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双语推荐：脊髓小脑共济失调

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背景：脊髓小脑性共济失调是临床上较常见的以进行性加重的四肢共济运动障碍为主要临床表现的中枢神经系统变性疾病，常规药物治疗效果欠佳。目的：观察自体骨髓间充质干细胞以及异体脐带间充质干细胞输注治疗脊髓小脑性共济失调的临床效果。方法：对接受间充质干细胞治疗的27例确诊脊髓小脑性共济失调患者进行综合统计分析，其中6例行自体骨髓间充质干细胞腰穿治疗，21例行异体脐带间充质干细胞腰穿结合静脉输注治疗，两组患者均采用世界神经病联合会国际合作共济失调量表(International Cooperative Ataxia Rating Scale，ICARS)对患者治疗前后神经功能进行评定。结果与结论：所有27例脊髓小脑共济失调患者行间充质干细胞治疗过程中以及治疗前后均未及明显不良反应。其中6例患者采用自体骨髓间充质干细胞治疗后效果均不明显，另外21例患者行异体脐带间充质干细胞输注治疗，治疗后3个月与治疗前比较，患者自觉症状均有一定程度改善，ICARS 评分明显降低(P <0.05)。说明脐带间充质干细胞治疗是安全的，可以一定程度地改善脊髓小脑性共济失调患者的临床症状，提高患者生活质量。
BACKGROUND: Spinocerebel ar ataxia is a common neurodegenerative disease characterized by slowly progressive movement incoordination of the limbs. It responds badly to common medication. OBJECTIVE: To observe the clinical effect of autologous bone marrow mesenchymal stem cells and al ogeneic umbilical cord mesenchymal stem cells tranfusion in the treatment of spinocerebel ar ataxia. METHODS: A total of 27 spinocerebel ar ataxia patients treated with mesenchymal stem cells treatment were included for comprehensive statistical analysis. Among these patients, six patients received autologous bone marrow mesenchymal stem cells lumbar puncture treatment and 21 patients received al ogeneic umbilical cord mesenchymal stem cells lumbar puncture treatment combined with intravenous infusion. The neurologic function of the patients in the two groups was evaluated with International Cooperative Ataxia Rating Scale before and after treatment. RESULTS AND CONCLUSION: There was no obvious adverse eff

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探讨神经节苷脂联合脐血干细胞移植治疗脊髓小脑性共济失调的近期疗效。方法:回顾性分析我院2010年1月~2012年1月间收治的10例脊髓小脑性共济失调患者的临床病历资料,对患者进行神经节苷脂联合脐血干细胞移植治疗,使用量表评定移植前和移植结束后患者的状态。结果:患者经联合治疗后,世界合作共济失调量表(International cooperative ataxia rating scale,ICARS)各项评分均有下降,治疗后动态功能、姿势和步态、言语障碍的评分明显低于治疗前(P0.05);日常生活能力量表(Activity of daily living scale,ADL)的两量表评分均有下降,治疗后躯体生活自理量表总分、工具性日常生活能力量表总分明显低于治疗前(P0.05)。结论:神经节苷脂联合脐血干细胞移植治疗脊髓小脑性共济失调的近期疗效显著,有效改善患者的症状,提高日常生活自理能力和生活质量。
Objective:To observe the short-term effects of combined ganglioside with transplantation of umbilical cord blood stem cells for treatment of spi-nocerebellar ataxia.Methods:The clinical data were retrospectively analyzed in 10 patients with spinocerebellar ataxia undergone treatment with combined ganglioside with umbilical cord blood stem cell transplantation in our hospital in January of 2010 to 2012.International Cooperative Ataxia Rating Scale ( ICARS) was used to assess the conditions of patients before and after transplantation .Results:After joint therapy,ICARS scoring was generally fallen and the improvement was significant compared to pretherapy regarding the dynamic function,posture and gait as well as speech disorder(P<0.05).The sco-ring on Activity of Daily Living Scale(ADL) was also declined,including lower scores on scales of physical self-maintenance and instrumental activities of daily living(P<0.05).Conclusion: Combined ganglioside with transplantation of umbilical cord

