Fressinaud C, Jean I, Dubas F
Département de Neurologie, UPRES EA 3143, CHU, Angers.
Rev Neurol (Paris). 2003 Mar;159(3):293-9.
Morphometric and immunocytochemical data obtained from nerve biopsies in six patients with necrotizing angiopathic neuropathy were compared with the data obtained in five control patients without neurologic disease. The density of myelinated fibers were greatly decreased in all patients (more than 55 p.cent), and reached 70 p.cent in four patients. The g myelination ratio was elevated in one patient, indicating demyelination. The number of fibers immunolabeled for the neurofilament (NF) light chain (L) was also decreased from 31 to 96 p.cent. Labeling for medium (M) and heavy (H) NF subunits was less affected. On the contrary, the number of fibers expressing beta-tubulin (TUB) increased by 38 to 141 p.cent and the ratio of the number of fibers expressing TUB and NFL (TUB/NFL) was increased 3 to 33-fold (p<0.05). We have previously reported similar, though less pronounced, anomalies in axonopathies of unknown cause. These results suggest that diverse etiologies may lead to the same type of lesions of the axon cytoskeleton.
对6例坏死性血管病性神经病患者神经活检获得的形态学和免疫细胞化学数据，与5例无神经系统疾病的对照患者的数据进行了比较。所有患者有髓纤维密度均大幅降低（超过55%），4例患者降低达70%。1例患者的g髓鞘形成比率升高，提示存在脱髓鞘。神经丝（NF）轻链（L）免疫标记的纤维数量也减少了31%至96%。中等（M）和重（H）NF亚基的标记受影响较小。相反，表达β-微管蛋白（TUB）的纤维数量增加了38%至141%，表达TUB和NFL的纤维数量之比（TUB/NFL）增加了3至33倍（p<0.05）。我们之前曾报道过原因不明的轴索性神经病中存在类似但不太明显的异常。这些结果表明，多种病因可能导致轴突细胞骨架出现相同类型的病变。
