Free light chains kappa can differentiate between myelitis and noninflammatory myelopathy
Conclusions With the hyperbolic reference range in quotient diagrams for FLC-k, it is possible to distinguish inflammatory myelitis from noninflammatory myelopathies. An FLC-k IF>78% can be a hint to suspect myelitis due to MS rather than NMOSD. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - September 18, 2020 Category: Neurology Authors: Süsse, M., Feistner, F., Grothe, M., Nauck, M., Dressel, A., Hannich, M. J. Tags: All Spinal Cord, Cerebrospinal Fluid, Multiple sclerosis, Devic's syndrome Article Source Type: research

Bickerstaff brainstem encephalitis with or without anti-GQ1b antibody
Conclusions BBE with anti-GQ1b antibody has homogeneous features. IVIG is the treatment used prevalently for BBE with anti-GQ1b antibody in Japan. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - September 15, 2020 Category: Neurology Authors: Yoshikawa, K., Kuwahara, M., Morikawa, M., Kusunoki, S. Tags: Autoimmune diseases, Guillain-Barre syndrome Article Source Type: research

A case of CIDP concurrent with MGUS IgG kappa responsive to autologous stem cell transplantation
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired immune-mediated disease of the peripheral nervous system, either being isolated or associated with other systemic diseases, most frequently with lymphoproliferative subtypes.1 CIDP concurrent with monoclonal gammopathy of undetermined significance (MGUS) accounts for up to 20%–30% of all cases,2 is often considered clinically indistinguishable from typical CIDP, and responds similarly to immunologic therapies.3 CIDP clinical course may be poorly responsive to conventional immunologic or chemotherapy treatment.4 Consequently, allogeneic an...
Source: Neurology Neuroimmunology and Neuroinflammation - September 15, 2020 Category: Neurology Authors: Colucci, G., Pabst, T., Bacher, U., Maggioli, C., Zecca, C., Gobbi, C. Tags: Chronic inflammatory demyelinating polyneuropathy Clinical/Scientific Notes Source Type: research

Is CSF neurofilament light chain measurement relevant for MS?
MS is a chronic inflammatory demyelinating disease of the CNS in which an autoimmune etiology targeting CNS myelin is implicated. Tremendous efforts to search for biomarkers that potentially reflect the inflammatory process in the CNS have been made, although MRI was the only reliable clinical method for that purpose until recent findings showing the usefulness of the measurement of neurofilaments. Neurofilament release is assumed to be a consequence of axonal damage, with some components appearing in the CSF and then in blood at extremely low concentrations.1 An ELISA for CSF neurofilament light chain protein (NfL) has sh...
Source: Neurology Neuroimmunology and Neuroinflammation - September 14, 2020 Category: Neurology Authors: Matsui, M. Tags: All Clinical Neurology, All Demyelinating disease (CNS), Multiple sclerosis Editorial Source Type: research

MRI activity in MS and completed pregnancy: Data from a tertiary academic center
Conclusions There was a high level of inflammatory radiographic disease activity which was related to relapses in postpartum patients with MS. Further studies are needed to determine whether hormonal fluctuations vs extended time off DMTs may be the underlying cause of our observations. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - September 11, 2020 Category: Neurology Authors: Houtchens, M., Bove, R., Healy, B., Houtchens, S., Kaplan, T. B., Mahlanza, T., Chitnis, T., Bakshi, R. Tags: MRI, Multiple sclerosis Article Source Type: research

Epidemiology of paraneoplastic neurologic syndromes and autoimmune encephalitides in France
Conclusions There was a significant widespread year-to-year increase in the incidence of diagnoses registered with the Reference Center for all subgroups of PNS and AE studied. The national observed incidence rate is likely underestimated due to underdiagnosis and underreporting. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - August 26, 2020 Category: Neurology Authors: Hebert, J., Riche, B., Vogrig, A., Muniz-Castrillo, S., Joubert, B., Picard, G., Rogemond, V., Psimaras, D., Alentorn, A., Berzero, G., Desestret, V., Rabilloud, M., Honnorat, J. Tags: Autoimmune diseases, Encephalitis, Paraneoplastic syndrome, Incidence studies Article Source Type: research

CSF neurofilament light chain testing as an aid to determine treatment strategies in MS
Objective To evaluate the use of CSF neurofilament light chain (NfL) measurements in clinical practice as well as their effect on treatment strategies and outcomes in patients with MS. Methods This was an observational cohort study of patients with MS who had a CSF NfL measurement between December 2015 and July 2018 as part of their routine clinical care. Treatment strategies were classified as "No Treatment/No Escalation" (no treatment or no escalation of treatment) or "Treatment/Escalation" (first-line injectable/oral disease-modifying therapies (DMTs), highly active DMTs, or treatment escalation). C...
Source: Neurology Neuroimmunology and Neuroinflammation - August 21, 2020 Category: Neurology Authors: Reyes, S., Smets, I., Holden, D., Carrillo-Loza, K., Christmas, T., Bianchi, L., Ammoscato, F., Turner, B., Marta, M., Schmierer, K., Giovannoni, G., Gnanapavan, S. Tags: All Clinical Neurology, All Demyelinating disease (CNS), Multiple sclerosis Article Source Type: research

