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Pitfalls and unmet needs of transition in epilepsy: Understanding the adult neurologist perspective 癫痫过渡时期的陷阱和未满足的需求:从成人神经科医生的角度理解
IF 2.3 3区 医学 Q2 BEHAVIORAL SCIENCES Pub Date : 2024-10-17 DOI: 10.1016/j.yebeh.2024.110100
Pietro Mattioli , Maria Margherita Mancardi , Alessandra Ferrari , Elisa Micalizzi , Dario Arnaldi , Irene Pappalardo , Francesca Bisulli , Chiara Pastori , Paola Mandich , Lino Nobili , Francesca Darra , Flavio Villani , Commission on Transition of the Lega Italiana Contro l'Epilessia (LICE)
Transition from the paediatric holistic approach to the adult more individual care of epilepsy is a process that carries a high level of complexity and involves many actors. It is a mandatory step so to provide a gold-standard quality of care in young people with epilepsy. Its failure is strongly associated with a poor epilepsy, cognitive, and behavioural outcome.
Although the interest of the scientific community on transition has increased, in the clinical practice it is still considered a sensitive issue, in particular for adult neurologists. In this study we aimed at exploring pitfalls and unmet needs of transition from the adult neurologists’ perspective. A short survey, aimed to adult neurologists, was designed to explore i) whether transition is considered a problematic issue for the adult neurologist, ii) how transition impact his/her clinical practice, iii) which are the major problems related to the process, and iv) how transition may be improved. The results of the survey showed that transition is felt as a complex process that needs a shared action plan between child and adult neurologists, possibly with multidisciplinary team meetings as well as a higher training on childhood epilepsies for the adult neurologist.
从儿科的整体护理方法过渡到成人的个性化癫痫护理,是一个高度复杂的过程,涉及许多参与者。要为青少年癫痫患者提供黄金标准质量的护理,这是一个必须采取的步骤。虽然科学界对过渡时期的关注度有所提高,但在临床实践中,这仍被视为一个敏感问题,尤其是对成年神经科医生而言。在这项研究中,我们旨在从成人神经科医生的角度探讨过渡时期的陷阱和未满足的需求。我们设计了一项针对成年神经科医生的简短调查,旨在探讨 i) 过渡期是否被认为是成年神经科医生面临的一个问题;ii) 过渡期对其临床实践有何影响;iii) 过渡期过程中存在哪些主要问题;以及 iv) 如何改善过渡期。调查结果显示,人们认为过渡是一个复杂的过程,需要儿童神经科医生和成人神经科医生共同制定行动计划,可能需要召开多学科小组会议,以及对成人神经科医生进行更多有关儿童癫痫的培训。
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引用次数: 0
Response to Letter on “Cognitive outcomes after fetal exposure to carbamazepine, lamotrigine, valproate or levetiracetam monotherapy: Data from the EURAP neurocognitive extension protocol” 对关于 "胎儿暴露于卡马西平、拉莫三嗪、丙戊酸钠或左乙拉西坦单药治疗后的认知结果:EURAP神经认知扩展方案的数据"
IF 2.3 3区 医学 Q2 BEHAVIORAL SCIENCES Pub Date : 2024-10-15 DOI: 10.1016/j.yebeh.2024.110084
Susanna Stjerna , Yfke Huber-Mollema , Torbjörn Tomson , Emilio Perucca , Dina Battino , John Craig , Anne Sabers , Sanjeev Thomas , Frank Vajda , Eija Gaily
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引用次数: 0
Epilepsy in LEAT and other brain tumors: A focused review LEAT 和其他脑肿瘤的癫痫:重点回顾
IF 2.3 3区 医学 Q2 BEHAVIORAL SCIENCES Pub Date : 2024-10-15 DOI: 10.1016/j.yebeh.2024.110092
Catrin Mann , Nico Melzer , Dorothea Münch
Of all patients with brain tumors, about 30–50% suffer from epileptic seizures. The probability of developing epilepsy is particularly high in low-grade, epilepsy-associated brain tumors (LEAT). LEATs often show a pronounced network dysfunction with extensive EEG pathologies and cognitive deficits, and the epilepsies are often difficult to treat. In high-grade brain tumors, epileptic seizures determine morbidity and quality of life. The underlying mechanisms of epileptogenesis of brain tumors are increasingly understood and raise hope for personalized therapeutic approaches. This short, focused review provides an overview of the current understanding of brain tumor-related epilepsies.
This paper was presented at 16th International Epilepsy Course and Colloquium held in Frankfurt a.M., Germany, September 2024.
在所有脑肿瘤患者中,约有 30-50% 会出现癫痫发作。低级别癫痫相关脑肿瘤(LEAT)发生癫痫的概率尤其高。低级别脑肿瘤通常表现出明显的网络功能障碍,伴有广泛的脑电图病变和认知障碍,而且癫痫通常难以治疗。在高级别脑肿瘤中,癫痫发作决定了发病率和生活质量。人们对脑肿瘤癫痫发生的基本机制有了越来越多的了解,这为个性化治疗方法带来了希望。本文是一篇简短而集中的综述,概述了目前对脑肿瘤相关癫痫的认识。
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引用次数: 0
Temporal lobe encephaloceles: Electro-clinical characteristics and seizure outcome after tailored lesionectomy 颞叶脑瘤:量身定制的病灶切除术后的电临床特征和癫痫发作结果。
IF 2.3 3区 医学 Q2 BEHAVIORAL SCIENCES Pub Date : 2024-10-11 DOI: 10.1016/j.yebeh.2024.110066
Camilo Garcia-Gracia , Samer Riaz , Claudia Vallin , Andreas Alexopoulos , Badih Adada , William Bingaman , Imad Najm , Juan C. Bulacio

