Dilber B, Sahin S, Eyüboğlu I, Kamaşak T, Acar Arslan E, Durgut B Diler, Cansu A
Karadeniz Technical University, Department of Pediatric Neurology, Trabzon, Turkey.
Karadeniz Technical University, Department of Pediatric Neurology, Trabzon, Turkey.
J Stroke Cerebrovasc Dis. 2020 Mar;29(3):104600. doi: 10.1016/j.jstrokecerebrovasdis.2019.104600. Epub 2020 Jan 6.
Dyke-Davidoff-Masson syndrome (DDMS) was first described in 1933 as a clinical condition characterized by hemiatrophy, hyperpneumatization of paranasal sinuses, contralateral hemiparesis, facial asymmetry, seizures, and mental retardation. DDMS can be of 2 types: congenital and acquired. The congenital type can be caused by various conditions experienced during fetal or early childhood development, including ischemia, infarction, trauma, infections, and hemorrhage. The acquired type is mostly associated with hemorrhage, trauma, and infections experienced after 1 month of age. DDMS can manifest alone or can be accompanied by crossed cerebellar atrophy (CCA) which is a newly discovered radiological marker characterized by prominent cortical sulci and loss of cerebellar parenchyma. The congenital type of DDMS is known to be accompanied by ipsilateral cerebellar atrophy and the acquired type is known to be accompanied by contralateral cerebellar atrophy. Supratentorial events may lead to destruction in the cortico-ponto-cerebellar pathways, mostly in the contralateral side of the body (80%) due to decussation. In this report, we present 2 cases of DDMS accompanied by CCA to emphasize the possibility that the DDMS cases with severe intrauterine hemorrhage can be accompanied by contralateral CCA and migratory abnormalities rather than ipsilateral CCA and clinical survey.
戴克-戴维多夫-马森综合征（DDMS）于1933年首次被描述，是一种以半侧萎缩、鼻窦气化过度、对侧偏瘫、面部不对称、癫痫发作和智力发育迟缓为特征的临床病症。DDMS可分为两种类型：先天性和后天性。先天性类型可由胎儿期或幼儿期发育过程中经历的各种情况引起，包括缺血、梗死、创伤、感染和出血。后天性类型主要与1个月龄后经历的出血、创伤和感染有关。DDMS可单独出现，也可伴有交叉性小脑萎缩（CCA），这是一种新发现的影像学标志物，其特征为明显的皮质沟和小脑实质丧失。已知先天性DDMS伴有同侧小脑萎缩，后天性DDMS伴有对侧小脑萎缩。幕上病变可能导致皮质-脑桥-小脑通路受损，由于交叉，大多发生在身体的对侧（80%）。在本报告中，我们展示了2例伴有CCA的DDMS病例，以强调严重宫内出血的DDMS病例可能伴有对侧CCA和迁移异常，而不是同侧CCA和临床检查的可能性。