N2 in the time of COVID-19
This issue of Neurology® Neuroimmunology & Neuroinflammation reflects the impact of the COVID-19 pandemic with 15 studies related to inflammatory or autoimmune neurologic complications in patients with this disease, including Guillain-Barré syndrome, CNS inflammatory disorders, and several studies addressing the risk of COVID-19 in patients with neuromyelitis optica spectrum disorders (NMOSD) or multiple sclerosis (MS). Among the latter, a study conducted in New York by Parotta et al.1 included 72 patients with MS (55 relapsing and 17 progressive) or related disorders (4 patients) and confirmed (37 patients)...
Source: Neurology Neuroimmunology and Neuroinflammation - August 20, 2020 Category: Neurology Authors: Dalmau, J. Tags: Editor ' s Corner Source Type: research

Interleukin-6 in neuromyelitis optica spectrum disorder pathophysiology
Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disorder that preferentially affects the spinal cord and optic nerve. Most patients with NMOSD experience severe relapses that lead to permanent neurologic disability; therefore, limiting frequency and severity of these attacks is the primary goal of disease management. Currently, patients are treated with immunosuppressants. Interleukin-6 (IL-6) is a pleiotropic cytokine that is significantly elevated in the serum and the CSF of patients with NMOSD. IL-6 may have multiple roles in NMOSD pathophysiology by promoting plasmablast survival, stimulating the pr...
Source: Neurology Neuroimmunology and Neuroinflammation - August 20, 2020 Category: Neurology Authors: Fujihara, K., Bennett, J. L., de Seze, J., Haramura, M., Kleiter, I., Weinshenker, B. G., Kang, D., Mughal, T., Yamamura, T. Tags: Autoimmune diseases, Optic nerve, All Spinal Cord, Cerebrospinal Fluid, Devic's syndrome Views [amp ] Reviews Source Type: research

Phenome-wide examination of comorbidity burden and multiple sclerosis disease severity
Conclusion The phenome-wide approach enabled a systematic interrogation of the comorbidity burden and highlighted clinically relevant medical conditions associated with MS severity (beyond MS-specific consequences) and defines a roadmap for comprehensive investigation of comorbidities in chronic neurologic diseases. Further prospective investigation of the bidirectional relationship between disability and comorbidities could inform the individualized patient management. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - August 19, 2020 Category: Neurology Authors: Zhang, T., Goodman, M., Zhu, F., Healy, B., Carruthers, R., Chitnis, T., Weiner, H., Cai, T., De Jager, P., Tremlett, H., Xia, Z. Tags: Electronic medical records, Multiple sclerosis Article Source Type: research

EBV-specific CD8 T lymphocytes and B cells during glatiramer acetate therapy in patients with MS
Objective Infection with Epstein-Barr virus (EBV) has been associated with clinical activity and risk of developing MS. The purpose of this study is to investigate the impact of glatiramer acetate (GA) therapy on EBV-specific immune responses and disease course. Methods We characterized EBV-specific CD8 T lymphocytes and B cells during disease-modifying treatments in 2 groups of patients with MS. We designed a 2-pronged approach consisting of a cross-sectional study (39 untreated patients, 38 patients who had undergone 12 months of GA treatment, and 48 healthy donors compatible for age and sex with the patients with MS) a...
Source: Neurology Neuroimmunology and Neuroinflammation - August 17, 2020 Category: Neurology Authors: Guerrera, G., Ruggieri, S., Picozza, M., Piras, E., Gargano, F., Placido, R., Gasperini, C., Salvetti, M., Buscarinu, M. C., Battistini, L., Borsellino, G., Angelini, D. F. Tags: Medical care, All Immunology, Autoimmune diseases, All Clinical Neurology, Multiple sclerosis Article Source Type: research

Cladribine vs other drugs in MS: Merging randomized trial with real-life data
In this study, the effectiveness of cladribine vs other approved drugs in patients with relapsing-remitting MS (RRMS) was compared by matching RCT to observational data. Methods Data from the pivotal trial assessing cladribine tablets vs placebo (CLARITY) were propensity score matched to data from the Italian multicenter database i-MuST. This database included 3,150 patients diagnosed between 2010 and 2018 at 24 Italian MS centers who started a disease-modifying drug. The annualized relapse rate (ARR) over 2 years from treatment start and the 24-week confirmed disability progression were compared between patients treated ...
Source: Neurology Neuroimmunology and Neuroinflammation - August 14, 2020 Category: Neurology Authors: Signori, A., Sacca, F., Lanzillo, R., Maniscalco, G. T., Signoriello, E., Repice, A. M., Annovazzi, P., Baroncini, D., Clerico, M., Binello, E., Cerqua, R., Mataluni, G., Perini, P., Bonavita, S., Lavorgna, L., Zarbo, I. R., Laroni, A., Pareja-Gutierrez, Tags: Class III, Multiple sclerosis Article Source Type: research

