结节性硬化症复合体相关癫痫患儿接受癫痫手术的途径。

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique Pub Date : 2024-10-01 DOI:10.1016/j.neurol.2024.04.009

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引用次数: 0

摘要

背景：以往的研究表明，对经过严格挑选的结节性硬化综合征癫痫患儿进行癫痫手术治疗具有疗效。然而，对于如何进行选择以及接受手术治疗的患者的特征，目前仍缺乏充分的描述。通过对过去二十年的临床实践进行多中心回顾性队列研究，我们描述了结节性硬化综合症相关癫痫患儿接受癫痫手术的途径：我们通过比对两份详尽的遗传病登记表以及随后在两家法国神经儿科和癫痫中心进行的病历审查，确定了 84 名被诊断患有结节性硬化症综合征和癫痫的儿童。收集了人口统计学、临床、纵向、诊断和手术数据：结果：46%的患儿最初具有耐药性，19%的患儿接受了切除手术，其中大部分患儿在四岁之前就接受了手术。44%的病例在手术前进行了立体定向脑电图检查。分别有57%和43%的患者在术后一年和十年仍无癫痫发作。此外，在最初耐药但未接受手术的患者中，52%的患者在最后一次随访时没有癫痫发作。50%的患者在手术后所需的抗癫痫药物数量有所减少。婴儿痉挛症、智力障碍、自闭症谱系障碍或严重行为障碍并非手术禁忌症，但与较高的并发症发生率和较低的术后无癫痫发作率有关：结论：尽管假定结节性硬化综合征幼儿患有复杂的多灶性癫痫并存在实际困难，但手术的成功率与其他耐药性癫痫患者不相上下，而且未接受手术的患者可能会自发演变为对药物敏感的癫痫。

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Pathways to epilepsy surgery in children with tuberous sclerosis complex-associated epilepsy

Background

Previous studies showed the efficacy of epilepsy surgery in carefully selected children with epilepsy associated with tuberous sclerosis complex. However, how this selection is conducted, and the characteristics of the patients brought to surgery are still poorly described. By conducting a multicentric retrospective cohort study covering the practice of the last twenty years, we describe the paths leading to epilepsy surgery in children with epilepsy associated with tuberous sclerosis complex.

Methods

We identified 84 children diagnosed with tuberous sclerosis complex and epilepsy by matching two exhaustive registries of genetic diseases and subsequent medical records reviews within two French neuropediatric and epilepsy centers. Demographic, clinical, longitudinal, and diagnostic and surgical procedures data were collected.

Results

Forty-six percent of the children were initially drug-resistant and 19% underwent resective surgery, most often before the age of four. Stereotactic electroencephalography was performed prior to surgery in 44% of cases. Fifty-seven and 43% of patients remained seizure-free one and ten years after surgery, respectively. In addition, 52% of initially drug-resistant patients who did not undergo surgery were seizure-free at the last follow-up. The number of anti-seizure medications required decreased in 50% of cases after surgery. Infantile spasms, intellectual disability, autism spectrum disorder or severe behavioral disorders were not contraindications to surgery but were associated with a higher rate of complications and a lower rate of seizure freedom after surgery.

Conclusion

Despite the assumption of complex multifocal epilepsy and practical difficulties in young children with tuberous sclerosis complex, successful surgery results are comparable with other populations of patients with drug-resistant epilepsy, and a spontaneous evolution to drug-sensitive epilepsy may occur in non-operated patients.

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来源期刊

Revue neurologique 医学-临床神经学

CiteScore

4.80

自引率

0.00%

发文量

598

审稿时长

55 days

期刊介绍： The first issue of the Revue Neurologique, featuring an original article by Jean-Martin Charcot, was published on February 28th, 1893. Six years later, the French Society of Neurology (SFN) adopted this journal as its official publication in the year of its foundation, 1899. The Revue Neurologique was published throughout the 20th century without interruption and is indexed in all international databases (including Current Contents, Pubmed, Scopus). Ten annual issues provide original peer-reviewed clinical and research articles, and review articles giving up-to-date insights in all areas of neurology. The Revue Neurologique also publishes guidelines and recommendations. The Revue Neurologique publishes original articles, brief reports, general reviews, editorials, and letters to the editor as well as correspondence concerning articles previously published in the journal in the correspondence column.