Views [amp ] Reviews Source Type: research

Hypogammaglobulinemia and Infections in Patients With Multiple Sclerosis Treated With Rituximab
Background and Objectives To determine the frequency of hypogammaglobulinemia and infections in patients with multiple sclerosis (PwMS) receiving rituximab (RTX). Methods This prospective observational study included all consecutive PwMS receiving RTX at the university hospital of Marseille, France, between 2015 and 2020. Patient visits occurred at least every 6 months. Results We included 188 patients (151 with relapsing-remitting MS; the mean age was 43.4 years [SD 12.9], median disease duration 10 years [range 0–36], median Expanded Disability Status Scale 5 [range 0–8], median follow-up 3.5 years [range 1...
Source: Neurology Neuroimmunology and Neuroinflammation - November 23, 2021 Category: Neurology Authors: Perriguey, M., Maarouf, A., Stellmann, J.-P., Rico, A., Boutiere, C., Demortiere, S., Durozard, P., Pelletier, J., Audoin, B. Tags: All Infections, Multiple sclerosis, Cohort studies Article Source Type: research

Interim Analysis of Pregnancy Outcomes After Exposure to Dimethyl Fumarate in a Prospective International Registry
The objective was to provide pregnancy outcomes and DMF exposure information from an interim analysis from a prospective, international registry (TecGistry; NCT01911767). Methods Women exposed to DMF from the first day of their last menstrual period before conception or during pregnancy were evaluated. Data were obtained at enrollment; 6–7 months' gestation; 4 weeks after estimated due date; and 4, 12, and 52 weeks after birth. Outcomes included live births, gestational size, pregnancy loss, birth defects, and infant or maternal death after delivery. Outcomes were analyzed cumulatively from October 30, 2013 (the sta...
Source: Neurology Neuroimmunology and Neuroinflammation - November 23, 2021 Category: Neurology Authors: Hellwig, K., Rog, D., McGuigan, C., Houtchens, M. K., Bruen, D. R., Mokliatchouk, O., Branco, F., Peng, X., Everage, N. J. Tags: Neonatal, All Demyelinating disease (CNS), Multiple sclerosis Article Source Type: research

No Signs of Neuroinflammation in Women With Chronic Fatigue Syndrome or Q Fever Fatigue Syndrome Using the TSPO Ligand [11C]-PK11195
Discussion In contrast to what was previously reported for CFS, we found no significant difference in BPND of [11C]-PK11195 when comparing patients with CFS or QFS with healthy neighborhood controls. In this small series, we were unable to find signs of neuroinflammation in patients with CFS and QFS. Trial Registration Information EudraCT number 2014-004448-37. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - November 23, 2021 Category: Neurology Authors: Raijmakers, R., Roerink, M., Keijmel, S., Joosten, L., Netea, M., van der Meer, J., Knoop, H., Klein, H., Bleeker-Rovers, C., Doorduin, J. Tags: Article Source Type: research

Interleukin-6 Receptor Blockade in Treatment-Refractory MOG-IgG-Associated Disease and Neuromyelitis Optica Spectrum Disorders
Background and Objectives To evaluate the long-term safety and efficacy of tocilizumab (TCZ), a humanized anti–interleukin-6 receptor antibody in myelin oligodendrocyte glycoprotein–IgG–associated disease (MOGAD) and neuromyelitis optica spectrum disorders (NMOSD). Methods Annualized relapse rate (ARR), Expanded Disability Status Scale score, MRI, autoantibody titers, pain, and adverse events were retrospectively evaluated in 57 patients with MOGAD (n = 14), aquaporin-4 (AQP4)-IgG seropositive (n = 36), and seronegative NMOSD (n = 7; 12%), switched to TCZ from previous immunotherapies, particularly ritux...
Source: Neurology Neuroimmunology and Neuroinflammation - November 16, 2021 Category: Neurology Authors: Ringelstein, M., Ayzenberg, I., Lindenblatt, G., Fischer, K., Gahlen, A., Novi, G., Hayward-Könnecke, H., Schippling, S., Rommer, P. S., Kornek, B., Zrzavy, T., Biotti, D., Ciron, J., Audoin, B., Berthele, A., Giglhuber, K., Zephir, H., Kü Tags: All Demyelinating disease (CNS), Devic's syndrome, Optic neuritis; see Neuro-ophthalmology/Optic Nerve, Transverse myelitis Article Source Type: research