Lymphocyte recovery after fingolimod discontinuation in patients with MS
Conclusions Prolonged lymphopenia after fingolimod cessation exists in a subgroup of patients with MS and should be considered in clinical practice, particularly when changing treatment regimens. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - August 14, 2020 Category: Neurology Authors: Nagy, S., Kuhle, J., Derfuss, T. Tags: All Clinical Neurology, All Demyelinating disease (CNS), Multiple sclerosis Article Source Type: research

EBNA-1 titer gradient in families with multiple sclerosis indicates a genetic contribution
Conclusions In families with MS, the EBNA-1 IgG gradient being the highest in patients with MS, intermediate in their siblings, and lowest in biologically unrelated spouses indicates a genetic contribution to EBNA-1 IgG levels that is only partially explained by HLA-DRB1*1501 carriership. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - August 13, 2020 Category: Neurology Authors: Mescheriakova, J. Y., van Nierop, G. P., van der Eijk, A. A., Kreft, K. L., Hintzen, R. Q. Tags: Article Source Type: research

Long-term prognostic value of longitudinal measurements of blood neurofilament levels
Objective To assess the long-term prognostic value of an integral of longitudinal measurements of plasma neurofilament light chain levels (NfLlong) over 12 and 24 months vs single neurofilament light chain (NfL) measurements in patients with relapsing-remitting MS (RRMS) and its additional value when combined with clinical and MRI measures. Methods This analysis included continuously fingolimod-treated patients with RRMS from the 24-month FTY720 Research Evaluating Effects of Daily Oral therapy in Multiple Sclerosis (FREEDOMS)/12-month Trial Assessing Injectable Interferon vs FTY720 Oral in Relapsing–Remitting Multi...
Source: Neurology Neuroimmunology and Neuroinflammation - August 12, 2020 Category: Neurology Authors: Häring, D. A., Kropshofer, H., Kappos, L., Cohen, J. A., Shah, A., Meinert, R., Leppert, D., Tomic, D., Kuhle, J. Tags: Multiple sclerosis Article Source Type: research

Autoimmunity complicating SARS-CoV-2 infection in selective IgA-deficiency
(Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - August 12, 2020 Category: Neurology Authors: Pfeuffer, S., Pawlowski, M., Joos, G. S., Minnerup, J., Meuth, S. G., Dziewas, R., Wiendl, H. Tags: All Immunology, Endocrine, Guillain-Barre syndrome, Critical care, COVID-19 Clinical/Scientific Notes Source Type: research

Immune profiling of plasma-derived extracellular vesicles identifies Parkinson disease
Conclusions Immune profiling of plasmatic EVs represents a crucial step toward the identification of biomarkers of disease for PD and AP. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - August 12, 2020 Category: Neurology Authors: Vacchi, E., Burrello, J., Di Silvestre, D., Burrello, A., Bolis, S., Mauri, P., Vassalli, G., Cereda, C. W., Farina, C., Barile, L., Kaelin-Lang, A., Melli, G. Tags: Parkinson's disease/Parkinsonism Article Source Type: research

Progressive multifocal leukoencephalopathy and sarcoidosis under interleukin 7: The price of healing
Conclusions New targeted therapies in immunology and infectiology open the doors of more specific and more specialized therapies for patients with immunodeficiencies, autoimmune diseases, or cancers. However, before instauration of these treatments, the risk of immune reconstitution inflammatory syndrome and potential exacerbation of an underlying disease must be considered. It is particularly true in case of autoimmune disease such as sarcoidosis or lupus. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - August 11, 2020 Category: Neurology Authors: Guffroy, A., Solis, M., Gies, V., Dieudonne, Y., Kuhnert, C., Lenormand, C., Kremer, L., Molitor, A., Carapito, R., Hansmann, Y., Poindron, V., Martin, T., Hirschi, S., Korganow, A.-S. Tags: MRI, Autoimmune diseases, Viral infections, Aphasia, All Demyelinating disease (CNS) Article Source Type: research

Interleukin-26, preferentially produced by TH17 lymphocytes, regulates CNS barrier function
Conclusions Our study demonstrates that although IL-26 is preferentially expressed by TH17 lymphocytes, it promotes BBB integrity in vitro and in vivo and is protective in chronic EAE, highlighting the functional diversity of cytokines produced by TH17 lymphocytes. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - August 11, 2020 Category: Neurology Authors: Broux, B., Zandee, S., Gowing, E., Charabati, M., Lecuyer, M.-A., Tastet, O., Hachehouche, L., Bourbonniere, L., Ouimet, J.-P., Lemaitre, F., Larouche, S., Cayrol, R., Bouthillier, A., Moumdjian, R., Lahav, B., Poirier, J., Duquette, P., Arbour, N., Peele Tags: All Immunology, Autoimmune diseases, Multiple sclerosis Article Source Type: research

