非遗传性起源舞蹈症相关疾病

舞蹈样症状是很多常见疾病伴发的一种罕见的症状,同时又是神经科较罕见疾病(如亨廷顿病等)的一种常见症状,迄今为止在国内外文献中出现一些以舞蹈症为首发或主要症状的系统疾病的个案报道,对于舞蹈症相关疾病的鉴别诊断显得尤为困难且极具挑战性.虽然现在对舞蹈症的发病机制仍未完全查明,但其相关疾病的鉴别诊断应得到各学科临床医师的重视.     

单肢舞蹈症表现的脑梗死一例

偏身舞蹈症是单侧肢体和（或）面部的不自主、不规则的舞蹈样动作，症状出现较为突然，多数急性起病，少数亦可在数周内逐渐出现，其病因多样化，见于脑血管病、风湿热、颅脑肿瘤、外伤、变性疾病、丘脑手术后等。此病需与慢性进行性舞蹈症、小舞蹈症、肝豆状核变性等疾病相鉴别。     

糖尿病性非酮症性偏侧舞蹈症一例报道

舞蹈病由锥体外系病变引起，表现为肢体或头面部不自主、不规则的舞蹈样动作。病灶位于对侧锥体外系，包括尾状核、壳核、苍白球、黑质、丘脑底核等。病因较为复杂，临床常见于脑血管病、感染、遗传性疾病、代谢性疾病、药物损害以及神经系统退行性疾病等。糖尿病并发偏侧舞蹈症临床少见，容易误诊，现对作者医院神经内科住院的1例糖尿病性非酮症性偏侧舞蹈症患者进行报道。     

非酮症高血糖性舞蹈病的研究进展

<正>舞蹈症是一组累及头面部及肢体的连续、粗大、不规则的不自主运动,其表现形式多样,通常由对侧基底节(尤其是丘脑底核、尾状核和壳核)或其联系纤维的病变所致[1-3],可由脑血管病、代谢性疾病、颅内占位、神经变性疾病、免疫性疾病、毒物及遗传性疾病引起[2-5]。1960年Bidwell首次报道糖尿病合并偏身舞蹈症[6]。目前,非酮症高血糖性偏身舞蹈症(NKHCB)已被认为是一组以非酮症性高血糖、偏身舞蹈症及头颅MRI T1WI对侧基底节区高信号为特点的综合征[7-10]现对的研究进展综述如下     

神经元核内包涵体病

神经元核内包涵体病是一种罕见的神经系统变性疾病 ,典型可见智能减退 ,锥体系、锥体外系体征如肌强直、姿位异常、静止性震颤、舞蹈样手足徐动、肌阵挛、双侧腱反射亢进、双侧Babinski征 ,下运动神经元损害 ,感觉减退。行为功能障碍 ,步态不稳、小脑性共济失调、构音障碍等小脑体征 ,神经肌肉受累如吞咽困难、弥漫性肌萎缩以及眼球运动异常等 ,神经病理可见特征性的神经元核内包涵体。病程缓慢进展 ,无有效治疗。     

非酮症性高血糖性偏身舞蹈症

<正>障碍疾病的2.8%。多见于对侧基底核或者联系纤维受损而导致的单侧肢体伴(或不伴)面部的一些不自主、不规则的舞蹈样动作。舞蹈动作在情绪紧张、疲劳、兴奋以及劳动时加剧,在安静时可减轻,而睡眠中消失。偏身舞蹈症常见病因为脑血管病,其他少见病因包括感染、肿瘤、亨廷顿病(Huntington's disease,HD)、肝豆状核变性(hepatolenticular degeneration,HLD)以及全身性疾病,如系统性红斑狼疮等。1960年,     