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背景：脊髓小脑性共济失调是以小脑共济运动障碍为主要临床表现的遗传性变性疾病，迄今为止缺乏有效的药物治疗。目的：观察脐带间充质干细胞鞘内注射治疗脊髓小脑性共济失调的临床疗效。方法：38例脊髓小脑性共济失调患者给予脐带间充质干细胞鞘内注射治疗，1×106/(kg?次)，1次/周，4次为1个疗程。38例患者共接受52个疗程治疗，其中27例接受1个疗程治疗，8例接受2个疗程，3例接受3个疗程。采用世界神经病联合会国际合作共济失调量表(ICARS)对患者治疗前后神经功能进行评定，分值越高表示神经功能缺损越严重，采用日常生活能力量表(ADL)对患者治疗前后的日常生活能力量进行评估，分值越低，日常生活能力越强。治疗后对患者进行随访。结果与结论：38例脊髓小脑性共济失调患者52个疗程治疗总有效率为84.6%。治疗结束1个月与治疗前比较 ICARS及ADL评分均明显降低(P<0.01)。有效患者行走不稳、站立不稳、运动迟缓、上肢精细动作障碍、书写困难、构音障碍、眼球运动障碍等临床症状得到改善。治疗后常见的不良反应有头晕(1例)，腰痛(2例)，头痛(1例)，发热(2例)，均在1-3 d内消失。在中位随访39个月(11-59个月)治疗过程中，无治疗相关的不良反应发生。有效患者疾病稳定时间
BACKGROUND:Spinocerebel ar ataxia is a inherited neurodegenerative disease with progressive cerebel ar masonic movement disorders as the main clinical manifestation. So far, no drug is available to control the disease progression. OBJECTIVE:To observe the clinical effect of umbilical cord mesenchymal stem cells in treating spinocerebel ar ataxia by intrathecal injection. METHODS:Thirty-eight cases of spinocerebel ar ataxia were given umbilical cord mesenchymal stem cells by intrathecal injection, 1×106/kg once a week, four times as a course. These 38 cases received 52 courses. Among them, 27 cases received 1 course, 8 cases received 2 courses and 3 cases received 3 courses. International Cooperative Ataxia Rating Scale (ICARS) and Activity of Daily Living Scale (ADL) were used to evaluate patients’neural functions (the greater scores, the more severe damage) and ability of daily living (the lower score, the stronger the ability of daily living). After treatment, al patients

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共济失调是临床上常见的疾病,多有小脑、大脑脊髓和周围神经病变引起,内关穴是于厥阴心包经之络穴,为八脉交会穴之一。该穴具有安神定志,镇静除烦,宣达三焦,去痛止呕,益气安神之功能,应用范围广泛,是临床常用的重要的腧穴之一。人中穴又名鬼宫、鬼市,为督脉经穴位,位于口鼻之间,能沟通任督阴阳经气以协调阴阳,同时督脉入属于大脑,故针刺可开窍启闭,宁心安神。平衡区是小脑在头皮的解剖投影区,是临床针灸治疗小脑共济失调的首选针刺治疗区。针灸治疗共济失调具有独特的疗效,减轻患者的痛苦,提高患者的生活质量。
Ataxia is a common clinical disease , which is mainly caused by the pathological change of cerebellum , brain spinal cord and peripheral nerve .Neiguan is Luo-connecting point , located at Jueyin pericardium meridian , which is one of eight confluence points .This point has efficiency in tranquilization , sedation, dredging the triple energizer , removing the pain and stopping vomiting and benefiting qi for tranquillization.It is one of the commonly used clinically important acupoints with a wide field of application .Renzhong, also known as Guigong and Guishi , is channel point at governor vessel meridian .Located between nose and mouth , it communicates Ren and Du Me-ridians and yin and yang qi in order to coordinate yin and yang .Meanwhile , Ren and Du Meridians belong to the brain , so inducing re-suscitation and tranquilizing the mind can be achieved by acupuncture .Equilibrium area is the anatomic projection area of cerebellum on scalp, which is preferred acupuncture treatment ar