Regional microglial activation in the substantia nigra is linked with fatigue in MS
Objective The goal of our study is to assess the role of microglial activation in MS-associated fatigue (MSAF) using [F-18]PBR06-PET. Methods Fatigue severity was measured using the Modified Fatigue Impact Scale (MFIS) in 12 subjects with MS (7 relapsing-remitting and 5 secondary progressive) and 10 healthy control participants who underwent [F-18]PBR06-PET. The MFIS provides a total fatigue score as well as physical, cognitive, and psychosocial fatigue subscale scores. Standardized Uptake Value (SUV) 60–90 minute frame PET maps were coregistered to 3T MRI. Voxel-by-voxel analysis using Statistical Parametric Mappin...
Source: Neurology Neuroimmunology and Neuroinflammation - August 7, 2020 Category: Neurology Authors: Singhal, T., Cicero, S., Pan, H., Carter, K., Dubey, S., Chu, R., Glanz, B., Hurwitz, S., Tauhid, S., Park, M.-A., Kijewski, M., Stern, E., Bakshi, R., Silbersweig, D., Weiner, H. L. Tags: PET, Multiple sclerosis Article Source Type: research

Guillain-Barre syndrome and chronic inflammatory demyelinating polyradiculoneuropathy after alemtuzumab therapy in kidney transplant recipients
(Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - August 7, 2020 Category: Neurology Tags: Correction Source Type: research

Mitigating alemtuzumab-associated autoimmunity in MS: A "whack-a-mole" B-cell depletion strategy
Conclusions An anti-CD20 "whack-a-mole" B-cell depletion strategy may serve to mitigate alemtuzumab-associated secondary autoimmunity in MS by reducing the imbalance in B- and T-cell regulatory networks during immune reconstitution. We believe that these observations warrant further investigation. Classification of evidence This study provides Class IV evidence that for people with MS, low-dose rituximab following alemtuzumab treatment decreases the risk of alemtuzumab-associated secondary autoimmune diseases. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - August 7, 2020 Category: Neurology Authors: Meltzer, E., Campbell, S., Ehrenfeld, B., Cruz, R. A., Steinman, L., Parsons, M. S., Zamvil, S. S., Frohman, E. M., Frohman, T. C. Tags: All Immunology, Autoimmune diseases, All Clinical trials, All Demyelinating disease (CNS), Multiple sclerosis Article Source Type: research

Validation of the NEOS score in Chinese patients with anti-NMDAR encephalitis
Objective The performance of anti-NMDAR Encephalitis One-Year Functional Status (NEOS) in predicting the 1-year functional status in Chinese patients with anti-NMDAR encephalitis is unknown. Methods We recruited patients with anti-NMDAR encephalitis from the Multicenter and Prospective Clinical Registry Study of Anti-NMDAR Encephalitis in Beijing Area. Patients were followed up for 1 year. We defined the poor functional status as a modified Rankin Scale score of more than 2 and good functional status as a modified Rankin Scale score of no more than 2. We performed a receiver-operator characteristic analysis to assess the ...
Source: Neurology Neuroimmunology and Neuroinflammation - August 5, 2020 Category: Neurology Authors: Peng, Y., Dai, F., Liu, L., Chen, W., Yan, H., Liu, A., Zhang, X., Wang, X., He, J., Li, Y., Li, C., Chen, L., Zhao, Y., Li, L., Ma, Q., Wang, J. Tags: Autoimmune diseases, Encephalitis, Prognosis, Clinical trials Observational study (Cohort, Case control), Class III Article Source Type: research

High-throughput investigation of molecular and cellular biomarkers in NMOSD
Conclusions Integrative analyses of cytokines, chemokines, and immune cells in participants with NMOSD and HCs provide congruence with previously identified biomarkers of NMOSD and highlight CD16+CD56+ NK cells and CX3CL1 as potential novel biomarker candidates. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - August 4, 2020 Category: Neurology Authors: Yandamuri, S. S., Jiang, R., Sharma, A., Cotzomi, E., Zografou, C., Ma, A. K., Alvey, J. S., Cook, L. J., Smith, T. J., Yeaman, M. R., O'Connor, K. C., On behalf of the Guthy-Jackson Charitable Foundation CIRCLES Study Group Tags: Autoimmune diseases Article Source Type: research

