Teunissen L L, Notermans N C, Jansen G H, Banga J D, Veldman H, Wokke J H
Rudolf Magnus Institute of Neurosciences, Department of Neurology, University Medical Center, Utrecht, The Netherlands.
Acta Neuropathol. 2000 Oct;100(4):445-50. doi: 10.1007/s004010000193.
For chronic idiopathic axonal polyneuropathy (CIAP), even after extensive evaluation, no cause has yet been found. Considering the age and sex distribution of patients with this disease, it is possible that vascular disease plays a role in the development of this polyneuropathy. As endoneurial vessel abnormalities can be related to ischemia, we investigated endoneurial vessels in sural nerve biopsies of 18 patients with CIAP. As controls we used sural nerves of 4 patients with diabetes mellitus, 6 patients with hereditary motor and sensory neuropathy type II (HMSN type III) and 10 autopsy cases. Basal lamina area thickness, endothelial cell area, lumen area, and the number of basal laminae, endothelial cells and periendothelial cell nuclei were investigated. Basal lamina area thickness, endoneurial cell area and number of endothelial cell nuclei in CIAP were increased in comparison with HMSN type III, whereas the basal lamina area thickness of patients with CIAP and diabetes mellitus were in the same range. The structure of the basal lamina area in CIAP differed from diabetes mellitus; in diabetes mellitus there was a larger number of lamellae, whereas in CIAP there was an increase in collagen. There was no correlation between basal lamina area thickness and age. In CIAP patients with peripheral vascular disease of the legs, basal lamina area thickness was increased. The relation between basal lamina area thickening and peripheral vascular disease of the legs in CIAP may indicate a role for ischemia in the development of this polyneuropathy.
对于慢性特发性轴索性多发性神经病（CIAP），即便经过广泛评估，仍未找到病因。鉴于该疾病患者的年龄和性别分布情况，血管疾病有可能在这种多发性神经病的发病过程中发挥作用。由于神经内膜血管异常可能与缺血相关，我们对18例CIAP患者的腓肠神经活检样本中的神经内膜血管进行了研究。我们将4例糖尿病患者、6例II型遗传性运动和感觉神经病（HMSN III型）患者的腓肠神经以及10例尸检病例作为对照。研究了基膜面积厚度、内皮细胞面积、管腔面积以及基膜、内皮细胞和血管周细胞核的数量。与HMSN III型相比，CIAP患者的基膜面积厚度、神经内膜细胞面积和内皮细胞核数量增加，而CIAP患者和糖尿病患者的基膜面积厚度处于同一范围。CIAP患者的基膜结构与糖尿病患者不同；糖尿病患者的板层数量较多，而CIAP患者的胶原蛋白增加。基膜面积厚度与年龄之间无相关性。在患有下肢周围血管疾病的CIAP患者中，基膜面积厚度增加。CIAP患者基膜面积增厚与下肢周围血管疾病之间的关系可能表明缺血在这种多发性神经病的发病过程中起作用。
