Rituximab-Induced Hypogammaglobulinemia and Risk of Infection in Neuromyelitis Optica Spectrum Disorders: A 14-Year Real-Life Experience
Discussion Over 14 years of long-term RTX treatment, IgG levels gradually decreased, and the frequency of hypo-IgG increased to 41% of the patients. Patients with prolonged memory B-cell depletion after RTX, previous mitoxantrone history, hypo-IgG at baseline, or obesity were at risk of developing RTX-induced hypogammaglobulinemia. Nevertheless, infection rates remained low during treatment, and reduced immunoglobulin levels were not associated with an increased incidence of infections. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 19, 2022 Category: Neurology Authors: Kim, S.-H., Park, N. Y., Kim, K. H., Hyun, J.-W., Kim, H. J. Tags: All Demyelinating disease (CNS), Devic's syndrome Research Article Source Type: research

Increased Intrathecal Activity of Follicular Helper T Cells in Patients With Relapsing-Remitting Multiple Sclerosis
This study identified 2 phenotypically and functionally distinct Tfh cell populations: CD25– Tfh cells (Tfh1-like) and CD25int Tfh cells (Tfh17-like). Whereas minor differences in Tfh cell populations were found in blood between patients with MS and controls, we observed an increased frequency of CD25– Tfh cells in CSF of patients with RRMS and PPMS and CD25int Tfh cells in patients with RRMS, compared with controls. Increasing frequencies of CSF CD25– Tfh cells and the CD25– Tfh/Tfr ratio scaled with increasing IgG index in patients with RRMS. Despite an increased prevalence of intrathecal Tfh cell...
Source: Neurology Neuroimmunology and Neuroinflammation - July 14, 2022 Category: Neurology Authors: Holm Hansen, R., Talbot, J., Hojsgaard Chow, H., Bredahl Hansen, M., Buhelt, S., Herich, S., Schwab, N., Hellem, M. N. N., Nielsen, J. E., Sellebjerg, F., von Essen, M. R. Tags: Research Article Source Type: research

Immune and Genetic Signatures of Breast Carcinomas Triggering Anti-Yo-Associated Paraneoplastic Cerebellar Degeneration
Discussion These data confirm the role of genetic alterations of Yo antigens in triggering the immune tolerance breakdown but also outline a specific biomolecular profile in Yo-PCD BCs, suggesting a cancer-specific pathogenesis. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 12, 2022 Category: Neurology Authors: Peter, E., Treilleux, I., Wucher, V., Jougla, E., Vogrig, A., Pissaloux, D., Paindavoine, S., Berthet, J., Picard, G., Rogemond, V., Villard, M., Vincent, C., Tonon, L., Viari, A., Honnorat, J., Dubois, B., Desestret, V. Tags: Research Article Source Type: research

Novel Cell-Based Assay for Alpha-3 Nicotinic Receptor Antibodies Detects Antibodies Exclusively in Autoimmune Autonomic Ganglionopathy
(Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 12, 2022 Category: Neurology Tags: Correction Source Type: research

Paraneoplastic Cerebellar Degeneration With P/Q-VGCC vs Yo Autoantibodies
In this study, we compare neuropathologic characteristics of PCD with anti–P/Q-VGCC and anti-Yo autoantibodies in an archival autopsy cohort. Methods We performed neuropathology, immunohistochemistry, and multiplex immunofluorescence on formalin-fixed and paraffin-embedded brain tissue of 1 anti–P/Q-VGCC, 2 anti–Yo-PCD autopsy cases and controls. Results Anti–Yo-PCD revealed a diffuse and widespread PC loss together with microglial nodules with pSTAT1+ and CD8+granzymeB+ T cells and neuronal upregulation of major histocompatibility complex (MHC) Class I molecules. Some neurons showed a cytoplasmic...
Source: Neurology Neuroimmunology and Neuroinflammation - July 7, 2022 Category: Neurology Authors: Winklehner, M., Bauer, J., Endmayr, V., Schwaiger, C., Ricken, G., Motomura, M., Yoshimura, S., Shintaku, H., Ishikawa, K., Tsuura, Y., Iizuka, T., Yokota, T., Irioka, T., Höftberger, R. Tags: Autoimmune diseases, Encephalitis, Paraneoplastic syndrome Research Article Source Type: research