糖尿病合并偏侧舞蹈症的临床及影像学特点

目的 探讨糖尿病合并偏侧舞蹈症的临床及影像学特点.方法 回顾性分析6例糖尿病合并偏侧舞蹈症患者的临床资料.结果 6例患者均在血糖控制不良的情况下急性起病,舞蹈样症状累及单侧肢体和/或面部,上肢为重;头颅CT显示出现舞蹈样症状肢体的对侧豆状核和/或尾状核早期呈高密度,MRI T1加权像呈高信号;2例DWI示病变处稍高信号;1例MRS示病灶处N-乙酰天冬氨酸/肌酸(NAA/Cr)低于对侧,胆碱/肌酸(Cho/Cr)高于对侧,出现乳酸(Lac)峰.经综合治疗(控制血糖、改善脑循环、抗氧自由基、以及口服氟哌啶醇、地西泮等)6例患者症状缓解.2周内复查,部分患者影像学有改善.结论 糖尿病合并偏侧舞蹈症与糖尿病血糖控制不佳有关,影像学显示脑深部灰质核团损伤;提示中枢神经系统微细血管病变可能是由糖尿病导致的;综合治疗近期疗效较好.     

糖尿病性偏侧舞蹈症临床特征研究(附二例患者临床报道)

目的 探讨糖尿病性偏侧舞蹈症的临床特点和影像学表现. 方法 回顾性分析日照市人民医院神经内科2010年10月9日、29日收治的2例糖尿病性偏侧舞蹈症患者的临床症状、影像学特征等资料,并复习相关文献. 结果 2例均为老年女性糖尿病患者,舞蹈症状均为急性起病(1例在应用胰岛素控制血糖的过程中发生,l例在未用胰岛素控制血糖的情况下发生),例2初为偏侧舞蹈症状,后出现双侧舞蹈症状.CT表现为舞蹈症状对侧的尾状核、壳核和苍白球的高密度影(CT值为50 Hu左右).MRI表现为T1像尾状核、壳核和苍白球片状高信号,T2像低信号,局部有轻度萎缩现象,病变部位无强化现象.服用氟哌啶醇及氯硝安定对症状控制有效. 结论 糖尿病性偏侧舞蹈症多见于血糖控制不佳的糖尿病患者,可以双侧先后受累,在舞蹈症状的对侧基底节区有特征性的影像学改变.控制血糖是治疗的基础,氟哌啶醇和氯硝安定有助于舞蹈症状的控制.     

脑梗塞所致舞蹈症及手足徐动症：附6例病例分析

脑梗塞根据病损部位不同,可有不同表现。在运动障碍方面,常见为锥体束损害,表现为瘫痪。少数病人在急性期或恢复期出现锥体外系损害的表现。本文报告6例由脑梗塞所致的舞蹈症(5例)及手足徐动症(1例)。     

非酮症性高血糖舞蹈症七例临床及神经影像学特点

目的 探讨非酮症性高血糖舞蹈症的临床及神经影像学特点.方法 对7例非酮症性高血糖舞蹈症患者进行临床及颅脑CT和MRI检查,分析其临床及影像学特征.结果 7例患者均有糖尿病病史,平素血糖控制不良,发病时血糖较高而酮体正常,表现为单侧肢体、双侧肢体或全身舞蹈样动作.颅脑CT和MRI可见单侧或双侧基底节区异常病灶.单纯药物控制舞蹈症效果不佳,降低血糖后舞蹈症状和神经影像改变可很快恢复,不留后遗症.结论 非酮症性高血糖舞蹈症多见于年龄较大的糖尿病患者,可能与大脑基底核在高血糖状况下脑细胞代谢出现异常有关.颅脑CT或MRI改变具有特征性.本病是可逆性的,对治疗反应较好,一般不留后遗症.     

