Meier C, Grahmann F, Engelhardt A, Dumas M
Abteilung für Neurologie, Universität Bern, Inselspital, Switzerland.
Acta Neuropathol. 1989;79(3):271-8. doi: 10.1007/BF00294661.
Clinical, cerebrospinal fluid and nerve biopsy findings from eight patients with peripheral nervous system complications of Lyme-Borreliosis are reported. Five cases showed the typical features of the Garin-Bujadoux-Bannwarth syndrome (meningoradiculoneuritis), one patient had a multiple mononeuritis associated with acrodermatitis chronica atrophicans Herxheimer. Two cases could not be classified under these diagnostic categories. In all patients we observed a prompt relief of signs and symptoms after antibiotic treatment. Nerve biopsy studies showed gross infiltrations of epineurial vasa nervorum and small infiltrations around endoneurial capillaries. The infiltrations consisted of lymphocytes, histiocytes and plasma cells. We did not find necrotizing changes of the vessel walls, but thrombosis and recanalization was observed in some epineurial vessels. Seven biopsies showed a significant loss of myelinated axons due to axonal degeneration. Only in one biopsy did we observe segmental demyelination next to axonal degeneration. We conclude that the PNS complications of Lyme-Borreliosis in early and late stages of the disease are angiopathic due to vasculitis of the vasa nervorum and primarily caused by axonal degeneration.
报告了8例莱姆病螺旋体病周围神经系统并发症患者的临床、脑脊液及神经活检结果。5例表现为加林-布雅杜-班瓦特综合征(脑膜神经根神经炎)的典型特征,1例患者患有多发性单神经炎,伴有赫克斯海默慢性萎缩性肢端皮炎。2例无法归入这些诊断类别。在所有患者中,我们观察到抗生素治疗后体征和症状迅速缓解。神经活检研究显示神经外膜血管有明显浸润,神经内膜毛细血管周围有小浸润。浸润由淋巴细胞、组织细胞和浆细胞组成。我们未发现血管壁的坏死性改变,但在一些神经外膜血管中观察到血栓形成和再通。7例活检显示由于轴突变性导致有髓轴突显著丢失。仅在1例活检中,我们在轴突变性旁观察到节段性脱髓鞘。我们得出结论,莱姆病螺旋体病在疾病早期和晚期的周围神经系统并发症是由于神经血管炎导致的血管病变,主要由轴突变性引起。