Dyck P J, Karnes J L, Lambert E H
Peripheral Neuropathy Research Laboratory, Mayo Clinic, Rochester, MN.
Neurology. 1989 Oct;39(10):1302-8. doi: 10.1212/wnl.39.10.1302.
We measured neuropathic deficit (neurologic disability score [NDS]) and attributes of nerve conduction in hereditary motor and sensory neuropathy (HMSN 1) in cross-sectional evaluation of 69 patients and in longitudinal evaluation over approximately 15 years in 31 of them. Neuropathic deficit worsened by 0.6 NDS point per year in patients 5 to 14 years old at first evaluation, by 1.1 points in patients 15 to 39 years old, and by 0.9 point in patients 40 or more years old. Neuropathic deficit was greater in HMSN 1b (the disorder linked to Duffy) than in HMSN 1a (not linked to Duffy). Nerve conduction attributes changed significantly depending on attribute studied, age, and nerve. In patients evaluated serially, ulnar conduction velocity (CV) increased by a few meters per second in patients who were 5 to 14 or 15 to 39 years old at first examination, but decreased in patients who were older. In serial measurements, peroneal nerve amplitude decreased in all 3 age groups. We found an association between CV and amplitude or NDS at first and last examinations, suggesting an association between severity of the CV abnormality and neuropathic deficit. The severity of the CV abnormality in the young appears to predict later neurologic abnormality.
我们对69例患者进行了横断面评估，对其中31例患者进行了约15年的纵向评估，测量了遗传性运动和感觉神经病（HMSN 1）患者的神经病变缺损（神经功能障碍评分[NDS]）和神经传导属性。在首次评估时，5至14岁患者的神经病变缺损每年恶化0.6个NDS点，15至39岁患者每年恶化1.1个点，40岁及以上患者每年恶化0.9个点。HMSN 1b（与达菲血型相关的疾病）患者的神经病变缺损比HMSN 1a（与达菲血型无关）患者更严重。神经传导属性根据所研究的属性、年龄和神经的不同而有显著变化。在接受系列评估的患者中，首次检查时年龄在5至14岁或15至39岁的患者，尺神经传导速度（CV）每秒增加几米，但年龄较大的患者则下降。在系列测量中，所有3个年龄组的腓总神经振幅均下降。我们发现在首次和末次检查时，CV与振幅或NDS之间存在关联，这表明CV异常的严重程度与神经病变缺损之间存在关联。年轻人CV异常的严重程度似乎可预测后期的神经异常。