Denervation study of synapses of organ of corti of old world monkeys

Fine structural characteristics of synapses in the spiral organ of Corti were examined, with reference to differences between inner and outer haircell systems, and to location of neurons of origin of efferent axons. Surgical interruption of crossed olivocochlear bundle, of vestibular nerve, of facial nerve, and excision of superior cervical ganglia were used to determine the pathways of efferent axons. Interruption of the vestibular nerve near the brainstem results in degeneration of all efferent terminals on outer hair cells. Mid-line lesions at, and caudal to, the facial colliculus result in degeneration of about half of these efferent terminals. Efferent synaptic bulbs to the inner hair-cell system are small, of the order of one micron, and form type 2 junctions with afferent dendrites. They tend to have more large dense-core vesicles (about 80 nm) than the large efferent terminals of the outer hair-cell system, and appear to be the terminals of axons in the habenula perforata, which exhibit varicosities laden with large dense core vesicles. The varicosities are unaffected by excision of the superior cervical ganglia. So far as our material can reveal, it appears that the varicosities in the habenula perforata do not survive vestibular root interruption, nor do the efferent processes in the internal spiral bundle or at the base of inner hair cells. Most interestingly, the afferent processes of the inner hair-cell system, as identified for example by their relation to pre-synaptic bodies in the inner hair cells, are subject to a trans-synaptic reaction after severance of the vestibular root. They undergo a dramatic cytological transformation, characterized by increase of volume, engorgement with microtubules, microfilaments, microvesicles of various sizes, and clusters of lysosomes. Thus, both the efferent and afferent terminals of the inner hair-cell system show marked cytological differences from the corresponding terminals of the outer hair cell system.     

Mechanism of action of tubocurarine on nicotinic cholinoreceptors of neurons of the sympathetic ganglia of rats

Tubocurarine (Tc) effect on membrane currents elicited by acetylcholine (ACh) was studied in isolated superior cervical ganglion neurons of rat using patch-clamp method in the whole-cell recording mode. The "use-dependent" block of ACh current by Tc was revealed in the experiments with ACh applications, indicating that Tc blocked the channels opened by ACh. Mean lifetime of Tc-open channel complex, tau, was found to be 9.8 +/- 0.5 s (n = 7) at -50 mV and 20-24 degrees C. tau exponentially increased with membrane hyperpolarization (e-fold change in tau corresponded to the membrane potential shift by 61 mV). Inhibition of the ACh-induced current by Tc (3-30 microM/1) was completely abolished by membrane depolarization to the level of 80-100 mV. Inhibition of ACh-induced current was augmented at increased ACh doses. It is concluded that the open channel block produced by Tc is likely to be the only mechanism for Tc action on nicotinic acetylcholine receptors in superior cervical ganglion neurons of rat.     

The effect of age of onset of PD on risk of dementia

Background Dementia occurs in the majority of patients with Parkinson’s disease (PD). Late onset of PD has been reported to be associated with a higher risk for dementia. However, age at onset (AAO) and age at baseline assessment are often correlated. The aim of this study was to explore whether AAO of PD symptoms is a risk factor for dementia independent of the general effect of age. Methods Two community-based studies of PD in New York (n = 281) and Rogaland county, Norway (n = 227) and two population-based groups of healthy elderly from New York (n = 180) and Odense, Denmark (n = 2414) were followed prospectively for 3–4 years and assessed for dementia according to DSM-IIIR. All PD and control cases underwent neurological examination and were followed with neurological and neuropsychological assessments. We used Cox proportional hazards regression based on three different time scales to explore the effect of AAO of PD on risk of dementia, adjusting for age at baseline and other demographic and clinical variables. Findings In both PD groups and in the pooled analyses, there was a significant effect of age at baseline assessment on the time to develop dementia, but there was no effect of AAO independent of age itself. Consistent with these results, there was no increased relative effect of age on the time to develop dementia in PD cases compared with controls. Interpretation This study shows that it is the general effect of age, rather than AAO that is associated with incident dementia in subjects with PD. Received in revised form: 22 December 2005     

Limits of deinstitutionalization--perspectives of relatives of psychiatric patients

After a hopeful beginning, the social process of the reintegration of those with severe mental illness has come to a standstill. I am led to wonder whether "the community" really wants to live together with people suffering from severe mental illness, and if so, how closely? As long as the medical treatment of mental illness provided by the general practitioners is fundamentally deficient, as they are not able to prescribe the necessary interventions--such as out-patient psychiatric nursing, and service providers in the out-patient sector are content with offering increasingly intensive forms of care for the less seriously ill at the cost of the Social Welfare System--the reintegration of those with serious mental illness remains an illusion--which is mainly to the benefit of providers of residential care in homes and hostels.     

Summary of Legislation of 1933 of interest to the Department of Mental Hygiene

Psychiatric Quarterly -