Analysis of Macrophages and Peptidergic Fibers in the Skin of Patients With Painful Diabetic Polyneuropathy
This study examined the possible skin markers of painful diabetic polyneuropathy (P-DPN): macrophages, substance P (SP), and calcitonin gene-related peptide (CGRP). Methods The participants were included from a large Danish cross-sectional clinical study of type 2 diabetes. We diagnosed definite diabetic polyneuropathy using the Toronto criteria and used the Neuropathic Pain Special Interest Group classification for defining P-DPN. We included 60 skin biopsies from patients with diabetic polyneuropathy—30 with P-DPN and 30 with nonpainful diabetic polyneuropathy (NP-DPN)—and 30 biopsies from healthy controls o...
Source: Neurology Neuroimmunology and Neuroinflammation - November 11, 2021 Category: Neurology Authors: Gylfadottir, S. S., Itani, M., Kristensen, A. G., Tankisi, H., Jensen, T. S., Sindrup, S. H., Bennett, D., Nyengaard, J. R., Finnerup, N. B., Karlsson, P. Tags: Peripheral neuropathy, Neuropathic pain Article Source Type: research

Impact of Dietary Intervention on Serum Neurofilament Light Chain in Multiple Sclerosis
We examined sNfL levels of 40 participants at baseline and at the end of the study after 6 months using single molecule array assay. Results sNfL levels were investigated in 9 controls, 14 participants on CR, and 17 participants on AKD. Correlation analysis showed an association of sNfL with age and disease duration; an association was also found between sNfL and the Multiple Sclerosis Functional Composite. AKD significantly reduced sNfL levels at 6 months compared with the common diet group (p = 0.001). Discussion For clinical or study use, consider that AKD may incline sNfL levels independent of relapse activity up to ...
Source: Neurology Neuroimmunology and Neuroinflammation - November 11, 2021 Category: Neurology Authors: Bock, M., Steffen, F., Zipp, F., Bittner, S. Tags: Clinical trials Randomized controlled (CONSORT agreement), Class II, Multiple sclerosis Article Source Type: research

Nicolau Syndrome After Glatiramer Acetate Injection in Close Proximity to Administration of SARS-CoV-2 mRNA Vaccine
(Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - November 10, 2021 Category: Neurology Authors: Sy, M. Y., Fromm, E., Doan, L., Rojek, N., Brandt, A. U. Tags: Multiple sclerosis Clinical/Scientific Note Source Type: research

Decrease in Serum Anti-MAG Autoantibodies Is Associated With Therapy Response in Patients With Anti-MAG Neuropathy: Retrospective Study
The objective of the retrospective analysis was to test the hypothesis that changes in serum anti-myelin-associated glycoprotein (MAG) autoantibodies are associated with clinical response to immunotherapy in patients with anti-MAG neuropathy. Methods As of January 29, 2020, we used anti-myelin-associated glycoprotein-related search strings in the Medline database to identify studies that provided information on anti-MAG immunoglobulin M (IgM) autoantibodies and clinical outcomes during immunotherapies. The relative change in anti-MAG IgM titers, paraprotein levels, or total IgM was determined before, during, or posttreatm...
Source: Neurology Neuroimmunology and Neuroinflammation - November 10, 2021 Category: Neurology Authors: Hänggi, P., Aliu, B., Martin, K., Herrendorff, R., Steck, A. J. Tags: All Immunology, Peripheral neuropathy Article Source Type: research