The BCL2 Inhibitor Venetoclax Plus Rituximab Is Active in MYD88 Wild-Type Polyneuropathy With Anti-MAG Antibodies
This study provides Class IV evidence that for a patient with relapsed anti-MAG antibody polyneuropathy, MYD88 wild-type, venetoclax plus rituximab is effective. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 7, 2022 Category: Neurology Authors: Briani, C., Visentin, A., Castellani, F., Cacciavillani, M., Trentin, L. Tags: All Clinical Neurology, Peripheral neuropathy, Class IV Clinical/Scientific Note Source Type: research

Long-term Effectiveness of IVIg Maintenance Therapy in 36 Patients With GAD Antibody-Positive Stiff-Person Syndrome
Discussion This is a large study in 36 patients with SPS demonstrating that monthly maintenance IVIg therapy offers long-term benefits in 67% of patients for a median 3.3-year period. Because 29.1% experienced diminishing benefit over time due to disease progression, the study highlights the need for more effective therapies. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 7, 2022 Category: Neurology Authors: Yi, J., Dalakas, M. C. Tags: Research Article Source Type: research

Younger Age at Onset Is Associated With Worse Long-term Behavioral Outcomes in Anti-NMDA Receptor Encephalitis
In this study, we evaluate the relationship between features from clinical presentation, including age, and long-term adaptive behavior in participants with anti-NMDARE. Methods Cross-sectional informant-reported data were collected between 2017 and 2019 from 41 individuals/caregivers of individuals with anti-NMDARE treated at 3 major academic hospitals. Neurologic disability was assessed by record review using the modified Rankin Scale (mRS). Functional outcomes were assessed using the validated Adaptive Behavior Assessment System, Third Edition (ABAS-3). Results The mean age at the time of study enrollment was 23.4 yea...
Source: Neurology Neuroimmunology and Neuroinflammation - July 6, 2022 Category: Neurology Authors: Yeshokumar, A., Gordon-Lipkin, E., Arenivas, A., Rosenfeld, M., Patterson, K., Blum, R., Banwell, B., Venkatesan, A., Lancaster, E., Panzer, J., Probasco, J. Tags: Research Article Source Type: research

AMPA Receptor Encephalitis in a Patient With Metastatic Breast Cancer Receiving Palbociclib: A Case Report
Discussion AMPARE is a well-described paraneoplastic syndrome. However, it is now understood that paraneoplastic syndromes can be driven by immunomodulatory medications, namely ICIs. Although palbociclib primarily prevents tumor proliferation, emerging data suggest that it may also be immunomodulatory. Given that our patient's AMPARE developed shortly after initiation of palbociclib while her cancer was responding to therapy, we postulate that it may have been unmasked by palbociclib, similarly to what has been reported with ICIs. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 6, 2022 Category: Neurology Authors: Matthews, E., Schmitt, B., Passeri, M., Mizenko, C., Orjuela, K., Piquet, A. Tags: Autoimmune diseases, Encephalitis, Paraneoplastic syndrome Clinical/Scientific Note Source Type: research

The Effect of Smoking on Long-term Gray Matter Atrophy and Clinical Disability in Patients with Relapsing-Remitting Multiple Sclerosis
Discussion Smoking was associated with brain atrophy and disability progression 10 years later in patients with RRMS. The findings imply that patients should be advised and offered aid in smoking cessation shortly after diagnosis, to prevent long-term disability progression. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - June 23, 2022 Category: Neurology Authors: Lie, I. A., Wesnes, K., Kvistad, S. S., Brouwer, I., Wergeland, S., Holmoy, T., Midgard, R., Bru, A., Edland, A., Eikeland, R., Gosal, S., Harbo, H. F., Kleveland, G., Sorenes, Y. S., Oksendal, N., Barkhof, F., Vrenken, H., Myhr, K.-M., Bo, L., Torkildsen Tags: Research Article Source Type: research

Comment: Humoral and T-cell Immunities to SARS-CoV-2 Vaccines: Safety, Efficacy, and Challenges in Autoimmune Neurology
(Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - June 21, 2022 Category: Neurology Authors: Dalakas, M. C., Dalmau, J. Tags: Comment Source Type: research

