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MRI detection of mild malformation of cortical development with oligodendroglial hyperplasia (MOGHE) on T1WI-CHESS 磁共振成像在 T1WI-CHESS 上检测到伴有少突胶质增生的轻度皮质发育畸形 (MOGHE)
IF 1.5 Q3 CLINICAL NEUROLOGY Pub Date : 2024-01-01 DOI: 10.1016/j.ebr.2024.100674
Elly Arizono , Zen-ichi Tanei , Keiya Iijima , Yukio Kimura , Yoko Shigemoto , Hiroyuki Maki , Midori Kusama , Kumiko Murayama , Masaki Iwasaki , Takashi Saito , Yuko Saito , Kazuhiro Saito , Noriko Sato

Mild malformation of cortical development with oligodendroglial hyperplasia (MOGHE) is a recently proposed epileptogenic entity that is difficult to detect on MRI. We present a case of MOGHE that was successfully detected on T1WI-chemical shift-selective saturation (CHESS) MRI. The clinical presentation, MRI including T1WI-CHESS, functional images, and pathology findings of a 14-year-old Japanese girl diagnosed with MOGHE are described. T1WI-CHESS revealed an abnormal high signal along the affected lesion, whereas the findings shown by the other MR sequences were less obvious; interictal fluorodeoxyglucose-positron emission tomography indicated slightly decreased accumulation in the lesion, and subtraction ictal single photon emission computed tomography co-registered to MRI showed an increased blood flow. Together these observations suggest that T1WI-CHESS may be a useful MR sequence for detecting the lesions in patients with MOGHE.

皮质发育轻度畸形伴少突胶质增生(MOGHE)是最近提出的一种致痫实体,很难在核磁共振成像中检测出来。我们介绍了一例在 T1WI-化学位移选择饱和(CHESS)磁共振成像上成功检测出的 MOGHE。本文描述了一名确诊为 MOGHE 的 14 岁日本女孩的临床表现、磁共振成像(包括 T1WI-CHESS)、功能图像和病理结果。T1WI-CHESS显示受影响病灶沿线出现异常高信号,而其他磁共振序列显示的结果则不太明显;发作间期氟脱氧葡萄糖正电子发射断层扫描显示病灶内的积聚略有减少,与磁共振成像共同登记的减影发作期单光子发射计算机断层扫描显示血流增加。这些观察结果表明,T1WI-CHESS 可能是检测 MOGHE 患者病变的有用 MR 序列。
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引用次数: 0
Opercular myoclonic-anarthric status (OMASE) secondary to anti-Hu paraneoplastic neurological syndrome 继发于抗胡副肿瘤性神经综合征的厣肌强直-无张力状态(OMASE)
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2024-01-01 DOI: 10.1016/j.ebr.2024.100703
César Romero, Alonso Quijada, Gabriel Abudinén, Catherine Céspedes, Ledda Aguilera

Focal Opercular Myoclonic – Anarthric Status Epilepticus (OMASE) is a rare form of focal motor status epilepticus caused by several etiologies. It is characterized by fluctuating dysarthria and epileptic myoclonus involving the bilateral glossopharyngeal musculature. We present the case of a 52-year-old woman who experienced gradual and progressive paralysis and myoclonus of facial and bulbar muscles; additional tests revealed the presence of right breast ductal adenocarcinoma and positive serum anti-Hu and anti-GAD65 antibodies. High doses of steroid pulses, anti-seizure therapy, and rituximab partially controlled myoclonus; the tumor resection improved dysphagia and dysarthria.