B Cells in the CNS at Postmortem Are Associated With Worse Outcome and Cell Types in Multiple Sclerosis
Background and Objectives To define the clinical and pathologic correlations of compartmentalized perivascular B cells in postmortem progressive multiple sclerosis (MS) brains. Methods Brain slices were acquired from 11 people with secondary progressive (SP) MS, 5 people with primary progressive (PP) MS, and 4 controls. Brain slices were immunostained for B lymphocytes (CD20), T lymphocytes (CD3), cytotoxic T lymphocytes (CD8), neuronal neurofilaments (NF200), myelin (SMI94), macrophages/microglia (CD68 and IBA1), astrocytes (glial fibrillary acidic protein [GFAP]), and mitochondria (voltage-dependent anion channel and cy...
Source: Neurology Neuroimmunology and Neuroinflammation - November 10, 2021 Category: Neurology Authors: Moccia, M., Haider, L., Eshaghi, A., van de Pavert, S. H. P., Brescia Morra, V., Patel, A., Wheeler-Kingshott, C. A. M., Barkhof, F., Ciccarelli, O. Tags: Multiple sclerosis Article Source Type: research

Anti-C2 Antibody ARGX-117 Inhibits Complement in a Disease Model for Multifocal Motor Neuropathy
Discussion Binding of IgM antibodies from patients with MMN to iPSC-derived MNs induces complement activation. By expressing complement regulatory proteins, particularly CD59, MNs are protected against complement-mediated lysis. Yet, because of expressing C3aR, the function of these cells may be affected by complement activation upstream of membrane attack complex formation. ARGX-117 inhibits complement activation upstream of C3 in this disease model for MMN and therefore represents an intervention strategy to prevent harmful effects of complement in MMN. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - November 10, 2021 Category: Neurology Authors: Budding, K., Johansen, L. E., Van de Walle, I., Dijkxhoorn, K., de Zeeuw, E., Bloemenkamp, L. M., Bos, J. W., Jansen, M. D., Curial, C. A. D., Silence, K., de Haard, H., Blanchetot, C., Van de Ven, L., Leusen, J. H. W., Pasterkamp, R. J., van den Berg, L. Tags: All Immunology, All Neuromuscular Disease Article Source Type: research

Targeted RNA Sequencing of VZV-Infected Brain Vascular Adventitial Fibroblasts Indicates That Amyloid May Be Involved in VZV Vasculopathy
Discussion Gene expression profiles and pathway enrichment analysis of VZV-infected HBVAFs, as well as phenotypic studies, reveal features of pathologic vascular remodeling (e.g., increased cell migration and changes in the extracellular matrix) that can contribute to cerebrovascular disease. Furthermore, the discovery of amyloid-associated transcriptional pathways and intracellular amyloid deposition in HBVAFs raise the possibility that VZV vasculopathy is an amyloid disease. Amyloid deposition may contribute to cell death and loss of vascular wall integrity, as well as potentiate chronic inflammation in VZV vasculopathy,...
Source: Neurology Neuroimmunology and Neuroinflammation - November 10, 2021 Category: Neurology Authors: Bubak, A. N., Como, C. N., Hassell, J. E., Mescher, T., Frietze, S. E., Niemeyer, C. S., Cohrs, R. J., Nagel, M. A. Tags: Vasculitis, Viral infections, All Cerebrovascular disease/Stroke, All Cognitive Disorders/Dementia, Gene expression studies Article Source Type: research