Objective

Our study aimed to investigate the management of patients with medically refractory epilepsy related to temporal encephaloceles, focusing on the use of ancillary testing in the pre-surgical evaluation to optimize surgical outcomes.

Methods

We conducted a retrospective analysis of electronic medical records from the Cleveland Clinic, covering the period from January 2000 to May 2020. Patients with drug-resistant temporal lobe epilepsy were included if they had temporal lobe encephaloceles and required surgical intervention. We reviewed the results of ancillary studies, including invasive EEG.

Results

A total of 19 patients with temporal lobe encephaloceles underwent resection for drug-resistant epilepsy treatment. Among them, 63 % reported experiencing auras commonly associated with mesial temporal lobe epilepsy, such as autonomic, psychic, and abdominal symptoms, followed by dialeptic seizures. Ictal patterns were consistently ipsilateral, with high amplitude delta or medium amplitude theta activity at onset, predominantly localized to the frontotemporal region in more than half of the cases. In 35 % of these patients, encephaloceles were only diagnosed during surgery. Stereo-EEG evaluation revealed two distinct ictal patterns: one characterized by localized low voltage fast activity in the temporal pole evolving into a 3–4 Hz high amplitude diffuse spiky activity, and the other exhibiting low amplitude rhythmic theta activity in the temporal pole with late involvement of the amygdala/hippocampus. Surgical resection strategy was based on clinical history and ancillary data analysis. At one-year follow-up after resection, 63 % of the patients attained Engel I seizure control over an average duration of 44 months (ranging from 6 months to 7.3 years). Additionally, 18 % of the patients achieved an Engel II outcome.

