Neurology® Neuroimmunology & Neuroinflammation最新文献

{"title":"Clinical and Paraclinical Characterizations, Management, and Prognosis in DPPX Antibody-Associated Encephalitis: A Systematic Review.","authors":"Er-Chuang Li, Tian-Yi Zhang, Meng-Ting Cai, Sheng-Yao Su, Chun-Hong Shen, Qi-Lun Lai, Yin-Xi Zhang","doi":"10.1212/NXI.0000000000200350","DOIUrl":"10.1212/NXI.0000000000200350","url":null,"abstract":"<p><p>In dipeptidyl-peptidase-like protein 6 (DPPX) antibody-associated encephalitis, DPPX antibodies from serum and CSF target the extracellular subunit of the voltage-gated potassium channel 4.2. This targeting leads to a characteristic clinical triad comprising gastrointestinal symptoms (predominantly diarrhea), cognitive-psychiatric dysfunction, and manifestations of CNS hyperexcitability, with hyperekplexia being a more specific feature. This rare disease typically presents with a subacute or chronic course and often affects middle-aged and older individuals. Patients may have a weak association with certain hematologic malignancies, particularly lymphoma and chronic lymphocytic leukemia. Brain MRI typically shows normal findings or nonspecific white matter changes. DPPX antibody-associated encephalitis responds well to immunotherapy, and most patients ultimately present with a good prognosis. However, relapses can occur. To improve our understanding of this rare but treatable autoimmune encephalitis and avoid misdiagnosis, we conduct a systematic review and summarize the current knowledge of its clinical and paraclinical features, management, and prognosis.</p>","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"12 2","pages":"e200350"},"PeriodicalIF":7.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11658814/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142854875","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 Characterization and Prognostic Risk Factors of Susac Syndrome: A Retrospective Multicenter Study.","authors":"Lior Fuchs, Adi Wilf-Yarkoni, Hadar Kolb, Ifat Vigiser, Keren Regev, Dinah Zur, Zohar Habot-Wilner, Yahav Oron, Viktoria Furer, Nitai Shimon, Mark A Hellmann, Itay Lotan, Eitan Auriel, Robert Rennebohm, Ori Elkayam, Arnon Karni","doi":"10.1212/NXI.0000000000200357","DOIUrl":"10.1212/NXI.0000000000200357","url":null,"abstract":"<p><strong>Background and objectives: </strong>Susac syndrome (SuS) is a rare disorder characterized by encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss, often accompanied by vertigo. Recent updates to diagnostic criteria and treatment guidelines have been made. This study examines clinical manifestations; disease activity; and risk factors of disability, dependency, and return to work in patients with SuS.</p><p><strong>Methods: </strong>A retrospective multicenter study was conducted on 20 consecutive patients with SuS with at least 2 years of follow-up. Clinical and paraclinical activities were assessed and rated according to the severity at onset and the end of follow-up. Cognitive function was assessed using the Montreal Cognitive Assessment while disability and dependence in daily activities were measured using the modified Rankin Scale. Employment status was graded.</p><p><strong>Results: </strong>The mean age at onset was 38.9 years, with a mean follow-up of 55.9 months. The female-to-male ratio was 1.86, and 45% of patients had the complete clinical triad. Severe cerebral involvement at onset was associated with a higher risk of cerebral exacerbations within the first year and with an increased long-term disability and dependency. Cognitive function improved in 75% of patients during follow-up. At disease onset, hearing loss excluding low frequencies occurred in 46.7%. Relapse of hearing loss was associated with greater impairment in daily activities. Male sex and elevated CSF protein levels were linked to poorer prognosis. Cerebral and inner ear exacerbations were most common in the first year while retinal exacerbations occurred more frequently, mainly within the first 2 years. Approximately 50% of patients resumed employment while 25% did not return to work.</p><p><strong>Discussion: </strong>Current treatment strategies for SuS do not fully prevent relapses. Severe brain manifestation at onset, male sex, and high CSF protein levels are risk factors of a worse prognosis of disability and dependence, indicating the need for intensive treatment. High-frequency hearing loss does not exclude SuS diagnosis.</p>","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"12 2","pages":"e200357"},"PeriodicalIF":7.