Impact of COVID-19 in Immunosuppressed Children With Neuroimmunologic Disorders
Background and Objectives To investigate whether children receiving immunosuppressive therapies for neuroimmunologic disorders had (1) increased susceptibility to SARS-CoV2 infection or to develop more severe forms of COVID-19; (2) increased relapses or autoimmune complications if infected; and (3) changes in health care delivery during the pandemic. Methods Patients with and without immunosuppressive treatment were recruited to participate in a retrospective survey evaluating the period from March 14, 2020, to March 30, 2021. Demographics, clinical features, type of immunosuppressive treatment, suspected or confirmed COV...
Source: Neurology Neuroimmunology and Neuroinflammation - November 10, 2021 Category: Neurology Authors: Olive-Cirera, G., Fonseca, E., Cantarin-Extremera, V., Vazquez-Lopez, M., Jimenez-Legido, M., Gonzalez-Alvarez, V., Ribeiro-Constante, J., Camacho-Salas, A., Marti, I., Cancho-Candela, R., Martinez-Gonzalez, M. J., Saiz, A., Armangue, T., on behalf of the Tags: Autoimmune diseases, All Pediatric, COVID-19, All Demyelinating disease (CNS) Article Source Type: research

COVID-19 Severity in Multiple Sclerosis: Putting Data Into Context
Background and Objectives It is unclear how multiple sclerosis (MS) affects the severity of COVID-19. The aim of this study is to compare COVID-19–related outcomes collected in an Italian cohort of patients with MS with the outcomes expected in the age- and sex-matched Italian population. Methods Hospitalization, intensive care unit (ICU) admission, and death after COVID-19 diagnosis of 1,362 patients with MS were compared with the age- and sex-matched Italian population in a retrospective observational case-cohort study with population-based control. The observed vs the expected events were compared in the whole MS...
Source: Neurology Neuroimmunology and Neuroinflammation - November 9, 2021 Category: Neurology Authors: Sormani, M. P., Schiavetti, I., Carmisciano, L., Cordioli, C., Filippi, M., Radaelli, M., Immovilli, P., Capobianco, M., De Rossi, N., Brichetto, G., Cocco, E., Scandellari, C., Cavalla, P., Pesci, I., Zito, A., Confalonieri, P., Marfia, G. A., Perini, P. Tags: COVID-19, Multiple sclerosis, Incidence studies, Risk factors in epidemiology Article Source Type: research

COVID-19 Vaccination Reactogenicity in Persons With Multiple Sclerosis
The objective of this retrospective cross-sectional study was to generate real-world multiple sclerosis–specific vaccine safety information, particularly in the context of specific DMTs, and provide information to mitigate specific concerns in vaccine hesitant PwMS. Methods Between 3/2021 and 6/2021, participants in iConquerMS, an online people-powered research network, reported SARS-CoV-2 vaccines, experiences of local (itch, pain, redness, swelling, or warmth at injection site) and systemic (fever, chills, fatigue, headache, joint pain, malaise, muscle ache, nausea, allergic, and other) reactions within 24 hours (...
Source: Neurology Neuroimmunology and Neuroinflammation - November 9, 2021 Category: Neurology Authors: Briggs, F. B. S., Mateen, F. J., Schmidt, H., Currie, K. M., Siefers, H. M., Crouthamel, S., Bebo, B. F., Fiol, J., Racke, M. K., O'Connor, K. C., Kolaczkowski, L. G., Klein, P., Loud, S., McBurney, R. N. Tags: Public health, Multiple sclerosis, All epidemiology Article Source Type: research

Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy
Background and Objectives To study the clinical and laboratory features of antineurofascin-155 (NF155)–positive autoimmune nodopathy (AN). Methods Patients with anti-NF155 antibodies detected on routine immunologic testing were included. Clinical characteristics, treatment response, and functional scales (modified Rankin Scale [mRS] and Inflammatory Rasch-built Overall Disability Scale [I-RODS]) were retrospectively collected at baseline and at the follow-up. Autoantibody and neurofilament light (NfL) chain levels were analyzed at baseline and at the follow-up. Results Forty NF155+ patients with AN were included. M...
Source: Neurology Neuroimmunology and Neuroinflammation - November 2, 2021 Category: Neurology Authors: Martin-Aguilar, L., Lleixa, C., Pascual-Goni, E., Caballero-Avila, M., Martinez-Martinez, L., Diaz-Manera, J., Rojas-Garcia, R., Cortes-Vicente, E., Turon-Sans, J., de Luna, N., Suarez-Calvet, X., Gallardo, E., Rajabally, Y., Scotton, S., Jacobs, B. C., B Tags: Chronic inflammatory demyelinating polyneuropathy, Class IV Article Source Type: research