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马查多-约瑟夫病(Machado-Joseph disease,MJD)也被称为脊髓小脑性共济失调3型(spinocerebellarataxia 3,SCA3),是最常见的遗传性脊髓小脑性共济失调亚型.发病机制尚不清楚,复杂的病情一直是临床工作者极大的挑战.得益于分子细胞遗传学迅猛发展,MJD症状前诊断及基因诊断成为现实并还在继续向前发展.逐步用于疾病诊断、鉴别诊断及治疗中,同时为遗传咨询提供科学依据.为寻求新突破,该文集中总结该疾病ATXN3突变致病机制及基因检测应用研究现状.更好地理解这些复杂的分子机制,探讨基因检测以及新基因突变位点的发现对这一致命性疾病的诊断及治疗意义.
Machado-Joseph disease (MJD),also known as spinocerebellar ataxia type 3 (SCA3),is the most common subtype of inherited spinocerebellar ataxia.The mechanism of MJD has not been very clear and the complex condition has always been a great challenge for clinical workers.Presymptomatic diagnosis and gene diagnosis of MJD have become reality profit from the rapid development of molecular cytogenetics and its continuing to move forward.This has gradually been successfully applied to the the clinical diagnosis,differential diagnosis and treatment of the disease,to provide the scientific basis for genetic counseling at the same time.To seek new breakthrough,we will summarizes the mutation mechanism of ATXN3 and the status of application of gene detection in the article.To explore the significance of gene detection and the discovery of new gene mutation,related to diagnosis and treatment,for this deadly disease through a better understanding of those complex molecular mechanisms.

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遗传性脊髓小脑共济失调17型是一种常染色体显性遗传的进行性神经系统退行性疾病,又称类Huntington舞蹈病4型,主要临床表现包括共济失调、肌张力障碍、精神症状等,其致病基因已被定位和克隆.该基因编码TATA结合蛋白,编码区内的CAG重复序列扩展突变可引起蛋白多聚谷氨酰胺链延伸从而致病.现就近年来SCA17在临床、病因、病理及发病机制等方面的研究进展进行综述.
Hereditary spinocerebellar ataxia type 17 (SCA17) is an autosomal dominantly inherited progressive degenerative disease of the nervous system.Also known as Huntington''s disease-like 4 (HDL4),SCA17 mainly features ataxia,muscle dystonia and psychiatric symptoms.The gene predisposing to SCA17 has been mapped and cloned,which encodes a TATA-binding protein (TBP).A CAG repeat expansion in the coding region of TBP gene can cause polyglutamine chain extension in the protein.This paper reviews recent progress in the research on SCA17 in regard to its clinical,etiology,pathology and pathogenesis.