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11658810/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142854877","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":"Profile and Usefulness of Serum Cytokines to Predict Prognosis in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.","authors":"Javier Villacieros-Álvarez, Carmen Espejo, Georgina Arrambide, Alessandro Dinoto, Patricia Mulero, Laura Rubio-Flores, Pablo Nieto, Carmen Alcalá, Jose E Meca-Lallana, Jorge Millan-Pascual, Pedro Martínez-García, Raphael Bernard-Valnet, Inés González-Suárez, Aída Orviz, Raquel Téllez, Laura Navarro Cantó, Silvia Presas-Rodríguez, Sergio Martínez-Yélamos, Juan Pablo Cuello, Ana Alonso, Raquel Piñar Morales, Gary Álvarez Bravo, Lakhdar Benyahya, Sophie Trouillet-Assant, Virginie Dyon-Tafan, Caroline Froment Tilikete, Aurélie Ruet, Bertrand Bourre, Romain Deschamps, Caroline Papeix, Elisabeth Maillart, Philippe Kerschen, Xavier Ayrignac, Àlex Rovira, Cristina Auger, Bertrand Audoin, Xavier Montalban, Mar Tintore, Sara Mariotto, Alvaro Cobo-Calvo, Romain Marignier","doi":"10.1212/NXI.0000000000200362","DOIUrl":"https://doi.org/10.1212/NXI.0000000000200362","url":null,"abstract":"<p><strong>Objectives: </strong>To characterize the serum cytokine profile in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) at onset and during follow-up and assess their utility for predicting relapses and disability.</p><p><strong>Methods: </strong>This retrospective multicentric cohort study included patients aged 16 years and older meeting MOGAD 2023 criteria, with serum samples collected at baseline (≤3 months from disease onset) and follow-up (≥6 months from the baseline), and age-matched and time to sampling-matched patients with multiple sclerosis (MS). Eleven cytokines were assessed using the ELLA system. Data comparisons and statistical analyses between cytokine levels and clinical outcomes were performed.</p><p><strong>Results: </strong>Eighty-eight patients with MOGAD and 32 patients with MS were included. Patients with MOGAD showed higher IL6 (<i>p</i> = 0.036), IL8 (<i>p</i> = 0.012), and IL18 (<i>p</i> = 0.026) baseline levels compared with those with MS, in non-optic neuritis (ON) presentations. BAFF values increased over time, especially in patients with MOGAD treated with anti-CD20 (<i>p</i> = 0.002). Baseline BAFF, CXCL10, IL10, and IL8 levels correlated with disease severity at MOGAD onset (all <i>p</i> < 0.05). Finally, higher baseline BAFF levels predicted lower risk of relapses (hazard ratio 0.41 [0.19; 0.89], <i>p</i> = 0.024).</p><p><strong>Discussion: </strong>This study suggests a proinflammatory Th17-dominant profile in non-ON MOGAD patients, with a novel finding of a potential protective role of BAFF on relapses. These results shed new light on the pathogenesis of MOGAD, potentially guiding therapeutic decisions.</p>","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"12 2","pages":"e200362"},"PeriodicalIF":7.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142927655","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Alterations in Gut Microbiome-Host Relationships After Immune Perturbation in Patients With Multiple Sclerosis.","authors":"Vinod K Gupta, Guneet S Janda, Heather K Pump, Nikhil Lele, Isabella Cruz, Inessa Cohen, William E Ruff, David A Hafler, Jaeyun Sung, Erin E Longbrake","doi":"10.1212/NXI.0000000000200355","DOIUrl":"10.1212/NXI.0000000000200355","url":null,"abstract":"<p><strong>Background and objectives: </strong>Gut microbial symbionts have been shown to influence the development of autoimmunity in multiple sclerosis (MS). Emerging research points to an important relationship between the microbial-IgA interface and MS pathophysiology. IgA-secreting B cells are observed in the MS brain, and shifts in gut bacteria-IgA binding have been described in some patients with MS. However, the relationships between the gut microbiome and the host immune response, particularly regarding B-cell-depleting immunomodulation, remain underexplored. This study aimed to evaluate the composition of the gut microbiome in patients with newly diagnosed MS at baseline and after B-cell depletion, using long-read sequencing for enhanced taxonomic resolution. We further aimed to investigate the host/microbiome interface by evaluating microbe/immunoglobulin A relationships.</p><p><strong>Methods: </strong>We collected stool samples from 43 patients with newly diagnosed, untreated MS and 42 matched healthy controls. Nineteen patients with MS initiated anti-CD20 monoclonal antibody treatment and donated additional stool samples after 6 months of treatment. We evaluated the host-microbial interface using bacterial flow cytometry and long-read 16S rRNA gene amplicon sequencing. We used Immune Coating Scores to compare the proportions of bacteria identified in the IgA-coated vs IgA-uncoated bacterial fractions.