Namba Y, Maegaki Y, Maeoka Y, Yoshimura M, Houdou S, Ishii S, Ohtani K, Takeshita K
Department of Pediatrics, Matsue Red Cross Hospital, Shimane.
No To Hattatsu. 1995 Sep;27(5):376-81.
Seven patients with Fukuyama type congenital muscular dystrophy were studied. Low density areas (LDs) in the cerebral white matter on cranial CT were present in all 4 patients younger than 13 years of age and in 1 of 3 adult patients. LDs corresponded to low signals on T1 weighted MRI image and high signals on T2 weighted MRI image. The follow-up MRI showed a decreased tendency of the abnormal signals in 2 patients. Short somatosensory evoked potentials (SSEPs) in two infants, aged 4 months and 8 months, showed absent or depressed N1 amplitudes and delayed interpeak latencies from P3 to N1. N1 amplitudes increased on follow-up studies. SSEPs of five patients, who were older than 2 years of age, showed normal N1-P3 latencies. Amplitude of N1 was low in 2 patients with LD. Since the absent or depressed amplitude and delayed latency of N1 improved with the decrease of abnormal signals on MRI, we considered that N1 abnormalities show delayed myelination.
对7例福山型先天性肌营养不良患者进行了研究。在所有4例13岁以下的患者以及3例成年患者中的1例中，头颅CT显示脑白质存在低密度区（LDs）。LDs在T1加权MRI图像上对应低信号，在T2加权MRI图像上对应高信号。随访MRI显示2例患者的异常信号有降低趋势。2例年龄分别为4个月和8个月的婴儿的短体感诱发电位（SSEPs）显示N1波幅缺失或降低，P3至N1的峰间期延迟。随访研究中N1波幅增加。5例年龄大于2岁患者的SSEPs显示N1 - P3潜伏期正常。2例有LD的患者N1波幅较低。由于随着MRI上异常信号的减少，N1波幅缺失或降低以及潜伏期延迟有所改善，我们认为N1异常表现为髓鞘形成延迟。