局灶性肌阵挛-失神状态癫痫(OMASE)是一种罕见的局灶性运动性癫痫,由多种病因引起。其特征是波动性构音障碍和癫痫性肌阵挛累及双侧舌咽肌。本病例是一名 52 岁的女性患者,她的面部和球部肌肉逐渐出现进行性瘫痪和肌阵挛;其他检查显示她患有右乳腺导管腺癌,血清中抗 Hu 和抗 GAD65 抗体呈阳性。大剂量类固醇脉冲、抗癫痫治疗和利妥昔单抗部分控制了肌阵挛;肿瘤切除术改善了吞咽困难和构音障碍。
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引用次数: 0
Exploring patients’ views on telephone consultations in the seizure clinic: A qualitative interview study 探索患者对癫痫发作门诊电话咨询的看法:定性访谈研究
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2024-01-01 DOI: 10.1016/j.ebr.2024.100705
Joseph Ford, Markus Reuber

The COVID-19 pandemic brought telemedicine into mainstream medical practice. Although it is widely agreed that telemedicine could be beneficial for patients with seizures, there has been little prior research investigating patients’ views on this subject. In this qualitative study, we conducted semi-structured interviews with 10 patients and one companion about their experiences of telemedicine. We also received written thoughts from one additional patient. Participants’ views fell under three broad themes. The first, “Convenience and practicality”, saw participants praising the flexibility of telephone consultations while noting that such consultations could introduce new practical problems. The second, “(Lack of) shared presence”, covered participants’ generally negative feelings about not being in the same room as their neurologists. The third, “Situation dependency”, saw participants drawing fine distinctions about the circumstances in which face-to-face and telephone consultations were suitable. Overall, although patients with seizures are generally positive about the convenience of telephone consultations, they have concerns about how they may lead to misunderstandings or affect the doctor-patient relationship. These concerns could be assuaged to some extent by offering video consultations or scheduling alternating telephone and face-to-face consultations.

COVID-19 大流行将远程医疗带入了主流医疗实践。尽管人们普遍认为远程医疗可以为癫痫发作患者带来益处,但此前很少有研究调查患者对这一问题的看法。在这项定性研究中,我们对 10 名患者和一名陪护进行了半结构化访谈,了解他们对远程医疗的体验。我们还收到了另外一名患者的书面意见。参与者的观点分为三大主题。第一个主题是 "便利性和实用性",参与者赞扬了电话会诊的灵活性,同时指出这种会诊可能会带来新的实际问题。第二个主题是"(缺乏)共同在场",与会者对不能与神经科医生同处一室普遍持消极态度。第三个问题是 "情况依赖性",与会者对适合面对面会诊和电话会诊的情况进行了细致的区分。总体而言,尽管癫痫发作患者对电话咨询的便利性普遍持肯定态度,但他们也担心电话咨询可能会导致误解或影响医患关系。通过提供视频会诊或交替安排电话会诊和面对面会诊,可以在一定程度上缓解这些担忧。
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引用次数: 0
Effects of steroids on super-refractory status epilepticus in tick-borne meningoencephalitis 类固醇对蜱传脑膜脑炎超级难治性癫痫状态的影响
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2024-01-01 DOI: 10.1016/j.ebr.2024.100710
Christine Heuer , Claudio Togni , Marian Galovic , Anna Czernuszenko , Giovanna Brandi , Ignazio de Trizio

We report a unique case of super-refractory status epilepticus (SRSE) secondary to tick-borne encephalitis (TBE) to evaluate the therapeutic challenges and potential benefits of steroid treatment in this context. A previously healthy 31-year-old woman was admitted to the hospital with fever, headache, vertigo, and meningismus, ultimately diagnosed with TBE. Despite empirical antimicrobial treatment, the patient’s condition deteriorated, leading to coma and SRSE. Various antiseizure medications and sedatives were administered without sustained success. Steroid treatment was initiated due to elevated intracranial pressure and persistent seizure activity. Following the administration of dexamethasone, electrographic status epilepticus resolved, though the patient developed clinical signs of increased intracranial pressure necessitating decompressive craniectomy. The patient’s condition stabilized with a combination of antiseizure medicazions. Despite cessation of SRSE, the patient remained in a minimally conscious state at discharge, showing only gradual improvement over time. The use of steroids in TBE is controversial, with limited reports of potential benefits. In this case, steroid administration coincided with the cessation of SRSE, and authors explore its potential benefit considering its immunomodulatory effects.