</p><p><strong>Results: </strong>Patients with untreated, newly diagnosed MS showed significant reductions in IgA-bound fecal microbiota compared with controls. Using multiple linear regression models adjusted for potential confounders, we observed significant (<i>p</i> < 0.05) changes in the abundance and prevalence of various strain-level gut bacteria amplicon sequence variants (ASVs) within both total and IgA-coated bacterial fractions. Some changes (e.g., decreased relative abundance of a <i>Faecalibacterium prausnitzii</i> variant in MS) were consistent with previous reports, while others (e.g., increased relative abundance and prevalence of <i>Monoglobus pectinyliticus</i> in MS) were novel. Immune Coating Scores identified subsets of organisms for which normal IgA-coating patterns were disrupted at the onset of MS, as well as those (particularly <i>Akkermansia muciniphila</i>) whose IgA-coating became more aligned with controls after therapy.</p><p><strong>Discussion: </strong>This analysis of gut microbial ASVs reveals shifts in taxonomic strains induced by immune modulation in MS.</p>","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"12 2","pages":"e200355"},"PeriodicalIF":7.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11741292/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143009109","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":"Modulator of VRAC Current 1 Is a Potential Target Antigen in Multiple Sclerosis.","authors":"Johannes Raffael Dahl, Alicia Weier, Christopher Winter, Maik Hintze, Veit Rothhammer, Thanos Tsaktanis, Anne-Katrin Proebstel, Tradite Neziraj, Elisabeth Poessnecker, Johanna Oechtering, Jens Kuhle, Boris-Alexander Kallmann, Gabriele Luber, Thorsten Heider, Luisa Klotz, Rittika Chunder, Stefanie Kuerten","doi":"10.1212/NXI.0000000000200374","DOIUrl":"https://doi.org/10.1212/NXI.0000000000200374","url":null,"abstract":"<p><strong>Background and objectives: </strong>Multiple sclerosis (MS) is a chronic immune-mediated demyelinating disease of the CNS. Highlighted by the success of B-cell-depleting therapies such as the monoclonal anti-CD20 antibodies rituximab, ocrelizumab, and ofatumumab, B cells have been shown to play a central role in the immunopathology of the disease. Yet, the target antigens of the pathogenic B-cell response in MS remain unclear.</p><p><strong>Methods: </strong>We combined polyclonal B-cell stimulation of peripheral blood mononuclear cells with a human proteome-wide protein microarray to identify target antigens of MS by comparing samples from 20 patients with MS with 9 age-matched and sex-matched healthy controls. Results were verified by enzyme-linked immunosorbent assay (ELISA) in 3 independent validation cohorts (N = 47 patients with MS in remission; N = 20 patients with MS during relapse; N = 25 HCs; N = 30 patients with other noninflammatory neurologic diseases; N = 9 patients with other inflammatory neurologic diseases). Experimental autoimmune encephalomyelitis (EAE) was used as an animal model to evaluate the pathogenicity of the antibodies of choice.</p><p><strong>Results: </strong>Our results corroborate the existing concept of a highly diverse autoimmune response in MS. Yet, a significantly elevated antibody response against the membrane protein modulator of VRAC current 1 (MLC1) was noted in B-cell culture supernatants and serum samples of patients with MS. Furthermore, significantly elevated titers to MLC1 were observed in the CSF of patients with neuroinflammatory diseases other than MS. Neurons and astrocytes were identified as the main cell types expressing MLC1 in the brain of a patient with MS. Injection of anti-MLC1 antibodies into mice with EAE led to strong in vivo binding to cerebral cortical neurons and to the death of 4 of the 7 injected mice.</p><p><strong>Discussion: </strong>Future studies will have to address the diagnostic and prognostic value of MLC1-specific antibodies in neuroinflammatory disorders such as MS and characterize the functional role of MLC1 expression in neurons and astrocytes.</p><p><strong>Trial registration information: </strong>The study has been registered in the German Clinical Trials Register (study number DRKS00015528).</p>","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"12 2","pages":"e200374"},"PeriodicalIF":7.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143399322","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Microstructural Damage and Repair in the Spinal Cord of Patients With Early Multiple Sclerosis and Association With Disability at 5 Years.","authors":"Malo Gaubert, Benoit Combès, Elise Bannier, Arthur Masson, Vivien Caron, Gaëlle Baudron, Jean-Christophe Ferré, Laure Michel, Emmanuelle Le Page, Bruno Stankoff, Gilles Edan, Benedetta Bodini, Anne Kerbrat","doi":"10.1212/NXI.0000000000200333","DOIUrl":"10.1212/NXI.0000000000200333","url":null,"abstract":"<p><strong>Background and objectives: </strong>The dynamics of microstructural spinal cord (SC) damage and repair in people with multiple sclerosis (pwMS) and their clinical relevance have yet to be explored. We set out to describe patient-specific profiles of microstructural SC damage and change during the first year after MS diagnosis and to investigate their associations with disability and SC atrophy at 5 years.