Gdynia H-J, Kurt A, Endruhn S, Ludolph A C, Sperfeld A-D
University of Ulm, Department of Neurology, Oberer Eselsberg 45, 89081 Ulm, Germany.
J Neurol Neurosurg Psychiatry. 2006 May;77(5):671-3. doi: 10.1136/jnnp.2005.078600.
Myocardial involvement in motor neuron diseases (MND) is an uncommon feature. In amyotrophic lateral sclerosis (ALS) abnormalities of the autonomic nervous system affecting cardiac function have been described, for the hereditary spastic paraplegias (HSP) comparable manifestations are unknown. This study observed ALS and HSP patients with coexisting cardiomyopathy without major cardial risk factors.
Four patients with definite ALS and two pHSP patients. In all patients detailed clinical, cardiological, electrophysiological and laboratory data were analysed. In two ALS patients skeletal muscle biopsy was performed.
In all investigated MND patients cardiomyopathy was present. Beside hyperlipoproteinaemia and mild hypertension in one case, none of the patients showed major cardiovascular risk factors. There was no evidence for a secondary cause of cardiomyopathy like coronary heart disease, myocarditis, or mitochondrial damage mimicking MND.
This report could not conclude that the occurrence of cardiomyopathy is rare logically. Although an underlying pathophysiological cause was not obvious, it is proposed that in all MND patients a routine cardiological evaluation should be performed.
运动神经元病（MND）累及心肌是一种不常见的特征。在肌萎缩侧索硬化症（ALS）中，已描述了影响心脏功能的自主神经系统异常，而对于遗传性痉挛性截瘫（HSP），类似表现尚不清楚。本研究观察了无主要心脏危险因素且合并心肌病的ALS和HSP患者。
4例确诊为ALS的患者和2例pHSP患者。对所有患者的详细临床、心脏、电生理和实验室数据进行分析。对2例ALS患者进行了骨骼肌活检。
在所有接受调查的MND患者中均存在心肌病。除1例患者有高脂蛋白血症和轻度高血压外，其他患者均未显示出主要的心血管危险因素。没有证据表明心肌病有继发原因，如冠心病、心肌炎或模仿MND的线粒体损伤。
本报告无法从逻辑上得出心肌病的发生罕见的结论。尽管潜在的病理生理原因并不明显，但建议对所有MND患者进行常规心脏评估。
