成人双侧屏状核受累的发热感染相关性癫癎综合征的临床和影像学特征(附1例报告及文献复习)  

Bilateral Claustrum Damage in Adults with Febrile Infection-related Epilepsy Syndrome

作　　者：韩丽娟[1] 荣蓉 徐运[1] 张馨[1] HAN Lijuan;RONG Rong;XU Yun;ZHANG Xin(Department of Neurology,Nanjing Drum Tower Hospital,the Affiliated Hospital of Nanjing University Medical School,Nanjing 210008,China)

机构地区：[1]南京大学医学院附属鼓楼医院神经内科,南京210008

出　　处：《中国临床神经科学》2024年第4期437-443,462,共8页Chinese Journal of Clinical Neurosciences

基　　金：江苏省科技厅医学重点项目(编号:BE2020620)。

摘　　要：目的探讨成人双侧屏状核受累的发热感染相关性癫癎综合征的临床及影像学特点,以提高临床医生对该病的认识。方法报道1例成人双侧屏状核受累的发热感染相关性癫癎综合征病例的临床资料,并在Pubmed、万方和CNKI数据库中以“屏状核”和“癫癎”或“癫癎发作”、“屏状核”和“癫癎持续状态”作为检索词,检索2023年6月30日之前发表的相关文献(收集13例患者)。通过文献复习对14例(包括本例)患者的临床及影像学特征进行总结。结果(1)本例患者女性,20岁。主因“发热半个月,反复发作性抽搐1周”入院。发作时肢体抽搐,双眼上翻,意识丧失,小便失禁,持续1~2 min。头颅MRI示双侧屏状核异常信号,随访时完全消失。脑电图示:间性发作期间双侧额极、颞、前额区可见每秒1.5~2.5次中幅尖慢波散在、周期性发放。经抗病毒、人免疫球蛋白、甲泼尼龙冲击和抗癫癎治疗后患者癫癎发作控制,反应迟钝以及行为异常好转出院。(2)文献收集13例患者及本例患者均有前驱感染史。急性期表现为难治性癫癎持续状态10/14例(71.4%);14例患者自身免疫性脑炎相关抗体检测结果均为阴性;9/14例(64.3%)患者接受了免疫治疗;8/14例(57.1%)脑脊液白细胞数轻度增多。急性期发作间期脑电图以慢波、周期性发放的尖、尖慢波为主,双侧脑电活动异常10例(90.9%)。头颅MRI双侧屏状核典型表现为T2/Flair高信号,9例在癫癎发作后8.7 d被发现,部分患者双侧屏状核异常信号完全吸收。慢性期有66.7%的患者遗留认知损害、行为异常;约1/4患者表现为慢性难治性癫癎。结论成人双侧屏状核受累的发热感染相关性癫癎综合征病程时间演变具有特异性;慢性期难治性癫癎持续时间长,耐药性高。Aim To investigate the clinical and image features of febrile infection-related epilepsy syndrome(FIRES)in adults with bilateral claustrum damage.Methods A case of bilateral claustrum damage following FIRES was reported.A search of similar published literature was performed.Details of clinical presentation,image results,treatments and prognosis in 14 adult patients were analyzed.Results A 20-year-old woman was admitted for evaluation of her frequent epileptic seizures of 1 week duration after 1 week of fever.She had repetitive tonic-clonic seizures usually lasting for 1 to 2 min.Bilateral MRI changes of claustrum in acute phase disappeared during follow up.Results of electroencephalogram examination showed that scattered and periodic slow waves of 1.5-2.5 Hz can be observed in the bilateral frontal pole,temporal and frontal lobes during epileptic seizures.She received anti-viral treatment,intravenous immunoglobulin and high dose intravenous steroids as well as antiseizure treatment.On discharge,she showed improvement in her neurological symptoms like seizures,cognitive impairment and behavior disturbances.Antecedent febrile illness preceding seizures occurred in all patients with an average of 6.2 days.71.4%patients were presented with refractory status epilepticus.Anti-nuclear antibodies associated with autoimmune encephalitis were negative in either blood or cerebral spinal fluid in each patient.Results of interictal EEG in acute phase demonstrated mainly as slow waves,periodical sharp and sharp/slow waves.Ten(90.9%)patients had abnormal electrical activity in bilateral hemispheres.Typical magnetic resonance image was shown as bilateral T2/Flair hyperintense claustrum lesions,some of which resolved during follow-up.Despite this,66.7%patients had cognitive deficits or abnormal behaviors in chronic phase.One quarter of patients were suffered with chronic refractory epilepsy.Conclusion Clinical and image features of adults with bilateral claustrum damage following FIRES were characterized with 3 typical phases.Pro

关 键 词：成人 双侧屏状核 发热感染相关性癫癎综合征 难治性癫癎 

分 类 号：R742.1[医药卫生—神经病学与精神病学]