Feiden W, Gerhard L, Borchard F
Institut für Neuropathologie, Ludwig-Maximilians-Universität München, Federal Republic of Germany.
Virchows Arch A Pathol Anat Histopathol. 1988;413(6):573-80. doi: 10.1007/BF00750399.
Three patients with the Guillain-Barré syndrome which followed the course of Landry's acute ascending paralysis died a sudden cardiac death. Autonomic dysfunction had appeared clinically, consisting of sphincter disturbances in one patient and fluctuating blood pressure and bradycardia in the other. In a twenty-three year old female patient cardiac function had been inconspicuous, apart from tachycardia, but the ECG showed S-T segment depression and flat T waves. Postmortem examination revealed acute inflammatory demyelinating polyradiculoneuritis involving the peripheral autonomic nervous system and especially the nerves of the heart. Immunohistochemically, the inflammatory cell infiltrations of this neuritis cordis consisted of macrophages (MAC 387 positive) and T lymphocytes (UCHL1 positive). No indication of a direct viral infection of the inflamed cardiac nerves was detectable by immunohistochemistry (HSV, CMV, influenza virus) nor by electron microscopy. The neuritis cordis was classified as an inflammatory cardio-neuropathy secondary to a patchy acute polyneuritis of the Guillain-Barré syndrome, involving the autonomic nervous system. Myocarditis could be discounted, and the neuritis cordis was thought to be responsible for the sudden cardiac death.
3例患格林-巴利综合征(病程呈Landry急性上升性麻痹)的患者死于心源性猝死。临床上出现了自主神经功能障碍,1例患者表现为括约肌功能紊乱,另1例患者表现为血压波动和心动过缓。在1例23岁女性患者中,除心动过速外,心脏功能并无明显异常,但心电图显示ST段压低和T波平坦。尸检发现急性炎症性脱髓鞘性多神经根神经炎累及外周自主神经系统,尤其是心脏神经。免疫组织化学检查显示,这种心脏神经炎的炎症细胞浸润由巨噬细胞(MAC 387阳性)和T淋巴细胞(UCHL1阳性)组成。免疫组织化学(单纯疱疹病毒、巨细胞病毒、流感病毒)和电子显微镜检查均未发现炎症性心脏神经有直接病毒感染的迹象。心脏神经炎被归类为格林-巴利综合征斑片状急性多发性神经炎继发的炎症性心脏神经病变,累及自主神经系统。可排除心肌炎,认为心脏神经炎是心源性猝死的原因。