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[一例“眼肌麻痹加征”病例的解剖学-临床研究,兼对肌肉、中枢神经、眼部、心肌及甲状腺病变的分析]

[Anatomo-clinical study of a case of "ophthalmoplegia plus" with analysis of muscular, central nervous, ocular, myocardial, and thyroid lesions].

作者信息

Castaigne P, Lhermitte F, Escourolle R, Chain F, Fardeau M, Hauw J J, Curet J, Flavigny C

出版信息

Rev Neurol (Paris). 1977 Jun-Jul;133(6-7):369-86.

PMID:929033
Abstract

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.

摘要

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。

相似文献

1
[Anatomo-clinical study of a case of "ophthalmoplegia plus" with analysis of muscular, central nervous, ocular, myocardial, and thyroid lesions].[一例“眼肌麻痹加征”病例的解剖学-临床研究,兼对肌肉、中枢神经、眼部、心肌及甲状腺病变的分析]
Rev Neurol (Paris). 1977 Jun-Jul;133(6-7):369-86.
2
Extraocular muscle biopsy in chronic progressive external ophthalmoplegia.慢性进行性眼外肌麻痹的眼外肌活检
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Neuropathy and mitochondrial myopathy.神经病变和线粒体肌病。
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Sporadic oculopharyngeal myopathy with abnormal mitochondria.伴有线粒体异常的散发性眼咽肌病
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[Ocular myopathy with mitochondrial abnormalities. Clinical and ultrastructural study].[伴有线粒体异常的眼肌病。临床与超微结构研究]
Arch Ophtalmol Rev Gen Ophtalmol. 1972 Oct;32(10):615-26.
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Sudden and unexpected death in a young Thai female due to poorly controlled Graves' disease: a case report.
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[A case of Kearns-Sayre-Shy syndrome with abnormal signal intensity on MRI in cerebral white matter and brainstem].1例伴有大脑白质和脑干MRI信号强度异常的卡恩斯-塞尔综合征-夏伊综合征
Rinsho Shinkeigaku. 1991 May;31(5):539-42.
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Chronic progressive external ophthalmoplegia. Clinical, electrophysiological, histochemical and ultrastructural studies of 14 cases.慢性进行性眼外肌麻痹。14例的临床、电生理、组织化学及超微结构研究
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Ocular abnormality in myotonic dystrophy.强直性肌营养不良的眼部异常。
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[Ocular myopathy with mitochondrial abnormalities. Observation of a case with histo-enzymologic and ultrastructural examinations].[伴有线粒体异常的眼肌病。1例经组织酶学和超微结构检查的观察]
Bull Soc Ophtalmol Fr. 1973 Mar;73(3):429-37.

引用本文的文献

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Mitochondrial cytopathy. A multisystem disorder with ragged red fibres on muscle biopsy.线粒体细胞病。一种多系统疾病,肌肉活检可见破碎红纤维。
Arch Dis Child. 1981 Oct;56(10):741-52. doi: 10.1136/adc.56.10.741.
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Intrinsic laryngeal muscles in oculocraniosomatic syndrome (OCSS). An autopsy study.眼颅躯体综合征(OCSS)中的喉内肌。一项尸检研究。
Arch Otorhinolaryngol. 1983;237(2):153-60. doi: 10.1007/BF00463615.
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Congenital Leigh's disease: panencephalomyelopathy and peripheral neuropathy.
Acta Neuropathol. 1984;64(2):167-71. doi: 10.1007/BF00695581.
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Retinal pathology in the Kearns-Sayre syndrome.卡恩斯-塞尔综合征的视网膜病理学
Br J Ophthalmol. 1985 Jan;69(1):63-75. doi: 10.1136/bjo.69.1.63.
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Ophthalmoplegia plus, an electro-oculographic study.眼肌麻痹叠加征:一项眼电图研究
Ital J Neurol Sci. 1985 Dec;6(4):429-39. doi: 10.1007/BF02331035.
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Mitochondrial encephalomyopathy. A variant with heart failure and liver steatosis.线粒体脑肌病。一种伴有心力衰竭和肝脂肪变性的变异型。
Acta Neuropathol. 1987;74(3):287-93. doi: 10.1007/BF00688194.
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Neuropathology in Kearns-Sayre syndrome.
Acta Neuropathol. 1990;80(5):541-6. doi: 10.1007/BF00294616.
8
Involvement of extraocular muscle in mitochondrial encephalomyopathy.
Acta Neuropathol. 1990;80(2):118-22. doi: 10.1007/BF00308913.