Kivelev Juri, Niemelä Mika, Kivisaari Riku, Dashti Reza, Laakso Aki, Hernesniemi Juha
Department of Neurosurgery, Helsinki University Central Hospital, Helsinki, Finland.
Neurosurgery. 2009 Sep;65(3):450-5; discussion 455. doi: 10.1227/01.NEU.0000346269.59554.DB.
Multiple cerebral cavernous malformations (MCCMs) typically occur in patients with a family history of these lesions. Literature on MCCMs is scarce, and little is known about their natural history.
Of 264 consecutive patients with cerebral cavernomas treated at the Department of Neurosurgery, Helsinki University Central Hospital, in the past 27 years, 33 patients had MCCMs. Lesions were categorized according to the Zabramski classification scale. Follow-up questionnaires were sent to all patients. Outcome was assessed using the Glasgow Outcome Scale, and amelioration of epilepsy was assessed using the Engel scale. All clinical data were analyzed retrospectively.
The mean age of patients at diagnosis was 44 years. Sex presentation was almost equal. Nine percent of all patients had a family history of the disease. Patients presented with epilepsy, acute headache, and focal neurological deficits. MCCMs were incidental findings in 2 patients. Altogether, 416 cavernomas were found: 70% supratentorial and 30% infratentorial. Fifteen patients had symptomatic hemorrhage before admission to our department. Surgery was performed on 18 patients. In most cases, the largest cavernoma was removed. Postoperatively, 1 patient experienced temporary hemiparesis, and another developed permanent motor dysphasia. No mortalities occurred. The mean follow-up time was 7.7 years. Twenty-six patients (79%) were in good condition. Among patients with epilepsy who underwent lesionectomy, 70% had an Engel class I outcome. On follow-up magnetic resonance imaging, 52 de novo cavernomas were found.
Surgical treatment of patients with MCCMs is safe. An extirpation of the clinically active cavernoma prevents further bleedings and improves outcome of epilepsy.
多发性脑海绵状血管畸形（MCCM）通常发生在有这些病变家族史的患者中。关于MCCM的文献稀少，对其自然病史了解甚少。
在过去27年里，赫尔辛基大学中心医院神经外科连续治疗的264例脑海绵状血管瘤患者中，33例患有MCCM。病变根据Zabramski分类量表进行分类。向所有患者发送随访问卷。使用格拉斯哥预后量表评估结果，使用恩格尔量表评估癫痫的改善情况。所有临床数据均进行回顾性分析。
患者诊断时的平均年龄为44岁。性别分布几乎相等。所有患者中有9%有该病的家族史。患者表现为癫痫、急性头痛和局灶性神经功能缺损。2例患者的MCCM为偶然发现。总共发现416个海绵状血管瘤：70%位于幕上，30%位于幕下。15例患者在入院前有症状性出血。18例患者接受了手术。在大多数情况下，切除了最大的海绵状血管瘤。术后，1例患者出现暂时性偏瘫，另1例出现永久性运动性失语。无死亡病例。平均随访时间为7.7年。26例患者（79%）情况良好。在接受病变切除术的癫痫患者中，70%的患者恩格尔分级为I级。在随访磁共振成像中，发现了52个新生海绵状血管瘤。
MCCM患者的手术治疗是安全的。切除临床上活跃的海绵状血管瘤可防止进一步出血并改善癫痫的预后。
