Castaigne P, Lhermitte F, Escourolle R, Chain F, Fardeau M, Hauw J J, Curet J, Flavigny C
Rev Neurol (Paris). 1977 Jun-Jul;133(6-7):369-86.
This study deals with a case of "ophtalmoplegia plus" with histochemistry and electron microscopy of a muscle biopsy and full pathological examination in a 29 year old woman. Ragged-red fibers with abnormal mitochondria, cerebral spongiosis mostly involving white matter, perimacular pigmentary retinopathy and scattered myocardial fibrosis interrupting the Hiss'bundle were found. A goiter with hypersecretion of thyroid hormones and thyroid and ocular muscles changes characteristics of Graves' disease without any hypermetabolism occurred in her late twenties. This could be related to the absence of response from the abnormal mitochondria to the thyroid hormones.
本研究涉及一名29岁女性“眼肌麻痹叠加症”病例,对其进行了肌肉活检的组织化学和电子显微镜检查以及全面的病理检查。发现有异常线粒体的破碎红纤维、主要累及白质的脑海绵状变性、黄斑周围色素性视网膜病变以及中断希氏束的散在心肌纤维化。她在二十多岁后期出现甲状腺激素分泌过多的甲状腺肿以及甲状腺和眼肌呈现格雷夫斯病的特征性改变,但无任何代谢亢进情况。这可能与异常线粒体对甲状腺激素无反应有关。