Significance

Tailored resection of the encephalocele and the surrounding temporal pole, while preserving the mesial temporal structures, can effectively control seizures in patients with temporal encephaloceles identified through MRI. Patients presenting with temporal lobe symptoms and scalp ictal patterns characterized by polymorphic high delta theta activity with frontotemporal evolution should be evaluated for temporal encephaloceles as a potential underlying cause of their seizures, especially when the MRI is otherwise unrevealing.
研究目的我们的研究旨在调查与颞叶脑瘤相关的药物难治性癫痫患者的管理情况,重点关注手术前评估中辅助检查的使用情况,以优化手术效果:我们对克利夫兰诊所 2000 年 1 月至 2020 年 5 月期间的电子病历进行了回顾性分析。耐药性颞叶癫痫患者如果患有颞叶脑瘤并需要手术治疗,我们将其纳入研究范围。我们回顾了包括侵入性脑电图在内的辅助研究结果:共有 19 名颞叶脑瘤患者接受了切除手术,以治疗耐药性癫痫。其中,63%的患者报告出现了与颞叶中叶癫痫常见的光环,如自主神经症状、精神症状和腹部症状,其次是透析性癫痫发作。ctal模式始终是同侧的,发病时有高振幅的delta或中等振幅的theta活动,半数以上的病例主要发生在额颞区。其中 35% 的患者在手术中才被确诊为脑瘤。立体电子脑电图(stereo-EEG)评估显示了两种不同的发作模式:一种是颞极局部的低电压快速活动演变为3-4赫兹的高振幅弥漫性棘波活动,另一种是颞极的低振幅节律性θ活动,晚期累及杏仁核/海马。手术切除策略基于临床病史和辅助数据分析。在切除术后一年的随访中,63%的患者在平均44个月(从6个月到7.3年不等)的时间内达到了恩格尔I型发作控制。此外,18%的患者达到了恩格尔II型:意义:在保留颞中叶结构的同时,对脑疝和周围颞极进行有针对性的切除,可有效控制通过磁共振成像发现的颞叶脑疝患者的癫痫发作。对于出现颞叶症状、头皮发作模式以多形性高δθ活动为特征并伴有额颞叶演变的患者,应将颞脑膜瘤作为其癫痫发作的潜在病因进行评估,尤其是在核磁共振成像无法揭示其他病因的情况下。
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引用次数: 0
Investigation of spontaneous abortion and stillbirth adverse events in epilepsy patients treated with levetiracetam: A pharmacovigilance study 调查接受左乙拉西坦治疗的癫痫患者的自然流产和死胎不良事件:药物警戒研究
IF 2.3 3区 医学 Q2 BEHAVIORAL SCIENCES Pub Date : 2024-10-11 DOI: 10.1016/j.yebeh.2024.110077
Qi Wang , Hao Sun , Jie Huang , Yanjie Chen , Jiameng Ni , Zaixiang Tang , Jingfang Liu

Background

The prescription of levetiracetam during pregnancy has become more common due to its lower teratogenic risk profile. However, due to a lack of data about its association with stillbirth and spontaneous abortion, worries remain.

Objective

To investigate information on any possible association of spontaneous abortion and stillbirth adverse events with levetiracetam in women with epilepsy.

Methods

This retrospective pharmacovigilance study used disproportionality analysis to detect signals of adverse reaction of interest reported with Levetiracetam in FAERS, the FDA Adverse Event Reporting System. The ratio of reporting odds (ROR) and information component (IC) indices were used to undertake disproportionality analyses, and change point analyses were carried out to identify variations in the frequency of reporting of relevant adverse events. Sensitivity analyses included subgroup analyses by indication, treatment regimen, and reporting region.

Results

Overall, 2870 cases of spontaneous abortion and stillbirth with commonly used antiseizure medications were analyzed. A total of 65.5 % of these cases had epilepsy as the indication. In the entire dataset, we observed disproportionality signals of spontaneous abortion for 6 ASMs (levetiracetam, carbamazepine, lamotrigine, oxcarbazepine, topiramate, valproic acid) and disproportionality signals of stillbirth for 4 ASMs (levetiracetam, carbamazepine, lamotrigine, oxcarbazepine). In the epileptic population, disproportionality signals for stillbirth (ROR0.25 = 4.60; IC0.25 = 1.30) and spontaneous abortion (ROR0.25 = 3.98; IC0.25 = 1.20) in levetiracetam was identified. These disproportionality signals have been consistently robust over the past years, according to a temporal assessment of them. Sensitivity studies proved how reliable the findings were.

