Pediatric Neurosurgery Unit, Rothschild Foundation Hospital, Paris, France.
Pediatric Neurosurgery Unit, Rothschild Foundation Hospital, Paris, France; Inserm, U663, Paris, F-75015 France, University Paris Descartes, 75005 Paris, France.
Seizure. 2017 Oct;51:163-170. doi: 10.1016/j.seizure.2017.08.010. Epub 2017 Aug 31.
Infantile spasms (IS) can occur as the only seizure type in children with surgically amenable epilepsies. Although early surgery has shown positive effects, little is known regarding outcomes.
We retrospectively reviewed all children with IS referred to our tertiary center between 2002 and 2014 and try to define factors of outcome.
Sixty-eight children with focal onset seizures were referred: twenty children with a hemispheric implication and 48 with one or more lobes involved. The age of onset was significantly earlier in the hemispheric population (8.0 versus 16.7 months in the focal population). There was no difference in the age of onset between anterior and posterior onset zones, as we could expect regarding the maturation gradient. The epilepsy began earlier in life in tuberous sclerosis than in DNET. Only three children of the 48 non-hemispheric patients had a normal MRI at the time of the surgery. Temporal lobe was involved only in a third of the population. More than 86% of the patients were operated on. Patients with hemispheric lesions were operated on younger (2.6 years+/- 2.1 years) compared to 4.6+/- 3.5 years in the whole population. The most frequent etiologies were in descending order: dysplasia, ganglioglioma or dysembryoplastic tumours and tuberous sclerosis. The global seizure outcome was favorable (Engel 1a) in 74.6% of the patients, and 87.9% if the delay between the first seizure and the surgery was less than 36 months. It fell to 64.7% if the delay exceeded 50 months.
Spasms of focal onset have a similar postsurgical outcome as other seizure types so surgery may be an excellent option for treating selected patients with focal infantile spasms. Volume and type but not topography of the lesion influence the age of onset. MRI is very helpful to locate the pathology in the pediatric population, since only a small portion had a normal MRI.
婴儿痉挛症（IS）可作为手术可治疗的癫痫儿童的唯一发作类型。尽管早期手术已显示出积极效果，但对于结果却知之甚少。
我们回顾性分析了 2002 年至 2014 年间转诊至我们三级中心的所有 IS 患儿，并尝试确定影响结果的因素。
共 68 例局灶性起病的癫痫患儿被转诊：20 例半球性受累患儿和 48 例累及 1 个或多个叶的患儿。半球性患儿的起病年龄明显早于局灶性患儿（8.0 个月对 16.7 个月）。根据成熟梯度，我们预计前区和后区起病的患儿起病年龄会有所不同，但实际上并无差异。与 DNET 相比，结节性硬化症的癫痫起病更早。48 例非半球性患者中，仅 3 例在手术时 MRI 正常。颞叶受累仅见于三分之一的患儿。超过 86%的患者接受了手术。半球性病变患儿的手术年龄较小（2.6 岁+/-2.1 岁），而全组患儿的手术年龄为 4.6 岁+/-3.5 岁。最常见的病因依次为：发育不良、神经节细胞瘤或发育不良性肿瘤和结节性硬化症。74.6%的患儿总体癫痫发作结局良好（Engel 1a），如果首次发作至手术的时间间隔小于 36 个月，则 87.9%的患儿结局良好。如果间隔超过 50 个月，则降至 64.7%。
局灶性起病的痉挛具有相似的术后结局，因此手术可能是治疗局灶性婴儿痉挛症的一种极佳选择。病变的容积和类型而不是部位影响起病年龄。MRI 对定位儿科人群中的病变非常有帮助，因为仅有一小部分患儿的 MRI 正常。
