Neurofilament Light in CSF and Plasma Is a Marker of Neuronal Damage in HTLV-1-Associated Myelopathy and Correlates With Neuroinflammation
Conclusions Nf-L in plasma and CSF has potential to be used as a biomarker of disease activity in HAM. Neuronal damage seems to be more intense early in disease but persists long term. Wheelchair-dependent patients have ongoing neuroinflammation. (Source: Neurology Neuroimmunology and Neuroinflammation)
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

Rituximab Treatment and Long-term Outcome of Patients With Autoimmune Encephalitis: Real-world Evidence From the GENERATE Registry
This study provides Class IV evidence that rituximab is an effective treatment for some types of AE. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - October 1, 2021 Category: Neurology Authors: Thaler, F. S., Zimmermann, L., Kammermeier, S., Strippel, C., Ringelstein, M., Kraft, A., Sühs, K.-W., Wickel, J., Geis, C., Markewitz, R., Urbanek, C., Sommer, C., Doppler, K., Penner, L., Lewerenz, J., Rössling, R., Finke, C., Prü Tags: All Immunology, Autoimmune diseases Article Source Type: research

CSF Levels of CXCL12 and Osteopontin as Early Markers of Primary Progressive Multiple Sclerosis
Conclusions At the time of diagnosis, a specific CSF protein profile can recognize the presence of early intrathecal inflammatory processes, possibly stratifying PPMS with respect to RRMS. Elevated CSF levels of CXCL12 and osteopontin suggested a key role of brain innate immunity and glia activity in MS. These molecules could represent useful candidate markers of MS progression, with implications for the pathogenesis and treatment of progressive MS. Classification of Evidence This study provides Class III evidence that CXCL12 and monocyte-related osteopontin may be correlated with PPMS, and IL-10 may be related to RRMS. I...
Source: Neurology Neuroimmunology and Neuroinflammation - September 29, 2021 Category: Neurology Authors: Marastoni, D., Magliozzi, R., Bolzan, A., Pisani, A. I., Rossi, S., Crescenzo, F., Montemezzi, S., Pizzini, F. B., Calabrese, M. Tags: Multiple sclerosis Article Source Type: research

Genome-wide Association Study Identifies 2 New Loci Associated With Anti-NMDAR Encephalitis
This study provides evidence for relevant genetic determinants of antibody-mediated autoimmune encephalitides outside the human leukocyte antigen (HLA) region. The results suggest that future studies with larger sample sizes will successfully identify additional genetic determinants and contribute to the elucidation of the pathomechanism. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - September 28, 2021 Category: Neurology Authors: Tietz, A. K., Angstwurm, K., Baumgartner, T., Doppler, K., Eisenhut, K., Elisak, M., Franke, A., Golombeck, K. S., Handreka, R., Kaufmann, M., Kraemer, M., Kraft, A., Lewerenz, J., Lieb, W., Madlener, M., Melzer, N., Mojzisova, H., Möller, P., Pfe Tags: Autoimmune diseases, Association studies in genetics Article Source Type: research

Neurological Autoimmunity Associated With Homer-3 Antibody: A Case Series From China
This study reports and analyzes cases with Homer-3 antibodies. Results Of the serum and CSF samples of 750 patients tested, 6 were positive for Homer-3 antibodies. All manifested subacute or insidious-onset cerebellar ataxia. Furthermore, 2 patients each exhibited encephalopathy, myeloradiculopathy, REM sleep behavior disorder, and autonomic dysfunction. Brain magnetic resonance images were normal (n = 1) or revealed cerebellar atrophy (n = 1), cerebellum and pons atrophy with the hot cross bun sign (n = 2), and bilateral cerebral abnormalities (n = 2). Definite leukocytosis was identified in the CSF of 2 patients, protei...
Source: Neurology Neuroimmunology and Neuroinflammation - September 27, 2021 Category: Neurology Authors: Liu, M., Ren, H., Fan, S., Zhang, W., Xu, Y., Zhao, W., Guan, H., and the Encephalitis Collaborative Group Tags: All Immunology, Gait disorders/ataxia Article Source Type: research

