From the Department of Neurology and Mayo Clinic Center for Multiple Sclerosis and Autoimmune Neurology (L.C., W.O.T., J.J.C., S.A.B., V.R., J.-M.T., Y.G., S.J.P., C.F.L., E.P.F.), Mayo Clinic, Rochester, MN; Vita-Salute San Raffaele University (L.C., F.M., M.A.R., M.F.); Neuroimaging Research Unit (L.C., M.A.R., M.F.), Division of Neuroscience, IRCCS San Raffaele Scientific Institute, Milan, Italy; Department of Radiology (P.M.), Department of Ophthalmology (J.J.C.), Mayo Clinic, Rochester, MN; Department of Neurology (P.E.), San Antonio Military Medical Center, Fort Sam Houston, TX; Neurology Unit (F.M., M.A.R., M.F.), IRCCS San Raffaele Scientific Institute, Milan; Department of Medical, Surgical and Experimental Sciences (E.S.), University of Sassari, Italy; Department of Neurology (A.S.L.-C.), Mayo Clinic, Jacksonville, FL; Department of Neurology (N.Z.), Mayo Clinic, Scottsdale, AZ; Neurorehabilitation Unit (M.F.), IRCCS San Raffaele Scientific Institute; Neurophysiology Service (M.F.), IRCCS San Raffaele Scientific Institute, Milan, Italy; and Laboratory Medicine and Pathology (S.J.P., E.P.F.), Mayo Clinic, Rochester, MN.
Neurology. 2023 Mar 28;100(13):e1418-e1432. doi: 10.1212/WNL.0000000000206820. Epub 2023 Jan 23.
Studies on tumefactive brain lesions in myelin oligodendrocyte glycoprotein-immunoglobulin G (IgG)-associated disease (MOGAD) are lacking. We sought to characterize the frequency clinical, laboratory, and MRI features of these lesions in MOGAD and compare them with those in multiple sclerosis (MS) and aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder (AQP4+NMOSD).
We retrospectively searched 194 patients with MOGAD and 359 patients with AQP4+NMOSD with clinical/MRI details available from the Mayo Clinic databases and included those with ≥1 tumefactive brain lesion (maximum transverse diameter ≥2 cm) on MRI. Patients with tumefactive MS were identified using the Mayo Clinic medical record linkage system. Binary multivariable stepwise logistic regression identified independent predictors of MOGAD diagnosis; Cox proportional regression models were used to assess the risk of relapsing disease and gait aid in patients with tumefactive MOGAD vs those with nontumefactive MOGAD.
We included 108 patients with tumefactive demyelination (MOGAD = 43; AQP4+NMOSD = 16; and MS = 49). Tumefactive lesions were more frequent among those with MOGAD (43/194 [22%]) than among those with AQP4+NMOSD (16/359 [5%], < 0.001). Risk of relapse and need for gait aid were similar in tumefactive and nontumefactive MOGAD. Clinical features more frequent in MOGAD than in MS included headache (18/43 [42%] vs 10/49 [20%]; = 0.03) and somnolence (12/43 [28%] vs 2/49 [4%]; = 0.003), the latter also more frequent than in AQP4+NMOSD (0/16 [0%]; = 0.02). The presence of peripheral T2-hypointense rim, T1-hypointensity, diffusion restriction (particularly an arc pattern), ring enhancement, and Baló-like or cystic appearance favored MS over MOGAD ( ≤ 0.001). MRI features were broadly similar in MOGAD and AQP4+NMOSD, except for more frequent diffusion restriction in AQP4+NMOSD (10/15 [67%]) than in MOGAD (11/42 [26%], = 0.005). CSF analysis revealed less frequent positive oligoclonal bands in MOGAD (2/37 [5%]) than in MS (30/43 [70%], < 0.001) and higher median white cell count in MOGAD than in MS (33 vs 6 cells/μL, < 0.001). At baseline, independent predictors of MOGAD diagnosis were the presence of somnolence/headache, absence of T2-hypointense rim, lack of T1-hypointensity, and no diffusion restriction (Nagelkerke = 0.67). Tumefactive lesion resolution was more common in MOGAD than in MS or AQP4+NMOSD and improved model performance.
Tumefactive lesions are frequent in MOGAD but not associated with a worse prognosis. The clinical, MRI, and CSF attributes of tumefactive MOGAD differ from those of tumefactive MS and are more similar to those of tumefactive AQP4+NMOSD with the exception of lesion resolution, which favors MOGAD.
髓鞘少突胶质细胞糖蛋白免疫球蛋白 G（MOG-IgG）相关疾病（MOGAD）的瘤样脑病变研究较少。我们旨在描述 MOGAD 瘤样病变的临床、实验室和 MRI 特征，并与多发性硬化（MS）和水通道蛋白 4 免疫球蛋白 G 阳性视神经脊髓炎谱系疾病（AQP4+NMOSD）进行比较。
我们回顾性检索了梅奥诊所数据库中具有临床/MRI 详细资料的 194 例 MOGAD 患者和 359 例 AQP4+NMOSD 患者的资料，并纳入了 MRI 上至少有 1 个瘤样脑病变（最大横径≥2cm）的患者。使用梅奥诊所病历链接系统确定瘤样 MS 患者。二元多变量逐步逻辑回归确定了 MOGAD 诊断的独立预测因素；使用 Cox 比例风险回归模型评估瘤样 MOGAD 患者与非瘤样 MOGAD 患者复发疾病和使用助行器的风险。
我们纳入了 108 例瘤样脱髓鞘病变患者（MOGAD=43 例，AQP4+NMOSD=16 例，MS=49 例）。MOGAD 瘤样病变的发生率（43/194[22%]）高于 AQP4+NMOSD（16/359[5%]，<0.001）。瘤样和非瘤样 MOGAD 患者的复发风险和使用助行器的需求相似。MOGAD 比 MS 更常见的临床特征包括头痛（18/43[42%]比 10/49[20%]， = 0.03）和嗜睡（12/43[28%]比 2/49[4%]， = 0.003），后者也比 AQP4+NMOSD 更常见（0/16[0%]， = 0.02）。外周 T2 低信号环、T1 低信号、弥散受限（特别是弧形模式）、环形增强和类似 Baló 或囊性外观的存在提示 MS 而非 MOGAD（≤0.001）。MOGAD 和 AQP4+NMOSD 的 MRI 特征大致相似，除了 AQP4+NMOSD 更常见弥散受限（10/15[67%]）而非 MOGAD（11/42[26%]， = 0.005）。CSF 分析显示，MOGAD 中寡克隆带阳性的频率（2/37[5%]）低于 MS（30/43[70%]，<0.001），而 MOGAD 的白细胞中位数高于 MS（33 比 6 个细胞/μL，<0.001）。基线时，MOGAD 诊断的独立预测因素是嗜睡/头痛、无 T2 低信号环、无 T1 低信号、无弥散受限（Nagelkerke = 0.67）。MOGAD 瘤样病变的缓解率高于 MS 或 AQP4+NMOSD，改善了模型性能。
MOGAD 瘤样病变较常见，但与预后不良无关。MOGAD 瘤样病变的临床、MRI 和 CSF 特征与 MS 和 AQP4+NMOSD 不同，与 AQP4+NMOSD 相似，除了病变缓解，这更有利于 MOGAD。
