Department of Internal Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire, USA.
Pediatr Neurol. 2011 Aug;45(2):83-8. doi: 10.1016/j.pediatrneurol.2011.05.001.
Identification of a single epileptogenic focus in patients with tuberous sclerosis complex is a challenge. Noninvasive imaging modalities, including subtraction ictal single-photon emission computed tomography coregistered to magnetic resonance imaging (SISCOM), have been used to determine the dominant epileptogenic focus for surgical resection. We assessed whether complete resection of SISCOM hyperperfusion abnormality correlates with seizure-free outcome in 6 children with tuberous sclerosis complex-related epilepsy. The median seizure onset age was 4 months (range 1 day to 16 months). The age at surgery ranged from 8 months to 13 years. A dominant SISCOM hyperperfusion focus was identified in 5 patients with multiple tubers. SISCOM provided additional localizing information for epilepsy surgery in 3 patients with nonlocalizing or discordant electrophysiologic and neuroimaging findings. At a minimum of 2 years' follow-up, 3 patients were free of seizures overall. Freedom from seizures was associated with complete resection of SISCOM abnormality in 2 patients. These findings demonstrate that SISCOM can be useful in identifying the epileptogenic zone and in guiding the location and extent of epilepsy surgery in children with tuberous sclerosis complex and multifocal abnormalities. In children with tuberous sclerosis complex and intractable epilepsy, complete resection of the SISCOM hyperperfusion abnormality is associated with freedom from seizures.
在结节性硬化症患者中识别单一的致痫灶是一项挑战。非侵入性成像方式，包括与磁共振成像配准的发作期单光子发射计算机断层扫描（SISCOM），已被用于确定手术切除的优势致痫灶。我们评估了 6 例结节性硬化症相关癫痫患者中，完全切除 SISCOM 高灌注异常是否与无癫痫发作结局相关。癫痫发作的中位起始年龄为 4 个月（范围 1 天至 16 个月）。手术年龄从 8 个月到 13 岁不等。5 例多结节患者中确定了一个优势 SISCOM 高灌注灶。SISCOM 为 3 例非局灶性或电生理和神经影像学结果不一致的患者提供了额外的癫痫手术定位信息。在至少 2 年的随访中，3 例患者总体无癫痫发作。2 例患者完全切除 SISCOM 异常与无癫痫发作相关。这些发现表明，SISCOM 可用于识别致痫区，并指导结节性硬化症和多灶性病变儿童的癫痫手术的位置和范围。在结节性硬化症和耐药性癫痫儿童中，完全切除 SISCOM 高灌注异常与无癫痫发作相关。