</p><p><strong>Methods: </strong>We performed a longitudinal monocentric cohort study among patients with relapsing-remitting MS: first relapse <1 year, no relapse <1 month, and high initial severity on MRI (>9 T2 lesions on brain MRI and/or initial myelitis). pwMS and age-matched healthy controls (HCs) underwent cervical SC magnetization transfer (MT) imaging at baseline and at 1 year for pwMS. Based on HC data, SC MT ratio <i>z</i>-score maps were computed for each person with MS. An index of microstructural damage was calculated as the proportion of voxels classified as normal at baseline and identified as damaged after 1 year. Similarly, an index of repair was also calculated (voxels classified as damaged at baseline and as normal after 1 year). Linear models including these indices and disability or SC cross-sectional area (CSA) change between baseline and 5 years were implemented.</p><p><strong>Results: </strong>Thirty-seven patients and 19 HCs were included. We observed considerable variability in the extent of microstructural SC damage at baseline (0%-58% of SC voxels). We also observed considerable variability in damage and repair indices over 1 year (0%-31% and 0%-20%), with 18 patients showing predominance of damage and 18 predominance of repair. The index of microstructural damage was associated positively with the Expanded Disability Status Scale score (<i>r</i> = 0.504, <i>p</i> = 0.002) and negatively with CSA change (<i>r</i> = -0.416, <i>p</i> = 0.02) at 5 years, independent of baseline SC lesion volume.</p><p><strong>Discussion: </strong>People with early relapsing-remitting MS exhibited heterogeneous profiles of microstructural SC damage and repair. Progression of microstructural damage was associated with disability progression and SC atrophy 5 years later. These results indicate a potential for microstructural repair in the SC to prevent disability progression in pwMS.</p>","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"12 1","pages":"e200333"},"PeriodicalIF":7.8,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11587990/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142687545","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":"Ten Years of <i>Neurology</i>® <i>Neuroimmunology & Neuroinflammation</i>: Decade in Review.","authors":"Josep Dalmau, Marinos C Dalakas, Dennis L Kolson, Anne-Katrin Pröbstel, Friedemann Paul, Scott S Zamvil","doi":"10.1212/NXI.0000000000200363","DOIUrl":"10.1212/NXI.0000000000200363","url":null,"abstract":"","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"12 1","pages":"e200363"},"PeriodicalIF":7.8,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11676263/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142896407","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":"\"Lupus Myelitis\" Revisited: A Retrospective Single-Center Study of Myelitis Associated With Rheumatologic Disease.","authors":"Jonathan D Krett, Angeliki G Filippatou, Paula Barreras, Carlos A Pardo, Allan C Gelber, Elias S Sotirchos","doi":"10.1212/NXI.0000000000200329","DOIUrl":"10.1212/NXI.0000000000200329","url":null,"abstract":"<p><strong>Background and objectives: </strong>Previous reports of patients with myelitis associated with rheumatologic disease may have had unrecognized aquaporin-4 (AQP4)-IgG seropositive neuromyelitis optica spectrum disorder (NMOSD) or myelin oligodendrocyte glycoprotein (MOG)-IgG-associated disease (MOGAD). We clinicoradiologically and serologically characterized patients with myelitis associated with rheumatologic disease evaluated in the era of availability of MOG-IgG and more sensitive AQP4-IgG cell-based assays.</p><p><strong>Methods: </strong>A retrospective cohort (2018-2023) at Johns Hopkins Medicine with diagnoses of myelopathy and rheumatologic comorbidity was identified by electronic medical record (EMR) query. All patients with myelitis unrelated to typical multiple sclerosis (MS) were included and analyzed by chart review.</p><p><strong>Results: </strong>Of 238 patients identified by EMR query, 197 were excluded (148 not meeting prespecified inclusion criteria, 49 had typical MS), resulting in 41 patients for review. The mean age at myelitis onset was 44 ± 15 years; 39 (95%) were female. Rheumatologic diagnoses included 17 (41.5%) with systemic lupus erythematosus (SLE), 10 (24.3%) Sjögren syndrome (SS), 6 (15%) undifferentiated connective tissue disease (UCTD), 5 (12%) combinations of SLE/SS/UCTD with antiphospholipid antibody syndrome, 1 (2.4%) rheumatoid arthritis, 1 (2.4%) psoriatic arthritis, and 1 (2.4%) Behçet disease. 