Aida N, Tamagawa K, Takada K, Yagishita A, Kobayashi N, Chikumaru K, Iwamoto H
Department of Radiology, Kanagawa Children's Medical Center, Yokohama, Japan.
AJNR Am J Neuroradiol. 1996 Apr;17(4):605-13.
To determine the MR characteristics of brain abnormalities in Fukuyama congenital muscular dystrophy (FCMD).
We reviewed 30 MR examinations of 21 patients with FCMD to assess cerebral and cerebellar cortical dysplasia, white matter changes, and miscellaneous abnormalities.
On MR images, all patients had thick and bumpy cortices with shallow sulci corresponding to polymicrogyria, and 12 patients had pachygyric cortices with smooth surfaces, corresponding to type II lissencephaly. Both types of cortical dysplasia had characteristic distributions: the first type involved the frontal lobe in all 21 patients and also the parietotemporal lobe in 6 patients; the second type involved the temporooccipital lobes. Eighteen patients had prolonged T1 and T2 signal in the white matter, which was indistinct in neonates and seen infrequently in adolescents. In four patients, abnormal vessels were seen within the pachygyric cortices.
MR studies of the brain show findings consistent with the known characteristics of FCMD. The MR detection of the two types of cerebral cortical dysplasia with characteristic distribution and cerebellar abnormalities is helpful in the differential and early diagnosis of FCMD.
确定福山型先天性肌营养不良（FCMD）脑异常的磁共振成像（MR）特征。
我们回顾了21例FCMD患者的30次MR检查，以评估大脑和小脑皮质发育异常、白质改变及其他异常情况。
在MR图像上，所有患者均有增厚且凹凸不平的皮质，脑沟变浅，符合多小脑回畸形，12例患者有表面光滑的巨脑回皮质，符合II型无脑回畸形。两种类型的皮质发育异常均有特征性分布：第一种类型在所有21例患者中均累及额叶，6例患者还累及顶颞叶；第二种类型累及颞枕叶。18例患者白质T1和T2信号延长，在新生儿中不明显，在青少年中少见。4例患者在巨脑回皮质内可见异常血管。
脑的MR研究显示的结果与FCMD的已知特征一致。MR检测具有特征性分布的两种类型的大脑皮质发育异常及小脑异常有助于FCMD的鉴别诊断和早期诊断。