Blocking Placental Class G Immunoglobulin Transfer Prevents NMDA Receptor Antibody Effects in Newborn Mice
Discussion FcRn blockade prevents placental transfer of IgG from patients with anti-NMDAR encephalitis and abrogates the synaptic and neurodevelopmental alterations caused by patients' antibodies. This model has potential therapeutic implications for other antibody-mediated diseases of the CNS during pregnancy. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - September 27, 2021 Category: Neurology Authors: Garcia-Serra, A., Radosevic, M., Rios, J., Aguilar, E., Maudes, E., Landa, J., Sabater, L., Martinez-Hernandez, E., Planaguma, J., Dalmau, J. Tags: Autoimmune diseases, Neonatal Article Source Type: research

Intrathymic Plasmablasts Are Affected in Patients With Myasthenia Gravis With Active Disease
Discussion Our findings confirmed a correlation between increased frequency of intrathymic plasmablasts and disease activity before thymectomy. We postulate that activated intrathymic plasmablasts endow pathogenic capacity in MG. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - September 24, 2021 Category: Neurology Authors: Yamamoto, Y., Matsui, N., Uzawa, A., Ozawa, Y., Kanai, T., Oda, F., Kondo, H., Ohigashi, I., Takizawa, H., Kondo, K., Sugano, M., Kitaichi, T., Hata, H., Kaji, R., Kuwabara, S., Yamamura, T., Izumi, Y. Tags: All Immunology, Autoimmune diseases, All Neuromuscular Disease, Myasthenia Article Source Type: research

T-Cell Specificity Influences Disease Heterogeneity in Multiple Sclerosis
Discussion Our observations reveal an association between autoantigen reactivity and features of disease heterogeneity that strongly supports an important role of T-cell specificity in MS pathogenesis. These data have the potential to improve patient classification in clinical practice and to guide the development of antigen-specific tolerization strategies. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - September 17, 2021 Category: Neurology Authors: Cruciani, C., Puthenparampil, M., Tomas-Ojer, P., Jelcic, I., Docampo, M. J., Planas, R., Manogaran, P., Opfer, R., Wicki, C., Reindl, M., Jelcic, I., Lutterotti, A., Martin, R., Sospedra, M. Tags: All Immunology, Autoimmune diseases, Multiple sclerosis Article Source Type: research

Retinal Optical Coherence Tomography in Neuromyelitis Optica
Discussion Our results emphasize that attack prevention is key to avoid severe neuroaxonal damage and vision loss caused by ON in NMOSD. Therapies ameliorating attack-related damage, especially during a first attack, are an unmet clinical need. Mild signs of neuroaxonal changes without apparent vision loss in ON-unaffected eyes might be solely due to contralateral ON attacks and do not suggest clinically relevant progression but need further investigation. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - September 15, 2021 Category: Neurology Authors: Oertel, F. C., Specovius, S., Zimmermann, H. G., Chien, C., Motamedi, S., Bereuter, C., Cook, L., Lana Peixoto, M. A., Fontanelle, M. A., Kim, H. J., Hyun, J.-W., Palace, J., Roca-Fernandez, A., Leite, M. I., Sharma, S., Ashtari, F., Kafieh, R., Dehghani, Tags: All Imaging, Retina, Visual loss, Devic's syndrome, Optic neuritis; see Neuro-ophthalmology/Optic Nerve Article Source Type: research

CSF Chitinase 3-Like 2 Is Associated With Long-term Disability Progression in Patients With Progressive Multiple Sclerosis
Conclusions Although further confirmatory studies are needed, we propose CSF CHI3L2 as a prognostic protein biomarker associated with long-term disability progression in patients with progressive MS. Classification of Evidence This study provides Class II evidence that high CSF CHI3L2 levels identified higher disability progression in patients with progressive MS. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - September 8, 2021 Category: Neurology Authors: Comabella, M., Sastre-Garriga, J., Borras, E., Villar, L. M., Saiz, A., Martinez-Yelamos, S., Garcia-Merino, J. A., Pinteac, R., Fissolo, N., Sanchez Lopez, A. J., Costa-Frossard, L., Blanco, Y., Llufriu, S., Vidal-Jordana, A., Sabido, E., Montalban, X. Tags: Class II, Multiple sclerosis Article Source Type: research

