Sakuma Hiroshi, Thomas Terrence, Debinski Carly, Eyre Michael, Han Velda X, Jones Hannah F, Kawano Go, Lee Vanessa W, Malone Stephen, Matsuishi Toyojiro, Mohammad Shekeeb S, Mori Takayuki, Nishida Hiroya, Nosadini Margherita, Takanashi Jun-Ichi, Mizuguchi Masashi, Lim Ming, Dale Russell C
Department of Brain & Neurosciences, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan.
Department of Paediatrics, Neurology Service, KK Women's and Children's Hospital, Singapore.
Dev Med Child Neurol. 2025 Feb;67(2):195-207. doi: 10.1111/dmcn.16067. Epub 2024 Aug 14.
To develop standardized diagnostic criteria for 'infection-triggered encephalopathy syndrome (ITES)' and five specific clinical syndromes of ITES.
The draft definitions were based on existing criteria, standardized, and discussed by a panel of international experts using nominal group technique over 18 months to achieve consensus. All criteria use the same format: (1) presence of infection/fever; (2) clinical features including encephalopathy; (3) neuroradiological features on magnetic resonance imaging; (4) exclusion of other causes.
We first highlighted differences between ITES and infectious and autoimmune encephalitis, which is the most important differential diagnosis. Consensus was achieved to define five specific ITESs: acute encephalopathy with biphasic seizures and late reduced diffusion; acute necrotizing encephalopathy; mild encephalopathy with a reversible splenial lesion; acute fulminant cerebral oedema; and acute shock with encephalopathy and multiorgan failure. Two further conditions that are currently classified as epilepsy syndromes but have similar features to ITES, namely febrile infection-related epilepsy syndrome and hemiconvulsion-hemiplegia-epilepsy syndrome, are also discussed.
The consensus definition is expected to improve awareness of this disease concept, provide diagnostic framework, and facilitate future international research and clinical trials.
制定“感染引发的脑病综合征（ITES）”及ITES五种特定临床综合征的标准化诊断标准。
定义草案基于现有标准制定，进行标准化处理，并由国际专家小组采用名义群体技术进行了18个月的讨论以达成共识。所有标准采用相同格式：（1）存在感染/发热；（2）临床特征包括脑病；（3）磁共振成像的神经放射学特征；（4）排除其他病因。
我们首先强调了ITES与感染性和自身免疫性脑炎之间的差异，这是最重要的鉴别诊断。就定义五种特定的ITES达成了共识：伴有双相发作和后期弥散受限的急性脑病；急性坏死性脑病；伴有可逆性胼胝体病变的轻度脑病；急性暴发性脑水肿；以及伴有脑病和多器官功能衰竭的急性休克。还讨论了另外两种目前归类为癫痫综合征但与ITES具有相似特征的病症，即发热感染相关癫痫综合征和偏瘫-偏瘫-癫痫综合征。
预计该共识定义将提高对这一疾病概念的认识，提供诊断框架，并促进未来的国际研究和临床试验。
