Matsui Naoko, Mitsui Takao, Ohshima Yasushi, Yokoi Kenji, Kunishige Makoto, Yagi Fumikazu, Vernino Steven, Matsumoto Toshio, Kaji Ryuji
Department of Neurology, Institute of Health Bioscience, Graduate School of Medicine, University of Tokushima, Tokushima, Japan.
Intern Med. 2010;49(1):73-7. doi: 10.2169/internalmedicine.49.2788. Epub 2010 Jan 1.
We encountered two patients with acute pandysautonomia who subacutely exhibited extensive autonomic dysfunction after antecedent infections. Although these patients had been suffering from autonomic disturbance for several months, they both had a good clinical course after plasma exchange and intravenous immunoglobulin therapy. Thin-layer chromatography (TLC)-immunostaining did not demonstrate any antibodies against gangliosides, but immunoblot analysis showed antibodies against a neuroblastoma cell line, SH-SY5Y, in serum samples. Furthermore, ganglionic acetylcholine receptor autoantibodies were detected in one patient. These findings suggest that neuronal antibodies against the autonomic nervous system play an important role in the pathogenesis of acute pandysautonomia.
我们遇到了两名急性全自主神经功能不全患者,他们在先前感染后亚急性地出现了广泛的自主神经功能障碍。尽管这些患者已经遭受自主神经紊乱数月,但在进行血浆置换和静脉注射免疫球蛋白治疗后,他们的临床病程均良好。薄层色谱(TLC)免疫染色未显示出任何针对神经节苷脂的抗体,但免疫印迹分析在血清样本中显示出针对神经母细胞瘤细胞系SH-SY5Y的抗体。此外,在一名患者中检测到了神经节乙酰胆碱受体自身抗体。这些发现表明,针对自主神经系统的神经元抗体在急性全自主神经功能不全的发病机制中起重要作用。