难治性癫痫相关脑血管畸形15例临床病理分析

Clinicopathologic features of patients with intractable epilepsy associated with cerebrovascular malformation:an analysis of 15 cases

目的分析并比较因脑血管畸形导致难治性癫痫从而接受癫痫外科手术的15例患者的临床及病理学特点。方法回顾性分析接受脑病灶及致痫灶切除手术的15例脑血管畸形患者的临床资料及病理资料。结果 15例患者均表现为难治性癫痫,其中包括8例海绵状血管瘤,7例Sturge-Weber综合征（SWS）。男性8例,女性7例,发病年龄1月龄~29岁,病程2~24年。手术年龄2~31岁。2例行单脑叶切除,12例行多脑叶切除,1例行功能性大脑半球切除。脑标本镜下可见海绵状血管瘤并局灶性皮质发育不良（FCD）Ⅲc型;软脑膜血管瘤病、灰质中弥漫钙化,脑实质内小血管壁钙化,并伴发局灶性皮质发育不良（FCD）Ⅲc型。术后随访0.5~8年,EngelⅠ级11例,EngelⅡ级2例,EngelⅢ级1例,EngelⅣ级1例。结论脑海绵状血管瘤患者接受癫痫外科手术后随访效果良好;SWS患者颅内病变程度可能与遗传学相关,SWSⅠ型患儿应尽早手术,术后效果良好;SWSⅢ型患者接受血管瘤病病灶及致痫灶切除术后,随访效果良好。

Objective To investigate the clinicopathologic features of 15 patients with refractory epilepsy induced by cerebrovascular malformation undergoing surgical operation for epilepsy. Methods A retrospective analysis was performed for the clinical and pathological data of 15 patients with cerebrovascular malformation who underwent the resection of brain lesions and epileptic lesions. Results All the 15 patients had refractory epilepsy; among them,8 had cavernous angioma and 7 had Sturge-Weber syndrome（ SWS）. There were 8 male and 7 female patients,with an age of onset of 1 month to 29 years and a course of the disease of 2- 24 years. The age when they underwent the surgery ranged 2- 31 years. Of all patients,2 underwent single lobe resection,12 underwent multilobar resection,and 1 underwent functional hemispherectomy. The microscopic evaluation of brain specimens showed cavernous angioma complicated by focal cortical dysplasia（ FCD） type IIIc,as well as hemangioma in pia mater,diffuse calcification in gray matter,calcification of small vessel wall in brain parenchyma,and FCD type IIIc. The patients were followed up for 0.5- 8 years after surgery,and of all patients,12 had Engel grade I,2 had Engel grade II,1 had Engel grade III,and 1 had Engel grade IV. Conclusions The patients with cavernous angioma have a good outcome during follow-up after surgical operation for epilepsy. In patients with SWS,the degree of intracranial lesions may be associated with genetics. The patients with SWS type I should undergo operation as soon as possible so as to achieve a good outcome during follow-up,and those with SWS type III should undergo resection of hemangioma lesions and epileptic lesions to achieve a good outcome.