Bautista-Lacambra Mario, Garayoa-Irigoyen Vanesa, Tique-Rojas Luisa-Fernanda, Seral-Moral María, Moles-Herbera Jesús, López-Lafuente Amparo, Barrena-Caballo Rosario, Tejero-Juste Carlos, Palacín-Larroy Marta, Tejada-Meza Herbert
Servicio de Neurología, Hospital Universitario Miguel Servet, 50009 Zaragoza, España.
Grupo de Investigación en Neurociencias, Instituto de Investigación Sanitaria (IIS) Aragón, 50009 Zaragoza, España.
Rev Neurol. 2025 Oct 30;80(9):37504. doi: 10.31083/RN37504.
Moyamoya angiopathy is a cerebrovascular disease characterized by progressive stenosis of the intracranial internal carotid arteries. There is limited literature addressing epilepsy in this condition, especially in Western countries.
This was a retrospective study conducted in the public hospitals of Aragón, analyzing data from all patients diagnosed with moyamoya angiopathy between 1981 and 2024. Epidemiological aspects of the disease were studied, as well as the presence of epilepsy and its management in this group of patients.
A total of 26 patients were included, with an estimated prevalence in Aragón of 1.71 cases per 100,000 inhabitants. The mean age at diagnosis was 36.64 years, with an equal sex distribution. Half of the patients presented with moyamoya syndrome. Fifty percent of the patients experienced a seizure and 42.31% of the total met diagnostic criteria for epilepsy. Most seizures were focal (81.8%), with a predominance of frontal lobe semiology. Levetiracetam was the most commonly used treatment. Up to four of the twelve patients with epilepsy met diagnostic criteria for drug-resistant epilepsy.
Although the prevalence of moyamoya in our series was lower than in Asian populations, the prevalence of epilepsy was significantly higher (50% of patients with seizures vs 0.9-18.9% in Asian series). In our cohort, epilepsy in moyamoya angiopathy was associated with the occurrence of syncope, cognitive impairment, affective disorders, and an earlier age at diagnosis. There are no other studies available addressing the percentage of drug-resistant epilepsy in these patients.
烟雾病是一种以颅内颈内动脉进行性狭窄为特征的脑血管疾病。关于这种疾病中癫痫的文献有限,尤其是在西方国家。
这是一项在阿拉贡公立医院进行的回顾性研究,分析了1981年至2024年间所有诊断为烟雾病的患者的数据。研究了该疾病的流行病学方面,以及这组患者中癫痫的存在情况及其管理。
共纳入26例患者,阿拉贡的估计患病率为每10万居民中有1.71例。诊断时的平均年龄为36.64岁,性别分布均衡。一半的患者表现为烟雾病综合征。50%的患者发生过癫痫发作,占总数42.31%的患者符合癫痫诊断标准。大多数癫痫发作是局灶性的(81.8%),以额叶症状学为主。左乙拉西坦是最常用的治疗药物。在12例癫痫患者中,多达4例符合耐药性癫痫的诊断标准。
尽管我们系列中烟雾病的患病率低于亚洲人群,但癫痫的患病率显著更高(癫痫发作患者中占50%,而亚洲系列中为0.9 - 18.9%)。在我们的队列中,烟雾病血管病中的癫痫与晕厥、认知障碍、情感障碍以及诊断时年龄较早有关。目前没有其他研究涉及这些患者中耐药性癫痫的比例。