Neurology® Neuroimmunology & Neuroinflammation最新文献

{"title":"Opsoclonus-Ataxia Syndrome in a Patient With Small-Cell Lung Cancer Treated With Immune Checkpoint Inhibitors.","authors":"Antonio Farina, Macarena Villagrán-García, Marie Benaiteau, Florian Lamblin, Anthony Fourier, Jérôme Honnorat, Bastien Joubert","doi":"10.1212/NXI.0000000000200287","DOIUrl":"10.1212/NXI.0000000000200287","url":null,"abstract":"<p><strong>Objectives: </strong>To describe a case of post-immune checkpoint inhibitor (ICI) opsoclonus-myoclonus-ataxia syndrome (OMAS), with complete clinical remission after treatment.</p><p><strong>Methods: </strong>A 52-year-old man was admitted because of subacute-onset vertigo, dysarthria, vomiting, and weight loss. He was under atezolizumab (anti-PD-L1) monotherapy (23 cycles) for metastatic small-cell lung cancer, with excellent response.</p><p><strong>Results: </strong>On examination (1 month after symptom onset), the patient had opsoclonus, dysarthria, severe truncal and gait ataxia, and mild appendicular ataxia without myoclonus (SARA score 26/40). Brain MRI showed mild cerebellar atrophy, and CSF analysis disclosed pleocytosis and oligoclonal bands. Anti-SOX1 antibodies were detected in serum and CSF. Atezolizumab was stopped, and corticosteroids and monthly IV immunoglobulins were administered. Chemotherapy (carboplatin and etoposide) was also started because of cancer progression. Three months later, examination showed regression of the opsoclonus, truncal ataxia, and dysarthria and persistence of very mild gait ataxia (SARA score 3.5/40), which completely regressed at last examination (20 months after onset).</p><p><strong>Discussion: </strong>The clinical pattern and reversibility bring the present case close to a few patients with paraneoplastic OMAS described before the ICI era. More research is needed to clarify the pathogenesis and outcomes of OMAS in the context of ICI.</p>","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"11 5","pages":"e200287"},"PeriodicalIF":7.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11262711/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141627265","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neurofilament Light Chain Serum Levels Mirror Age and Disability in Secondary Progressive Multiple Sclerosis: A Cross-Sectional Study.","authors":"Leila Husseini, Jakob Jung, Natalie Boess, Niels Kruse, Stefan Nessler, Christine Stadelmann, Imke Metz, Michael Haupts, Martin S Weber","doi":"10.1212/NXI.0000000000200279","DOIUrl":"10.1212/NXI.0000000000200279","url":null,"abstract":"<p><strong>Objectives: </strong>To assess neurofilament light chain serum (sNfL) levels in patients with secondary progressive multiple sclerosis (SP-MS).</p><p><strong>Methods: </strong>Using a single molecule array, we analyzed sNfL levels in a cross-sectional cohort study of 153 patients with SP-MS hospitalized for rehabilitation in a clinic specialized in the care for patients with multiple sclerosis (MS). In addition, we investigated the correlation of disease activity with sNfL levels in 36 patients with relapsing-remitting MS (RR-MS).</p><p><strong>Results: </strong>Mean sNfL levels in patients with SP-MS were consistently elevated when compared with age-matched controls and patients with RR-MS. In SP-MS, age dependency of sNfL levels was pronounced, whereas patients with RR-MS younger than 41 years without recent disease activity were not distinguishable from age-matched healthy controls. In a multivariate analysis, clinical disability was a risk factor for elevated sNfL levels in SP-MS, whereas no correlation with comorbidities, such as cardiovascular disease, diabetes mellitus, smoking status, or vitamin D serum levels, could be detected.</p><p><strong>Discussion: </strong>These findings highlight that measurement of sNfL levels represents a useful tool to assess the extent of neuroaxonal damage as a surrogate for clinical progression in patients with SP-MS, when age and disease activity as major confounders are taken into account.</p>","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"11 5","pages":"e200279"},"PeriodicalIF":7.