破碎红纤维”(ragged-red fiber, RRF):即病理性线粒体聚积于肌膜下,是线粒体肌(脑)病的诊断金标准。线粒体脑肌病伴乳酸酸中毒和卒中样发作(mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes,MELAS):常为多个脑叶联合受累,以颞枕或颞顶叶多见,病灶大,累及皮质下白质。神经元退行性变以脑干和小脑为主,前者好发部位为黑质、红核、前庭神经核及动眼神经核,后者常表现为Purkinje细胞的脱失而很 ......