Neurology Neuroimmunology and Neuroinflammation This is an RSS file. You can use it to subscribe to this data in your favourite RSS reader or to display this data on your own website or blog.

Multiple Sclerosis Disease Activity and Disability Following Cessation of Fingolimod for Pregnancy
Discussion Relapses during pregnancy after fingolimod cessation are common. Approximately 6% of women will retain clinically meaningful disability from these pregnancy-related, fingolimod cessation relapses 1 year postpartum. This information should be shared with women on fingolimod desiring pregnancy, and optimizing MS treatment with nonteratogenic approaches should be discussed. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - May 22, 2023 Category: Neurology Authors: Hellwig, K., Tokic, M., Thiel, S., Hemat, S., Timmesfeld, N., Ciplea, A. I., Gold, R., Langer-Gould, A. M. Tags: Multiple sclerosis Research Article Source Type: research

Anti-GABA-A Receptor Antibody-Mediated Epilepsia Partialis Continua After Treatment With Alemtuzumab: A Case Report
We present a young woman with prediagnosis of relapse remitting multiple sclerosis (MS), treated with interferons, natalizumab, and alemtuzumab. Six months after one and only cycle of alemtuzumab, speech arrest and behavioral changes with aggressive and anxious traits appeared. She showed increasing motor convulsions resulting in focal status epilepticus. Results Anti–GABA-A receptor antibodies in CSF and serum were confirmed in different external laboratories, in a more extensive analysis after antibodies against NMDAR, CASPR2, LGI1, GABABR, and AMPAR were ruled out during in-house examination. Clinical condition i...
Source: Neurology Neuroimmunology and Neuroinflammation - May 18, 2023 Category: Neurology Authors: Ratuszny, D., Skripuletz, T., Stüber, T., Valizada, E., Gehring, K., Ertl, P., Müller, J. A., Wattjes, M. P., Feuerhake, F., Sühs, K.-W. Tags: Autoimmune diseases, Status epilepticus, Multiple sclerosis Clinical/Scientific Note Source Type: research

Jo-1 Antibodies From Myositis Induce Complement-Dependent Cytotoxicity and TREM-1 Upregulation in Muscle Endothelial Cells
Discussion Jo-1 antibodies from Jo-1 antibody myositis show complement-dependent cellular cytotoxicity in muscle endothelial cells. IgGs from patients with Jo-1, SRP, and DM increase the TREM-1 expression in endothelial cells and muscles. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - May 5, 2023 Category: Neurology Authors: Honda, M., Shimizu, F., Sato, R., Mizukami, Y., Watanabe, K., Takeshita, Y., Maeda, T., Koga, M., Kanda, T. Tags: All Neuromuscular Disease, Muscle disease Research Article Source Type: research

Prognostic Value of Persistent CSF Antibodies at 12 Months in Anti-NMDAR Encephalitis
This study aimed to determine the prognostic value of persistent CSF NMDAR-Abs during follow-up. Methods This retrospective observational study included patients diagnosed with anti-NMDAR encephalitis in the French Reference Center for Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis and for whom CSF samples were obtained at diagnosis and >4 months of follow-up to evaluate CSF NMDAR-Ab persistence. Because patients were tested for CSF NMDAR-Abs at different time points, samples were stratified into different periods of follow-up (i.e., 12 months was considered for the 9- to 16-month follow-up period). ...
Source: Neurology Neuroimmunology and Neuroinflammation - May 5, 2023 Category: Neurology Authors: Ciano-Petersen, N. L., Robert, M., Muniz-Castrillo, S., Wucher, V., Klich, A., Vogrig, A., Villagran Garcia, M., Farina, A., Goncalves, D., Picard, G., Rogemond, V., Joubert, B., Oliver-Martos, B., Serrano-Castro, P. J., Maucort-Boulch, D., Honnorat, J. Tags: All Immunology, Autoimmune diseases Research Article Source Type: research

Deep Phenotyping of Neurologic Postacute Sequelae of SARS-CoV-2 Infection
Discussion CSF immune dysregulation and neurocirculatory abnormalities after SARS-CoV-2 infection in the setting of disabling neuro-PASC call for further evaluation to confirm these changes and explore immunomodulatory treatments in the context of clinical trials. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - May 5, 2023 Category: Neurology Authors: Mina, Y., Enose-Akahata, Y., Hammoud, D. A., Videckis, A. J., Narpala, S. R., O'Connell, S. E., Carroll, R., Lin, B. C., McMahan, C. C., Nair, G., Reoma, L. B., McDermott, A. B., Walitt, B., Jacobson, S., Goldstein, D. S., Smith, B. R., Nath, A. Tags: Autonomic diseases, All Immunology, Viral infections, Post-infectious, Clinical trials Observational study (Cohort, Case control) Research Article Source Type: research

