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共济失调

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These antioxidant agents represent a new hope of treating Friedreich's Ataxia and there is, at the present moment, a study testing their efficiency on a group of patients.

这些抗氧化剂给予了治疗Friedreich共济失调以新的希望,目前,一项对一组患者进行的研究正在测试它们的有效性。

The genetic studies, undertaken since the end of 1980, have lead to discovery in 1996 of the Friedreich's ataxia gene.

自1980年末开始进行的遗传学研究,已经于1996年发现了Friedreich共济失调的基因。

These signs are followed by ataxia .

这些症状之后就是共济失调

The intraperitonealLD_(50) values in mice of VES and VDPG were 0.16mg/kg and 0.39mg/kg,respectively. The intraperitoneal LD_(50) value in cockroach of VDPG andVES were 2.32μg/g and 1.87μg/g, respectively. After intraperitonealinjection of the venom in mice, the main poisoning symptoms includedlethargy, ataxia, diaphoresis, convulsion, anorexia, shortness of breath,and difficulty to open eyes, etc. Whereas the mice injected withphysiological saline acted normally.

两种粗毒都对小鼠和蜚蠊有较强的毒性,其中毒囊粗毒和电刺激粗毒对小鼠的LD_(50)值分别为0.39mg/kg和0.16mg/kg;毒囊粗毒和电刺激粗毒对蜚蠊的LD_(50)值分别为2.32μg/g和1.87μg/g体重;小鼠经腹腔注射粗毒后,出现无生气、共济失调、排汗、痉挛、食欲减退、呼吸短促、睁眼困难等症状,而注射生理盐水的小鼠活动正常。

At 14 postoperative days, the paralytic limbs'function of the survived rats were evaluated with the Ducker's method (grade 0: pamplegia grade 1: automatic action but could not support the body mass grade 2: could stand but could not walk grade 3: could walk but with difficulty and accompanied by spasm grade 4: could run accompanied by mild incoordination grade 5: normal movement).

术后14d将两组存活的实验鼠按Ducker's评级方法进行瘫痪肢体功能测定(0级:完全瘫;1级:能自主运动,但不能支撑体质量;2级:能站立但不能行走;3级:能行走,但困难伴痉孪;4级:能跑,但有轻度共济失调;5级:正常运动)。

Clinically, there may be a variety of similar to the performance of lacunar syndrome, such as pure athletic hemiparesis, pure sensory stroke, ataxia hemiparesis, dysarthria - clumsy hand syndrome, sensorimotor stroke and so on.

临床上可出现各种类似腔隙性综合征的表现,如单纯运动性轻偏瘫,单纯感觉性卒中,共济失调轻偏瘫,构音障碍——手笨拙综合征,感觉运动性卒中等。

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。

The chicks show an ataxia not unlike the ataxia of vitamin E deficiency known as encephalomalacia or crazy chick disease.

雏鸡显示共济失调,但与维生素E缺乏的称为小脑软化症或疯狂病不同。

The chick s show an ataxia not unlike the ataxia of vitamin E deficiency known as encephalomalacia or crazy chick disease.

雏鸡显示共济失调,但与维生素E缺乏的称为小脑软化症或疯狂病不同。

L Nervous system: Agitation, confusion, hyperkinesia, ataxia, CNS depression, nightmares, nervousness, psychiatric disturbance, hallucinations, insomnia, anxiety, dizziness, thinking abnormality.

神经系统:激动、意识混乱、运动机能亢进、共济失调、CNS抑制、梦魇、神经过敏、精神障碍、幻觉、失眠、焦虑、头昏、思维异常。

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