我们报告了一例继发于蜱传脑炎(TBE)的独特的超级难治性癫痫状态(SRSE)病例,以评估类固醇治疗在这种情况下的治疗挑战和潜在益处。一名先前健康的 31 岁女性因发热、头痛、眩晕和脑膜炎入院,最终被诊断为蜱传脑炎。尽管接受了经验性抗菌治疗,但患者病情恶化,导致昏迷和 SRSE。患者接受了多种抗癫痫药物和镇静剂治疗,但效果不佳。由于颅内压升高和癫痫持续发作,患者开始接受类固醇治疗。使用地塞米松后,电图癫痫状态缓解,但患者出现了颅内压增高的临床症状,必须进行减压开颅手术。在联合使用抗癫痫药物后,患者的病情趋于稳定。尽管停止了 SRSE,但患者出院时仍处于微清醒状态,只是随着时间的推移逐渐好转。类固醇在结核性脑病中的应用尚存争议,有关其潜在益处的报道有限。在本病例中,类固醇的使用与 SRSE 的停止同时进行,考虑到其免疫调节作用,作者对其潜在益处进行了探讨。
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引用次数: 0
Delineating abnormal individual structural covariance brain network organization in pediatric epilepsy with unilateral resection of visual cortex 单侧切除视觉皮层的小儿癫痫患者异常个体结构协方差脑网络组织的划分
IF 1.5 Q3 CLINICAL NEUROLOGY Pub Date : 2024-01-01 DOI: 10.1016/j.ebr.2024.100676
Liang Zhang , Bei Zhuang , Mengyuan Wang , Jie Zhu , Tao Chen , Yang Yang , Haoting Shi , Xiaoming Zhu , Li Ma

Although several previous studies have used resting-state functional magnetic resonance imaging and diffusion tensor imaging to report topological changes in the brain in epilepsy, it remains unclear whether the individual structural covariance network (SCN) changes in epilepsy, especially in pediatric epilepsy with visual cortex resection but with normal functions. Herein, individual SCNs were mapped and analyzed for seven pediatric patients with epilepsy after surgery and 15 age-matched healthy controls. A whole-brain individual SCN was constructed based on an automated anatomical labeling template, and global and nodal network metrics were calculated for statistical analyses. Small-world properties were exhibited by pediatric patients after brain surgery and by healthy controls. After brain surgery, pediatric patients with epilepsy exhibited a higher shortest path length, lower global efficiency, and higher nodal efficiency in the cuneus than those in healthy controls. These results revealed that pediatric epilepsy after brain surgery, even with normal functions, showed altered topological organization of the individual SCNs, which revealed residual network topological abnormalities and may provide initial evidence for the underlying functional impairments in the brain of pediatric patients with epilepsy after surgery that can occur in the future.

