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[遗传性脊髓小脑变性中的心脏改变]

[Cardiac changes in hereditary spinocerebellar degenerations].

作者信息

Illarioshkin S N, Borisenko V V, Ivanova-Smolenskaia I A

出版信息

Ter Arkh. 1990;62(10):88-92.

PMID:2084900
Abstract

Echocardiographic alterations were compared with the clinical picture in 32 patients with different forms of hereditary spinocerebellar degenerations (HSCD). Three groups of patients were examined. Group I included patients with Friedreich's ataxia (FA), group II consisted of patients with familial cerebellar degeneration, and group III of those with sporadic cerebellar degeneration. Echographic alterations associated with FA were recorded in 71.4% of cases. Cardiomyopathy was confirmed to be a characteristic feature of FA. Echographic alterations in FA were noted to be pleomorphic: apart from typical hypertrophy of the myocardium, a considerable enlargement of the left ventricle was detectable more seldom. In familial cerebellar degeneration, different echocardiographic alterations were recorded in 81.8% of cases, whereas in sporadic cerebellar degeneration, in 78.6% of cases. Dilated cardiomyopathy was revealed in 3 cases (in patients belonging to groups II and III). It is assumed that cardiac pathology may be one of the extraneural manifestations not only in FA but also in other forms of HSCD.

摘要

对32例不同类型遗传性脊髓小脑变性(HSCD)患者的超声心动图改变与临床表现进行了比较。检查了三组患者。第一组包括弗里德赖希共济失调(FA)患者,第二组由家族性小脑变性患者组成,第三组为散发性小脑变性患者。71.4%的FA病例记录到了与FA相关的超声心动图改变。心肌病被证实是FA的一个特征性表现。FA的超声心动图改变呈多形性:除了典型的心肌肥厚外,较少能检测到左心室明显扩大。在家族性小脑变性中,81.8%的病例记录到了不同的超声心动图改变,而在散发性小脑变性中,这一比例为78.6%。3例(属于第二组和第三组的患者)发现有扩张型心肌病。据推测,心脏病变可能不仅是FA,也是其他形式HSCD的神经外表现之一。

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