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首页> 外文期刊>Neuromuscular disorders: NMD >Decreased cerebral perfusion in Duchenne muscular dystrophy patients
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Decreased cerebral perfusion in Duchenne muscular dystrophy patients

机译：杜氏肌营养不良症患者的脑灌注减少

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Duchenne muscular dystrophy is caused by dystrophin gene mutations which lead to the absence of the protein dystrophin. A significant proportion of patients suffer from learning and behavioural disabilities, in addition to muscle weakness. We have previously shown that these patients have a smaller total brain and grey matter volume, and altered white matter microstructure compared to healthy controls. Patients with more distal gene mutations, predicted to affect dystrophin isoforms Dp140 and Dp427, showed greater grey matter reduction. Now, we studied if cerebral blood flow in Duchenne muscular dystrophy patients is altered, since cerebral expression of dystrophin also occurs in vascular endothelial cells and astrocytes associated with cerebral vasculature. T1-weighted anatomical and pseudo-continuous arterial spin labeling cerebral blood flow images were obtained from 26 patients and 19 age-matched controls (ages 8-18 years) on a 3 tesla MRI scanner. Group comparisons of cerebral blood flow were made with and without correcting for grey matter volume using partial volume correction. Results showed that patients had a lower cerebral blood flow than controls (40.0 +/- 6.4 and 47.8 +/- 6.3 mL/100 g/min respectively, p = 0.0002). This reduction was independent of grey matter volume, suggesting that they are two different aspects of the pathophysiology. Cerebral blood flow was lowest in patients lacking Dp140. There was no difference in CBF between ambulant and non-ambulant patients. Only three patients showed a reduced left ventricular ejection fraction. No correlation between cerebral blood flow and age was found. Our results indicate that cerebral perfusion is reduced in Duchenne muscular dystrophy patients independent of the reduced grey matter volume.
机译：杜氏肌营养不良症是由抗肌萎缩蛋白基因突变引起的，导致抗肌萎缩蛋白缺失。除了肌肉无力外，很大一部分患者还患有学习和行为障碍。我们之前已经表明，与健康对照组相比，这些患者的总脑和灰质体积较小，白质微观结构也发生了变化。具有更多远端基因突变的患者，预计会影响抗肌萎缩蛋白亚型 Dp140 和 Dp427，显示出更大的灰质减少。现在，我们研究了杜氏肌营养不良症患者的脑血流是否改变，因为抗肌萎缩蛋白的脑表达也发生在与脑脉管系统相关的血管内皮细胞和星形胶质细胞中。在 3 特斯拉 MRI 扫描仪上从 26 名患者和 19 名年龄匹配的对照组（8-18 岁）那里获得了 T1 加权解剖学和伪连续动脉自旋标记脑血流图像。使用部分体积校正对灰质体积进行和不使用脑血流量校正的脑血流量的组比较。结果显示，患者的脑血流量低于对照组（分别为 40.0 +/- 6.4 和 47.8 +/- 6.3 mL/100 g/min，p = 0.0002）。这种减少与灰质体积无关，表明它们是病理生理学的两个不同方面。缺乏 Dp140 的患者脑血流量最低。卧床和非卧床患者的CBF无差异。只有 3 例患者左心室射血分数降低。未发现脑血流量与年龄之间的相关性。我们的结果表明，杜氏肌营养不良症患者的脑灌注减少与灰质体积减少无关。

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