20 patients (49%) were diagnosed with AQP4-IgG seropositive NMOSD, 3 (7%) with MOGAD, and 18 (44%) had \"double-seronegative\" myelitis. Of these 18, 3 were diagnosed with AQP4-IgG seronegative NMOSD, 1 neuro-Behçet disease, and 14 other (unclassifiable) myelitis. Excluding 1 patient with neuro-Behçet disease, 18 (90%) of 20 AQP4-IgG seropositive patients had longitudinally extensive cord lesions compared with 5 (29%; <i>p</i> < 0.001) of 17 \"double-seronegative\" patients and 2 (67%) of 3 with MOGAD. \"Double-seronegative\" patients more commonly had CSF-restricted oligoclonal bands. Functional outcomes did not differ by diagnosis, and most patients received acute immunotherapy at the time of initial myelitis diagnosis with at least partial recovery over a median follow-up of 38 (interquartile range: 9-74) months.</p><p><strong>Discussion: </strong>Approximately half of our rheumatologic disease cohort with myelitis unrelated to MS had AQP4-IgG seropositive NMOSD while MOGAD accounted for a small but clinically relevant proportion of patients. Further research is needed to characterize myelitis etiology in patients who are seronegative for both AQP4-IgG and MOG-IgG.</p>","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"12 1","pages":"e200329"},"PeriodicalIF":7.8,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11727606/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142504995","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":"Cerebellar Involvement in Attacks of Aquaporin-4-IgG Positive Neuromyelitis Optica Spectrum Disorder.","authors":"Alessandro Dinoto, Laura Cacciaguerra, Karl N Krecke, John Jing-Wei Chen, Dean M Wingerchuk, Brian G Weinshenker, Samantha A Banks, Alfonso Sebastian Lopez-Chiriboga, Cristina Valencia-Sanchez, Elia Sechi, Sean J Pittock, Eoin P Flanagan","doi":"10.1212/NXI.0000000000200344","DOIUrl":"10.1212/NXI.0000000000200344","url":null,"abstract":"<p><strong>Objectives: </strong>To characterize the frequency and clinicoradiologic phenotype of cerebellar involvement in attacks of aquaporin-4-IgG positive neuromyelitis optica spectrum disorder (AQP4+NMOSD) which are incompletely captured in current diagnostic criteria.</p><p><strong>Methods: </strong>Brain MRI scans from patients with AQP4+NMOSD in the Mayo Clinic database were reviewed, and those with cerebellar T2-hyperintense lesions ≤30 days from attack onset were included for clinical and radiologic characterization.</p><p><strong>Results: </strong>From 432 patients with AQP4+NMOSD, we identified 17 (4%) with cerebellar attacks. The median age at attack onset was 47 years (range, 7-74). Cerebellar symptoms and signs were noted in 16 (94%) of 17 and the remaining patient was intubated preventing a detailed cerebellar exam. The median Expanded Disability Status Scale score at nadir was 5 (range, 2-9.5). Sixteen (94%) had other regions involved during the attack, most frequently with brainstem or area postrema involvement. Cerebellar MRI T2-lesions (8 single; 11 contiguous with the brainstem; 6/15 [35%] enhancing) were located in cerebellar peduncles, 15 (inferior, 5; middle, 10; superior, 10), and cerebellar parenchyma, 8 (dentate, 4; medial, 2; lateral, 4). T2-lesions persisted in 9 (82%) of 11 beyond 6 months.</p><p><strong>Discussion: </strong>Cerebellar involvement during attacks of AQP4+NMOSD is rare but the associated neurologic deficits tend to be severe. Cerebellar peduncle or dentate nucleus T2-lesions are frequent MRI accompaniments. Clinical features and MRI lesion patterns of cerebellar involvement could be incorporated into future iterations of AQP4+NMOSD criteria.</p>","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"12 1","pages":"e200344"},"PeriodicalIF":7.8,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11637506/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142813866","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":"Pharmacokinetics and Pharmacodynamics of Natalizumab 6-Week Dosing vs Continued 4-Week Dosing for Relapsing-Remitting Multiple Sclerosis.","authors":"","doi":"10.1212/NXI.0000000000200354","DOIUrl":"10.1212/NXI.0000000000200354","url":null,"abstract":"","PeriodicalId":19472,"journal":{"name":"Neurology® Neuroimmunology & Neuroinflammation","volume":"12 1","pages":"e200354"},"PeriodicalIF":7.8,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11620544/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142780801","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}