Conclusion

Using validated pharmacovigilance methods, we found significant disproportional signals for spontaneous abortion and stillbirth associated with levetiracetam. Of these, the signals for spontaneous abortion were observed after 2011 and for stillbirth after 2014, which may be related to the rise in levetiracetam prescriptions during pregnancy in recent years. The association of spontaneous abortion and stillbirth adverse events with levetiracetam and potential biases confounding this association merit further investigation.
背景由于左乙拉西坦的致畸风险较低,妊娠期处方左乙拉西坦已变得越来越普遍。方法这项回顾性药物警戒研究使用不相称性分析来检测FDA不良事件报告系统FAERS中报告的左乙拉西坦相关不良反应信号。采用报告几率比(ROR)和信息成分指数(IC)进行比例失调分析,并进行变化点分析以确定相关不良事件报告频率的变化。敏感性分析包括按适应症、治疗方案和报告地区进行亚组分析。结果共分析了2870例使用常用抗癫痫药物的自然流产和死胎病例。其中,65.5%的病例以癫痫为适应症。在整个数据集中,我们观察到 6 种 ASM(左乙拉西坦、卡马西平、拉莫三嗪、奥卡西平、托吡酯、丙戊酸)的自然流产比例失调信号,以及 4 种 ASM(左乙拉西坦、卡马西平、拉莫三嗪、奥卡西平)的死胎比例失调信号。在癫痫人群中,发现左乙拉西坦有死胎(ROR0.25=4.60;IC0.25=1.30)和自然流产(ROR0.25=3.98;IC0.25=1.20)的不相称性信号。根据对其进行的时间评估,这些比例失调信号在过去几年中一直很稳定。结论通过使用经过验证的药物警戒方法,我们发现了与左乙拉西坦相关的自发性流产和死胎的重大比例失调信号。其中,自然流产信号出现在 2011 年之后,死胎信号出现在 2014 年之后,这可能与近年来妊娠期左乙拉西坦处方量增加有关。自然流产和死胎不良事件与左乙拉西坦的关联以及混淆这种关联的潜在偏倚值得进一步研究。
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引用次数: 0
Predictive factors for additional surgeries in drug-resistant epilepsy after mesial temporal laser interstitial thermal therapy 颞中叶激光间质热疗后耐药癫痫患者进行额外手术的预测因素。
IF 2.3 3区 医学 Q2 BEHAVIORAL SCIENCES Pub Date : 2024-10-11 DOI: 10.1016/j.yebeh.2024.110074
Behnaz Esmaeili , Andrew L. Ko , John W. Miller
We aimed to identify factors predicting additional surgical interventions in patients with drug-resistant epilepsy who continue experiencing seizures after mesial temporal laser interstitial thermal therapy (LITT). In a retrospective observational study, we analyzed consecutive patients with drug-resistant temporal epilepsy treated with mesial temporal LITT at the University of Washington between 2013–2022. The primary outcome was subsequent epilepsy surgery to improve seizure control after LITT. Logistic regression was used to assess how clinical factors and travel distance within tertiary center referral regions predict subsequent surgery occurrence. We identified 145 patients treated with mesial temporal LITT. Among the patients, 25 underwent subsequent surgeries, including 17 temporal lobectomies, 5 repeat LITT, 2 responsive neurostimulation, and 1 with deep brain stimulation. Further surgery was associated with higher Engel scores and shorter travel distance to our tertiary epilepsy center. Factors like age, sex, epilepsy duration, surgery on dominant hemisphere, and frequency of convulsive seizures were not significant. Patients who continue to have seizures after LITT are potential candidates for more surgery. Patients who must travel farther are less likely to receive follow-up surgery. Healthcare inequity presents a significant barrier in accessing epilepsy surgery, limiting the potential to achieve improved outcomes for individuals with epilepsy.
我们的目的是找出耐药性癫痫患者在接受颞中叶激光间质热疗(LITT)后继续出现癫痫发作的额外手术干预的预测因素。在一项回顾性观察研究中,我们分析了 2013-2022 年间在华盛顿大学接受颞中叶激光间质热疗的连续耐药颞叶癫痫患者。主要结果是 LITT 后为改善癫痫发作控制而进行的后续癫痫手术。逻辑回归用于评估三级中心转诊区域内的临床因素和旅行距离如何预测后续手术的发生。我们确定了 145 名接受颞中叶 LITT 治疗的患者。其中 25 人接受了后续手术,包括 17 例颞叶切除术、5 例重复 LITT、2 例反应性神经刺激和 1 例脑深部刺激。进一步手术与较高的恩格尔评分和较短的前往三级癫痫中心的路程有关。年龄、性别、癫痫持续时间、优势半球手术和惊厥发作频率等因素均无显著影响。LITT后仍有癫痫发作的患者有可能接受更多手术。必须长途跋涉的患者接受后续手术的可能性较低。医疗不公平是接受癫痫手术的一大障碍,限制了癫痫患者获得更好治疗效果的可能性。
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引用次数: 0
Clinical presentation of young persons with epilepsy who have not transitioned to adult health care systems: A single-site retrospective cohort 尚未过渡到成人医疗系统的青少年癫痫患者的临床表现:单一地点回顾性队列。
IF 2.3 3区 医学 Q2 BEHAVIORAL SCIENCES Pub Date : 2024-10-11 DOI: 10.1016/j.yebeh.2024.110058
Meha Joshi , Laura D. Fonseca , Gogi Kumar

