Pasquier B, Couderc P, Pasquier D
Sem Hop. 1975;51(31-34):2103-7.
The authors report the pathological and clinical findings in two cases of familial Dejerine-Sottas neuropathy, including the peripheral nerve lesions of an unusual type producing extensive sub-perineural separation. In one case, there was onion bulb hypertrophy of the schwann layer and definite demyelinisation and above all, endoneural nodular structures known as Renaut bodies. The presence of Renaut bodies has not, to our knowledge, yet been reported in Dejerine-Sotta's disease. It is rare but well known during other neural diseases, which are often familial and in various pathological circumstances, such as syringomylia, diabetes and hypothyroidism. The fibroblastic origin of these structures has been proved. Their possible pathogenic role and their significance are still the object of discussion.
作者报告了两例家族性德热里纳 - 索塔斯神经病的病理和临床发现，包括一种不寻常类型的周围神经病变，可导致广泛的神经束膜下分离。在其中一例中，施万层有洋葱球样肥大，明确的脱髓鞘，最重要的是，存在被称为勒诺体的神经内膜结节状结构。据我们所知，勒诺体在德热里纳 - 索塔斯病中尚未见报道。它在其他神经疾病中罕见但为人所知，这些疾病通常是家族性的，且存在于各种病理情况下，如脊髓空洞症、糖尿病和甲状腺功能减退症。这些结构的成纤维细胞起源已得到证实。它们可能的致病作用及其意义仍是讨论的对象。
