共济失调前的

Gene mutations were detected in 25 patients and a 18-year-old girl among 16 families. Trinucleotide repeats of CAG were 73～79. The fragments of abnormal alleles were 380～402bp, and all patients were heterozygous. The copy numbers of normal alleles were 18～40, fragments from 202～270bp. SCV reduction was much obvious compared to MCV, MCV and SCV in lower limb ?ere much more slow than that in upper's. BAEP, VEP were also delayed in latency. The anticipation in parental sex bias were much more obvioius than that in matental's. Cerebellar ataxia was most severe, the next were dysarthria and bulging eyes. Amytrophy was seen only in bed ridden patients. Cerebellar atrophy was more severe than brain stem, cord atrophy was n't observed in all MJD.

结果检出10个家系25例病人及1例症状前18岁女孩有MJD基因突变，CAG三核苷酸重复73～79次，异常等位基因片段长380～402bp，均为杂合子；正常人CAG三核苷酸重复18～40次，等位片段长200～270bp，电生理发现MJD的SCV减慢比MCV明显，而下肢的MCV、SCV又较上肢明显，BAEP、VEP均有不同程度的潜伏期延长或波的异常；MJD的父亲遗传早于母亲，进展也较块，临床以小脑性共济失调为突出症状，其次为构音障碍、突眼等，肌肉萎缩仅见于晚期病人；MRI示小脑萎缩较明显，脑干萎缩并不严重，未见明显的颈髓萎缩。

Patients' symptoms were assessed in accordance with Unified Parkinson's Disease Rating Scale (partsⅠ,Ⅱ,Ⅲ,Ⅳ were respectively used to evaluate mental activity, activities of daily living, motor nerve characteristics, complication therapy), International Cooperative Ataxia Rating Scale (including gait, cooperative ataxia, dysarthrosis, ocular dyscinesia), Activities of Daily Living Scale and mini-mental state examination.

分别于移植前及移植后2个月，采用统一帕金森病评定量表（partⅠ、Ⅱ、Ⅲ、Ⅳ分别评价心理活动、日常生活活动、运动神经特性、并发症的治疗）、国际共济失调量表（包括姿势步态、肢体共济失调、构音障碍、眼球运动障碍4项因子）、日常生活能力量表、简易智力量表对患者临床症状的变化进行评定。

The former group showed severe sensory ataxia and predominantly large myelinated fiber loss in the sural nerve.

前一组表现为严重的感觉性共济失调，主要是大的有髓纤维缺失，特别是在腓肠神经。

This one mode pays close attention to network credence foundation of the businessman very much.

这一模式非常关注商人的网络信用基础。

Cell morphology of bacterial ghost of Pasteurella multocida was observed by scanning electron microscopy and inactivation ratio was estimated by CFU analysi.

扫描电镜观察多杀性巴氏杆菌细菌幽灵和菌落形成单位评价遗传灭活率。