CCR5 Blockade in Inflammatory PML and PML-IRIS Associated With Chronic Inflammatory Diseases' Treatments
This study provides Class IV evidence showing that adding maraviroc to the management of iatrogenic iPML/PML-IRIS does not improve the outcome. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - November 2, 2021 Category: Neurology Authors: Bernard-Valnet, R., Moisset, X., Maubeuge, N., Lefebvre, M., Ouallet, J.-C., Roumier, M., Lebrun-Frenay, C., Ciron, J., Biotti, D., Clavelou, P., Godeau, B., Du Pasquier, R. A., Martin-Blondel, G. Tags: Viral infections, Class IV, Multiple sclerosis Article Source Type: research

GRP78 Antibodies Are Associated With Blood-Brain Barrier Breakdown in Anti-Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disorder
Discussion GRP78 antibodies may be associated with BBB dysfunction in MOG-Ab–associated disorder. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - November 1, 2021 Category: Neurology Authors: Shimizu, F., Ogawa, R., Mizukami, Y., Watanabe, K., Hara, K., Kadono, C., Takahashi, T., Misu, T., Takeshita, Y., Sano, Y., Fujisawa, M., Maeda, T., Nakashima, I., Fujihara, K., Kanda, T. Tags: Autoimmune diseases, All Demyelinating disease (CNS) Article Source Type: research

Myelin Oligodendrocyte Glycoprotein-Immunoglobulin G in the CSF: Clinical Implication of Testing and Association With Disability
This study provides Class II evidence that the presence of CSF MOG-IgG can improve the diagnosis of MOGAD in the absence of an MS phenotype, and intrathecal synthesis of MOG-IgG was associated with increased disability. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - October 28, 2021 Category: Neurology Authors: Kwon, Y. N., Kim, B., Kim, J.-S., Mo, H., Choi, K., Oh, S.-i., Kim, J.-E., Nam, T.-S., Sohn, E. H., Heo, S. H., Kim, S. B., Park, K.-C., Yoon, S. S., Oh, J., Baek, S.-H., Kim, B.-J., Park, K. S., Sung, J.-J., Jung, J. H., Kim, S.-J., Park, S.-H., Waters, Tags: Class II, All Demyelinating disease (CNS) Article Source Type: research

CSF Findings in Acute NMDAR and LGI1 Antibody-Associated Autoimmune Encephalitis
Discussion NMDAR-E and LGI-E differ in their typical extent of CSF inflammation. In addition, the patterns formed by the different inflammatory CSF parameters and their relationship with disease severity, age, and disease duration are subtype-characteristic. Moreover, signs for multiple sclerosis-like chronic inflammation are present in a subgroup of patients with NMDAR-E. These CSF patterns might be markers for the different immunopathogeneses of LGI1-E and NMDAR-E. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - October 25, 2021 Category: Neurology Authors: Dürr, M., Nissen, G., Sühs, K.-W., Schwenkenbecher, P., Geis, C., Ringelstein, M., Hartung, H.-P., Friese, M. A., Kaufmann, M., Malter, M. P., Madlener, M., Thaler, F. S., Kümpfel, T., Senel, M., Häusler, M. G., Schneider, Tags: Autoimmune diseases, Encephalitis Article Source Type: research

Evaluation of Age-Dependent Immune Signatures in Patients With Multiple Sclerosis
Discussion Our data illustrate that aging in MS is associated with a dysbalance between costimulatory and immunoregulatory signals provided by CD8 T cells favoring a proinflammatory phenotype and, more importantly, a pattern of premature immune aging in the CD8 T-cell compartment of young patients with MS with potential implications for disease severity. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - October 19, 2021 Category: Neurology Authors: Eschborn, M., Pawlitzki, M., Wirth, T., Nelke, C., Pfeuffer, S., Schulte-Mecklenbeck, A., Lohmann, L., Rolfes, L., Pape, K., Eveslage, M., Bittner, S., Gross, C. C., Ruck, T., Wiendl, H., Meuth, S. G., Klotz, L. Tags: Autoimmune diseases, Multiple sclerosis Article Source Type: research