Mutational Profile in 75 Patients With Anti-Myelin-Associated Glycoprotein Neuropathy: Clinical and Hematologic Therapy Response and Hints on New Therapeutic Targets
The objective of our study was to assess the prevalence of MYD88L265P and CXCR4S338X gene variants in patients with anti-MAG antibody neuropathy. Secondary aims were to evaluate possible correlations between the mutational profile and neuropathy severity, antibody titers, and treatment response. Methods Seventy-five patients (47 men, mean age at molecular analysis 70.8 ± 10.2 years; mean disease duration 5.1 ± 4.9 years) with anti-MAG antibody neuropathy were recruited. Among them, 38 (50.7%) had IgM monoclonal gammopathy of undetermined significance, 29 (38.7%) Waldenstrom macroglobulinemia (WM), and 8 (10....
Source: Neurology Neuroimmunology and Neuroinflammation - May 3, 2023 Category: Neurology Authors: Castellani, F., Visentin, A., Schirinzi, E., Salvalaggio, A., Cacciavillani, M., Candiotto, C., Barate, C., Cellini, A., Bertorelle, R., Siciliano, G., Trentin, L., Briani, C. Tags: Autoimmune diseases, All Clinical Neurology, All Neuromuscular Disease, Peripheral neuropathy Research Article Source Type: research

Isolated Area Postrema Syndrome Preceding the Diagnosis of Giant Cell Arteritis: A Case Report
We report a case of biopsy-proven giant cell arteritis after an initial presentation of area postrema syndrome. Methods A 65-year-old man was evaluated using MRI, temporal artery biopsy, and ultrasound. Results The patient presented with refractory nausea, vomiting, and hiccups that caused weight loss without any other neurologic or clinical symptoms. His MRI scan 15 days later revealed a hyperintense sign on the area postrema with no abnormal diffusion or contrast enhancement, compatible with isolated area postrema syndrome. An extensive workup for inflammation and other etiologies including neuromyelitis optica spectru...
Source: Neurology Neuroimmunology and Neuroinflammation - April 25, 2023 Category: Neurology Authors: Silva, M. A. d., Carvalho, B. M. d., Queiroz, A. L. G. d., Lima, K. D. F., Teixeira, H. S., Schmid, M. F., Campos, C. M. d. S., Baeta, A. M. Tags: Autoimmune diseases, Devic's syndrome Clinical/Scientific Note Source Type: research

Arterial Spin Labeling Changes Parallel Asymmetric Perisylvian and Perirolandic Symptoms in 3 Pediatric Cases of Anti-NMDAR Encephalitis
Discussion We found that ASL may be a suitable early imaging biomarker to highlight perfusion changes corresponding to the functional localization of NMDAR AE in pediatric patients. We briefly highlight the neuroanatomic parallels between working models of schizophrenia, chronic NMDAR antagonist administration (ketamine abuse), and NMDAR AE affecting primarily language centers. The regional specificity seen in NMDAR hypofunction may make ASL a reasonable early and specific biomarker of NMDAR AE disease activity. Future studies are necessary to evaluate regional changes in those patients who present with primarily psychiatr...
Source: Neurology Neuroimmunology and Neuroinflammation - April 24, 2023 Category: Neurology Authors: Sandweiss, A. J., Kannan, V., Desai, N. K., Kralik, S. F., Muscal, E., Fisher, K. S. Tags: MRI, All Immunology, Autoimmune diseases, All Pediatric Research Article Source Type: research

Effect of Ocrelizumab on B- and T-Cell Receptor Repertoire Diversity in Patients With Relapsing Multiple Sclerosis From the Randomized Phase III OPERA Trial
Discussion Our data illustrate that the diversity of CD4+/CD8+ T-cell receptor repertoires remained unaltered in OCR-treated patients with relapsing MS. Persistence of a highly diverse T-cell repertoire suggests that aspects of adaptive immunity remain intact despite extended anti-CD20 therapy. Trial Registration Information This is a substudy (BE29353) of the OPERA I (WA21092; NCT01247324) trial. Date of registration, November 23, 2010; first patient enrollment, August 31, 2011. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - April 24, 2023 Category: Neurology Authors: Laurent, S. A., Strauli, N. B., Eggers, E. L., Wu, H., Michel, B., Demuth, S., Palanichamy, A., Wilson, M. R., Sirota, M., Hernandez, R. D., Cree, B. A. C., Herman, A. E., von Büdingen, H.- C. Tags: Multiple sclerosis Research Article Source Type: research

Treatment With Erythropoietin for Patients With Optic Neuritis: Long-term Follow-up
This study provides Class II evidence that for patients with acute optic neuritis, EPO as an adjunct to methylprednisolone is well tolerated and does not improve long-term visual outcomes. Trial Registration Information The trial was preregistered before commencement at clinicaltrials.gov (NCT01962571). (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - April 24, 2023 Category: Neurology Authors: Küchlin, S., Ihorst, G., Grotejohann, B., Beisse, F., Heinrich, S. P., Albrecht, P., Ungewiss, J., Wörner, M., Hug, M. J., Wolf, S., Diem, R., Lagreze, W. A., for the TONE Study Group Tags: All Clinical trials, Multiple sclerosis, Optic neuritis; see Neuro-ophthalmology/Optic Nerve Research Article Source Type: research

