Epilepsy surgery in a patient with Lennox-Gastaut syndrome and cortical dysplasia

Epilepsy surgery in a patient with Lennox-Gastaut syndrome and cortical dysplasia
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DOI:
10.1016/j.braindev.2006.07.013
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发表时间:
2007-04-01
影响因子:
1.7
通讯作者:
Ko, Tae-Sung
Ko, Tae-Sung
中科院分区:
医学4区
文献类型:
--
作者:
You, Su Jeong;Lee, Jung-Kyo;Ko, Tae-Sung

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Lennox-Gastaut综合征(LGS)是一种全身性癫痫,抗癫痫药物治疗无效。虽然胼胝体切开术有时可以控制跌倒发作,但治疗性癫痫手术在LGS中很少可能。我们报告,在这里,LGS和局灶性皮质发育不良的患者,谁成为癫痫手术后无癫痫。患者是一名24个月大的男孩,无围产期损伤,出生后7天开始癫痫发作,并出现发育迟缓。脑磁共振成像(MRI)显示右额叶区局灶性皮质病变伴钙化。在13个月大时,他的癫痫发作类型从强直性癫痫发作变为头下垂(失张力型)和非典型失神性癫痫发作。他的发作间期脑电图(EEG)显示广泛的缓慢棘波和波放电,他被诊断为LGS。他的癫痫发作是难以治疗的,生酮饮食无效。在手术前通过长期视频EEG监测对患者进行了评价,监测发现许多癫痫发作包括突然发作的跌倒发作(失张力型),间歇性,随后为非典型失神发作,伴弥漫性慢波爆发,随后为发作性EEG、脑正电子发射断层扫描(PET)和MRI中的慢棘波和波放电。进行了右额叶病变切除术和术中皮质电图(EcoG)。从病灶切除术到现在,患者一直没有癫痫发作,并一直正常发展。我们的研究结果表明,局部切除手术应考虑与LGS和局灶性癫痫病变的患者。(c)2006 Elsevier B. V.保留所有权利。
Lennox-Gastaut syndrome (LGS) is classified as a generalized epilepsy, and is often intractable to antiepileptic drugs. Although corpus callosotomy may sometimes control drop attacks, curative epilepsy surgery is rarely possible in LGS. We report, here, a patient with LGS and focal cortical dysplasia, who became seizure-free after epilepsy surgery. The patient was a 24-month-old boy without perinatal insult in whom seizures began 7 days after birth and who experienced development delay. Brain magnetic resonance imaging (MRI) showed focal cortical lesion with calcification in the right frontal area. At age 13 months, his seizure type changed from tonic seizures to head drops (atonic types) and atypical absence seizures. His interictal electroencephalogram (EEG) showed generalized slow spike and wave discharges, and he was diagnosed with LGS. His seizures were intractable to medical treatment, and a ketogenic diet was not effective. He was evaluated prior to surgery by long-term video-EEG monitoring, which detected many seizures consisted of a sudden onset of falling attacks (atonic type) intermittently followed by atypical absence seizures with diffuse slow wave bursts followed by slow spike and wave discharges in ictal EEG, brain positron emission tomography (PET) and MRI. A right frontal lesionectomy with intra-operative electrocorticography (EcoG) was performed. From the time of lesionectomy to the present, the patient has been seizure free and has been developing normally. Our results suggest that focal resective surgery should be considered in patients with LGS and focal epileptic lesions. (c) 2006 Elsevier B.V. All rights reserved.