Diagnostic MRI Score to Differentiate Susac Syndrome from Primary Angiitis of the Central Nervous System and Multiple Sclerosis

IF 7.7 1区医学 Q1 CLINICAL NEUROLOGY Annals of Neurology Pub Date : 2024-07-26 DOI:10.1002/ana.27043

Mariano Marrodan MD, Ismael L. Calandri MD, Diana I. Bocancea MSc, Maria C. Ysrraelit MD, Enrique Gomez Figueroa MD, Montserrat Massó Páez MD, José D.J. Flores MD, Juan I. Rojas MD, Ethel Ciampi MD, Pablo Ioli MD, Gisela Zanga MD, Carolina Ardohain MD, Maria E. Fracaro MD, Mariela Amaya MD, Verónica Tkachuk MD, Victoria C. Fernandez MD, Gustavo José MD, Emanuel Silva MD, Geraldine Luetic MD, Edgar Carnero Contentti MD, Eduardo Köhler MD, Fatima Pagani Cassara MD, Dolores Moran MD, Carla Seimandi MD, Juan P. Paviolo MD, Brenda D'elio MD, Gustavo Da Prat MD, Emilia Gatto MD, Edgardo Cristiano MD, Virginia Pujol Lereis MD, Sebastian F. Ameriso MD, Marcela P. Fiol MD, Jorge Correale MD

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Abstract

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

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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。

目的：苏萨克综合征（SuS）、多发性硬化症（MS）和中枢神经系统原发性血管炎（PACNS）的临床特征相互重叠，给诊断带来了挑战。我们的目标是通过开发 SPAMS（SuS、PACNS、MS）评分这一实用的放射学工具来提高诊断的精确性：这项多中心研究包括来自南美国家的 99 名患者（43 名 SuS、37 名 MS、19 名 PACNS）。通过弹性网模型确定关键变量，从而确定相关的磁共振成像特征：SPAMS评分为雪球状病变得2分，辐条状病变得1分，或胼胝体中有4个以上病变、胼胝体受累或小脑受累得1分。如果出现钆增强病变，则减去 1 分；如果出现道森指，则减去 4 分。Bootstrapping 验证了最佳分界点为 2 点，其诊断性能为曲线下面积 = 0.931、灵敏度 = 88%、特异性 = 89%、阳性预测值 = 88%、阴性预测值 = 89%、准确性 = 88%：当特定的 MRI 检查结果同时存在时，SPAMS 评分可将 SuS 与 MS 和 PACNS 区分开来。核磁共振成像和标准方案序列使其成为及时诊断和治疗的重要工具，有可能防止残疾进展和严重的临床后果。ann neurol 2024.

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来源期刊

Annals of Neurology 医学-临床神经学

CiteScore

18.00

自引率

1.80%

发文量

270

审稿时长

3-8 weeks

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.