Objective

We aimed to determine the demographics and clinical presentation of young persons with epilepsy (YPE) 18-years and older who have not transitioned to adult health care systems (AHCS) and to investigate the number of neurology-related hospital encounters in this population before and after attaining adulthood.

Methods

We conducted a single-site retrospective cohort study at Dayton Children’s Hospital (DCH) between 12/01/2021 and 5/31/2022. We included epilepsy patients 18-years and older. Patient demographics, insurance, number of hospitalizations and Emergency Department (ED) visits before and after 18 years of age, clinical presentation, and comorbidities were collected.

Results

251 patient charts were reviewed. The median age was 20.9 years. The most common comorbidities were intellectual disability (36 %) and psychiatric illnesses (59 %). The majority had generalized epilepsy (52 %) and tonic-clonic seizures (56 %). 58 % of the patients had infrequent seizures in the past year. Documentation of transition to AHCS discussion occurred in only 3 % of patients before age 18 compared to 35 % of patients after age 18. There was a decrease in 30 % for ED visits and 15 % for neurology related hospitalizations after the age of 18.

Conclusion

YPE 18-years of age and older who were managed by a pediatric neurologist at DCH had significant co-morbidities. However, they were clinically stable and rarely required admission to the hospital. Most of them did not have documentation about transition discussions. At DCH, YPE who have not transitioned to AHCS are clinically stable and well managed.
目的我们旨在确定 18 岁及以上尚未过渡到成人医疗保健系统 (AHCS) 的年轻癫痫患者 (YPE) 的人口统计学特征和临床表现,并调查该人群在成年前后与神经病学相关的医院就诊次数:我们在 2021 年 1 月 12 日至 2022 年 5 月 31 日期间在代顿儿童医院(DCH)开展了一项单点回顾性队列研究。研究对象包括 18 岁及以上的癫痫患者。我们收集了患者的人口统计学资料、保险情况、18 岁之前和之后的住院和急诊科就诊次数、临床表现和合并症。中位年龄为 20.9 岁。最常见的合并症是智力障碍(36%)和精神疾病(59%)。大多数患者患有全身性癫痫(52%)和强直阵挛发作(56%)。58% 的患者在过去一年中癫痫发作不频繁。在 18 岁之前,只有 3% 的患者有过渡到 AHCS 讨论的记录,而在 18 岁之后,则有 35% 的患者有此记录。18 岁以后,急诊室就诊率下降了 30%,神经内科相关住院率下降了 15%:结论:由大昌医院儿科神经科医生治疗的 18 岁及以上的 YPE 患者有严重的并发症。然而,他们的临床状况稳定,很少需要入院治疗。他们中的大多数人都没有关于转院讨论的文件记录。在 DCH,未转入 AHCS 的 YPE 临床病情稳定,管理良好。
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引用次数: 0
Interictal head-turning sign in patients with idiopathic generalized epilepsy during initial medical interview: A matched multicenter study 初诊时特发性全身性癫痫患者发作间期的转头征:一项匹配的多中心研究
IF 2.3 3区 医学 Q2 BEHAVIORAL SCIENCES Pub Date : 2024-10-11 DOI: 10.1016/j.yebeh.2024.110039
Shuichiro Neshige , Shiro Aoki , Narumi Ohno , Megumi Nonaka , Hidetada Yamada , Yoshiko Takebayashi , Haruka Ishibashi , Takeo Shishido , Dai Agari , Yu Yamazaki , Koji Iida , Hirofumi Maruyama

