Mariano Marrodan, Ismael L Calandri, Diana I Bocancea, Maria C Ysrraelit, Enrique Gomez Figueroa, Montserrat Massó Páez, José D J Flores, Juan I Rojas, Ethel Ciampi, Pablo Ioli, Gisela Zanga, Carolina Ardohain, Maria E Fracaro, Mariela Amaya, Verónica Tkachuk, Victoria C Fernandez, Gustavo José, Emanuel Silva, Geraldine Luetic, Edgar Carnero Contentti, Eduardo Köhler, Fatima Pagani Cassara, Dolores Moran, Carla Seimandi, Juan P Paviolo, Brenda D'elio, Gustavo Da Prat, Emilia Gatto, Edgardo Cristiano, Virginia Pujol Lereis, Sebastian F Ameriso, Marcela P Fiol, Jorge Correale
{"title":"区分苏萨克综合征与中枢神经系统原发性血管炎和多发性硬化症的磁共振成像诊断评分。","authors":"Mariano Marrodan, Ismael L Calandri, Diana I Bocancea, Maria C Ysrraelit, Enrique Gomez Figueroa, Montserrat Massó Páez, José D J Flores, Juan I Rojas, Ethel Ciampi, Pablo Ioli, Gisela Zanga, Carolina Ardohain, Maria E Fracaro, Mariela Amaya, Verónica Tkachuk, Victoria C Fernandez, Gustavo José, Emanuel Silva, Geraldine Luetic, Edgar Carnero Contentti, Eduardo Köhler, Fatima Pagani Cassara, Dolores Moran, Carla Seimandi, Juan P Paviolo, Brenda D'elio, Gustavo Da Prat, Emilia Gatto, Edgardo Cristiano, Virginia Pujol Lereis, Sebastian F Ameriso, Marcela P Fiol, Jorge Correale","doi":"10.1002/ana.27043","DOIUrl":null,"url":null,"abstract":"<p><strong>Objective: </strong>Susac syndrome (SuS), multiple sclerosis (MS), and primary angiitis of the central nervous system (PACNS) present diagnostic challenges due to overlapping clinical features. We aimed to enhance diagnostic precision by developing the SPAMS (SuS, PACNS, MS) score, a practical radiological tool.</p><p><strong>Methods: </strong>This multicenter study included 99 patients (43 SuS, 37 MS, 19 PACNS) from South American countries. Relevant MRI features were identified through an elastic-net model determined key variables.</p><p><strong>Results: </strong>The SPAMS score assigned 2 points for snowball lesions, 1 point for spokes-like lesions, or if there are more than 4 lesions in the corpus callosum, corpus callosum involvement, or cerebellar involvement. It subtracted 1 point if gadolinium-enhancing lesions or 4 points if Dawson's fingers are present. Bootstrapping validated the optimal cutoff at 2 points, exhibiting a diagnostic performance of area under the curve = 0.931, sensitivity = 88%, specificity = 89%, positive predictive value = 88%, negative predictive value = 89%, and accuracy = 88%.</p><p><strong>Interpretation: </strong>When specific MRI findings coexisted, the SPAMS score differentiated SuS from MS and PACNS. Access to MRI and standard protocol sequences makes it a valuable tool for timely diagnosis and treatment, potentially preventing disability progression and severe clinical outcomes. ANN NEUROL 2024;96:846-854.</p>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":null,"pages":null},"PeriodicalIF":8.1000,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Diagnostic MRI Score to Differentiate Susac Syndrome from Primary Angiitis of the Central Nervous System and Multiple Sclerosis.\",\"authors\":\"Mariano Marrodan, Ismael L Calandri, Diana I Bocancea, Maria C Ysrraelit, Enrique Gomez Figueroa, Montserrat Massó Páez, José D J Flores, Juan I Rojas, Ethel Ciampi, Pablo Ioli, Gisela Zanga, Carolina Ardohain, Maria E Fracaro, Mariela Amaya, Verónica Tkachuk, Victoria C Fernandez, Gustavo José, Emanuel Silva, Geraldine Luetic, Edgar Carnero Contentti, Eduardo Köhler, Fatima Pagani Cassara, Dolores Moran, Carla Seimandi, Juan P Paviolo, Brenda D'elio, Gustavo Da Prat, Emilia Gatto, Edgardo Cristiano, Virginia Pujol Lereis, Sebastian F Ameriso, Marcela P Fiol, Jorge Correale\",\"doi\":\"10.1002/ana.27043\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Objective: </strong>Susac syndrome (SuS), multiple sclerosis (MS), and primary angiitis of the central nervous system (PACNS) present diagnostic challenges due to overlapping clinical features. We aimed to enhance diagnostic precision by developing the SPAMS (SuS, PACNS, MS) score, a practical radiological tool.