Bova Stefania Maria, Serafini Ludovica, Capetti Pietro, Dallapiccola Andrea Riccardo, Doneda Chiara, Gadda Arianna, Lonoce Luisa, Vittorini Alessandra, Mannarino Savina, Veggiotti Pierangelo
Pediatric Neurology Unit, V. Buzzi Children's Hospital, ASST Fatebenefratelli Sacco, Milan, Italy.
Department of Biomedical and Clinical Science, University of Milan, Milan, Italy.
Front Pediatr. 2022 Aug 12;10:932208. doi: 10.3389/fped.2022.932208. eCollection 2022.
To describe neurological involvement in multisystem inflammatory syndrome in children (MIS-C) and to evaluate whether neurological manifestations are related to the degree of multiorgan involvement and inflammation.
The authors conducted a retrospective analysis of clinical, electroencephalographic (EEG), neuroradiological (MRI), and CSF parameters in 62 children with MIS-C (45 M, age 8 months-17 years, mean age 9 years) hospitalized between October 1, 2020 and March 31, 2022.
Neurological involvement was documented in 58/62 (93.5%) patients. Altered mental status was observed in 29 (46.7%), focal neurological signs in 22 (35.4%), and non-specific symptoms in 54 (87%). EEG was performed in 26/62 children: 20 showed EEG slowing, diffuse or predominantly over the posterior regions. Ten patients underwent brain MRI: three showed a cytotoxic lesion of the corpus callosum. CSF analysis, performed in six patients, was normal. On the basis of the clinical and EEG findings, two profiles of neurological involvement were identified: 16/62 (26%) patients presented encephalitis with rapid-onset encephalopathy, focal neurological signs, and EEG slowing; 42/62 (68%) showed mild neurological involvement with mild or non-specific neurological signs. All patients received intravenous immunoglobulin and methylprednisolone (MTP), low-molecular-weight heparin, and therapeutic-dose anticoagulant treatment. Children with severe encephalopathy received intravenous MTP at 30 mg/kg/day for 3 days, obtaining rapid clinical and EEG improvement. Neurological assessment at discharge was normal in all cases. Children with encephalitis were younger than those without (median age 5 and 10 years, respectively); no differences between the two groups were found in the other parameters: comorbidities, fever, number of organs and systems involved, shock, hospitalization, pediatric intensive care unit admission, non-invasive ventilation, inotropic support, laboratory data.
Neurological involvement in MIS-C is frequent but not serious in most cases: around two thirds of the affected children had mild and short-lasting symptoms. It seems to be related to age, but not to the degree of multiorgan involvement and inflammation. In children with acute immune-mediated encephalitis, the clinical picture was dominated by encephalopathy that disappeared with immunomodulatory therapy. Neurological assessment allowed timely diagnosis and treatment.
描述儿童多系统炎症综合征（MIS-C）中的神经受累情况，并评估神经表现是否与多器官受累及炎症程度相关。
作者对2020年10月1日至2022年3月31日期间住院的62例MIS-C患儿（45例男性，年龄8个月至17岁，平均年龄9岁）的临床、脑电图（EEG）、神经放射学（MRI）和脑脊液参数进行了回顾性分析。
62例患者中有58例（93.5%）记录有神经受累。29例（46.7%）出现精神状态改变，22例（35.4%）出现局灶性神经体征，54例（87%）出现非特异性症状。62例患儿中有26例进行了脑电图检查：20例显示脑电图减慢，弥漫性或主要在后部区域。10例患者接受了脑部MRI检查：3例显示胼胝体细胞毒性病变。6例患者进行了脑脊液分析，结果正常。根据临床和脑电图检查结果，确定了两种神经受累类型：62例中有16例（26%）表现为伴有快速发作性脑病、局灶性神经体征和脑电图减慢的脑炎；62例中有42例（68%）表现为轻度神经受累，伴有轻度或非特异性神经体征。所有患者均接受了静脉注射免疫球蛋白和甲泼尼龙（MTP）、低分子量肝素以及治疗剂量的抗凝治疗。患有严重脑病的儿童接受30mg/kg/天的静脉注射MTP，持续3天，临床和脑电图迅速改善。所有病例出院时的神经评估均正常。患有脑炎的儿童比未患脑炎的儿童年龄小（中位数年龄分别为5岁和10岁）；两组在其他参数方面无差异：合并症、发热、受累器官和系统数量、休克、住院时间、儿科重症监护病房入住情况、无创通气、血管活性药物支持、实验室数据。
MIS-C中的神经受累很常见，但在大多数情况下并不严重：约三分之二的患病儿童症状轻微且持续时间短。它似乎与年龄有关，但与多器官受累及炎症程度无关。在患有急性免疫介导性脑炎的儿童中，临床表现以脑病为主，免疫调节治疗后消失。神经评估有助于及时诊断和治疗。
