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无典型症状的麦克尔迪氏病

[McArdle's disease without typical symptoms].

作者信息

Watanabe M, Matsubara E, Amari M, Okamoto K, Hirai S

机构信息

Department of Neurology, Gunma University School of Medicine.

出版信息

Rinsho Shinkeigaku. 1990 Nov;30(11):1247-51.

PMID:2085931
Abstract

A 25-year-old female with McArdle's disease was reported. She had no characteristic symptoms for McArdle's disease such as muscle cramp and brown urine, but had general fatiguability from childhood. On examination, she showed no neurological abnormalities including muscle atrophy and weakness. On laboratory examination, serum creatinine kinase (CK) level was elevated, though serum lactic acid level remained unchanged after the ischemic forearm exercise test. Muscle biopsy from the biceps brachii showed almost completely absent phosphorylase activity both histochemically and biochemically. Thus, she was diagnosed as having McArdle's disease. The skinned fiber test of the muscle showed no enhanced Ca induced Ca release (CICR), and serum VLDL level was normal. Her 27-year-old elder brother had similar clinical symptoms and serological abnormalities and may also have McArdle's disease, although muscle biopsy was not performed. A possibility of McArdle's disease should be considered when we encounter a patient who has only general fatigue and high serum CK level.

摘要

报告了一名25岁患麦克尔迪氏病的女性。她没有麦克尔迪氏病的典型症状,如肌肉痉挛和棕色尿液,但自幼就有全身易疲劳的症状。检查时,她未表现出包括肌肉萎缩和无力在内的神经学异常。实验室检查发现,尽管缺血性前臂运动试验后血清乳酸水平未变,但血清肌酸激酶(CK)水平升高。肱二头肌肌肉活检显示,组织化学和生物化学检测均几乎完全缺乏磷酸化酶活性。因此,她被诊断为患有麦克尔迪氏病。肌肉的皮肤纤维试验未显示钙诱导的钙释放(CICR)增强,血清极低密度脂蛋白(VLDL)水平正常。她27岁的哥哥有类似的临床症状和血清学异常,尽管未进行肌肉活检,但也可能患有麦克尔迪氏病。当我们遇到仅有全身疲劳和血清CK水平升高的患者时,应考虑麦克尔迪氏病的可能性。

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