Kalashnikova L A, Sakharova A V, Dobrynina L A, Mir-Kasimov M F, Chaĭkovskaia R P, Konovalov R N, Protskiĭ S V, Shabalina A A, Kostyreva M V
Zh Nevrol Psikhiatr Im S S Korsakova. 2010;110(4 Suppl 2):3-11.
The vascular wall weakness caused by dysplastic alterations predisposes to the spontaneous dissection of cerebral arteries. The authors hypothesized for the first time that dysplasia might be the result of mitochondrial cytopathy. To test this hypothesis, the muscle biopsy was conducted in 3 male patients, aged 30-38 years, with the spontaneous dissection of the internal carotid (2) and posterior cerebral (1) arteries. Clinically dissections manifested by ischemic stroke (2) or the peripheral paresis of the hypoglossal nerve (1). The morphological study of fresh frozen sections of muscle by modified Gomori trichrome method revealed ragged-red fibers The histochemical study showed the severe decrease of the stain on succinate dehydrogenase and cytochrome-c-oxidase as well as the focal intensive staining of peripheral regions of muscle fibers. The complex of found changes is characteristic for a mitochondrial pathology. No patients had A3243G tRNA gene mutation, the most common mutation for MELAS. The serum lactate level was elevated only in one patient. We suggest that the mitochondrial disorder occurs not only in muscle, but also in cerebral artery wall--mitochondrial arteriopathy, which predisposes to spontaneous cerebral artery dissection.
发育异常改变导致的血管壁薄弱易引发脑动脉自发性夹层形成。作者首次提出假说，认为发育异常可能是线粒体细胞病的结果。为验证这一假说，对3例年龄在30 - 38岁的男性患者进行了肌肉活检，这些患者分别出现了颈内动脉（2例）和大脑后动脉（1例）的自发性夹层形成。临床上，夹层形成表现为缺血性卒中（2例）或舌下神经周围性麻痹（1例）。采用改良Gomori三色法对肌肉新鲜冰冻切片进行形态学研究，发现了破碎红纤维。组织化学研究显示琥珀酸脱氢酶和细胞色素c氧化酶染色严重减少，以及肌纤维周边区域的局灶性强染色。所发现的这些变化组合是线粒体病理学的特征。患者均无A3243G tRNA基因突变，这是线粒体脑肌病伴乳酸血症和卒中样发作（MELAS）最常见的突变。仅1例患者血清乳酸水平升高。我们认为线粒体功能障碍不仅发生在肌肉中，也发生在脑动脉壁——线粒体动脉病，这易导致自发性脑动脉夹层形成。
