Laswell Emily M, Chambers Kasandra D, Whitsel Danielle R, Poudel Kiran
Cedarville University School of Pharmacy, Cedarville, Ohio.
The Clinical Neuroscience Institute, Miami Valley Hospital, Dayton, Ohio.
Pharmacotherapy. 2015 Jun;35(6):e106-10. doi: 10.1002/phar.1595. Epub 2015 Jun 4.
New-onset refractory status epilepticus (NORSE) is defined as a sudden onset of refractory status epilepticus in patients who do not have a history of epilepsy. It is a neurologic emergency, and determining the underlying etiology is an important factor for effectively managing and predicting the prognosis of NORSE. We describe the case of a 28-year-old woman who was hospitalized with NORSE secondary to an unknown etiology. She did not respond to traditional anticonvulsant therapy, including benzodiazepines, fosphenytoin, propofol, and levetiracetam. The patient was placed on continuous electroencephalography (EEG) monitoring and was treated further with multiple antiepileptics, which were titrated aggressively based on EEG readings and therapeutic drug levels; despite this treatment, EEG monitoring revealed continued seizures. Thus, high-dose corticosteroids were started for seizure control. Her workup included computed tomography and magnetic resonance imaging of the head, a lumbar puncture, toxicology screening, and extensive testing for multiple infectious and inflammatory etiologies. The patient's history revealed recent exposure to a new cat. Serologic results were positive for Bartonella henselae, and she was diagnosed with cat-scratch disease (CSD). She did not have the typical presentation of symptoms of lymphadenopathy, however, which is common in CSD. Doxycycline 100 mg and rifampin 300 mg twice daily were added to the patient's anticonvulsant and corticosteroid therapy. She was hospitalized for a total of 26 days and discharged with only minor neurologic impairment (short-term memory deficits and minor cognitive problems). The patient was discharged receiving antiepileptics, antibiotics, and a corticosteroid taper. To our knowledge, this is the first clinically known case of NORSE secondary to CSD without typical CSD symptoms in the adult population. The patient failed to respond to traditional anticonvulsant therapy alone. With the addition of high-dose corticosteroids to aggressive anticonvulsant therapy, seizure control was achieved. Further studies are needed to determine the effectiveness of high-dose corticosteroid and anticonvulsant therapy followed by antibiotics in patients with NORSE secondary to CSD.
新发难治性癫痫持续状态(NORSE)定义为既往无癫痫病史的患者突然发生难治性癫痫持续状态。这是一种神经系统急症,确定潜在病因是有效管理和预测NORSE预后的重要因素。我们描述了一名28岁女性的病例,她因病因不明的NORSE住院。她对包括苯二氮䓬类、磷苯妥英、丙泊酚和左乙拉西坦在内的传统抗惊厥治疗无反应。患者接受了持续脑电图(EEG)监测,并进一步接受了多种抗癫痫药物治疗,根据EEG读数和治疗药物水平积极调整剂量;尽管进行了这种治疗,EEG监测仍显示癫痫持续发作。因此,开始使用大剂量皮质类固醇来控制癫痫发作。她的检查包括头部计算机断层扫描和磁共振成像、腰椎穿刺、毒理学筛查以及针对多种感染和炎症病因的广泛检测。患者病史显示近期接触过一只新猫。血清学结果显示汉赛巴尔通体阳性,她被诊断为猫抓病(CSD)。然而,她没有CSD常见的典型淋巴结病症状表现。在患者的抗惊厥和皮质类固醇治疗中加用了每日两次100毫克强力霉素和300毫克利福平。她总共住院26天,出院时仅有轻微神经功能损害(短期记忆缺陷和轻微认知问题)。患者出院时接受抗癫痫药物、抗生素治疗以及逐渐减量的皮质类固醇治疗。据我们所知,这是成年人群中首例临床上已知的继发于CSD且无典型CSD症状的NORSE病例。患者单独对传统抗惊厥治疗无效。在积极的抗惊厥治疗基础上加用大剂量皮质类固醇后,实现了癫痫发作的控制。需要进一步研究以确定大剂量皮质类固醇和抗惊厥治疗后加用抗生素对继发于CSD的NORSE患者的有效性。