Objectives

Lennox-Gastaut syndrome (LGS) and Dravet syndrome (DS) are rare treatment-resistant epileptic encephalopathies with limited data describing the relationship between seizures and quality of life (QoL). The objective of this cross-sectional pilot study was to assess the impact on QoL of seizures and seizure-free days for the generation of utility values.

Methods

Surveys were conducted in the UK and France, whereby patients and/or caregivers of patients with LGS, DS, or other epilepsies were asked to score health state vignettes for a hypothetical patient with LGS or DS. Respondents evaluated QoL for health states based on the number of seizures and seizure-free days per month, using a visual analog scale (VAS). Visual analog scale scores were converted to the 0–1 scale as a proxy estimate for utility values. Surveys were pilot tested and respondents were recruited from October 2018 to August 2019.

Results

Patient respondents were mainly treatment-responsive (n = 43/55) whereas caregiver respondents mainly cared for patients with treatment-resistant epilepsy (n = 38/43). Most respondents and patients were aged ≥18 years. Results from LGS and DS surveys in the UK (n = 58) and France (n = 40) suggested that health states with fewer seizures and more seizure-free days had higher QoL scores for hypothetical patients. For DS, QoL scores for patient health states ranged from 0.20 (32 convulsive seizures and 4 seizure-free days/month, UK) to 0.92 (seizure-free, France). For LGS, scores ranged from 0.14 (130 drop seizures and 1 seizure-free day/month, France) to 0.83 (seizure-free, UK). In all surveys, seizure-free days had a greater impact on QoL than seizure frequency (P < 0.001).

Conclusions

Fewer seizures and additional seizure-free days improved QoL in patients with LGS or DS; seizure-free days had the greatest impact on QoL.

中文翻译：

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癫痫发作频率对 Lennox-Gastaut 综合征或 Dravet 综合征患者生活质量的影响

目标

Lennox-Gastaut 综合征 (LGS) 和 Dravet 综合征 (DS) 是罕见的难治性癫痫性脑病，描述癫痫发作与生活质量 (QoL) 之间关系的数据有限。这项横断面试点研究的目的是评估癫痫发作和无癫痫发作天数对产生效用值的 QoL 的影响。

方法

在英国和法国进行了调查，要求 LGS、DS 或其他癫痫患者的患者和/或护理人员对假设的 LGS 或 DS 患者的健康状况进行评分。受访者使用视觉模拟量表 (VAS) 根据每月癫痫发作次数和无癫痫发作天数评估健康状况的 QoL。视觉模拟量表分数被转换为 0-1 量表，作为效用值的代理估计。调查进行了试点测试，并于 2018 年 10 月至 2019 年 8 月招募了受访者。

结果

患者受访者主要对治疗有反应 ( n  = 43/55)，而照顾者受访者主要照顾难治性癫痫患者 ( n  = 38/43)。大多数受访者和患者年龄≥18 岁。英国 ( n  = 58) 和法国 ( n = 40) 表明，对于假设患者而言，癫痫发作较少且无癫痫发作天数较多的健康状况具有更高的 QoL 评分。对于 DS，患者健康状况的 QoL 评分范围从 0.20（32 次惊厥发作和 4 天/月无癫痫发作，英国）到 0.92（无癫痫发作，法国）。对于 LGS，评分范围从 0.14（130 次癫痫发作和 1 天/月无癫痫发作，法国）到 0.83（无癫痫发作，英国）。在所有调查中，无癫痫发作天数对 QoL 的影响大于癫痫发作频率 ( P <  0.001)。

结论

更少的癫痫发作和额外的无癫痫发作天数改善了 LGS 或 DS 患者的 QoL；无癫痫发作天数对 QoL 的影响最大。