Tailoring B cell depletion therapy in MS according to memory B cell monitoring
Conclusion The results of this study show that the memory B cell–based RTX reinfusion protocol is able to reduce the mean number of RTX reinfusions with persistent reduction of disease activity. Classification of evidence This study provides Class IV evidence that for patients with MS, a memory B cell–based RTX reinfusion protocol can reduce the mean number of RTX reinfusions with persistent reduction of disease activity. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - August 4, 2020 Category: Neurology Authors: Novi, G., Bovis, F., Fabbri, S., Tazza, F., Gazzola, P., Maietta, I., Curro, D., Bruschi, N., Roccatagliata, L., Boffa, G., Lapucci, C., Pesce, G., Cellerino, M., Solaro, C., Laroni, A., Capello, E., Mancardi, G., Sormani, M., Inglese, M., Uccelli, A. Tags: All Immunology, Class IV, Multiple sclerosis Article Source Type: research

Effect of the sphingosine-1-phosphate receptor modulator ozanimod on leukocyte subtypes in relapsing MS
Conclusion Ozanimod induced dose-dependent reductions in circulating B- and T-cell counts and differential effects on naive and memory CD4+ and CD8+ T cells and CD19+ B cells. Data characterized with both a novel epigenetic cell-counting method and flow cytometry support ozanimod's MOA. Clinical trial registration: clinicaltrials.gov NCT02797015. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 31, 2020 Category: Neurology Authors: Harris, S., Tran, J. Q., Southworth, H., Spencer, C. M., Cree, B. A. C., Zamvil, S. S. Tags: All Neuropsychology/Behavior Article Source Type: research

Treatment and outcome of aquaporin-4 antibody-positive NMOSD: A multinational pediatric study
Objective To describe the clinical phenotypes, treatment response, and outcome of children with antibodies against aquaporin-4 (AQP4-Ab) neuromyelitis optica spectrum disorder (NMOSD). Methods Retrospective, multicenter, and multinational study of patients with AQP4-Ab NMOSD aged
Source: Neurology Neuroimmunology and Neuroinflammation - July 30, 2020 Category: Neurology Authors: Paolilo, R. B., Hacohen, Y., Yazbeck, E., Armangue, T., Bruijstens, A., Lechner, C., Apostolos-Pereira, S. L., Martynenko, Y., Breu, M., de Medeiros Rimkus, C., Wassmer, E., Baumann, M., Papetti, L., Capobianco, M., Kornek, B., Rostasy, K., da Paz, J. A., Tags: Autoimmune diseases, Devic's syndrome, Optic neuritis; see Neuro-ophthalmology/Optic Nerve, Transverse myelitis Article Source Type: research

Clinically based score predicting cryptogenic NORSE at the early stage of status epilepticus
Objective To determine whether a clinically based score predicts cryptogenic new-onset refractory status epilepticus (C-NORSE) at the early stage of status epilepticus (SE) with prominent motor symptoms (SE-M) of unclear etiology. Methods The score (range 0–6) included 6 clinical features: highly refractoriness to antiseizure drugs, previously healthy individual, presence of prodromal fever, absence of prodromal psychobehavioral or memory alterations, absence of dyskinesias, and symmetric brain MRI abnormalities (the first 2 mandatory). We retrospectively assessed the usefulness of a high scale score (≥5) in pred...
Source: Neurology Neuroimmunology and Neuroinflammation - July 29, 2020 Category: Neurology Authors: Yanagida, A., Kanazawa, N., Kaneko, J., Kaneko, A., Iwase, R., Suga, H., Nonoda, Y., Onozawa, Y., Kitamura, E., Nishiyama, K., Iizuka, T. Tags: MRI, Autoimmune diseases, Encephalitis, All Epilepsy/Seizures Article Source Type: research

Infliximab treatment in pathology-confirmed neurosarcoidosis
Conclusion Infliximab is an effective treatment in neurosarcoidosis leading to remission or improvement in 70%. The mortality rate in infliximab-treated patients was substantial, indicating the severity of disease and treatment-associated complications. Classification of evidence This study provides Class IV evidence that in people with pathology-confirmed neurosarcoidosis, infliximab is beneficial. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 27, 2020 Category: Neurology Authors: Fritz, D., Timmermans, W. M. C., van Laar, J. A. M., van Hagen, P. M., Siepman, T. A. M., van de Beek, D., Brouwer, M. C. Tags: All Immunology, Class IV, Cohort studies Article Source Type: research

Isotyping paranodal antibodies in inflammatory neuropathies: One step closer to precision care
The discovery of autoantibodies against paranodal proteins such as neurofascin-155 (NF155), contactin-1 (CNTN1), or contactin-associated protein-1 (CASPR1) in inflammatory neuropathies has led to the description of subsets of patients with specific phenotypic features.1 These antibodies mostly belong to the immunoglobulin G (IgG)4 subclass, but IgG1, IgG2, or IgG3 autoantibodies have also been described.2–4 Different autoantibody isotypes are associated with different effector mechanisms causing nerve damage5,6; testing them may have implications to inform therapeutic choices or to predict prognosis. (Source: Neurolo...
Source: Neurology Neuroimmunology and Neuroinflammation - July 24, 2020 Category: Neurology Authors: Lleixa, C., Querol, L. Tags: Autoimmune diseases, All Neuromuscular Disease, Peripheral neuropathy, Guillain-Barre syndrome, Chronic inflammatory demyelinating polyneuropathy Editorial Source Type: research