Background

Utilizing interictal manifestations for the diagnosis of epilepsy is challenging. We investigated whether an interictal “head-turning sign,” typically indicative of dependence on others observed in Alzheimer’s disease, can act as a behavioral marker of idiopathic generalized epilepsy.

Methods

This multicenter study examined 579 consecutive patients, with a mean age of 36.8 ± 20.4 years, who did not have an intellectual disability and had their first outpatient visit for epilepsy evaluation between 2019 and 2023. Patients were categorized into IGE, non-IGE epilepsy, non-epileptic, and psychiatric conditions based on their ultimate diagnostic outcomes to identify difference of the occurrence of the head-turning sign among them. Additionally, we extracted data from patients under the age of 40, specifically adolescents and young adults (AYA). Then we used propensity score matching to confirm the reproducibility of observed differences and to identify associated factors within the AYA age group.

Results

The occurrence of the head-turning sign was significantly more prevalent in the IGE group compared to the non-IGE group (20.4 % vs. 2.2 %; P<0.0001) and non-epileptic group (20.4 % vs. 8.3 %; P=0.033). Following the matching, the head-turning sign was still evident in IGE relative to non-IGE patients (14.6 % vs. 4.5; P=0.004), yielding a 94 % specificity for IGE. IGE diagnosis (P<0.0001), myoclonic seizure (P<0.0001), being visited by a parent (P=0.017), and comorbidity with headache (P=0.021) were significantly associated with the head-turning sign. Multivariate analysis revealed that IGE (odds ratio: OR=2.80, P=0.028), attending with a parent (OR=2.92, P=0.029), and comorbidity with headache (OR=4.06, P=0.016) were independently associated with the head-turning sign.

Conclusions

We confirmed a substantial association between the interictal “head-turning sign” and IGE. This unique sign may reflect a tendency towards dependence on others in IGE, and may serve as a promising diagnostic auxiliary marker for identifying IGE in the AYA age group.
背景利用发作间期表现诊断癫痫具有挑战性。我们研究了发作间期 "转头征"(通常表示阿尔茨海默病中观察到的对他人的依赖)是否可以作为特发性全身性癫痫的行为标记。方法这项多中心研究对 579 名连续患者进行了检查,这些患者平均年龄(36.8 ± 20.4)岁,没有智力障碍,并且在 2019 年至 2023 年期间进行了首次门诊癫痫评估。根据最终诊断结果,我们将患者分为IGE、非IGE癫痫、非癫痫和精神疾病患者,以确定他们之间出现转头征的差异。此外,我们还提取了 40 岁以下患者的数据,特别是青少年和年轻人(AYA)。结果与非 IGE 组(20.4% vs. 2.2%;P<0.0001)和非癫痫组(20.4% vs. 8.3%;P=0.033)相比,IGE 组的转头征发生率明显更高。配对后,与非 IGE 患者相比,IGE 患者的转头征仍然明显(14.6% 对 4.5;P=0.004),IGE 的特异性为 94%。IGE诊断(P<0.0001)、肌阵挛发作(P<0.0001)、父母探视(P=0.017)和合并头痛(P=0.021)与转头征显著相关。多变量分析显示,IGE(几率比:OR=2.80,P=0.028)、父母陪同就诊(OR=2.92,P=0.029)和头痛合并症(OR=4.06,P=0.016)与转头征独立相关。我们证实了发作间期 "转头征 "与 IGE 有很大的关联。这种独特的征象可能反映了 IGE 患者对他人的依赖倾向,可作为识别青壮年 IGE 的辅助诊断标志物。
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引用次数: 0
What do ketogenic dietitians and physicians need? A survey from the KetoCollegeAdvance™ training in United Kingdom 生酮营养师和医生需要什么?来自英国 KetoCollegeAdvance™ 培训的一项调查。
IF 2.3 3区 医学 Q2 BEHAVIORAL SCIENCES Pub Date : 2024-10-11 DOI: 10.1016/j.yebeh.2024.110071
Eric H. Kossoff , Zoe Simpson , Julie Fountain

