Viteva Ekaterina, Vasilev Petar, Vasilev Georgi, Chompalov Kostadin
Department of Neurology, Faculty of Medicine, Medical University of Plovdiv, 4000 Plovdiv, Bulgaria.
Clinic of Neurology, UMHAT "Sv. Georgi", 4000 Plovdiv, Bulgaria.
Neurol Int. 2024 Aug 20;16(4):869-879. doi: 10.3390/neurolint16040065.
Moyamoya disease is a cerebrovascular pathology characterized by progressive stenosis of the internal carotid arteries and their branches, leading to ischemic and/or hemorrhagic disorders of the cerebral circulation, primarily affecting children and young adults. We present a case of a 23-year-old woman with a history of recurrent cerebrovascular accidents since childhood. Despite experiencing focal motor seizures and transient ischemic attacks, her condition remained undiagnosed until 2006, when, at the age of 7, a digital subtraction angiography revealed characteristic bilateral internal carotid artery occlusions. Subsequent diagnostic challenges and treatments preceded a worsening of symptoms in adulthood, including generalized tonic-clonic seizures. Upon presentation to our clinic, the patient exhibited upper motor neuron syndrome and occipital lobe syndrome, consistent with the disease's pathophysiology, neuroimaging, and clinical manifestations. Imaging studies confirmed multiple ischemic lesions throughout the cerebral vasculature. Treatment adjustments were made due to the increased incidence of seizures, and the dose of her anti-seizure medication-divalproex sodium-was increased. This case underscores the diagnostic complexities and challenges in managing moyamoya disease, emphasizing the importance of early recognition and prompt intervention.
烟雾病是一种脑血管病变，其特征为颈内动脉及其分支进行性狭窄，导致脑循环的缺血性和/或出血性疾病，主要影响儿童和年轻人。我们报告一例23岁女性病例，该患者自童年起就有复发性脑血管意外病史。尽管经历了局灶性运动性癫痫发作和短暂性脑缺血发作，但她的病情直到2006年才被诊断出来，当时她7岁，数字减影血管造影显示双侧颈内动脉有特征性闭塞。随后在成年期症状恶化之前出现了诊断难题和治疗情况，包括全身性强直阵挛发作。在到我们诊所就诊时，患者表现出上运动神经元综合征和枕叶综合征，这与该疾病的病理生理学、神经影像学和临床表现相符。影像学研究证实整个脑血管系统存在多处缺血性病变。由于癫痫发作发生率增加，对治疗进行了调整，增加了她的抗癫痫药物丙戊酸钠的剂量。该病例强调了烟雾病诊断的复杂性和管理挑战，凸显了早期识别和及时干预的重要性。
