Periventricular nodular heterotopia: Epileptogenic findings

Periventricular nodular heterotopia: Epileptogenic findings
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DOI:
10.1111/j.1528-1157.1997.tb01213.x
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发表时间:
1997-11-01
期刊:
影响因子:
5.6
通讯作者:
Avanzini, G
Avanzini, G
中科院分区:
医学1区
文献类型:
--
作者:
Battaglia, G;Granata, T;Avanzini, G

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目的:为进一步研究室周结节性异位症(PNH)的电临床表现和癫痫发作的症状学特征,对17例PNH患者进行了磁共振成像(MRI)检查。仔细分析患者的临床和家族史,并随访10个月至22年的癫痫电临床特征和病程。结果:本组患者分为双侧PNH 7例,单侧PNH 10例。前者主要表现为大脑后窝的结构异常和多种癫痫发作类型;后者表现为异常的三叉神经旁位置,并且经常表现为视觉或听觉发作的原发性癫痫发作。局灶性癫痫多数耐药。发作间期脑电图异常总是局灶性的,与PNH的位置一致。一个以前未报告的光驱动的后方背景活动,观察在所有患者中,总是一致的PNH location.Conclusions:我们目前的研究结果和以前报道的数据表明,双边和单边PNH的情况下,是不同的形态和电临床特征,并可能由不同的病因。女性占优势,频繁的家族发生,并积极的癫痫家族史表明,遗传因素可能参与了双边和对称性PNH的成因,而产前危险因素的存在及其在分水岭旁三角区的位置表明,血管机制可能决定单侧PNH。
Purpose: We studied 17 patients with periventricular nodular heterotopia (PNH) to further investigate the electroclinical pictures and semiology of the associated seizures.Methods: PNH was diagnosed by means of magnetic resonance imaging (MRI). The patients' clinical and familial histories were carefully analyzed, and their electroclinical features and course of epilepsy followed for periods ranging from 10 months to 22 years. The electroclinical data were compared with those of previously reported PNH cases.Results: The patients were subdivided into those with bilateral (7) and unilateral (10) PNH. The former were mainly characterized by structural abnormalities in the posterior cerebral fossa and multiple seizure types; the latter were characterized by the paratrigonal location of the malformation and, frequently, by elementary seizures with a visual or auditory onset. Focal seizures were drug resistant in most cases. The interictal EEG abnormalities were always focal and consistent with the location of the PNH. A previously unreported photic driving of posterior background activity was observed in all patients and was always consistent with the PNH location.Conclusions: Our present findings and previously reported data show that bilateral and unilateral PNH cases are different in their morphological and electroclinical features and may be determined by different etiologies. The female predominance, frequent familial occurrence, and positive family history for epilepsy suggest that genetic factors may be involved in the genesis of bilateral and symmetrical PNH, whereas the presence of prenatal risk factors and its location in the watershed paratrigonal area suggest that vascular mechanisms may determine unilateral PNH.