尽管之前的一些研究利用静息态功能磁共振成像和弥散张量成像报告了癫痫患者大脑拓扑结构的变化,但目前仍不清楚癫痫患者的个体结构协方差网络(SCN)是否会发生变化,尤其是在视觉皮层切除但功能正常的小儿癫痫患者中。在此,我们绘制并分析了 7 名手术后的儿科癫痫患者和 15 名年龄匹配的健康对照者的单个 SCN。根据自动解剖标记模板构建了全脑单个 SCN,并计算了全局和节点网络指标进行统计分析。脑部手术后的儿科患者和健康对照组均表现出小世界特性。与健康对照组相比,脑部手术后的小儿癫痫患者楔骨处的最短路径长度更高,全局效率更低,节点效率更高。这些结果表明,脑部手术后的小儿癫痫患者即使功能正常,也会表现出单个SCN拓扑组织的改变,这揭示了残留的网络拓扑异常,可能为小儿癫痫患者手术后大脑潜在的功能障碍提供了初步证据。
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引用次数: 0
Cognitive outcomes after magnetic resonance-guided laser interstitial thermal therapy for mesial temporal lobe epilepsy in adolescent patients 磁共振引导下的激光间质热疗治疗青少年颞叶中叶癫痫后的认知效果
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2024-01-01 DOI: 10.1016/j.ebr.2024.100723
Jonathon M. Cavaleri , Jenna A. Chiang , Danielle M. Wishart , Keiko M. Kang , Patrick R. Ng , Leanne Mendoza , Kenneth Hartline , Michele Van Hirtum-Das , Latanya D. Agurs , Madeline Kahan , Brittany Jordan , Charles Y. Liu , Brian Lee , Peter A. Chiarelli , Jason K. Chu
Surgical treatment of medication-resistant mesial temporal lobe epilepsy (MTLE) is associated with cognitive deficits. Magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) for MTLE has been shown to result in superior cognitive outcomes in adults when compared to open surgical resection. However, data regarding postoperative cognitive outcomes in adolescent and pediatric patients is limited. We retrospectively reviewed sequential cases of pediatric patients who underwent MRgLITT for MTLE between 2017 and 2023. Patients who had complete preoperative and 12 month postoperative neuropsychological evaluation were analyzed for changes in the neuropsychological domains of cognition, memory, executive functioning, visual scanning, graphomotor speed, and fine motor speed/dexterity. Six adolescent patients who underwent MRgLITT for MTLE (x̄ age = 19.0 years, SD = 1.2) and had complete preoperative and postoperative neuropsychological evaluations were included in the analysis. There were no statistically significant changes across neuropsychological domains when comparing pre- and postoperative cognitive evaluations, including verbal memory scores. Clinically significant changes in phonemic fluency were observed when examining side-specific effects and improved for patients who received right-sided MRgLITT but declined for patients who received left-sided MRgLITT. 50 % of patients achieved Engel I outcome at last follow-up. Our preliminary results suggest minimal adverse neuropsychologic effects following MRgLITT for adolescent MTLE, including preservation of verbal memory. Clinical outcomes were similar with those reported in the literature.
耐药性颞叶间叶癫痫(MTLE)的手术治疗与认知障碍有关。磁共振引导下的激光间质热疗(MRgLITT)治疗MTLE与开放性手术切除相比,成人患者的认知疗效更佳。然而,有关青少年和儿童患者术后认知效果的数据却很有限。我们对2017年至2023年间接受MRgLITT治疗MTLE的儿科患者病例进行了回顾性连续回顾。我们对术前和术后12个月进行了完整神经心理学评估的患者进行了分析,以了解他们在认知、记忆、执行功能、视觉扫描、图形运动速度和精细运动速度/灵活性等神经心理学领域的变化。分析对象包括六名因MTLE接受MRgLITT治疗的青少年患者(x̄年龄=19.0岁,SD=1.2),他们均接受了完整的术前和术后神经心理学评估。比较术前和术后的认知评估结果,各神经心理学领域均无统计学意义上的显著变化,包括言语记忆得分。在检查特定侧效应时,观察到语音流畅性发生了临床意义上的变化,接受右侧 MRgLITT 的患者的语音流畅性有所改善,但接受左侧 MRgLITT 的患者的语音流畅性有所下降。50%的患者在最后一次随访时达到了恩格尔I型结果。我们的初步研究结果表明,MRgLITT 对青少年 MTLE 的不良神经心理影响极小,包括保留了言语记忆。临床结果与文献报道的结果相似。
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引用次数: 0
Self-reported exercise engagement and seizure control – A preliminary survey of people with epilepsy at a safety-net hospital 自我报告的运动参与度与癫痫发作控制--对一家安全网医院癫痫患者的初步调查
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2024-01-01 DOI: 10.1016/j.ebr.2024.100724
Ariel Farb , Joseph Sisto , Janine Barrett , Abrar Al-Faraj , Shelby Goodson , Janice Weinberg , Jane B. Allendorfer , Myriam Abdennadher
Benefits of exercise on general health and wellbeing are undeniable. The International League Against Epilepsy has provided some guidance into exercise and sports for epilepsy. However, people with epilepsy are typically misinformed and restricted by fear and lack of evidence about exercise benefits in epilepsy. Our study seeks to investigate engagement in exercise in epilepsy at our center and identify potential barriers to physical activity. We conducted an anonymous survey at the Epilepsy Clinic using a clinically validated measure of exercise (IPAQ) at various levels: vigorous, moderate, and walking, and a questionnaire of 21 potential reasons for inactivity. Data were collected in REDCap. Statistical analysis was performed on SAS. We collected responses from 72 epilepsy participants between January and April 2024. Participants with controlled seizures were more likely to engage in moderate exercise compared to respondents with uncontrolled seizures. The top two general perceived barriers were having no one to exercise with and not liking exercise. Epilepsy-specific barriers were higher in respondents with uncontrolled seizures compared to those with controlled seizures, with the top two reasons being fear of “exercise-induced” seizures and lack of guidance on appropriate exercises. Our findings showed that there were more participants with controlled seizures who perform moderate exercise compared to those with uncontrolled seizures. Future studies are needed to evaluate whether exercise can have an impact on improving seizure control. Our study also highlights opportunities to educate health care providers, patients, and community members about exercise to facilitate engagement in exercise and improve epilepsy outcomes.