New BBB Model Reveals That IL-6 Blockade Suppressed the BBB Disorder, Preventing Onset of NMOSD
Discussion These results suggest that (1) our triple-cultured in vitro and in ex vivo BBB models are ideal for evaluating barrier function, leukocyte transmigration, and intracerebral transferability; (2) NMO-IgG increased the intracerebral transferability of NMO-IgG via decreasing barrier function and induced secretion of IL-6 from astrocytes causing more dysfunction of the barrier and disrupting controlled cellular infiltration; and (3) satralizumab, which can pass through the BBB in the presence of NMO-IgG, suppresses the BBB dysfunction and the infiltration of inflammatory cells, leading to prevention of onset of NMOSD...
Source: Neurology Neuroimmunology and Neuroinflammation - October 19, 2021 Category: Neurology Authors: Takeshita, Y., Fujikawa, S., Serizawa, K., Fujisawa, M., Matsuo, K., Nemoto, J., Shimizu, F., Sano, Y., Tomizawa-Shinohara, H., Miyake, S., Ransohoff, R. M., Kanda, T. Tags: Autoimmune diseases, Devic's syndrome Article Source Type: research

CNS Involvement in Chronic Inflammatory Demyelinating Polyneuropathy: Subtle Retinal Changes in Optical Coherence Tomography
Discussion Our data reveal subtle retinal neurodegeneration in patients with CIDP, providing evidence for visual pathway involvement, detectable by OCT. The results need corroboration in independent, larger cohorts. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - October 19, 2021 Category: Neurology Authors: Ingwersen, J., Graf, J., Kluge, J., Weise, M., Dietrich, M., Lee, J.-I., Harmel, J., Hartung, H.-P., Ruck, T., Meuth, S. G., Albrecht, P., Aktas, O., Ringelstein, M. Tags: Peripheral neuropathy, Chronic inflammatory demyelinating polyneuropathy, All Demyelinating disease (CNS) Article Source Type: research

Primary Angiitis of the CNS: A Systematic Review and Meta-analysis
Discussion PACNS is associated with disabling symptoms, frequent relapses, and significant mortality. Differences in symptoms and neuroimaging results and low overlap between biopsy and angiogram suggest that biopsy- and angiogram-confirmed cases represent different histopathologic types of PACNS. The optimal treatment is unknown. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - October 18, 2021 Category: Neurology Authors: Beuker, C., Strunk, D., Rawal, R., Schmidt-Pogoda, A., Werring, N., Milles, L., Ruck, T., Wiendl, H., Meuth, S., Minnerup, H., Minnerup, J. Tags: Vasculitis, All Cerebrovascular disease/Stroke Views [amp ] Reviews Source Type: research

Neuromyelitis Optica Spectrum Disorders in Africa: A Systematic Review
Discussion The majority of studies on NMOSD from the African continent are retrospective, and most countries do not report any data. Our systemic review shows that data derived from patients living in Africa correspond well to what has been previously published in meta-analyses on patients of African ancestry with NMOSD who live outside of Africa, except for a younger age at onset and a lower proportion of females. We advocate for systematic data collection to adequately capture and monitor the burden of NMOSD, for expansion of research efforts and facilities to perform fundamental and clinical research, and for improved a...
Source: Neurology Neuroimmunology and Neuroinflammation - October 18, 2021 Category: Neurology Authors: Musubire, A. K., Derdelinckx, J., Reynders, T., Meya, D. B., Bohjanen, P. R., Cras, P., Willekens, B. Tags: All global neurology, Underserved populations, Devic's syndrome, Transverse myelitis, All epidemiology Views [amp ] Reviews Source Type: research