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11256980/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141590942","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical and Immunologic Effects of Paraprobiotics in Long-COVID Patients: A Pilot Study.","authors":"María José Docampo, Mattei Batruch, Pietro Oldrati, Ernesto Berenjeno-Correa, Marc Hilty, Gabriel Leventhal, Andreas Lutterotti, Roland Martin, Mireia Sospedra","doi":"10.1212/NXI.0000000000200296","DOIUrl":"10.1212/NXI.0000000000200296","url":null,"abstract":"<p><strong>Background and objectives: </strong>After the enormous health burden during the acute stages of the COVID-19 pandemic, we are now facing another important challenge, that is, long-COVID, a clinical condition with often disabling signs and symptoms of the neuropsychiatric, gastrointestinal, respiratory, cardiovascular, and immune systems. While the pathogenesis of this syndrome is still poorly understood, alterations of immune function and the gut microbiota seem to play important roles. Because affected individuals are frequently unable to work for prolonged periods and suffer numerous health compromises, effective treatments represent a major unmet medical need. Multiple potential therapies have been tried, but none is approved yet. Approaches that are able to influence the immune system and gut microbiota such as probiotics and paraprobiotics, i.e., nonviable probiotics, seem promising candidates. We, therefore, evaluated the clinical and immunologic effects of paraprobiotics in a small pilot study.</p><p><strong>Methods: </strong>A total of 6 patients with long-COVID were followed systematically for more than 12 months after disease onset using standardized validated questionnaires, a smartphone app, and wearable sensors to assess neurocognitive function, fatigue, depressiveness, autonomic nervous system alterations, and quality of life. We then offered patients defined paraprobiotics for 4 weeks and evaluated them at the end of the treatment period using the same questionnaires, smartphone app, and wearable sensors. In addition, a comprehensive immunophenotyping and gut microbiota analysis was performed before and after treatment.</p><p><strong>Results: </strong>Improvements in several of the neurologic symptoms such as dysautonomia, fatigue, and depression were documented using both patient-reported outcomes and data from the smartphone app and wearable sensors. Of interest, the expression of activation markers on some immune cell populations such as B cells and nonclassical monocytes and the expression of toll-like receptor 2 (TLR2) on T cells were reduced after paraprobiotics treatment.</p><p><strong>Discussion: </strong>Our results suggest that paraprobiotics might exert positive effects in patients with long-COVID most likely by modulating immune cell activation and expression of TLR2 on T cells. Further studies with paraprobiotics should confirm the promising observations of this small pilot study and hopefully not only improve the outcome of long-COVID but also unravel the pathomechanisms of this condition.</p><p><strong>Classification of evidence: </strong>This study provides Class IV evidence that paraprobiotics increase the probability of favorable changes of clinical and immunologic markers in patients with long-COVID.</p>","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"11 5","pages":"e200296"},"PeriodicalIF":7.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11318528/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141897937","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Presentation and Outcome in S1P-RM and Natalizumab-Associated Progressive Multifocal Leukoencephalopathy: A Multicenter Cohort Study.","authors":"","doi":"10.1212/NXI.0000000000200310","DOIUrl":"10.1212/NXI.0000000000200310","url":null,"abstract":"","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"11 5","pages":"e200310"},"PeriodicalIF":7.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11340933/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142018207","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Different Complement Activation Patterns Following C5 Cleavage in MOGAD and AQP4-IgG+NMOSD.","authors":"Kimihiko Kaneko, Hiroshi Kuroda, Yuki Matsumoto, Naohiro Sakamoto, Naoya Yamazaki, Naoki Yamamoto, Shu Umezawa, Chihiro Namatame, Hirohiko Ono, Yoshiki Takai, Toshiyuki Takahashi, Juichi Fujimori, Ichiro Nakashima, Yasuo Harigaya, Hans Lassmann, Kazuo Fujihara, Tatsuro Misu, Masashi Aoki","doi":"10.1212/NXI.0000000000200293","DOIUrl":"10.1212/NXI.0000000000200293","url":null,"abstract":"<p><strong>Objectives: </strong>In myelin oligodendrocyte glycoprotein IgG-associated disease (MOGAD) and aquaporin-4 IgG+ neuromyelitis optica spectrum disorder (AQP4+NMOSD), the autoantibodies are mainly composed of IgG1, and complement-dependent cytotoxicity is a primary pathomechanism in AQP4+NMOSD. We aimed to evaluate the CSF complement activation in MOGAD.</p><p><strong>Methods: </strong>CSF-C3a, CSF-C4a, CSF-C5a, and CSF-C5b-9 levels during the acute phase before treatment in patients with MOGAD (n = 12), AQP4+NMOSD (n = 11), multiple sclerosis (MS) (n = 5), and noninflammatory neurologic disease (n = 2) were measured.