Huang Yuyao, Wang Xinpeng, Wang Ling, Wang Juan, Tan Chengbing, Cheng Min, Jiang Yan, Jiang Li
Department of Neurology Children's Hospital of Chongqing Medical University National Clinical Research Center for Child Health and Disorders Ministry of Education Key Laboratory of Child Development and Disorders Chongqing Key Laboratory of Translational Medical Research in Cognitive Development and Learning and Memory Disorders Chongqing China.
Pediatr Discov. 2023 Aug 14;1(2):e23. doi: 10.1002/pdi3.23. eCollection 2023 Sep.
The clinical data of patients with Kikuchi-Fujimoto disease (KFD) combined with the involvement of the central system is limited, particularly for children. This study aimed to investigate the clinical features and prognosis of KFD associated with the central nervous system in children. We collected the clinical data of patients diagnosed with KFD combined with the involvement of the central system, hospitalized at Children's Hospital of Chongqing Medical University (CHCMU). Furthermore, we summarized the clinical features and outcomes in children by reviewing the published literature. Together with the two patients from CHMCU enrolled in this study, we identified 19 children diagnosed with KFD combined with the involvement of the central nervous system, including eight males and 11 females. The onset age ranges from 6 to 18 years (the median age was 14 and the interquartile range was 9-16). Sixteen patients were diagnosed with aseptic meningitis. Headache (78.9%, 15/19) was the most common symptom. Cerebrospinal fluid pressure was increased in 3 patients (60.0%, 3/5), cell count was elevated in 14 patients (73.7%, 14/19), glucose was decreased in 4 patients (21.0%, 4/19), and protein was elevated in 12 patients (63.1%, 12/19). A total of 9 patients had cranial imaging changes. Electroencephalogram (EEG) was performed on seven patients and 4 patients presented abnormal EEG (57.1%, 4/7). A total of 12 patients received steroid therapy; two were combined with antiepileptic treatment. In children, KFD complicated with the involvement of the central nervous system was rare and aseptic meningitis was the primary manifestation. Most patients had a good prognosis and a few had residual sequelae of neurological damage.
菊池-藤本病（KFD）合并中枢系统受累的患者临床资料有限，尤其是儿童患者。本研究旨在探讨儿童KFD合并中枢神经系统受累的临床特征及预后。我们收集了重庆医科大学附属儿童医院（CHCMU）收治的诊断为KFD合并中枢系统受累患者的临床资料。此外，我们通过回顾已发表的文献总结了儿童的临床特征及结局。连同本研究纳入的CHCMU的两名患者，我们共确定了19例诊断为KFD合并中枢神经系统受累的儿童，其中男性8例，女性11例。发病年龄为6至18岁（中位年龄为14岁，四分位间距为9 - 16岁）。16例患者被诊断为无菌性脑膜炎。头痛（78.9%，15/19）是最常见的症状。5例患者中3例（60.0%，3/5）脑脊液压力升高，19例患者中14例（73.7%，14/19）细胞计数升高，19例患者中4例（21.0%，4/19）葡萄糖降低，19例患者中12例（63.1%，12/19）蛋白质升高。共有9例患者有头颅影像学改变。7例患者进行了脑电图（EEG）检查，4例患者脑电图异常（57.1%，4/7）。共有12例患者接受了类固醇治疗；2例联合抗癫痫治疗。在儿童中，KFD合并中枢神经系统受累较为罕见，无菌性脑膜炎是主要表现。大多数患者预后良好，少数有神经损伤的残留后遗症。