运动对一般健康和福祉的益处是不可否认的。国际抗癫痫联盟(International League Against Epilepsy)为癫痫患者的运动和体育锻炼提供了一些指导。然而,癫痫患者通常对运动有益于癫痫的信息存在误解,并受到恐惧和缺乏证据的限制。我们的研究旨在调查我们中心的癫痫患者参与运动的情况,并找出体育锻炼的潜在障碍。我们在癫痫诊所进行了一项匿名调查,使用了经临床验证的不同级别的运动量(IPAQ):剧烈、中等和步行,以及一份包含 21 个不运动潜在原因的问卷。数据在 REDCap 中收集。统计分析在 SAS 中进行。我们在 2024 年 1 月至 4 月期间收集了 72 名癫痫参与者的回复。与癫痫发作未得到控制的受访者相比,癫痫发作得到控制的受访者更有可能进行适度锻炼。一般认为的前两大障碍是没有人一起锻炼和不喜欢锻炼。与发作受控制的受访者相比,发作未受控制的受访者的癫痫特定障碍更高,前两个原因是害怕 "运动诱发 "癫痫发作和缺乏适当运动的指导。我们的研究结果表明,与发作未受控制的受访者相比,发作受控的受访者中进行适度运动的人数更多。今后还需要进行研究,以评估运动是否能对改善癫痫发作控制产生影响。我们的研究还强调了对医疗服务提供者、患者和社区成员进行运动教育的机会,以促进他们参与运动并改善癫痫预后。
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引用次数: 0
Comparative analysis of processing speed impairments in TLE, FLE, and GGE: Theoretical insights and clinical Implications 对 TLE、FLE 和 GGE 处理速度障碍的比较分析:理论见解和临床意义
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2024-01-01 DOI: 10.1016/j.ebr.2024.100722
Adam Falah , Gavin P. Winston
In this narrative review, we explore the differences in processing speed (PS) impairments among three epilepsy conditions; Temporal Lobe Epilepsy (TLE), Frontal Lobe Epilepsy (FLE) and Genetic Generalized Epilepsy (GGE) with a focus on Juvenile Myoclonic Epilepsy (JME). Despite the large body of research focusing on cognition in epilepsy, the intricacies of PS impairments in the epilepsy syndromes have not been fully explored. We investigate the cognitive profiles with focus on PS associated with each of the three conditions, and the neuropsychological methods employed. Furthermore, we evaluate PS in epilepsy within the theoretical frameworks of PS, such as the Relative Consequence Model, the Limited Time Mechanism Model, and the Neural Noise Hypothesis. We find the main challenge of PS research in epilepsy is the inconsistency of assessment methods utilized in different studies. Furthermore, PS impairments are not isolated but rather interconnected to other cognitive domains. Thus, future studies need to standardize PS assessment tools, and incorporate innovative solutions such as technology and neuroimaging techniques to further enhance our understanding of PS impairments in epilepsy.
在这篇叙述性综述中,我们探讨了颞叶癫痫(TLE)、额叶癫痫(FLE)和遗传性广泛性癫痫(GGE)这三种癫痫疾病在处理速度(PS)障碍方面的差异,重点是青少年肌阵挛性癫痫(JME)。尽管有大量研究关注癫痫的认知问题,但尚未充分探讨癫痫综合征中 PS 损伤的复杂性。我们研究了三种情况下的认知概况,重点是与每种情况相关的 PS,以及所采用的神经心理学方法。此外,我们还在 PS 理论框架(如相对后果模型、有限时间机制模型和神经噪音假说)内对癫痫中的 PS 进行了评估。我们发现,癫痫中的 PS 研究面临的主要挑战是不同研究采用的评估方法不一致。此外,PS 损伤并不是孤立的,而是与其他认知领域相互关联。因此,未来的研究需要将 PS 评估工具标准化,并结合创新的解决方案,如技术和神经影像技术,以进一步加深我们对癫痫患者 PS 损伤的理解。
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引用次数: 0
Case reports of identical twins with developmental and epileptic encephalopathy with STXBP1 gene mutations for whom different CBD supplementations were markedly effective 患有STXBP1基因突变的发育不良和癫痫性脑病的同卵双胞胎的病例报告,补充不同的CBD对他们明显有效
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2024-01-01 DOI: 10.1016/j.ebr.2024.100720
Yuji Masataka , Naoko Miki , Kozo Akino , Hitoshi Yamamoto , Ichiro Takumi
Cannabidiol (CBD) is a compound found specifically in the cannabis plant. Although a clinical trial for intractable epilepsy started in Japan in 2023, it is also available in the market as a dietary supplement. Herein, we report two cases of identical twins with developmental and epileptic encephalopathy with STXBP1 gene mutation who achieved seizure suppression through different regimens of CBD supplementation. The observation that different trace ingredients produced different effects in patients with identical genetic backgrounds is a crucial finding that has implications for the future regulation and clinical application of cannabinoid products.
大麻二酚(CBD)是一种专门存在于大麻植物中的化合物。虽然日本于 2023 年开始对难治性癫痫进行临床试验,但它也可以作为膳食补充剂在市场上销售。在此,我们报告了两例同卵双胞胎,他们患有STXBP1基因突变的发育性癫痫性脑病,通过不同的CBD补充方案实现了癫痫发作的抑制。观察到不同的微量成分对具有相同遗传背景的患者产生了不同的效果,这是一个至关重要的发现,对未来大麻素产品的监管和临床应用具有重要意义。
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引用次数: 0
Anticipatory anxiety of seizures: What is the best treatment? 癫痫发作的预期焦虑:最佳治疗方法是什么?
IF 1.5 Q3 CLINICAL NEUROLOGY Pub Date : 2024-01-01 DOI: 10.1016/j.ebr.2024.100673
Coraline Hingray , Herve Javelot , Frank Lach , Alexis Tarrada

