Wu Chengyuan, Sperling Michael R, Falowski Steven M, Chitale Ameet V, Werner-Wasik Maria, Evans James J, Andrews David W, Sharan Ashwini D
Thomas Jefferson University Hospitals, Department of Neurological Surgery, 909 Walnut Street, Third Floor, Philadelphia, PA, USA.
Thomas Jefferson University, Department of Neurology, Philadelphia, PA, USA.
Epilepsy Behav Case Rep. 2012 Nov 7;1:1-6. doi: 10.1016/j.ebcr.2012.10.004. eCollection 2013.
Periventricular heterotopia (PVH) is a neuronal migration disorder characterized by masses of gray matter located along the lateral ventricles that commonly cause epilepsy. The benefit of surgical resection of the PVH has been demonstrated in case reports to date; however, the location of the PVH in the paratrigonal region of the lateral ventricles can present significant surgical challenges. Noninvasive modalities of ablating this epileptogenic focus must therefore be considered. We present a small series of three patients who underwent stereotactic radiosurgery (SRS) for inoperable unilateral dominant hemisphere PVHs in order to illustrate the potential benefits and risks of this treatment modality. A total dose of 37.5-65 Gy resulted in seizure freedom for at least 14 months at the time of their last follow-up, even in patients harboring a second independent epileptic focus. Whether intracranial electrode recording truly offers added value is therefore uncertain. The two patients who received higher radiation doses suffered from symptomatic radiation necrosis and associated cerebral edema, requiring further medical intervention, and persistent monocular visual loss in one patient. While a longer interval prior to re-treatment may have been attempted, neither patient demonstrated radiographic findings typically associated with seizure remission. Refractory epilepsy due to PVH may be successfully treated with radiation therapy; but further work is needed to define the optimal dosing parameters in order to lower toxicity to normal tissue.
室周异位(PVH)是一种神经元迁移障碍疾病,其特征是沿侧脑室有大量灰质团块,通常会引发癫痫。迄今为止,病例报告已证实手术切除PVH的益处;然而,PVH位于侧脑室三角区旁的位置可能带来重大手术挑战。因此,必须考虑采用非侵入性方法来消除这个致痫灶。我们报告了一小系列3例患者,他们因无法手术的单侧优势半球PVH接受了立体定向放射外科治疗(SRS),以说明这种治疗方式的潜在益处和风险。总剂量37.5 - 65 Gy使患者在最后一次随访时至少14个月无癫痫发作,即使是患有第二个独立致痫灶的患者。因此,颅内电极记录是否真的能提供附加价值尚不确定。接受较高辐射剂量的2例患者出现了有症状的放射性坏死及相关脑水肿,需要进一步的医学干预,其中1例患者还持续存在单眼视力丧失。虽然可能尝试了更长的再治疗间隔时间,但这2例患者均未出现通常与癫痫缓解相关的影像学表现。由PVH引起的难治性癫痫可能通过放射治疗成功治疗;但需要进一步研究来确定最佳剂量参数,以降低对正常组织的毒性。