Purpose

Clinicians and researchers often focus on the primary cause of seizures and epilepsy, but outcomes in individual patients also depend on multiple other variables, which might be easy to adjust. Previous studies suggest mutual interactions between endocrine disorders and epilepsy. We therefore hypothesized that combined pituitary hormone deficiency (CPHD) facilitates seizures and epilepsy.

Methods

This is a retrospective study from a pediatric center. We determined the proportion of CPHD patients with epilepsy and examined basic clinical features in this group. Patients with super-refractory status epilepticus (SRSE) were reviewed to identify subjects with co-morbid CPHD. Those cases were analyzed in detail.

Results

12 of 73 CPHD patients (16%) also had epilepsy. Various etiologies of CPHD were represented, though five subjects had a cranial tumor or cortical malformation. Epilepsy was drug resistant in all but one patient. Among 12 identified patients with SRSE, 4 were unexpected new-onset cases. Three of these subjects also had CPHD with ACTH deficiency and a febrile infection prior to SRSE. Another common feature was the devastating clinical course: In all three patients, initial MRI already suggested severe neuronal damage, SRSE persisted for at least one week with ongoing need for anesthetic coma, and outcome was poor (two patients survived with major sequelae, one child deceased during the episode).

Conclusion

Our findings indicate that CPHD may predispose for drug-resistant epilepsy and refractory seizures with catastrophic outcome. We suggest that in children with new-onset SRSE, screening for CPHD should be considered.

中文翻译：

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对合并垂体激素缺乏症患儿耐药性癫痫和灾难性癫痫持续状态的促进作用

目的

临床医生和研究人员通常关注癫痫发作和癫痫的主要原因，但个别患者的结果还取决于多个其他变量，这些变量可能很容易调整。先前的研究表明内分泌失调与癫痫之间存在相互作用。因此，我们假设联合垂体激素缺乏症 (CPHD) 会促进癫痫发作和癫痫。

方法

这是一项来自儿科中心的回顾性研究。我们确定了 CPHD 癫痫患者的比例并检查了该组的基本临床特征。对超难治性癫痫持续状态 (SRSE) 的患者进行审查，以确定患有共病 CPHD 的受试者。对这些案例进行了详细分析。

结果

73 名 CPHD 患者中有 12 名 (16%) 也患有癫痫。CPHD 的各种病因都有代表，但有五名受试者患有颅内肿瘤或皮质畸形。除一名患者外，其他所有患者的癫痫均具有耐药性。在 12 名已确定的 SRSE 患者中，4 名是意外的新发病例。其中三名受试者在 SRSE 之前也患有 CPHD，ACTH 缺乏症和发热性感染。另一个共同特征是毁灭性的临床病程：在所有 3 名患者中，最初的 MRI 已经表明严重的神经元损伤，SRSE 持续了至少一周并且持续需要麻醉昏迷，结果很差（两名患者因严重后遗症幸存下来，一名儿童在剧集中去世）。

结论

我们的研究结果表明，CPHD 可能易患耐药性癫痫和难治性癫痫发作，并带来灾难性的后果。我们建议对于新发 SRSE 的儿童，应考虑筛查 CPHD。