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目的:根据腓骨肌萎缩症腓肠神经的形态计量学研究,为本病的临床诊断和遗传咨询提供可靠的指标。方法:测定7例腓骨肌萎缩症(遗传性运动感觉神经病,HMSNⅠ和Ⅱ型)患者腓肠神经的节间长度(IL)、有髓纤维(MF)和无髓纤维(UMF)的密度以及轴突直径与纤维直径之比(g比率),并比较HMSNⅠ和Ⅱ型间上述参数的差异。结果:HMSNⅠ型中MF密度、g比率有显著下降,Ⅱ型的MF密度、g比率实际数值也明显低于对照组。Ⅰ型患者运动神经传导速度(MCV)与MF密度有直线相关关系。结论:MF及UMF密度、有髓纤维g比率及IL在HMSNⅠ和Ⅱ型中均有较稳定的改变,对反映两型的病理改变具有肯定意义;MCV是反映病理改变最敏感的指标,可作为本病家系及群体调查时初步筛选和分型根据之一。
OBJECTIVE: To provide a reliable indicator of the clinical diagnosis and genetic counseling of this disease based on the morphometric study of the sural nerve of Charcot-Marie Tooth. METHODS: The intima-media length (IL), the density of myelinated (MF) and non-myelinated (UMF) fibers of the sural nerve in 7 patients with Charcot-Marie-Tooth Syndrome (hereditary motor sensory neuropathy, HMSNⅠ and Ⅱ) The ratio of the diameter of the protuberances to the diameter of the fibers (g ratio), and the differences between the above parameters of HMSN I and II were compared. Results: The MF density and g ratio of HMSN Ⅰ decreased significantly. The actual values of type Ⅱ MF density and g ratio were also significantly lower than those of the control group. Type I patients had a linear correlation between motor nerve conduction velocity (MCV) and MF density. CONCLUSION: The density of MF and UMF, g-ratio of myelinated fiber and IL in HMSN Ⅰ and Ⅱ are more stable, reflecting the pathological changes of both types. MCV is the most sensitive indicator of pathological changes and can be used as The disease pedigree and group investigation of the initial screening and typing based on one.