Introduction

The ketogenic diet is a valuable nonpharmacologic therapy for the treatment of refractory epilepsy in children and adults. It can be time-intensive for ketogenic teams, typically comprised of a physician and dietitian at a minimum. Challenges and barriers to providing ketogenic diet services to patients by members of these teams has not been studied extensively.

Methods

A survey was created and distributed to attendees at a ketogenic diet training conference (KetoCollegeAdvance™) held 21–23 May 2024 in United Kingdom. Questions included Likert scales and fill-in responses. Surveys were provided by 63 attendees (mostly dietitians) from 17 countries.

Results

Respondents were mostly dietitians (45/63, 71 %) and from the United Kingdom. In regards to perceived interest levels in KD in general in their countries, dietitians were perceived as 80 % very or extremely interested, parents (66 %), and neurologists (45 %). The majority of teams included a dietitian (79 %) and physician (78 %). The majority, 43 (68 %) of respondents, assumed care of all aspects of epilepsy care once the KD was started. Common barriers to starting KD services included a long waiting list, lack of adult KD services, funding dietitians, and low referrals. Barriers to continuing KD services included poor patient compliance, a lack of financial resources for some families to afford foods, and a need for more pre-made ketogenic foods including bread, pizza, pasta, potato fries, and chocolates.

Conclusions

These results from a conference of international ketogenic dietitians and physicians highlights common difficulties in providing the ketogenic diet successfully. Addressing these barriers may help expand the usage of this therapy for more patients with epilepsy.
简介:生酮饮食是治疗儿童和成人难治性癫痫的重要非药物疗法。生酮小组通常至少由一名医生和一名营养师组成,这可能会耗费大量时间。目前尚未对这些团队成员为患者提供生酮饮食服务所面临的挑战和障碍进行广泛研究:在 2024 年 5 月 21-23 日于英国举行的生酮饮食培训会议(KetoCollegeAdvance™)上,我们制作并向与会者分发了一份调查问卷。问题包括李克特量表和填写式回答。来自 17 个国家的 63 名与会者(大部分是营养师)提供了调查问卷:受访者大多是营养师(45/63,71%),来自英国。就各国对 KD 的兴趣程度而言,80% 的营养师、66% 的家长和 45% 的神经科医生对 KD 非常或非常感兴趣。大多数团队包括一名营养师(79%)和一名医生(78%)。大多数受访者(43 人,占 68%)在开始接受 KD 后,会负责癫痫护理的所有方面。开始接受 KD 服务的常见障碍包括等候名单过长、缺乏成人 KD 服务、营养师经费不足以及转诊率低。持续开展生酮治疗服务的障碍包括患者依从性差、一些家庭缺乏经济来源买不起食物,以及需要更多的预制生酮食物,包括面包、披萨、意大利面、薯条和巧克力:这些来自国际生酮营养师和医生会议的结果凸显了成功提供生酮饮食的共同困难。解决这些障碍可能有助于扩大这种疗法在更多癫痫患者中的应用。
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引用次数: 0
Neuromodulation strategies in developmental and epileptic encephalopathies 发育性和癫痫性脑病的神经调控策略。
IF 2.3 3区 医学 Q2 BEHAVIORAL SCIENCES Pub Date : 2024-10-10 DOI: 10.1016/j.yebeh.2024.110067
Debopam Samanta , Zulfi Haneef , Gregory W. Albert , Sunil Naik , Puck C. Reeders , Puneet Jain , Taylor J. Abel , Ruba Al-Ramadhani , George M. Ibrahim , Aaron E.L. Warren