</p><p><strong>Methods: </strong>This multicenter study included 99 patients (43 SuS, 37 MS, 19 PACNS) from South American countries. Relevant MRI features were identified through an elastic-net model determined key variables.</p><p><strong>Results: </strong>The SPAMS score assigned 2 points for snowball lesions, 1 point for spokes-like lesions, or if there are more than 4 lesions in the corpus callosum, corpus callosum involvement, or cerebellar involvement. It subtracted 1 point if gadolinium-enhancing lesions or 4 points if Dawson's fingers are present. Bootstrapping validated the optimal cutoff at 2 points, exhibiting a diagnostic performance of area under the curve = 0.931, sensitivity = 88%, specificity = 89%, positive predictive value = 88%, negative predictive value = 89%, and accuracy = 88%.</p><p><strong>Interpretation: </strong>When specific MRI findings coexisted, the SPAMS score differentiated SuS from MS and PACNS. Access to MRI and standard protocol sequences makes it a valuable tool for timely diagnosis and treatment, potentially preventing disability progression and severe clinical outcomes. ANN NEUROL 2024;96:846-854.</p>\",\"PeriodicalId\":127,\"journal\":{\"name\":\"Annals of Neurology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":8.1000,\"publicationDate\":\"2024-11-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Annals of Neurology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1002/ana.27043\",\"RegionNum\":1,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/7/26 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q1\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Annals of Neurology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1002/ana.27043","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/7/26 0:00:00","PubModel":"Epub","JCR":"Q1","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
Diagnostic MRI Score to Differentiate Susac Syndrome from Primary Angiitis of the Central Nervous System and Multiple Sclerosis.
Objective: Susac syndrome (SuS), multiple sclerosis (MS), and primary angiitis of the central nervous system (PACNS) present diagnostic challenges due to overlapping clinical features. We aimed to enhance diagnostic precision by developing the SPAMS (SuS, PACNS, MS) score, a practical radiological tool.
Methods: This multicenter study included 99 patients (43 SuS, 37 MS, 19 PACNS) from South American countries. Relevant MRI features were identified through an elastic-net model determined key variables.
Results: The SPAMS score assigned 2 points for snowball lesions, 1 point for spokes-like lesions, or if there are more than 4 lesions in the corpus callosum, corpus callosum involvement, or cerebellar involvement. It subtracted 1 point if gadolinium-enhancing lesions or 4 points if Dawson's fingers are present. Bootstrapping validated the optimal cutoff at 2 points, exhibiting a diagnostic performance of area under the curve = 0.931, sensitivity = 88%, specificity = 89%, positive predictive value = 88%, negative predictive value = 89%, and accuracy = 88%.
Interpretation: When specific MRI findings coexisted, the SPAMS score differentiated SuS from MS and PACNS. Access to MRI and standard protocol sequences makes it a valuable tool for timely diagnosis and treatment, potentially preventing disability progression and severe clinical outcomes. ANN NEUROL 2024;96:846-854.
期刊介绍:
Annals of Neurology publishes original articles with potential for high impact in understanding the pathogenesis, clinical and laboratory features, diagnosis, treatment, outcomes and science underlying diseases of the human nervous system. Articles should ideally be of broad interest to the academic neurological community rather than solely to subspecialists in a particular field. Studies involving experimental model system, including those in cell and organ cultures and animals, of direct translational relevance to the understanding of neurological disease are also encouraged.