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伴有线粒体脑肌病伴乳酸血症和卒中样发作(MELAS)的卒中样发作早期多支脑动脉血管扩张

Vasodilatation of multiple cerebral arteries in early stage of stroke-like episode with MELAS.

作者信息

Minobe Shoko, Matsuda Akiko, Mitsuhashi Tetsuya, Ishikawa Motonao, Nishimura Yoshiko, Shibata Koichi, Ito Eiichi, Goto Yu-ichi, Nakaoka Takashi, Sakura Hiroshi

机构信息

Department of Internal Medicine, Tokyo Woman's Medical University Medical Center East, 2-1-10 Nishiogu, Arakawa, Tokyo 116-8567, Japan.

Department of Internal Medicine, Tokyo Woman's Medical University Medical Center East, 2-1-10 Nishiogu, Arakawa, Tokyo 116-8567, Japan.

出版信息

J Clin Neurosci. 2015 Feb;22(2):407-8. doi: 10.1016/j.jocn.2014.05.021. Epub 2014 Aug 12.

DOI:10.1016/j.jocn.2014.05.021
PMID:25128282
Abstract

We describe a patient with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), with multiple cerebral vasodilatations in a stroke-like episode visualised by using magnetic resonance angiography (MRA) and CT angiography (CTA). In the acute stroke-like episode stage, T2-weighted and fluid-attenuated inversion recovery MRI showed high-intensity areas in the left occipital area. In addition, MRA and CTA revealed prominent dilatation of the left posterior cerebral artery and temporal branches of the middle cerebral artery with focal hyperperfusions using CT perfusion (CTP) that corresponded to the MRI. After 10 days, with the development of aphasia, MRI indicated the lesions had spread to the temporal and parietal regions, and this distribution was not confined to major vascular territories. The patient's symptoms gradually improved, accompanied by the attenuation of MRI, CTA, and CTP findings. These characteristic features along with the MRI changes that spread beyond vascular boundaries and the multiple cerebral vasodilatations prior to the development of clinical symptoms are not fully explained by the mitochondrial angiopathy or cytopathy theories. These findings provide further evidence supporting neuronal hyperexcitability in stroke-like episodes of MELAS.

摘要

我们描述了一名患有线粒体肌病、脑病、乳酸酸中毒和卒中样发作(MELAS)的患者,在一次卒中样发作中通过磁共振血管造影(MRA)和CT血管造影(CTA)观察到多处脑血管扩张。在急性卒中样发作阶段,T2加权和液体衰减反转恢复序列MRI显示左侧枕叶区域有高强度区域。此外,MRA和CTA显示左侧大脑后动脉及大脑中动脉颞支显著扩张,使用CT灌注(CTP)显示对应于MRI的局灶性高灌注。10天后,随着失语症的出现,MRI显示病变已扩散至颞叶和顶叶区域,且这种分布并不局限于主要血管区域。患者症状逐渐改善,同时MRI、CTA和CTP表现也逐渐减轻。这些特征以及超出血管边界的MRI变化和临床症状出现之前的多处脑血管扩张,线粒体血管病或细胞病理论无法完全解释。这些发现为支持MELAS卒中样发作中神经元过度兴奋提供了进一步证据。

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