Effects of a Small-Molecule Perforin Inhibitor in a Mouse Model of CD8 T Cell-Mediated Neuroinflammation
In this study, we used an experimental mouse model to evaluate the ability of a small-molecule perforin inhibitor to prevent neuroinflammation resulting from cytotoxic CD8 T cell–mediated damage of BBB-ECs. Results Using an in vitro coculture system, we first identified perforin as an essential molecule for killing of BBB-ECs by CD8 T cells. We then found that short-term pharmacologic inhibition of perforin commencing after disease onset restored motor function and inhibited the neuropathology. Perforin inhibition resulted in preserved BBB-EC viability, maintenance of the BBB, and reduced CD8 T-cell accumulation in ...
Source: Neurology Neuroimmunology and Neuroinflammation - April 20, 2023 Category: Neurology Authors: Gonzalez-Fierro, C., Fonte, C., Dufourd, E., Cazaentre, V., Aydin, S., Engelhardt, B., Caspi, R. R., Xu, B., Martin-Blondel, G., Spicer, J. A., Trapani, J. A., Bauer, J., Liblau, R. S., Bost, C. Tags: Autoimmune diseases Research Article Source Type: research

Anti-AGO1 Antibodies Identify a Subset of Autoimmune Sensory Neuronopathy
In this study, we aim to reveal (1) the frequency of AGO1 Abs in sensory neuronopathy (SNN), (2) titers and IgG subclasses, and (3) their clinical pattern including response to treatment. Methods This retrospective multicentric case/control study screened 132 patients with SNN, 301 with non-SNN neuropathies, 274 with autoimmune diseases (AIDs), and 116 healthy controls (HCs) for AGO1 Abs through ELISA. Seropositive cases were also tested for IgG subclasses, titers, and conformation specificity. Results AGO1 Abs occurred in 44 patients, comprising significantly more of those with SNN (17/132 [12.9%]) than those with non-S...
Source: Neurology Neuroimmunology and Neuroinflammation - April 18, 2023 Category: Neurology Authors: Moritz, C. P., Tholance, Y., Vallayer, P.-B., Do, L.-D., Muniz-Castrillo, S., Rogemond, V., Ferraud, K., La Marca, C., Honnorat, J., Killian, M., Paul, S., Camdessanche, J.-P., Antoine, J.-C. G. Tags: All Immunology, Autoimmune diseases, Peripheral neuropathy Research Article Source Type: research

Natalizumab Promotes Activation of Peripheral Monocytes in Patients With Multiple Sclerosis
In this study, we report that in patients with MS, NTZ treatment is associated with an enhanced activation status of peripheral monocytes. Results Expression of 2 independent activation markers, CD69 and CD150, was significantly higher on blood monocytes from NTZ-treated patients when compared with those from matched untreated patients with MS, while other properties such as cytokine production remained unchanged. Discussion These findings consolidate the concept that peripheral immune cells remain fully competent on NTZ treatment, an excellent asset rare among MS treatments. However, they also suggest that NTZ may exert...
Source: Neurology Neuroimmunology and Neuroinflammation - April 18, 2023 Category: Neurology Authors: Frisch, E. S., Häusler, D., Weber, M. S. Tags: Multiple sclerosis Clinical/Scientific Note Source Type: research

Therapies in Stiff-Person Syndrome: Advances and Future Prospects Based on Disease Pathophysiology
This article is focused on the rationale of specific therapeutic strategies based on the SPS pathophysiology targeting both the impaired reciprocal GABAergic inhibition to symptomatically improve the main clinical manifestations of stiffness in the truncal and proximal limb muscles, gait dysfunction, and episodic painful muscle spasms and the autoimmunity to enhance improvement and slow down disease progression. A practical, step-by-step therapeutic approach is provided, highlighting the importance of combination therapies with the preferred gamma-aminobutyric acid–enhancing antispasmodic drugs, such as baclofen, tiz...
Source: Neurology Neuroimmunology and Neuroinflammation - April 14, 2023 Category: Neurology Authors: Dalakas, M. C. Tags: All Immunology, Autoimmune diseases, Stiff person syndrome Review Source Type: research

Frontal Intermittent Rhythmic Delta Activity Is a Useful Diagnostic Tool of Neurotoxicity After CAR T-Cell Infusion
This study aimed to determine whether EEG patterns may be considered as diagnostic tools for ICANS. Methods Patients who received CAR T-cell therapy at Montpellier University Hospital between September 2020 and July 2021 were prospectively enrolled. Neurologic signs/symptoms and laboratory parameters were monitored daily for 14 days after CAR T-cell infusion. EEG and brain MRI were performed between day 6 and 8 after CAR T-cell infusion. EEG was performed again on the day of ICANS occurrence, if outside this time window. All collected data were compared between patients with and without ICANS. Results Thirty-eight consec...
Source: Neurology Neuroimmunology and Neuroinflammation - April 14, 2023 Category: Neurology Authors: Huby, S., Gelisse, P., Tudesq, J.-J., Labauge, P., Duflos, C., Cartron, G., Gallerand, M.-A., Platon, L., Badiou, S., Lamure, S., Menjot de Champfleur, N., Ayrignac, X., Taieb, G. Tags: Hematologic, All Oncology, EEG; see Epilepsy/Seizures, Class III, EEG Research Article Source Type: research