论文部分内容阅读
大多数非Friedreich型脊髓小脑变性症(SCD)的末梢神经损害(PN)已被明确具有比较共同的特征。本文主要讨论少数具有不同病理学改变的非典型例的表现。研究对象是根据腓肠神经活检诊断为单发型OPCA的13例,Menzed型SCD10例,23例中有10例其有髓神经纤维密度在6500/mm~2以下,有髓神经纤维明显减少,20%左右的末梢神经呈现中度的节段性脱髓鞘与再生的病理改变,称之为SCD+PN群。5例有髓神经纤维密度在7000/mm~2以上,残存的有髓神经纤维((?))大致正常,称为SCD群。多数病例属于以上两群中的任一群,但有3例神经纤维密度在7000/mm~2以上的正常范围而残存纤维却有明显异常,它不属于上两群,故称其为第一特殊型(S-Ⅰ)。还有一例神经纤维密度
Most peripheral nerve damage (PN) in non-Friedreich spinocerebellar degeneration (SCD) has been clearly characterized by more common features. This article focuses on the performance of a few atypical cases with different pathological changes. Thirteen patients were diagnosed as single-staged OPCA by sural nerve biopsy and 10 patients with Menzed type SCD. The density of myelinated nerve fibers was below 6500 / mm ~ 2 in 10 of 23 patients, and the number of myelinated fibers was significantly reduced. Peripheral nerves around% showed moderate segmental demyelination and pathological changes of regeneration, which was called SCD + PN group. 5 cases of myelinated nerve fiber density 7000 / mm ~ 2 above, the remaining myelinated nerve fibers ((?)) Roughly normal, known as the SCD group. Most cases belong to any one of the above two groups, but there are 3 cases of nerve fiber density in the normal range of 7000 / mm ~ 2 and the remaining fibers are obviously abnormal, it does not belong to the last two groups, it is called the first special Type (S-Ⅰ). There is another example of nerve fiber density