背景:某些罕见结节性硬化症(TSC)患者的耐药性癫痫(DRE)可能受益于切除性癫痫手术。此外,相关的神经精神障碍(TAND)在TSC患者中很常见;然而,关于手术如何影响神经精神合并症的长期数据很少.
方法:采用两种回顾性方法对发病年龄<18岁的TSC和DRE患儿进行鉴定。研究组(手术)通过瑞典国家癫痫手术登记(n=17)确定,自1990年以来在全国范围内进行登记,自1995年以来进行前瞻性患者登记。通过搜索从瑞典南部三级医院检索的医疗记录来确定参考组(非手术)(n=52)。邀请符合条件的参与者完成经过验证的TAND寿命清单。那些没有完成清单的人,从来没有DRE,或年龄<7岁的患者被排除在研究之外。参照组与研究组在推定的混杂因素方面保持平衡,按以下分层顺序:调查时的DRE,癫痫发作的年龄,随访年龄,和性爱。
结果:在平衡过程之后,两组均由13名参与者组成.研究组和参照组从癫痫发作到调查的中位时间分别为18.5年(范围:7.75-40.25)和16.0年(7.33-33.5),分别。从手术到调查的中位时间为13年(范围:4-22)。在行为问题上没有发现显著差异,自闭症谱系障碍的诊断或症状,或者群体之间的智力残疾,不管手术。无癫痫发作的个体(n=11)在社交技能方面表现更好(p=0.016),智力技能(p=0.029),和总体TAND评分(p=0.005)比非无癫痫组(n=15)。
结论:这是第一项评估TSC患者癫痫手术后长期随访中TAND合并症的研究。我们没有发现输卵管切除术后TAND合并症的不良反应的证据。然而,需要一项更大的研究,以便更好地调整混杂因素。根据以前的研究,与未控制的癫痫组相比,无癫痫个体在大多数TAND领域的症状较少,表明症状不太严重。
BACKGROUND: Drug-resistant epilepsy (DRE) in selected individuals with the rare tuberous sclerosis complex (TSC) may benefit from resective epilepsy surgery. Furthermore, associated neuropsychiatric disorders (TAND) are common in patients with TSC; however, long-term data on how surgery affects neuropsychiatric comorbidities are sparse.
METHODS: Two retrospective approaches were used to identify children with TSC and DRE with onset at < 18 years of age. The study group (surgical) was identified through the Swedish National Epilepsy Surgery Registry (n = 17), a registry with complete national coverage since 1990 and prospective patient enrolment since 1995. The reference group (non-surgical) was identified by searching medical records retrieved from the tertiary hospital of Southern Sweden (n = 52). Eligible participants were invited to complete the validated TAND lifetime checklist. Those who did not complete the checklist, never had DRE, or were aged < 7 years old were excluded from the study. The reference group was balanced with the study group for putative confounders, in the following hierarchical order: DRE at the survey, age at seizure onset, age at follow-up, and sex.
RESULTS: After the balancing procedure, both groups comprised 13 participants. The median time from epilepsy onset to the survey was 18.5 (range: 7.75-40.25) and 16.0 (7.33-33.5) years in the study and reference groups, respectively. The median time from surgery to the survey was 13 years (range: 4-22). No significant differences were found in behavioural problems, autism spectrum disorder diagnosis or symptoms, or intellectual disability between the groups, regardless of surgery. Seizure-free individuals (n = 11) performed better in social skills (p = 0.016), intellectual skills (p = 0.029), and overall TAND scores (p = 0.005) than the non-seizure-free group (n = 15).
CONCLUSIONS: This is the first study to evaluate TAND comorbidities during the long-term follow-up after epilepsy surgery in patients with TSC. We found no evidence of the adverse effects of TAND comorbidities after tuberectomy. However, a larger study that allows for a better adjustment for confounders is needed. Following previous studies, seizure-free individuals had fewer symptoms within most TAND domains compared with the group with uncontrolled epilepsy, indicating less severe symptomatology.