Barbieri F, Santoro L, Crisci C, Massini R, Russo E, Campanella G
Acta Neuropathol. 1986;69(3-4):213-9. doi: 10.1007/BF00688296.
The bioptical morphometric and ultrastructural study of sural nerve in a 17-year-old boy with ataxia-telangiectasia (AT) is reported. Our findings include a loss of fibers, particularly of large ones, axonal degenerative changes, Schwann cell inclusions of various type, and rare signs of primary demyelination. Teased-fiber study showed paranodal myelin enlargements, segmental demyelination, shortening, and/or variability of internodal length. This picture is similar to that in Friedreich's ataxia (FA), although they differ in degree and time of onset. A correct neuropathologic diagnosis of AT cannot be made on the basis of sural nerve biopsy alone.
报道了对一名患有共济失调毛细血管扩张症（AT）的17岁男孩腓肠神经进行的生物光学形态计量学和超微结构研究。我们的发现包括纤维丧失，尤其是大纤维丧失、轴突退行性改变、各种类型的施万细胞包涵体以及罕见的原发性脱髓鞘迹象。 teased-fiber研究显示结旁髓鞘增大、节段性脱髓鞘、缩短和/或节间长度变异性。尽管它们在程度和发病时间上有所不同，但这种情况与弗里德赖希共济失调（FA）相似。仅根据腓肠神经活检不能做出AT的正确神经病理学诊断。
