线粒体脑病学习教案

1、会计学1第一页，共18页。第1页/共18页第二页，共18页。n定义n由于遗传缺损引起线粒体代谢缺陷，导致ATP合成障碍，能量产生(chnshng)不足而出现的一组多系统疾病。n分类第2页/共18页第三页，共18页。第3页/共18页第四页，共18页。第4页/共18页第五页，共18页。第5页/共18页第六页，共18页。第6页/共18页第七页，共18页。第7页/共18页第八页，共18页。1.2660.8261.172 0.765第8页/共18页第九页，共18页。1.0820.8310.851第9页/共18页第十页，共18页。第10页/共18页第十一页，共18页。第11页/共18页第十二页，共18页。

2、nFig. 1 MRI exams were realized at admission (D0), at 15 days (D15) of evolution, and for control 6 (M6) and 12 months later. Conventional FLAIR and DWI data arenrepresented in Fig. 1. FLAIR and DWI sequences are represented at two levels; the first 2 left columns corresponding to a view at the temp

3、oral level, and the 2 right columnsnto the occipital level. Rows represent successively MRI exams realized at D0, D15, and M6 (MRIs at M12 were not represented as they were similar to images obtained 6nmonths earlier).nAt admission, recent left temporal lesion appeared with a hyper intensity on FLAI

4、R sequence (1a), and ADCs were heterogeneous; elevated in anterior localization, andndiminished in posterior region (1b). There were no signal abnormalities on FLAIR or DWI views in the occipital regions (2a and 2b).nAt D15, bilateral occipital FLAIR hyperintensities appeared (2c). ADCs increased in

5、 these regions (2d), and became homogeneously elevated in the left temporal lesionn(1d).nAt M6, FLAIR hyperintensities diminished in the temporal lesion, replaced with gliosis (1e), and disappeared in the occipital region (2e). Lesion regression was more markednin those regions of the temporal lobe

6、in which ADCs were previously the most elevated (white arrow). FLAIR abnormalities disappeared completely in occipital regions (2e),nand ADCs reached normal values (2f).第12页/共18页第十三页，共18页。na mild energy failure resulting in moderate cellular dysfunction, responsible for vasogenic edema (high ADCs)na severe energy failure resulting in an irreversible cellular failure, with cytotoxic edema (low ADCs).第13页/共18页第十四页，共18页。第14页/共18页第十五页，共18页。第15页/共18页第十六页，共18页。n(a) MRA o