Cortical Dysplasia in Congenital Muscular Dystrophy with Central Nervous System Involvement (Fukuyama Type)
Cortical Dysplasia in Congenital Muscular Dystrophy with Central Nervous System Involvement (Fukuyama Type)
复制标题
累及中枢神经系统的先天性肌营养不良症的皮质发育不良（福山型）
DOI：
10.1097/00005072-198407000-00005
复制
发表时间：
1984
影响因子：
3.2
通讯作者：
K. Takada
中科院分区：
文献类型：
--
作者：
K. Takada
我们报告五例先天性肌营养不良症与中枢神经系统受累的福山型（FCMD），大脑皮质发育不良是不均匀的，即使在同一个大脑。我们将发育不良分为三种主要类型，尽管个体差异，但每种类型都具有可预测的地形。小脑微多回症局限于每个半球的背侧。除一例病例外，所有病例均在小脑背表面的蛛网膜下腔发现了与小多脑回症密切相关的异常有髓神经纤维束。我们讨论FCMD的皮质发育不良的特点，特别是在有关的步行者的无脑，发病机制，以及中枢神经系统和骨骼肌病变之间的关系。
We report five cases of congenital muscular dystrophy with central nervous system involvement of the Fukuyama type (FCMD) in which cerebral cortical dysplasia was not uniform even in the same brain. We have categorized the dysplasia into three major patterns, each with a predictable topography despite individual variations. Cerebellar micropolygyria was localized to the dorsal halves of each hemisphere. Aberrant fascicles of myelinated nerve fibers, closely associated with micropolygyria, were found in the subarachnoid space of the dorsal cerebellar surface in all but one case. We discuss the characteristics of the cortical dysplasia of FCMD, particularly in relation to that of Walker's lissencephaly, pathogenesis, and the relationship between lesions of the central nervous system and skeletal muscle.