Department of Neurology, School of Medicine, University of Hacettepe, Ankara, Turkey.
Epilepsy Res. 2020 Nov;167:106479. doi: 10.1016/j.eplepsyres.2020.106479. Epub 2020 Sep 29.
Aphasic status epilepticus (ASE), although rare, may often remain underdiagnosed. We aimed to report the patients diagnosed with ASE and describe their clinical presentation, etiology, electrophysiological findings, neuroimaging, treatment options, and outcome.
We retrospectively analyzed a series of 28 patients diagnosed with ASE between January 2010 and August 2019 in our tertiary referral center. We reviewed medical files, patient charts, and short- and long-term intermittent electroencephalogram (EEG) recordings. Demographical, historical, clinical, neuroimaging, electrophysiological parameters, administered antiseizure medications, and prognosis of all patients were recorded. Furthermore, EEGs were re-evaluated according to Salzburg criteria.
Most patients presented with tumors (n = 11) and cerebrovascular disease (CVD) (n = 11), while the rest were diagnosed with hyperglycemia (n = 2), autoimmune encephalitis (n = 1), remote intracranial abscess (n = 1), mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) attack (n = 1), or unknown cause (n = 1). Seventy-one percent of patients had prior history of epilepsy. The most common EEG findings were rhythmic delta activity with fluctuation and epileptiform discharges ≤ 2.5 Hz with spatiotemporal evolution (66 %). Magnetic resonance imaging (MRI) and EEG findings indicated dominant hemisphere involvement in all patients. No casualties occurred; 71 % patients exhibited total recovery, while 29 % had mild-moderate sequela aphasia. None of the patients received aggressive treatment for ASE.
A relatively higher number of ASE patients could be contributed to the literature through this study. ASE should be considered in patients with prolonged unexplained aphasia, especially with pre-existing risk factors, such as prior history of epilepsy, structural lesion, and metabolic disorders accompanied by concordant EEG findings. Although the underlying cause is a determining prognostic factor, this study shows a significant number of patients with complete remission; thus, the prognosis of ASE may be considered more favorable than other types of nonconvulsive status epilepticus.
失语性癫痫持续状态（ASE）虽然罕见，但往往容易漏诊。我们旨在报告确诊为 ASE 的患者，并描述其临床表现、病因、电生理发现、神经影像学、治疗选择和结局。
我们回顾性分析了 2010 年 1 月至 2019 年 8 月期间在我们的三级转诊中心确诊的 28 例 ASE 患者的系列病例。我们查阅了病历、患者图表以及短期和长期间歇性脑电图（EEG）记录。记录了所有患者的人口统计学、病史、临床、神经影像学、电生理参数、给予的抗癫痫药物和预后。此外，根据萨尔茨堡标准重新评估了 EEG。
大多数患者表现为肿瘤（n = 11）和脑血管病（CVD）（n = 11），其余患者被诊断为高血糖（n = 2）、自身免疫性脑炎（n = 1）、远处颅内脓肿（n = 1）、线粒体脑肌病伴乳酸酸中毒和卒中样发作（MELAS）发作（n = 1）或原因不明（n = 1）。71%的患者有癫痫既往史。最常见的 EEG 表现为节律性 δ 活动伴波动和 ≤2.5 Hz 的癫痫样放电，具有时空演变（66%）。磁共振成像（MRI）和 EEG 结果显示所有患者均累及优势半球。无死亡病例；71%的患者完全恢复，29%的患者有轻度至中度的后遗症失语症。没有患者接受 ASE 的积极治疗。
通过这项研究，可以为文献提供更多的 ASE 患者病例。ASE 应考虑在伴有持续性不明原因失语症的患者中，特别是伴有既往癫痫病史、结构性病变和代谢紊乱等危险因素的患者，同时伴有一致的 EEG 发现。尽管潜在病因是决定预后的一个因素，但本研究显示有相当数量的患者完全缓解；因此，ASE 的预后可能比其他类型的非惊厥性癫痫持续状态更有利。