</p><p><strong>Results: </strong>CSF-C3a and CSF-C5a levels were significantly higher in MOGAD (mean ± SD, 5,629 ± 1,079 pg/mL and 2,930 ± 435.8 pg/mL) and AQP4+NMOSD (6,017 ± 3,937 pg/mL and 2,544 ± 1,231 pg/mL) than in MS (1,507 ± 1,286 pg/mL and 193.8 ± 0.53 pg/mL). CSF-C3a, CSF-C4a, and CSF-C5a did not differ between MOGAD and AQP4+NMOSD while CSF-C5b-9 (membrane attack complex, MAC) levels were significantly lower in MOGAD (17.4 ± 27.9 ng/mL) than in AQP4+NMOSD (62.5 ± 45.1 ng/mL, <i>p</i> = 0.0019). Patients with MOGAD with severer attacks (Expanded Disability Status Scale [EDSS] ≥ 3.5) had higher C5b-9 levels (34.0 ± 38.4 ng/m) than those with milder attacks (EDSS ≤3.0, 0.9 ± 0.7 ng/mL, <i>p</i> = 0.044).</p><p><strong>Discussion: </strong>The complement pathway is activated in both MOGAD and AQP4+NMOSD, but MAC formation is lower in MOGAD, particularly in those with mild attacks, than in AQP4+NMOSD. These findings may have pathogenetic and therapeutic implications in MOGAD.</p>","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"11 5","pages":"e200293"},"PeriodicalIF":7.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11379436/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141971545","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Single-Cell RNA Sequencing Reveals Transcriptional Landscape of Neutrophils and Highlights the Role of TREM-1 in EAE.","authors":"Moyuan Quan, Huining Zhang, Xianxian Han, Yongbing Ba, Xiaoyang Cui, Yanwei Bi, Le Yi, Bin Li","doi":"10.1212/NXI.0000000000200278","DOIUrl":"10.1212/NXI.0000000000200278","url":null,"abstract":"<p><strong>Background and objectives: </strong>Neutrophils, underestimated in multiple sclerosis (MS), are gaining increased attention for their significant functions in patients with MS and the experimental autoimmune encephalomyelitis (EAE) animal model. However, the precise role of neutrophils in cervical lymph nodes (CLNs), the primary CNS-draining lymph nodes where the autoimmune response is initiated during the progression of EAE, remains poorly understood.</p><p><strong>Methods: </strong>Applying single-cell RNA sequencing (scRNA-seq), we constructed a comprehensive immune cell atlas of CLNs during development of EAE. Through this atlas, we concentrated on and uncovered the transcriptional landscape, phenotypic and functional heterogeneity of neutrophils, and their crosstalk with immune cells within CLNs in the neuroinflammatory processes in EAE.</p><p><strong>Results: </strong>Notably, we observed a substantial increase in the neutrophil population in EAE mice, with a particular emphasis on the significant rise within the CLNs. Neutrophils in CLNs were categorized into 3 subtypes, and we explored the specific roles and developmental trajectories of each distinct neutrophil subtype. Neutrophils were found to engage in extensive interactions with other immune cells, playing crucial roles in T-cell activation. Moreover, our findings highlighted the strong migratory ability of neutrophils to CLNs, partly regulated by triggering the receptor expressed on myeloid cells 1 (TREM-1). Inhibiting TREM1 with LR12 prevents neutrophil migration both in vivo and in vitro. In addition, in patients with MS, we confirmed an increase in peripheral neutrophils with an upregulation of TREM-1.</p><p><strong>Discussion: </strong>Our research provides a comprehensive and precise single-cell atlas of CLNs in EAE, highlighting the role of neutrophils in regulating the periphery immune response. In addition, TREM-1 emerged as an essential regulator of neutrophil migration to CLNs, holding promise as a potential therapeutic target in MS.</p>","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"11 5","pages":"e200278"},"PeriodicalIF":7.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11221915/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141492860","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association of Levels of CSF Osteopontin With Cortical Atrophy and Disability in Early Multiple Sclerosis.","authors":"Damiano Marastoni, Ermanna Turano, Agnese Tamanti, Elisa Colato, Anna Isabella Pisani, Arianna Scartezzini, Silvia Carotenuto, Valentina Mazziotti, Valentina Camera, Daniela Anni, Stefano Ziccardi, Maddalena Guandalini, Francesca B Pizzini, Federica Virla, Raffaella Mariotti, Roberta Magliozzi, Bruno Bonetti, Lawrence Steinman, Massimiliano Calabrese","doi":"10.1212/NXI.0000000000200265","DOIUrl":"10.1212/NXI.0000000000200265","url":null,"abstract":"<p><strong>Background and objectives: </strong>To evaluate CSF inflammatory markers with accumulation of cortical damage as well as disease activity in patients with early relapsing-remitting MS (RRMS).