Anti-Interleukin-6 and Janus Kinase Inhibitors for Severe Neurologic Toxicity of Checkpoint Inhibitors
Discussion Our case suggests that some patients with severe, corticoresistant CNS immune-related toxicities of ICIs may benefit from cytokine blockade. Cytokine measurement in serum and CSF might help in selecting patients for personalized treatment strategies. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - September 8, 2021 Category: Neurology Authors: Picca, A., Valyraki, N., Birzu, C., Kramkimel, N., Hermine, O., Zahr, N., Berzero, G., Psimaras, D. Tags: All Immunology, Autoimmune diseases, All Oncology, Cerebrospinal Fluid, Transverse myelitis Clinical/Scientific Notes Source Type: research

Balancing Potential Benefits and Risks of Bruton Tyrosine Kinase Inhibitor Therapies in Multiple Sclerosis During the COVID-19 Pandemic
Bruton tyrosine kinase inhibitors (BTKis) encompass a new class of therapeutics currently being evaluated for the treatment of multiple sclerosis (MS). Whether BTKis affect COVID-19 risk or severity or reduce vaccine efficacy are important but unanswered questions. Here, we provide an overview on BTKi mechanisms relevant to COVID-19 infection and vaccination and review preliminary data on BTKi use in patients with COVID-19. BTKis block B-cell receptor– and myeloid fragment crystallizable receptor–mediated signaling, thereby dampening B-cell activation, antibody class-switching, expansion, and cytokine productio...
Source: Neurology Neuroimmunology and Neuroinflammation - September 8, 2021 Category: Neurology Authors: Weber, M. S., Nicholas, J. A., Yeaman, M. R. Tags: COVID-19, Multiple sclerosis Views [amp ] Reviews Source Type: research

Targeted RNA Sequencing of Formalin-Fixed, Paraffin-Embedded Temporal Arteries From Giant Cell Arteritis Cases Reveals Viral Signatures
Discussion The detection of viral and immune activation pathways in GCA TAs supports a role for virus infection in GCA pathogenesis. In addition, the detection of viral pathways in control/VZV-positive TAs, along with vascular remodeling pathways, suggests that these samples may represent early infection with progression to clinical disease, depending on host and other environmental factors. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - September 7, 2021 Category: Neurology Authors: Bubak, A. N., Mescher, T., Mariani, M., Frietze, S. E., Hassell, J. E., Niemeyer, C. S., Como, C. N., Burnet, A. M., Subramanian, P. S., Cohrs, R. J., Mahalingam, R., Nagel, M. A. Tags: All Immunology, Vasculitis, Viral infections, All Cerebrovascular disease/Stroke, Gene expression studies Article Source Type: research

Increased Prevalence of Familial Autoimmune Disease in Children With Opsoclonus-Myoclonus Syndrome
The objective of this study was to investigate the prevalence of autoimmune disease in first-degree relatives of pediatric patients with paraneoplastic and nonparaneoplastic OMS. Methods A single-center cohort study of consecutively evaluated children with OMS was performed. Parents of patients were prospectively administered surveys on familial autoimmune disease. Rates of autoimmune disease in first-degree relatives of pediatric patients with OMS were compared using Fisher exact t test and 2 analysis: (1) between those with and without a paraneoplastic cause and (2) between healthy and disease (pediatric multiple sclero...
Source: Neurology Neuroimmunology and Neuroinflammation - September 2, 2021 Category: Neurology Authors: Santoro, J. D., Kerr, L. M., Codden, R., Casper, T. C., Greenberg, B. M., Waubant, E., Kong, S. W., Mandl, K. D., Gorman, M. P. Tags: All Immunology, Autoimmune diseases, All Neuro-ophthalmology, Paraneoplastic syndrome Article Source Type: research