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[先天性和内源性内分泌性肌病]

[Congenital and endogenous endocrine myopathy].

作者信息

Wenninger S, Schoser B

机构信息

Friedrich-Baur-Institut, Neurologische Klinik, Interdisziplinäres Zentrum für Neuromuskuläre Erkrankungen der Ludwig-Maximilians-Universität München, Deutschland.

出版信息

Z Rheumatol. 2011 Nov;70(9):760-2, 764-6. doi: 10.1007/s00393-011-0787-5.

Abstract

Disorders in endocrinological pathways rarely lead to manifest acquired or endogenous myopathy so that an interdisciplinary evaluation between neurology and endocrinology is essential for these disorders. Asymptomatic or forme fruste variants may be more common and even underdiagnosed in these circumstances. Dysbalance disorders of protein synthesis, electrolytes and carbohydrates can lead to several rare forms of myopathy due to the dependence on hormonal metabolism. In general, the main neuromuscular symptom is proximal weakness, sometimes in addition to myalgia and muscle atrophy. Endocrine myopathies are usually reversible by treatment of the underlying disease. The severity of the endocrinopathy is of fundamental importance for the long-term clinical outcome.

摘要

内分泌途径紊乱很少导致明显的后天性或内源性肌病,因此神经科和内分泌科之间的多学科评估对于这些疾病至关重要。在这些情况下,无症状或顿挫型变体可能更常见,甚至可能诊断不足。由于对激素代谢的依赖,蛋白质合成、电解质和碳水化合物的失衡紊乱可导致几种罕见的肌病形式。一般来说,主要的神经肌肉症状是近端肌无力,有时还伴有肌痛和肌肉萎缩。内分泌性肌病通常通过治疗基础疾病可逆转。内分泌病的严重程度对长期临床结果至关重要。

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