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共济失调

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Joubert syndrome is an autosomal-recessive disorder, characterized by clinical presentation of hypotonia, ataxia, and global developmental delay.

jouber综合症是一种常染色体遗传性病变,临床表现为张力减低、共济失调、大脑半球发育延迟。

The EAE rats were induced by injecting subcutaneously an emulsion of GPSCH and CFA. In addition, each rat received an intracutaneous injection of pertussis. Albumin in serum and CSF samples was examined on the days of 4,6,8,10,12,14,16,18 and 20 p.i.. The integrity of BBB was assessed by caculating CSF to serum albumin quotient. At the same time the brains, spinal cords, thymus and inguinal lymph nodes were removed for paraffin sections cutting. These sections were used for HE staining and ICAM-1 semiquantitative immunohistochemical staining. The results showed as follows:1. None of EAE rats on days 4,6 and 8 p.i. showed clinical signs whichbecame apparent on day 10 p.i.(2.33±0.76; n=6). On days 12 and 14 p.i.

检测p.i。第4天、6天、8天、10天、12天、14天、16天、18天、20天血清和CSF中ALB含量,其CSF/S ALB比值作为评价BBB损害的指标;同时段取动物脑、脊髓、胸腺和腹股沟淋巴结制成石蜡切片行HE染色和ICAM-1半定量免疫组化分析,结果显示如下: 1、EAE大鼠p.i.4-8天无任何临床表现;p.i.10天症状开始明显,症状评分为2.33±0.76(n=6);p.i.12-14天为发病高峰期,症状评分为3.75±0.54(n=12),病情由最初的活动减少,体重减轻逐渐发展为肢体无力、瘫痪,共济失调,甚至濒死状态;p.i.16天大部分动物症状开始缓解,评分为2.08±0.66(n=6);p.i.20天疾病症状完全消失。

Spinocerebellar ataxia Olivopontocerebellar atrophy Retinal pigmental degeneration Trinucleotide repeat

脊髓小脑性共济失调;橄榄桥脑小脑萎缩;视网膜色素变性;三核苷酸重复

Clinical signs in penguins included anorexia, behaior changes, depression, regurgitation, ataxia, recumbency, and seizures, and some penguins did not hae any clinical signs.

企鹅感染东方马脑炎的临床表现包括厌食、行为异常、抑郁、返食、共济失调、斜卧和癫痫发作等,部分企鹅没有任何临床征象。

The results hereinbefore indicated that MK-801 at 0.6mg/kg could induce hyperlocomotor, stereotypy and ataxia, and was suitable for the further mechanical experiment.

以上结果表明,MK-801 0.6 mg/kg既可以诱导小鼠的运动亢进,又表现有明显的定型行为和共济失调,适合进行下面的一些神经生化学实验研究,便于深入探讨精神分裂症的发病机理。

For the birth cohort 1980 to 1996 (n=9 128), 53.9% of the CP children had a bilateral spastic cerebral palsy, 31.0% had unilateral spastic CP, 6.6% were dyskinetic and 4.1% ataxic.

在1980至1996年间的出生组群中(n=9128),53.9%的脑瘫患儿为双侧痉挛性脑瘫,31.0%的为单侧痉挛性脑瘫,6.6%为运动障碍,4.1%为共济失调

Nerve conduction assessment indicated an axonal neuropathy pattern in both groups, while sensory action potentials were more markedly diminished in the sensory ataxic form.

神经传导评估提示两组都存在轴索性神经病,在感觉性共济失调型中感觉动作电位显著降低。

Child cerebral form is the most common form of ALD.Its main clinical characteristics are mental impairment,limbs weakness,alalia,ataxia,seizures,viral and auditive disturbances.Some patients accompanied by visaul nerve atrophy,adrenal cortex and testicle impairment.ALD can be diagnosed by clinical characteristics combined with cerebral MRI.

结论ALD以儿童脑型常见,主要表现为智能下降、肢体无力、言语不清、共济失调、视听力下降及癫发作等症状,可伴有视神经萎缩、肾上腺皮质及睾丸损害,依据临床症状和典型的MRI表现可以进行临床诊断。

The age of onset were 7 to 11 years old.Three patients revealed mental impairment and limbs weakness.Other symptoms included alalia,ataxia in three cases and visual and auditive disturbances in 2 case,One case had visual nerve atrophy and seizures.Testost in one case and 17-hydroxide steroid in another case were degressive.Cerebral biopsy in one case suggest lipide sediment.Testicle impairment in one case was found by ultrasonography.The cerebral magnetic resonance imaging of all the 4 patients showed the characteristic pattern of symmetrical long T1 and long T2 signal in the parieto-occipital region.

结果发病年龄7~11岁,均有智能下降、肢体无力,3例伴言语不清、共济失调,2例伴视听力下降,1例伴视神经萎缩、癫发作,血睾酮降低及血17-羟类固醇降低各1例,B超示双侧睾丸弥漫性病变、脑活检示类脂质沉积病各1例,4例磁共振均见双侧枕顶叶对称性蝶翼状长T1、长T2信号灶,可累及颞叶、内囊和脑干白质。

Results Wernicke-Korsakoff Syndrome clinical Manifection included Ophthalmopegia,dysphrenia and ataxia or Polyneuropathy,and so on.

结果Wernicke-Korsakoff综合征的临床表现为眼外肌瘫、精神障碍和共济失调及多发性神经病等表现,磁共振表现为乳头体、第三脑室、双侧丘脑、中脑导水管周围区域异常信号。

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