Hamida C B, Samouda F
J Neurol Sci. 1980 Mar;45(2-3):379-89. doi: 10.1016/0022-510x(80)90182-3.
The muscolo-cutaneous nerve, and skin biopsies from 3rd and 5th finger-tips and the back were studied in a 8-year-old girl with congenital indifference to pain, and in a control child of the same age. The tips of the fingers, and the toes were the most damaged areas. The diameters of myelinated and unmyelinated fibers of the nerve in the sick child and the control child were compared. A loss of 54% of myelinated fibers and 33% of unmyelinated fibers was observed. In the skin, the loss of myelinated fibers was 82% in the 3rd finger-tip, 78% in the 5th, and 35% in the back, and of unmyelinated fibers, 97%, 87%, and 8%, respectively. Eighty four per cent of free endings were absent in the 3rd finger-tip and they were completely absent in the 5th. As for Meissner's corpuscles, 97% were absent in the 3rd finger, and 75% in the 5th. The absence of degenerating fibers and the unimodal distribution of unmyelinated fibers raise the problem of the nosological position of this case of congenital indifference to pain, as compared to the sensory neuropathies.
对一名患有先天性无痛觉的8岁女孩以及一名同龄对照儿童的肌皮神经、来自第三和第五指尖及背部的皮肤活检样本进行了研究。手指尖和脚趾是受损最严重的部位。比较了患病儿童和对照儿童神经中髓鞘纤维和无髓鞘纤维的直径。观察到髓鞘纤维损失了54%,无髓鞘纤维损失了33%。在皮肤中,第三指尖的髓鞘纤维损失率为82%,第五指尖为78%,背部为35%;无髓鞘纤维的损失率分别为97%、87%和8%。第三指尖84%的游离神经末梢缺失,第五指尖则完全没有。至于迈斯纳小体,第三指中97%缺失,第五指中75%缺失。与感觉神经病相比,无变性纤维以及无髓鞘纤维的单峰分布引发了这例先天性无痛觉病例的疾病分类定位问题。