</p><p><strong>Methods: </strong>CSF levels of osteopontin (OPN) and 66 inflammatory markers were assessed using an immune-assay multiplex technique in 107 patients with RRMS (82 F/25 M, mean age 35.7 ± 11.8 years). All patients underwent regular clinical assessment and yearly 3T MRI scans for 2 years while 39 patients had a 4-year follow-up. White matter lesion number and volume, cortical lesions (CLs) and volume, and global cortical thickness (CTh) were evaluated together with the 'no evidence of disease activity' (NEDA-3) status, defined by no relapses, no disability worsening, and no MRI activity, including CLs.</p><p><strong>Results: </strong>The random forest algorithm selected OPN, CXCL13, TWEAK, TNF, IL19, sCD30, sTNFR1, IL35, IL16, and sCD163 as significantly associated with changes in global CTh. OPN and CXCL13 were most related to accumulation of atrophy after 2 and 4 years. In a multivariate linear regression model on CSF markers, OPN (<i>p</i> < 0.001), CXCL13 (<i>p</i> = 0.001), and sTNFR1 (<i>p</i> = 0.024) were increased in those patients with accumulating atrophy (adjusted R-squared 0.615). The 10 markers were added in a model that included all clinical, demographic, and MRI variables: OPN (<i>p</i> = 0.002) and IL19 (<i>p</i> = 0.022) levels were confirmed to be significantly increased in patients developing more CTh change over the follow-up (adjusted R-squared 0.619). CXCL13 and OPN also revealed the best association with NEDA-3 after 2 years, with OPN significantly linked to disability accumulation (OR 2.468 [1.46-5.034], <i>p</i> = 0.004) at the multivariate logistic regression model.</p><p><strong>Discussion: </strong>These data confirm and expand our knowledge on the prognostic role of the CSF inflammatory profile in predicting changes in cortical pathology and disease activity in early MS. The data emphasize a crucial role of OPN.</p>","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"11 5","pages":"e200265"},"PeriodicalIF":7.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11203401/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141451086","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Racial and Ethnic Disparities in the Incidence of Anti-NMDA Receptor Encephalitis.","authors":"Samir Alsalek, Kathryn B Schwarzmann, Sakar Budhathoki, Viridiana Hernandez-Lopez, Jessica B Smith, Bonnie H Li, Annette Langer-Gould","doi":"10.1212/NXI.0000000000200255","DOIUrl":"10.1212/NXI.0000000000200255","url":null,"abstract":"<p><strong>Objectives: </strong>To estimate the incidence of anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis.</p><p><strong>Methods: </strong>We conducted a retrospective cohort study of >10 million person-years of observation from members of Kaiser Permanente Southern California, 2011-2022. The electronic health record of individuals with text-string mention of <i>NMDA</i> and <i>encephalitis</i> were reviewed to identify persons who met diagnostic criteria for anti-NMDAR encephalitis. Age-standardized and sex-standardized incidences stratified by race and ethnicity were estimated according to the 2020 US Census population.</p><p><strong>Results: </strong>We identified 70 patients who met diagnostic criteria for anti-NMDAR encephalitis. The median age at onset was 23.7 years (IQR = 14.2-31.0 years), and 45 (64%) were female patients. The age-standardized and sex-standardized incidence of anti-NMDAR encephalitis per 1 million person-years was significantly higher in Black (2.94, 95% CI 1.27-4.61), Hispanic (2.17, 95% CI 1.51-2.83), and Asian/Pacific Island persons (2.02, 95% CI 0.77-3.28) compared with White persons (0.40, 95% CI 0.08-0.72). Ovarian teratomas were found in 58.3% of Black female individuals and 10%-28.6% in other groups.</p><p><strong>Discussion: </strong>Anti-NMDA receptor encephalitis disproportionately affected Black, Hispanic, or Asian/Pacific Island persons. Ovarian teratomas were a particularly common trigger in Black female individuals. Future research should seek to identify environmental and biological risk factors that disproportionately affect minoritized individuals residing in the United States.</p>","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"11 4","pages":"e200255"},"PeriodicalIF":7.8,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11089538/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140904825","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Synaptic Protein Loss in Extracellular Vesicles Reflects Brain and Retinal Atrophy in People With Multiple Sclerosis.","authors":"Dimitrios C Ladakis, Michael Vreones, Joseph Blommer, Kimystian L Harrison, Matthew D Smith, Eleni S Vasileiou, Hussein Moussa, Gelareh Ahmadi, Omar Ezzedin, Anna L DuVal, Blake E Dewey, Jerry L Prince, Kathryn C Fitzgerald, Elias S Sotirchos, Shiv Saidha, Peter A Calabresi, Dimitrios Kapogiannis, Pavan Bhargava","doi":"10.1212/NXI.0000000000200257","DOIUrl":"10.1212/NXI.0000000000200257","url":null,"abstract":"<p><strong>Objectives: </strong>To assess whether the rate of change in synaptic proteins isolated from neuronally enriched extracellular vesicles (NEVs) is associated with brain and retinal atrophy in people with multiple sclerosis (MS).