Safety and Immune Effects of Blocking CD40 Ligand in Multiple Sclerosis
This study provides Class IV evidence on the safety, pharmacokinetics, and immune effects of an mAb to CD40L in patients with RRMS. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - October 15, 2021 Category: Neurology Authors: Fadul, C. E., Mao-Draayer, Y., Ryan, K. A., Noelle, R. J., Wishart, H. A., Channon, J. Y., Kasper, I. R., Oliver, B., Mielcarz, D. W., Kasper, L. H. Tags: All Immunology, Autoimmune diseases, All Clinical Neurology, All Clinical trials, Multiple sclerosis Article Source Type: research

Teriflunomide Promotes Oligodendroglial 8,9-Unsaturated Sterol Accumulation and CNS Remyelination
Discussion TF enhances zymosterol accumulation in oligodendrocytes and CNS myelin repair, a beneficial off-target effect that should be investigated in patients with multiple sclerosis. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - October 12, 2021 Category: Neurology Authors: Martin, E., Aigrot, M.-S., Lamari, F., Bachelin, C., Lubetzki, C., Oumesmar, B. N., Zalc, B., Stankoff, B. Tags: Multiple sclerosis Article Source Type: research

Autopsy Case of Meningoencephalomyelitis Associated With Glial Fibrillary Acidic Protein Antibody
Discussion This case illustrates autoimmune meningoencephalomyelitis associated with GFAP antibody in the CSF and coexistent neuroendocrine tumor. The autopsy findings were nonspecific and did not demonstrate astrocyte involvement. Further accumulation of cases is warranted to delineate the utility and pathogenic significance of the GFAP autoantibody. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - October 12, 2021 Category: Neurology Authors: Yamakawa, M., Hogan, K. O., Leever, J., Jassam, Y. N. Tags: All Immunology, Autoimmune diseases, Paraneoplastic syndrome Article Source Type: research

Neurofilament Light in CSF and Plasma Is a Marker of Neuronal Damage in HTLV-1-Associated Myelopathy and Correlates With Neuroinflammation
Background and Objectives To evaluate the usefulness of CSF and plasma neurofilament light (Nf-L) as a biomarker for human T-cell lymphotropic virus type 1 (HTLV-1)-associated myelopathy (HAM). Methods Nf-L, CXCL10, and neopterin were measured by ELISA in 83 CSF samples obtained from 49 individuals living with HTLV-1/2. Plasma Nf-L was also measured by single molecule array. Results were correlated with duration of disease, age, mobility, CSF cell counts, CSF protein, and HTLV-1 proviral load. Results Nf-L was detected in all CSF samples (median [range] = 575 [791.8–2,349] pg/mL) and positively correlated with mark...
Source: Neurology Neuroimmunology and Neuroinflammation - October 5, 2021 Category: Neurology Authors: Rosadas, C., Zetterberg, H., Heslegrave, A., Haddow, J., Borisova, M., Taylor, G. P. Tags: Viral infections, Cerebrospinal Fluid Article Source Type: research

Neurosarcoidosis: Pathophysiology, Diagnosis, and Treatment
Although often regarded as a protean illness with myriad clinical and imaging manifestations, neurosarcoidosis typically presents as recognizable syndromes that can be approached in a rational, systematic fashion. Understanding of neurosarcoidosis has progressed significantly in recent years, including updated diagnostic criteria and advances in treatment. The diagnosis of neurosarcoidosis is established by the clinical syndrome, imaging and histopathological findings, and exclusion of other causes. Mounting evidence supports the use of tumor necrosis factor inhibitors as an important addition to the therapeutic armamentar...
Source: Neurology Neuroimmunology and Neuroinflammation - October 4, 2021 Category: Neurology Authors: Bradshaw, M. J., Pawate, S., Koth, L. L., Cho, T. A., Gelfand, J. M. Tags: Optic neuritis; see Neuro-ophthalmology/Optic Nerve, Transverse myelitis Views [amp ] Reviews Source Type: research