Neurology-Stroke Unit, Ospedale del Mare Hospital, Naples, Italy.
Neurology-University Hospital of Torrecardenas, Almeria, Spain.
Neurol Sci. 2022 Jul;43(7):4555-4558. doi: 10.1007/s10072-022-06106-8. Epub 2022 May 4.
A new onset of status epilepticus in a previously healthy adult preceded by a recent minor febrile infection represents a diagnostic and therapeutic challenge in clinical practice. Considering the broad spectrum of epileptic encephalopathies caused by autoimmune mechanisms, differential diagnosis for new-onset refractory status epilepticus (NORSE) should include febrile infection-related epilepsy syndrome (FIRES), in order to not underestimate the underlying etiological condition triggering epilepsy in non-epileptic patients (Hon et al. in Recent Pat Inflamm Allergy Drug Discov 12:128-135, 2018). We report a case of acute encephalopathy with refractory seizures after a febrile illness (FIRES) in a young adult with complete remission of symptoms as well as dramatic improvement of EEG abnormalities following intravenous immunoglobulin and proper antiepileptic medications. We conducted an extensive workup including lumbar puncture, blood tests, EEG serial monitoring, MRI brain, total body CT scan, and PET brain with FDG to shed light on the etiology of the disease.
先前健康的成年人在近期轻微发热感染后出现新发癫痫持续状态，这在临床实践中构成了诊断和治疗方面的挑战。鉴于自身免疫机制引起的癫痫性脑病谱广泛，新发难治性癫痫持续状态（NORSE）的鉴别诊断应包括发热感染相关性癫痫综合征（FIRES），以免低估非癫痫患者中引发癫痫的潜在病因（Hon 等人，《Recent Pat Inflamm Allergy Drug Discov》，12:128-135, 2018）。我们报告了一例年轻成年人在发热疾病后出现急性脑病伴难治性癫痫发作（FIRES）的病例，在静脉注射免疫球蛋白和适当的抗癫痫药物治疗后，症状完全缓解，脑电图异常显著改善。我们进行了广泛的检查，包括腰椎穿刺、血液检查、脑电图连续监测、脑 MRI、全身 CT 扫描和脑 PET 检查，以阐明疾病的病因。