Developmental and epileptic encephalopathies (DEEs) are a group of childhood-onset epilepsy syndromes characterized by frequent seizures, severe cognitive and behavioral impairments, and poor long-term outcomes. These conditions are typically refractory to currently available medical therapies, prompting recent exploration of neuromodulation treatments such as deep brain stimulation (DBS) and responsive neurostimulation (RNS), which aim to modulate epileptic networks spanning cortical and subcortical regions. These advances have occurred alongside an improved understanding of syndrome-specific and interictal epileptiform discharge/seizure-specific brain networks. By targeting key nodes within these networks, DBS and RNS hold promise for influencing seizures and associated cognitive and behavioral comorbidities. Initial experiences with centromedian (CM) thalamic DBS for Lennox-Gastaut syndrome (LGS) have shown modest efficacy across multiple seizure types. Reports also indicate the application of DBS and RNS across various genetic and structural etiologies commonly associated with DEEs, with mixed success. Although DBS and RNS are increasingly used in LGS and other DEEs, their mixed efficacy highlights a knowledge gap in understanding why some patients with LGS do not respond and which neuromodulation approach is most effective for other DEEs. To address these issues, this review first discusses recent neuroimaging studies showing similarities and differences in the epileptic brain networks underlying various DEEs, revealing the common involvement of the thalamus and the default-mode network (DMN) across multiple DEEs. We then examine thalamic DBS for LGS to illustrate how such network insights may be used to optimize neuromodulation. Although network-based neuromodulation is still in its infancy, the LGS model may serve as a framework for other DEEs, where optimal treatment necessitates consideration of the underlying epileptic networks. Lastly, the review suggests future research directions, including individualized connectivity assessment and biomarker identification through collaborative efforts, which may enhance the therapeutic potential of neuromodulation for individuals living with DEEs.
发育性癫痫性脑病(DEEs)是一组儿童期发病的癫痫综合征,其特点是癫痫频繁发作、严重的认知和行为障碍以及长期疗效不佳。这些病症通常对现有的药物疗法具有难治性,促使人们最近开始探索神经调控疗法,如脑深部刺激(DBS)和反应性神经刺激(RNS),其目的是调节跨越皮层和皮层下区域的癫痫网络。在取得这些进展的同时,人们对综合征特异性和发作间期癫痫样放电/癫痫特异性大脑网络的了解也在不断加深。通过靶向这些网络中的关键节点,DBS 和 RNS 有希望影响癫痫发作及相关的认知和行为合并症。丘脑中央定位脑电切除术(CM)治疗伦诺克斯-加斯豪特综合征(LGS)的初步经验显示,该疗法对多种癫痫发作类型均有一定疗效。报告还显示,DBS 和 RNS 可应用于与 DEEs 常见的各种遗传和结构病因,但疗效参差不齐。虽然 DBS 和 RNS 越来越多地用于 LGS 和其他 DEEs,但它们的疗效参差不齐,这凸显了在理解为什么一些 LGS 患者没有反应以及哪种神经调控方法对其他 DEEs 最有效方面存在知识空白。为了解决这些问题,本综述首先讨论了最近的神经影像学研究,这些研究显示了各种 DEEs 的基础癫痫脑网络的异同,揭示了丘脑和默认模式网络 (DMN) 在多种 DEEs 中的共同参与。然后,我们研究了丘脑 DBS 治疗 LGS 的情况,以说明如何利用这种网络洞察力来优化神经调控。尽管基于网络的神经调控仍处于起步阶段,但 LGS 模型可作为其他 DEE 的框架,在这些 DEE 中,优化治疗需要考虑潜在的癫痫网络。最后,综述提出了未来的研究方向,包括通过合作进行个体化连通性评估和生物标记物鉴定,这可能会提高神经调控对 DEEs 患者的治疗潜力。
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Epilepsy & Behavior
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