Antiparanodal antibodies and IgG subclasses in acute autoimmune neuropathy
Conclusion Our data (1) confirm and extend previous observations that antiparanodal IgG2/3 but not IgG4 antibodies can occur in acute-onset neuropathies manifesting as monophasic GBS, (2) suggest association of IgG3 to a favorable response to IVIg, and (3) lend support to the hypothesis that in some patients, an IgG subclass switch from IgG3 to IgG4 may be the correlate of a secondary progressive or relapsing course following a GBS-like onset. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 24, 2020 Category: Neurology Authors: Appeltshauser, L., Brunder, A.-M., Heinius, A., Körtvelyessy, P., Wandinger, K.-P., Junker, R., Villmann, C., Sommer, C., Leypoldt, F., Doppler, K. Tags: Autoimmune diseases, All Neuromuscular Disease, Peripheral neuropathy, Guillain-Barre syndrome, Chronic inflammatory demyelinating polyneuropathy Article Source Type: research

Genetic determinants of the humoral immune response in MS
Conclusion Although some HLA alleles seem to primarily drive the extent of humoral immune responses in the CNS by increasing CSF B cells and plasmablasts, genetic variants at the immunoglobulin heavy chain constant locus might regulate intrathecal immunoglobulins levels via different mechanisms. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 16, 2020 Category: Neurology Authors: Gasperi, C., Andlauer, T. F. M., Keating, A., Knier, B., Klein, A., Pernpeintner, V., Lichtner, P., Gold, R., Zipp, F., Then Bergh, F., Stangel, M., Tumani, H., Wildemann, B., Wiendl, H., Bayas, A., Kümpfel, T., Zettl, U. K., Linker, R. A., Zieman Tags: Autoimmune diseases, Cerebrospinal Fluid, Multiple sclerosis, Association studies in genetics Article Source Type: research

Motor cortex transcriptome reveals microglial key events in amyotrophic lateral sclerosis
Conclusions DAM has a central role in microglia-related neuroinflammatory changes in the motor cortex of patients with ALS, and these alterations are coupled with a reduced expression of postsynaptic transcripts. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 15, 2020 Category: Neurology Authors: Dols-Icardo, O., Montal, V., Sirisi, S., Lopez-Pernas, G., Cervera-Carles, L., Querol-Vilaseca, M., Munoz, L., Belbin, O., Alcolea, D., Molina-Porcel, L., Pegueroles, J., Turon-Sans, J., Blesa, R., Lleo, A., Fortea, J., Rojas-Garcia, R., Clarimon, J. Tags: Amyotrophic lateral sclerosis, Gene expression studies Article Source Type: research

High {kappa} free light chain is a potential biomarker for double seronegative and ocular myasthenia gravis
Conclusions Elevated serum FLC may serve as a biomarker for MG in suspected patients who are double seronegative and in those with only ocular manifestations when serology is inconclusive. Classification of evidence This study provides Class III evidence that high FLC levels distinguished patients with MG, including those who were double seronegative, from healthy controls. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 14, 2020 Category: Neurology Authors: Wilf-Yarkoni, A., Alkalay, Y., Brenner, T., Karni, A. Tags: Autoimmune diseases, Myasthenia, Class III Article Source Type: research

Guillain-Barre syndrome related to SARS-CoV-2 infection
In the Article "Guillain-Barré syndrome related to SARS-CoV-2 infection" by K. Bigaut et al.,1 there are several errors in table 1. The age noted for the patient described in the first case should be 43; additionally, the protein level in CSF should be listed as 0.94 g/L for this patient. Lastly, for the patient described in the second case, the protein level in CSF should be listed as 1.06 g/L. The authors regret the errors. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 9, 2020 Category: Neurology Tags: CORRECTION Source Type: research

COVID-19 outcomes in MS: Observational study of early experience from NYU Multiple Sclerosis Comprehensive Care Center
Conclusions Most patients with MS with COVID-19 do not require hospitalization despite being on DMTs. Factors associated with critical illness were similar to the general at-risk patient population. DMT use did not emerge as a predictor of poor COVID-19 outcome in this preliminary sample. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 9, 2020 Category: Neurology Authors: Parrotta, E., Kister, I., Charvet, L., Sammarco, C., Saha, V., Charlson, R. E., Howard, J., Gutman, J. M., Gottesman, M., Abou-Fayssal, N., Wolintz, R., Keilson, M., Fernandez-Carbonell, C., Krupp, L. B., Zhovtis Ryerson, L. Tags: COVID-19, All Demyelinating disease (CNS), Multiple sclerosis Article Source Type: research

