Druschky A, Spitzer A, Platsch G, Claus D, Feistel H, Druschky K, Hilz M J, Neundörfer B
Department of Neurology, University Erlangen-Nuernberg, Erlangen, Germany.
Acta Neurol Scand. 1999 May;99(5):308-14. doi: 10.1111/j.1600-0404.1999.tb00681.x.
Involvement of the autonomic cardiac nervous system in early stages of amyotrophic lateral sclerosis (ALS) was evaluated in 40 patients. I-123-metaiodobenzylguanidine-single photon emission computed tomography (MIBG-SPECT) and heart rate variability (HRV) yielded information about sympathetic and parasympathetic innervation of the heart. MIBG-SPECT is a sensitive diagnostic method for demonstration of early cardiac sympathetic denervation. Both sympathetic and parasympathetic dysfunction was observed in 16 (40%) out of 40 patients. Mean cardiac MIBG uptake as demonstrated by the heart/mediastinum ratio was significantly reduced in all ALS patients in comparison with controls (P<0.01). The global MIBG-SPECT score was clearly abnormal in 29% and slightly abnormal in 22% of patients. HRV was diminished in 6 of 38 patients, 4 of whom having an abnormal MIBG-SPECT score as well. The presented results indicate that ALS patients with mild to moderate impairment may have evidence of postganglionic sympathetic adrenergic cardiac or cardiovagal denervation. To our knowledge, this is the first study indicating possible postganglionic sympathetic denervation in ALS. The original concept of ALS as an isolated degeneration of motor neurons seems to extend to a more widespread understanding of the disease which possibly represents different entities.
对40例肌萎缩侧索硬化症（ALS）患者自主心脏神经系统的早期受累情况进行了评估。I-123-间碘苄胍单光子发射计算机断层扫描（MIBG-SPECT）和心率变异性（HRV）提供了有关心脏交感神经和副交感神经支配的信息。MIBG-SPECT是一种用于显示早期心脏交感神经去神经支配的敏感诊断方法。40例患者中有16例（40%）观察到交感神经和副交感神经功能障碍。与对照组相比，所有ALS患者心脏/纵隔比值所显示的平均心脏MIBG摄取显著降低（P<0.01）。29%的患者MIBG-SPECT整体评分明显异常，22%的患者轻度异常。38例患者中有6例HRV降低，其中4例MIBG-SPECT评分也异常。研究结果表明，轻度至中度受损的ALS患者可能存在节后交感肾上腺素能心脏或心迷走神经去神经支配的证据。据我们所知，这是第一项表明ALS可能存在节后交感神经去神经支配的研究。ALS最初作为运动神经元孤立性变性的概念似乎扩展到了对该疾病更广泛的理解，这可能代表了不同的实体。
