TY - JOUR
T1 - Diagnostic MRI Score to Differentiate Susac Syndrome from Primary Angiitis of the Central Nervous System and Multiple Sclerosis
AU - Marrodan, Mariano
AU - Calandri, Ismael L.
AU - Bocancea, Diana I.
AU - Ysrraelit, Maria C.
AU - Gomez Figueroa, Enrique
AU - Massó Páez, Montserrat
AU - Flores, José D. J.
AU - Rojas, Juan I.
AU - Ciampi, Ethel
AU - Ioli, Pablo
AU - Zanga, Gisela
AU - Ardohain, Carolina
AU - Fracaro, Maria E.
AU - Amaya, Mariela
AU - Tkachuk, Verónica
AU - Fernandez, Victoria C.
AU - José, Gustavo
AU - Silva, Emanuel
AU - Luetic, Geraldine
AU - Carnero Contentti, Edgar
AU - Köhler, Eduardo
AU - Pagani Cassara, Fatima
AU - Moran, Dolores
AU - Seimandi, Carla
AU - Paviolo, Juan P.
AU - D'elio, Brenda
AU - da Prat, Gustavo
AU - Gatto, Emilia
AU - Cristiano, Edgardo
AU - Pujol Lereis, Virginia
AU - Ameriso, Sebastian F.
AU - Fiol, Marcela P.
AU - Correale, Jorge
N1 - Publisher Copyright:
© 2024 American Neurological Association.
PY - 2024/11/1
Y1 - 2024/11/1
N2 - 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.
AB - 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.
UR - https://www.scopus.com/pages/publications/85199755953
U2 - 10.1002/ana.27043
DO - 10.1002/ana.27043
M3 - Article
C2 - 39056308
SN - 0364-5134
VL - 96
SP - 846
EP - 854
JO - Annals of neurology
JF - Annals of neurology
IS - 5
ER -