High proportion of Guillain-Barre syndrome associated with chikungunya in Northeast Brazil
From 2013 to 2015, sanitary authorities reported an increased incidence of Guillain-Barré syndrome (GBS) associated with Zika virus (ZIKV) in French Polynesia, Caribbean, and Brazil.1–3 After the end of ZIKV epidemics, GBS cases where still above the usual limits in countries where the arrival of chikungunya virus (CHIKV) was also a concern.3 (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 6, 2020 Category: Neurology Authors: Matos, A. d. M. B., Maia Carvalho, F. M., Malta, D. L., Rodrigues, C. L., Felix, A. C., Pannuti, C. S., Lima, A. D. d. R., Esposito, D. L. A., dos Santos, L. M. B., von Glehn, F., Colares, J. K. B., da Fonseca, B. A. L., de Oliveira, A. C. P., Romano, C. Tags: Autoimmune diseases, All Infections, Viral infections, Post-infectious, Guillain-Barre syndrome Clinical/Scientific Notes Source Type: research

Increased CSF levels of IL-1{beta}, IL-6, and ACE in SARS-CoV-2-associated encephalitis
(Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 1, 2020 Category: Neurology Authors: Bodro, M., Compta, Y., Llanso, L., Esteller, D., Doncel-Moriano, A., Mesa, A., Rodriguez, A., Sarto, J., Martinez-Hernandez, E., Vlagea, A., Egri, N., Filella, X., Morales-Ruiz, M., Yagüe, J., Soriano, A., Graus, F., Garcia, F., on behalf of the " Tags: Encephalitis, COVID-19 Clinical/Scientific Notes Source Type: research

CSF chitinase 3-like-1 association with disability of primary progressive MS
Conclusions This analysis supports the association between CSF CHI3L1 levels and neurologic disability according to EDSS scores in patients with PPMS. Other chitinase-like proteins such as CHI3L2 may also be involved. Classification of evidence This study provides Class II evidence that CSF CHI3L1 is associated with neurologic disability in patients with PPMS. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 1, 2020 Category: Neurology Authors: Perez-Miralles, F., Prefasi, D., Garcia-Merino, A., Gascon-Gimenez, F., Medrano, N., Castillo-Villalba, J., Cubas, L., Alcala, C., Gil-Perotin, S., Gomez-Ballesteros, R., Maurino, J., Alvarez-Garcia, E., Casanova, B. Tags: Cerebrospinal Fluid, Multiple sclerosis, Cohort studies, Natural history studies (prognosis), Risk factors in epidemiology Article Source Type: research

Extending rituximab dosing intervals in patients with MS during the COVID-19 pandemic and beyond?
Conclusions An extended dosing interval for rituximab for patients with stable MS during the COVID-19 pandemic may be associated with a low risk of disease activity. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - June 25, 2020 Category: Neurology Authors: Maarouf, A., Rico, A., Boutiere, C., Perriguey, M., Demortiere, S., Pelletier, J., Audoin, B., Under the aegis of OFSEP Tags: COVID-19, Multiple sclerosis, Cohort studies Article Source Type: research

COVID-19-associated ophthalmoparesis and hypothalamic involvement
We describe 2 patients with COVID-19 presenting with ophthalmoparesis and characteristic MRI findings. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - June 25, 2020 Category: Neurology Authors: Pascual-Goni, E., Fortea, J., Martinez-Domeno, A., Rabella, N., Tecame, M., Gomez-Oliva, C., Querol, L., Gomez-Anson, B. Tags: Encephalitis, Viral infections, Neuroendocrinology, Ocular motility, COVID-19 Clinical/Scientific Notes Source Type: research

Painful trigeminal neuropathy associated with anti-Plexin D1 antibody
Conclusions Plexin D1-IgG, which binds to pain-conducting small TG neurons in addition to DRG neurons, can be present in IPTN as well as limb and trunk NP. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - June 25, 2020 Category: Neurology Authors: Fujii, T., Yamasaki, R., Miyachi, Y., Iinuma, K., Hashimoto, Y., Isobe, N., Matsushita, T., Kira, J.-i. Tags: Autoimmune diseases, Neuropathic pain Article Source Type: research

Altered fovea in AQP4-IgG-seropositive neuromyelitis optica spectrum disorders
Conclusions Foveal morphometry reveals a wider and flatter fovea in NMOSD in comparison to MS and HC. Comparison to MS and accounting for ON suggest this effect to be at least in part independent of ON. This supports a primary retinopathy in AQP4-IgG–seropositive NMOSD. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - June 23, 2020 Category: Neurology Authors: Motamedi, S., Oertel, F. C., Yadav, S. K., Kadas, E. M., Weise, M., Havla, J., Ringelstein, M., Aktas, O., Albrecht, P., Ruprecht, K., Bellmann-Strobl, J., Zimmermann, H. G., Paul, F., Brandt, A. U. Tags: Retina, Devic's syndrome Article Source Type: research

