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Classification of myasthenia gravis and congenital myasthenic syndromes in dogs and cats

机译:猪和猫的肌肌肌无力分类和先天性肌炎综合征

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摘要

Myasthenia, a syndrome of impaired neuromuscular transmission, occurs as either an acquired or congenital condition. Myasthenia gravis (MG) is an acquired autoimmune disorder with autoantibodies against the neuromuscular junction (NMJ) of skeletal muscle whereas congenital myasthenic syndromes (CMSs) are a clinically heterogeneous group of genetic disorders affecting the NMJ with a young age of onset. Both conditions are diseases for which recognition is important with regard to treatment and outcome. We review the published literature on MG and CMSs in dogs and cats, and by comparison with published classification used in humans, propose a classification system for MG and CMSs in dogs and cats. Myasthenia gravis is first classified based on focal, generalized, or acute fulminating presentation. It then is subclassified according to the autoimmune disease mechanism or seronegativity. Autoimmune disease mechanism relates to the presence or absence of a thymoma, or administration of thiourylene medication in cats. Congenital myasthenic syndromes are classified according to the affected NMJ component, the mechanism of the defect of neuromuscular transmission, the affected protein, and ultimately the mutated gene responsible. In proposing this categorization of MG and CMSs, we hope to aid recognition of the disease groups for both conditions, as well as guide treatment, refine prognosis, and provide a framework for additional studies of these conditions.
机译:Myasthenia是一种受损神经肌肉传播症的综合征,作为获得的或先天性病症发生。 MyAsthenia Gravis(Mg)是一种具有骨骼肌的神经肌肉接合量(NMJ)的自身免疫性疾病,而先天性肌炎综合征(CMS)是一种影响NMJ的临床异质遗传紊乱组,具有年轻的发病时期。这两种情况都是疾病在治疗和结果方面具有重要疾病。我们在狗和猫的MG和CMS上审查发表的文献,并通过与人类使用的公布分类进行比较,提出了狗和猫的MG和CMS的分类系统。基于焦点,广义或急性颤动的介绍,首先分类MyAsthenia。然后根据自身免疫性疾病机制或血清术来分类。自身免疫性疾病机制涉及胸腺瘤的存在或不存在,或在猫中施用硫代脲药物。先天性染发素综合征根据受影响的NMJ组分进行分类,神经肌肉传播缺陷的机制,受影响的蛋白质,最终突变基因负责。在提出MG和CMS的这种分类时,我们希望能够识别疾病群体的病症,以及指导治疗,细化预后,并提供额外研究这些条件的框架。

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