Martínez Juana, Ramón César, Morís César, Pascual Julio, Morís Germán
Juana Martínez, César Ramón, Julio Pascual, Germán Morís, Neurology Department, Hospital Universitario Central Asturias, 33006 Oviedo, Spain.
World J Clin Cases. 2014 Jun 16;2(6):211-4. doi: 10.12998/wjcc.v2.i6.211.
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that almost exclusively involves motor neurons although autonomic dysfunction has also been reported. We present an 84-year-old female with no documented history of heart disease, who was admitted with negative T waves in the electrocardiogram precordial leads mimicking myocardial ischaemia. No other abnormalities were shown in the rest of the cardiologic evaluation, suggesting autonomic nervous system dysfunction. A neurophysiological study demonstrated acute and chronic denervation in multiple muscles with normal nerve conduction studies, confirming ALS diagnosis. Previous studies have shown that subclinical sympathetic hyperfunction and parasympathetic hypofunction might result in cardiovascular dysfunction in ALS patients. It is important to detect disturbances of autonomic cardiac control because this dysfunction may influence survival and quality of life, leading to a decrease in life expectancy in ALS patients. This Case Report may support the impairment of cardiac autonomic control in patients with ALS.
肌萎缩侧索硬化症(ALS)是一种进行性神经退行性疾病,几乎仅累及运动神经元,尽管也有自主神经功能障碍的报道。我们报告一例84岁女性,既往无心脏病史,因心电图胸前导联T波倒置,类似心肌缺血而入院。心脏评估的其他方面未显示其他异常,提示自主神经系统功能障碍。神经生理学研究显示多块肌肉存在急性和慢性失神经支配,神经传导研究正常,确诊为ALS。既往研究表明,亚临床交感神经功能亢进和副交感神经功能减退可能导致ALS患者出现心血管功能障碍。检测心脏自主控制的紊乱很重要,因为这种功能障碍可能影响生存率和生活质量,导致ALS患者预期寿命缩短。本病例报告可能支持ALS患者存在心脏自主控制受损。