</p><p><strong>Methods: </strong>People with MS were followed with serial blood draws, MRI (MRI), and optical coherence tomography (OCT) scans. NEVs were immunocaptured from plasma, and synaptopodin and synaptophysin proteins were measured using ELISA. Subject-specific rates of change in synaptic proteins, as well as brain and retinal atrophy, were determined and correlated.</p><p><strong>Results: </strong>A total of 50 people with MS were included, 46 of whom had MRI and 45 had OCT serially. The rate of change in NEV synaptopodin was associated with whole brain (rho = 0.31; <i>p</i> = 0.04), cortical gray matter (rho = 0.34; <i>p</i> = 0.03), peripapillary retinal nerve fiber layer (rho = 0.37; <i>p</i> = 0.01), and ganglion cell/inner plexiform layer (rho = 0.41; <i>p</i> = 0.006) atrophy. The rate of change in NEV synaptophysin was also correlated with whole brain (rho = 0.31; <i>p</i> = 0.04) and cortical gray matter (rho = 0.31; <i>p</i> = 0.049) atrophy.</p><p><strong>Discussion: </strong>NEV-derived synaptic proteins likely reflect neurodegeneration and may provide additional circulating biomarkers for disease progression in MS.</p>","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"11 4","pages":"e200257"},"PeriodicalIF":8.8,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11131364/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140956892","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Primary Angiitis of the CNS: Differences in the Profile Between Subtypes and Outcomes From an Indian Cohort.","authors":"Naveen K Paramasivan, Dev P Sharma, S M Krishna Mohan, Soumya Sundaram, Sapna E Sreedharan, P Sankara Sarma, P N Sylaja","doi":"10.1212/NXI.0000000000200262","DOIUrl":"10.1212/NXI.0000000000200262","url":null,"abstract":"<p><strong>Background and objectives: </strong>Primary angiitis of the CNS (PACNS) is a rare disease that has significant morbidity and mortality. Subtypes of PACNS can have different presentations that could be missed with certain diagnostic modalities, further increasing diagnostic complexity. We sought to distinguish the subtypes of PACNS and describe their outcomes in an Indian cohort.</p><p><strong>Methods: </strong>Adult patients in this retrospective single-center cohort study were reviewed from the PACNS database between 2000 and 2019. Diagnosis was made as per Calabrese and Malleck criteria. Small and medium vessel vasculitis was defined, and their clinical and radiologic profile, treatment, and outcomes were compared. Functional outcomes were noted at 6-month, 1-year, and at last follow-up, while relapses were noted at last follow-up. A poor outcome was defined as modified Rankin Scale >2.</p><p><strong>Results: </strong>Seventy-two patients fulfilled the inclusion criteria of whom 50 (69.4%) were male. The small vessel vasculitis subtype had a younger age at onset (30.5 vs 40.5 years, <i>p</i> = 0.014), presented less often as a stroke (22% vs 62%, <i>p</i> = 0.001), and had greater delay in diagnosis and treatment initiation (median of 620 days vs 118 days, <i>p</i> = 0.001) compared with medium vessel vasculitis subtype. Although no difference was noted at 6 months, the small vessel vasculitis group had poor outcomes at 1-year and last follow-up (57% vs 20%, <i>p</i> = 0.011 and 72% vs 34%, <i>p</i> = 0.005, respectively) and had more relapses at last follow-up (89% vs 30%, <i>p</i> < 0.001) when compared with the medium vessel vasculitis group. On analyzing the entire cohort, 50 of 72 (69%) and 37 of 53 (69.8%) patients had a good outcome at 6 months and 1 year, respectively. Relapse was noted in 35 of 72 (49%) at final follow-up. The choice of the treatment regimen did not predict outcomes or relapses.</p><p><strong>Discussion: </strong>The small vessel vasculitis subtype of PACNS is a distinct entity that has diagnostic and treatment delays with poor long-term outcomes and more relapses. Recognizing the different subtypes of PACNS may help to expedite diagnosis and plan treatment.</p>","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"11 4","pages":"e200262"},"PeriodicalIF":7.8,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11197987/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141301109","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}