Anxiety disorders affect roughly 25% of people with epilepsy (PWE), and are associated with a strong impairment of quality of life and a poorer stabilization of epilepsy. Anticipatory anxiety of seizure (AAS), defined by the persistent worry or fear to have another seizure, is highly frequent and associated with avoidant behavior. Unfortunately, AAS is often overlooked and untreated. Here, we present the case of a 35-year-old patient suffering from AAS secondary to focal epilepsy. We aimed to provide practical guidelines and tools for the screening and treatment of anxiety disorders in PWE. Regarding psychotropic medication, Sertraline or Citalopram might be good options for first-line treatment of AAS, since they are efficient against anxiety and well-tolerated in epilepsy.

约有 25% 的癫痫患者(PWE)会患上焦虑症,焦虑症与生活质量的严重受损和癫痫的稳定性较差有关。癫痫发作预期焦虑症(AAS)的定义是持续担心或害怕再次癫痫发作,这种焦虑症的发病率很高,并且与回避行为有关。不幸的是,癫痫发作预期焦虑症常常被忽视而得不到治疗。在此,我们介绍了一名继发于局灶性癫痫的 35 岁 AAS 患者的病例。我们旨在提供实用的指南和工具,用于筛查和治疗残疾人焦虑症。在精神药物治疗方面,舍曲林或西酞普兰可能是一线治疗焦虑症的不错选择,因为这两种药物对焦虑症有效,而且在癫痫患者中耐受性良好。
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引用次数: 0
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Epilepsy and Behavior Reports
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