Unilateral cortical FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES): characterization of a distinct clinico-radiographic syndrome
Unilateral cortical FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES): characterization of a distinct clinico-radiographic syndrome
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DOI：
10.1007/s00415-019-09440-8
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发表时间：
2019-10-01
影响因子：
6
通讯作者：
Nicolle, M. W.
中科院分区：
文献类型：
--
作者：
Budhram, A.;Mirian, A.;Nicolle, M. W.
目的探讨抗MOG相关性癫痫发作（FLAMES）患者单侧大脑皮层FLAIR高信号病变的临床特征和MRI表现。方法本文报告一例单侧皮质FLAMES的病例报告，并对文献进行系统回顾。对病例进行审查，以确定临床症状（癫痫发作、头痛、发热和与FLAMES位置相关的皮质症状）的频率，并确定MRI异常是否仅限于该综合征的单侧皮质。结果20例单侧皮质FLAMES患者。其中，17/20例（85%）有癫痫发作，14/20例（70%）有头痛，13/20例（65%）有发热，11/20例（55%）报告了与FLAMES位置相关的皮质症状，19/20例（95%）报告了这四种结果中的至少两种。MRI上4/20例（20%）对侧大脑半球皮质信号异常，6/20例（30%）有脑膜炎的MRI表现。结论单侧皮质FLAMES患者临床表现以癫痫发作、头痛、发热及与FLAMES部位相关的皮质症状多见。虽然最初被描述为单侧皮质脑炎，但双侧皮质受累和可能的脑膜炎可能表明疾病谱更广。识别这种独特的临床放射学综合征可能有助于及时诊断和治疗。
Objective To characterize the clinical symptoms and magnetic resonance imaging (MRI) findings of unilateral cortical FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES). Methods This is a case report and systematic review of the literature to identify cases of unilateral cortical FLAMES. Cases were reviewed to determine the frequency of clinical symptoms (seizures, headache, fever and cortical symptoms referable to FLAMES location), and to determine whether MRI abnormalities are restricted to the unilateral cortex in this syndrome. Results We identified 20 cases of unilateral cortical FLAMES for review. Among them, 17/20 (85%) had seizures, 14/20 (70%) had headache, 13/20 (65%) had fever, 11/20 (55%) reported cortical symptoms referable to the FLAMES location, and 19/20 (95%) reported at least two of these four findings. On MRI 4/20 (20%) had some contralateral hemispheric cortical signal abnormality, and 6/20 (30%) had MRI findings concerning for meningeal inflammation. Conclusions In patients with unilateral cortical FLAMES, the clinical symptoms of seizures, headache, fever and cortical symptoms referable to the FLAMES location are frequent. Although initially described as a unilateral cortical encephalitis, bilateral cortical involvement and possible meningeal inflammation could indicate a broader disease spectrum. Recognition of this distinct clinico-radiographic syndrome may facilitate prompt diagnosis and treatment.