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中枢神经系统表面含铁血黄素沉积症是一种罕见的综合征[1],Hamill在1908年首先做出了临床病理描述[2].临床表现特点为感觉神经性耳聋、小脑性共济失调、痴呆、脊髓病和慢性蛛网膜下隙出血.我科于2013年4月收治1例此病患者,经过精心的治疗与护理,患者病情好转出院,现报道如下.
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目的 探讨齿状核红核苍白球路易氏体萎缩症(dentatorubral-pallidoluysian atrophy,DRPLA)患者的临床特征和基因突变特点.方法 应用基于荧光标记的毛细管电泳片段分析法对708个常染色体显性遗传脊髓小脑共济失调(spinocerebellar ataxias,SCA)家系的先证者和119例临床拟诊为SCA的散发患者进行DRPLA基因CAG重复次数分析.结果 共检出3例患者存在DRPLA基因CAG重复扩展突变.片段分析显示其CAG重复次数分别为16/58、16/58和14/54次,长片段重复次数达到异常范围.3个家系的先证者均为成年起病,以共济失调为主要症状,患者可伴发抽搐、颈部扭转等表现.结论 在827例共济失调病例中仅发现3例DRPLA,说明该病在中国人群中较为罕见.DRPLA的临床表现复杂多样,存在变异.对DRPLA患者的临床特征和突变特点的细致分析有助于同其他SCA类型进行鉴别诊断.
Objective To investigate genetics and clinical characteristics of dentatorubralpallidoluysian atrophy (DRPLA) in Chinese kindreds.Methods Fragment analysis with laser-induced fluorescence in capillary electrophoresis was performed for the cytosine-adenine-guanine (CAG) repeats of DRPLA gene in 708 probands of autosomal dominant ataxia pedigrees and 119 sporadic ataxia cases.Results Expanded CAG repeats of DRPLA gene were detected in probands of three ataxia pedigrees,with the numbers of repeats being 16/58,16/58 and 14/54,respectively.In addition to ataxia,patients with adultonset disease also exhibited spasm and neck torsion.Conclusion Only three cases of DRPLA have been identified among 827 cases,which suggested that DRPLA is a relatively rare subtype of SCA in Chinese population.Clinical variation among the patients suggested DRPLA has a wide spectrum of phenotype.

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线粒体自身形态和功能异常出现于很多神经退行性疾病,这些疾病包括极为常见的帕金森病(PD)和阿尔茨海默病(AD),也包括较为罕见的亨廷顿疾病(HD)和肌萎缩性脊髓侧索硬化症(ALS)以及脊髓小脑性共济失调(SCA)等.某些疾病所特有的线粒体形态与功能的改变或损伤已经被体内外的实验所证明.线粒体自身功能的受损会对正常神经细胞功能造成损伤,引起神经系统功能障碍,导致疾病的发生.本文将对线粒体自身形态和功能的损伤与神经系统退行性疾病之间的关系加以综述.
The abnormal of mitochondrial morphology and function discover in many neurodegenerative disorders,including Parkinson''s disease (PD) and Alzheimer''s disease (AD) which are extremely common diseases,also including rare types of neurodegenerative diseases,such as Huntington s disease (HD),amyotrophic lateral sclerosis (ALS),spinocerebellar ataxia (SCA) and so on.The specific changes of mitochondrial morphology and function or mitochondrial defects in some diseases have been demonstrated in vivo and vitro experiments.Mitochondrial defects that lead to the damage of normal neurons and the dysfunction of nervous system,eventually result in the neurodegenerative disorders.Here is to make a review of abnormalities of mitochondrial function and morphology and relationships between with the degenerative diseases.

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α突触核蛋白(α-Syn)是一种小分子酸性可溶性蛋白，是帕金森病发病的病理基础，在帕金森病的发生中起着重要作用。该文对α-Syn的结构、功能及其导致帕金森病的发病机制等方面进行了综述，结合近年来对遗传性脊髓小脑型共济失调2型(SCA2)与α-Syn关系的研究来阐述带有帕金森症状的SCA2的发病是否与帕金森病的发病有共同的发病基础，是否也是α-Syn异常聚集所致，尚待进一步考证。
α-synuclein is a small molecule of acid soluble proteins,the incidence of the pathological ba-sis of Parkinson′s disease,plays an important role in the genesis of Parkinson′s disease. Aspects of this pa-per,the α-synuclein structure, function and lead to the pathogenesis of Parkinson′s disease were reviewed, combined with recent hereditary spinocerebellar ataxia (SCA2) 2 andα-synaptic nuclear protein-to illustrate whether hereditary spinocerebellar ataxia (SCA2) 2 with the onset of Parkinson′s symptoms have a common basis of the incidence and onset of Parkinson′s disease, whether it is abnormal aggregation of α-synuclein due,pending further research.

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