Prevalence, clinical characteristics and outcomes of seizures in neurofibromatosis type 1: A systematic review and single arm meta-analysis

IF 2 4区医学 Q3 CLINICAL NEUROLOGY

Epilepsy Research Pub Date : 2024-11-08 DOI:10.1016/j.eplepsyres.2024.107476

Fan Wu , Xinna Ji , Mengxiao Shen , Peidi Cheng , Yanyan Gao , Wanting Liu , Jinxiao Chen , Shuo Feng , Huanhuan Wu , Fei Di , Yunlin Li , Jianhua Wang , Xue Zhang , Qian Chen

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引用次数: 0

Abstract

Background

Neurofibromatosis type 1 (NF1) is a common neurocutaneous syndrome that predisposes patients to seizures. We aimed to estimate the prevalence, clinical characteristics and outcomes of seizures in NF1 patients, and analyze prognostic factors of seizures.

Methods

Systematic searches were conducted in the PubMed, EMBASE, Web of Science, Scopus, Cochrane Library, and grey literature databases from inception to April 2024. We identified observational studies that included NF1 patients with seizures. Clinical characteristics of seizures were summarized and meta-analyses of prevalence and outcomes were conducted. We assessed publication bias using funnel plots and conducted sensitivity analysis to assess stability and reliability. Individual patient data were analyzed to evaluate prognostic factors.

Results

Fourteen studies were identified from 1021 records. A total of 337 patients with seizures from 4946 NF1 patients were included. Meta-analysis indicated the overall prevalence of seizures in NF1 patients was 8.1 % (95 % CI= 5.9–10.3 %). The first seizure usually occurs in childhood, with a median age range of 3.5–12.0 years. Focal seizures (54.2 %) and generalized tonic-clonic seizures (16.8 %) were the most common types. The seizure freedom rate was 68.5 % (95 % CI= 57.5–79.5 %), mostly with one or two antiseizure medications. Cortical malformation/hippocampus sclerosis emerged as an independent risk factor for persistent seizures (OR=5.19, 95 %CI=1.27–21.18, P=0.02).

Conclusion

Though NF1 patients face a higher risk of seizures than the general population, the majority achieve seizure freedom. Patients with cortical malformation or hippocampus sclerosis were at a higher risk of persistent seizures.

查看原文本刊更多论文

1型神经纤维瘤病癫痫发作的患病率、临床特征和预后：系统综述和单臂荟萃分析。

背景：神经纤维瘤病1型（NF1）是一种常见的神经皮肤综合征，易导致患者癫痫发作。我们的目的是估计 NF1 患者癫痫发作的患病率、临床特征和结果，并分析癫痫发作的预后因素：方法：我们在 PubMed、EMBASE、Web of Science、Scopus、Cochrane Library 和灰色文献数据库中进行了系统检索，检索时间从开始到 2024 年 4 月。我们确定了纳入 NF1 癫痫发作患者的观察性研究。我们总结了癫痫发作的临床特征，并对发病率和结果进行了荟萃分析。我们使用漏斗图评估了发表偏倚，并进行了敏感性分析以评估稳定性和可靠性。我们对患者的个体数据进行了分析，以评估预后因素：从 1021 份记录中确定了 14 项研究。共纳入了 4946 名 NF1 患者中的 337 名癫痫发作患者。元分析表明，NF1患者癫痫发作的总患病率为8.1%（95% CI= 5.9-10.3%）。首次癫痫发作通常发生在儿童时期，中位年龄为 3.5-12.0 岁。最常见的类型是局灶性发作（54.2%）和全身强直阵挛发作（16.8%）。癫痫发作自由率为 68.5%（95 % CI= 57.5-79.5%），大多数情况下只需服用一种或两种抗癫痫药物。皮质畸形/海马硬化是癫痫持续发作的独立风险因素（OR=5.19，95 %CI=1.27-21.18，P=0.02）：尽管NF1患者面临的癫痫发作风险高于普通人群，但大多数患者都能摆脱癫痫发作。皮质畸形或海马硬化的患者癫痫持续发作的风险较高。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Epilepsy Research 医学-临床神经学

CiteScore

0.10

自引率

4.50%

发文量

143

审稿时长

62 days

期刊介绍： Epilepsy Research provides for publication of high quality articles in both basic and clinical epilepsy research, with a special emphasis on translational research that ultimately relates to epilepsy as a human condition. The journal is intended to provide a forum for reporting the best and most rigorous epilepsy research from all disciplines ranging from biophysics and molecular biology to epidemiological and psychosocial research. As such the journal will publish original papers relevant to epilepsy from any scientific discipline and also studies of a multidisciplinary nature. Clinical and experimental research papers adopting fresh conceptual approaches to the study of epilepsy and its treatment are encouraged. The overriding criteria for publication are novelty, significant clinical or experimental relevance, and interest to a multidisciplinary audience in the broad arena of epilepsy. Review articles focused on any topic of epilepsy research will also be considered, but only if they present an exceptionally clear synthesis of current knowledge and future directions of a research area, based on a critical assessment of the available data or on hypotheses that are likely to stimulate more critical thinking and further advances in an area of epilepsy research.