Recent cannabis use in HIV is associated with reduced inflammatory markers in CSF and blood
Conclusions Recent cannabis use was associated with lower levels of inflammatory biomarkers, both in CSF and blood, but in different patterns. These results are consistent with compartmentalization of immune effects of cannabis. The principal active components of cannabis are highly lipid soluble and sequestered in brain tissue; thus, our findings are consistent with specific anti-neuroinflammatory effects that may benefit HIV neurologic dysfunction. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - June 17, 2020 Category: Neurology Authors: Ellis, R. J., Peterson, S. N., Li, Y., Schrier, R., Iudicello, J., Letendre, S., Morgan, E., Tang, B., Grant, I., Cherner, M. Tags: HIV, Cerebrospinal Fluid Article Source Type: research

"Time to recharge"
This has been a challenging spring, and we all hope that the summer will bring the needed relief. To help you unwind, the July issue of Neurology® Neuroimmunology & Neuroinflammation contains excellent articles on a wide variety of topics that should hold your interest and help charge your brain batteries. Here I highlight 4 studies that I choose for the common theme of dealing with rare inflammatory disorders. I took the liberty of including a study of pregnant and lactating women with MS/NMOSD because investigations of this cohort are relatively rare. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - June 11, 2020 Category: Neurology Authors: Dalmau, J. Tags: Editor ' s Corner Source Type: research

Diagnostic accuracy of intrathecal kappa free light chains compared with OCBs in MS
Conclusion All evaluated KFLC metrics have excellent accuracy, and both KFLC intrathecal fraction and CSF KFLC/CSF albumin are at least as good as OCB in separating patients with MS from a control group. Classification of evidence This study provides Class III evidence that CSF KFLC accurately distinguishes patients with MS from healthy controls. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - June 11, 2020 Category: Neurology Authors: Duell, F., Evertsson, B., Al Nimer, F., Sandin, A., Olsson, D., Olsson, T., Khademi, M., Hietala, M. A., Piehl, F., Hansson, M. Tags: Diagnostic test assessment, Multiple sclerosis Article Source Type: research

Should interferons take front stage as an essential MS disease-modifying therapy in the era of coronavirus disease 2019?
In the unprecedented pandemic of the coronavirus disease 2019 (COVID-19) along with a limited clinical understanding on effective vaccines and therapies, there are currently many unknowns for patients with autoimmune conditions, such as MS, who require ongoing treatment with immunotherapies. As information is currently lacking on the immune effects of COVID-19 in the context of MS disease-modifying therapies (DMTs), a challenging clinical question being faced by patients and neurologists is whether to continue current DMTs for patients with MS and risk potentially greater morbidity and mortality due to COVID-19 infection v...
Source: Neurology Neuroimmunology and Neuroinflammation - June 11, 2020 Category: Neurology Authors: Maguire, C., Frohman, T., Zamvil, S. S., Frohman, E., Melamed, E. Tags: All Immunology, All Clinical trials, COVID-19, Multiple sclerosis Clinical/Scientific Notes Source Type: research

CNS inflammatory vasculopathy with antimyelin oligodendrocyte glycoprotein antibodies in COVID-19
A 44-year-old right-handed woman reported a gradual onset of right hand incoordination seven days after the onset of minor respiratory symptoms and pruritus due to COVID-19 infection. Over 48 hours, the patient developed word-finding difficulties and progression in right arm weakness leading to presentation to the emergency department as a suspected stroke. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - June 10, 2020 Category: Neurology Authors: Pinto, A. A., Carroll, L. S., Nar, V., Varatharaj, A., Galea, I. Tags: MRI, Other cerebrovascular disease/ Stroke, Encephalitis, COVID-19 Clinical/Scientific Notes Source Type: research

COVID-19-associated acute necrotizing myelitis
A 69-year-old otherwise healthy woman presented to our clinic with irradiated cervical pain, imbalance, and motor weakness and numbness in the left hand, which had been ongoing for 7 days. Eight days before the onset of these symptoms, she had fever and dry cough. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - June 10, 2020 Category: Neurology Authors: Sotoca, J., Rodriguez-Alvarez, Y. Tags: MRI, All Spinal Cord, COVID-19, All Demyelinating disease (CNS), Transverse myelitis Clinical/Scientific Notes Source Type: research

Severe paradoxical disease activation following alemtuzumab treatment for multiple sclerosis
A 39-year-old right-handed agricultural service engineer developed rapidly evolving severe relapsing-remitting multiple sclerosis (MS). MRI showed multiple T2 hyperintensities throughout his neuroaxis (figure, A). Several lesions showed restricted diffusion, and 2 enhanced. He received steroids for each relapse, making a full recovery (Expanded Disability Status Score [EDSS] 0). (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - June 10, 2020 Category: Neurology Authors: Brannigan, J., Jones, J. L., Stacpoole, S. R. L. Tags: Autoimmune diseases, Encephalitis, Multiple sclerosis, Acute disseminated encephalomyelitis Clinical/Scientific Notes Source Type: research