Guzzetta F, Ferrière G
Acta Neuropathol. 1985;68(3):185-90. doi: 10.1007/BF00690192.
A case of congenital neuropathy associated with an unclassified chronic brain disorder is described. Morphological findings differ from the reported congenital neuropathies where primary myelin change have been usually found. In contrast, sural nerve biopsy showed marked signs of active or past axonal degeneration; at the teasing and morphometric analysis there was also some evidence of segmental demyelination. Atypical onion bulb formations (Evans and Murray type) and a very poor demyelination activity stressed the prevailing axonal involvement with possibly secondary segmental demyelination.
本文描述了一例与未分类慢性脑部疾病相关的先天性神经病变。形态学发现与已报道的先天性神经病变不同，后者通常发现有原发性髓鞘改变。相比之下，腓肠神经活检显示有明显的活动性或既往轴索性变性迹象；在 teased 检查和形态计量分析中，也有一些节段性脱髓鞘的证据。非典型洋葱球样结构（Evans 和 Murray 型）以及极弱的脱髓鞘活性强调了主要的轴索受累，并可能伴有继发性节段性脱髓鞘。
