Aida N
Department of Radiology, Kanagawa Children's Medical Center, Yokohama, Japan.
J Magn Reson Imaging. 1998 Mar-Apr;8(2):317-26. doi: 10.1002/jmri.1880080211.
We reviewed neuroradiologic findings of Fukuyama congenital muscular dystrophy (FCMD) and correlated them with the known neuropathology. All patients showed thick and bumpy cortices with shallow sulci corresponding to polymicrogyria, and approximately half of the patients showed pachygyric cortex with smooth surface corresponding to type II lissencephaly. The two types of cortical dysplasias presented characteristic distributions: the former demonstrated frontal lobe involvement in all and parietotemporal lobe involvement in some, whereas the latter involved the temporo-occipital lobes. Most patients showed prolonged T1 and T2 signal in the white matter, which was indistinct in neonates and infrequently seen in adolescents. Cerebellar polymicrogyria depicted as disorganized cerebellar foliation accompanying cysts were found more than 90% of the patients. In conclusion, brain MRI demonstrates findings consistent with the known neuropathology of FCMD. The detection of the two types of cerebral cortical dysplasia with characteristic distribution and cerebellar abnormalities is helpful in the differential and early diagnosis.
我们回顾了福山型先天性肌营养不良（FCMD）的神经放射学表现，并将其与已知的神经病理学进行关联。所有患者均显示皮质增厚且凹凸不平，脑沟变浅，符合多小脑回畸形，约一半患者显示皮质表面光滑的巨脑回，符合II型无脑回畸形。这两种类型的皮质发育异常呈现出特征性分布：前者在所有患者中均累及额叶，部分患者累及顶颞叶，而后者累及颞枕叶。大多数患者白质T1和T2信号延长，在新生儿中不明显，在青少年中少见。超过90%的患者发现小脑多小脑回畸形表现为小脑叶排列紊乱并伴有囊肿。总之，脑部MRI显示的结果与FCMD已知的神经病理学一致。检测具有特征性分布的两种类型的大脑皮质发育异常和小脑异常有助于鉴别诊断和早期诊断。