Immune Response and Safety of SARS-CoV-2 mRNA-1273 Vaccine in Patients With Myasthenia Gravis
This study provides Class IV evidence that patients with MG receiving the mRNA-1273 vaccine did not show clinical worsening after vaccination and that most of the patients achieved high cellular or immune response levels. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - June 21, 2022 Category: Neurology Authors: Reyes-Leiva, D., Lopez-Contreras, J., Moga, E., Pla-Junca, F., Lynton-Pons, E., Rojas-Garcia, R., Turon-Sans, J., Querol, L., Olive, M., Alvarez-Velasco, R., Caballero-Avila, M., Carbayo, A., Vesperinas-Castro, A., Domingo, P., Illa, I., Gallardo, E., Cor Tags: Autoimmune diseases, Myasthenia, Class IV, COVID-19 Research Article Source Type: research

Refractory Anti-NMDA Receptor Encephalitis in Early Pregnancy: A Case Report of Treatment Course and Pregnancy Outcomes
We present a case of refractory anti-NMDAR encephalitis during the first and second trimesters of pregnancy with the successful use of rituximab and cyclophosphamide and resultant healthy pregnancy. Results The patient was treated with an escalating immunotherapy regimen from 11 to 15 weeks of gestation, including steroids, plasma exchange, IV immunoglobulins, and rituximab, with no clinical response. At 16 weeks of gestation, she received cyclophosphamide with clinical improvement after 4 weeks. She subsequently gave birth to a healthy, term baby boy, who continued to do well at the follow-up. Discussion This case illus...
Source: Neurology Neuroimmunology and Neuroinflammation - June 21, 2022 Category: Neurology Authors: Fredrich, S., Wang, C., Narayan, R., Tardo, L., Blackburn, K. M., Vernino, S. Tags: Clinical/Scientific Note Source Type: research

Early Reduction of MRI Activity During 6 Months of Treatment With Cladribine Tablets for Highly Active Relapsing Multiple Sclerosis: MAGNIFY-MS
This study (MAGNIFY-MS) evaluates the onset of action of cladribine tablets by observing changes in combined unique active (CUA) MRI lesion counts during the first 6 months of treatment in patients with highly active relapsing MS. Methods MRI was performed at screening, baseline, and at months 1, 2, 3, and 6 after initiating treatment with cladribine tablets 3.5 mg/kg. CUA lesion counts, defined as the sum of T1 gadolinium-enhancing (Gd+) lesions and new or enlarging active T2 lesions (without T1 Gd+), were compared between postbaseline and the baseline period and standardized to the period length and the number of MRIs p...
Source: Neurology Neuroimmunology and Neuroinflammation - June 14, 2022 Category: Neurology Authors: de Stefano, N., Barkhof, F., Montalban, X., Achiron, A., Derfuss, T., Chan, A., Hodgkinson, S., Prat, A., Leocani, L., Schmierer, K., Sellebjerg, F., Vermersch, P., Wiendl, H., Keller, B., Roy, S., MAGNIFY-MS Study Group Tags: MRI, Class IV, Multiple sclerosis RESEARCH Article Source Type: research

Disease-Modifying Drugs for Multiple Sclerosis and Association With Survival
We examined the association between the disease-modifying drugs (DMDs) for multiple sclerosis (MS) and survival in a multiregion population-based study. Methods We accessed multiple administrative health databases from 4 Canadian provinces. Persons with MS were identified and followed from the most recent of the first MS or demyelinating event or January 1, 1996 (index date), until death, emigration, or December 31, 2017. Association between the first-generation and second-generation DMDs and all-cause mortality was examined using stratified Cox proportional hazard models, reported as adjusted hazard ratios (aHRs). Timing...
Source: Neurology Neuroimmunology and Neuroinflammation - June 14, 2022 Category: Neurology Authors: Ng, H. S., Zhu, F., Kingwell, E., Yao, S., Ekuma, O., Evans, C., Fisk, J. D., Marrie, R. A., Zhao, Y., Tremlett, H. Tags: Research Article Source Type: research