帕金森病患者睡眠障碍的相关性分析

目的：评价帕金森病（PD）患者的睡眠质量,探讨PD患者睡眠障碍的具体表现及影响因素。方法：选取54例PD患者,进行帕金森病睡眠量表（PDSS）,爱泼沃斯嗜睡量表（Ess）和Hoetm—Yahr（H—Y）的评分。结果：PD患者睡眠障碍的发生率为62．96％（34／54）。以入睡困难和睡眠维持困难最常见,占42．86％（21／49）。H—Y分期、病程以及左旋多巴日平均剂量与PDSS评分呈正相关。H—Y分期、左旋多巴日平均剂量与ESS评分呈正相关。结论：PD患者睡眠障碍发生率高,且和病情严重程度、病程、多巴胺药物应用等因素有关;与性别和年龄无关。     

新发帕金森病的急性多巴反应性评价

目的：建立急性左旋多巴负荷试验用于鉴别新发帕金森病（PD）与新发帕金森综合征（PDS）患者,并筛选评价指标的临界值。方法：选择89例有PD样表现但未服用过左旋多巴的患者,根据临床诊断分为PD组（n=48）和PDS组（n=41）,进行了急性左旋多巴／卡比多巴（100／25mg）试验。根据两组患者统-PD评分运动分量表（UPDRS—Ⅲ）评分的平均最大改善率进行比较,并建立受试者工作特征（ROC）曲线,以该曲线上最大Youden指数对应的运动评分最大改善率作为临界值。结果：PD组在服用左旋多巴-卡比多巴后的平均UPDRS-Ⅲ评分平均最大改善率高于PDS组,差异有统计学意义（P〈0．01）。建立的ROC曲线在鉴别PD和PDS差异具有统计学意义,ROC曲线下面积为0．827（P〈0．01）,对应上网UPDRS运动评分改善率的最佳临界值为12．45％（Youden指数0．565,敏感度80．9％,特异度75．6％）。结论：急性左旋多巴-卡比多巴试验可作为PD疗效和诊断的一种辅助参考方法。     

不同剂量左旋多巴对帕金森病运动并发症大鼠模型行为学的影响

目的：比较不同剂量左旋多巴长期注射对帕金森病（PD）运动并发症大鼠模型异常不自主运动（AIM）和疗效减退的影响,以确定合适的左旋多巴用药剂量和行为学评价标准。方法：立体定向SD大鼠右侧前脑内侧束,注射6羟基多巴建立PD大鼠模型。成模PD大鼠随机分为3组,分别腹腔注射左旋多巴酯：小剂量组（20mg·kg（-1）·d（-1））、中剂量组（50mg·kg（-1）·d（-1））和大剂量组（100mg·kg（-1）·d（-1））,每天2次,持续21d。比较3组PD大鼠模型AIM各组份的评分情况及对侧旋转反应时间。结果：左旋多巴3种剂量均可诱发PD大鼠出现相似程度的口面、前肢、轴向AIM,但大剂量组大鼠旋转AIM的评分高于小剂量组（P〈0.05）,且旋转反应时间缩短幅度大于中剂量组和小剂量组（P〈0.05）。结论：小剂量左旋多巴就可诱发PD大鼠出现明显的AIM,又能避免过于剧烈的旋转运动对AIM评分的干扰;但观察疗效减退现象需要选用大剂量左旋多巴以诱导出稳定下降的旋转反应时间。     

不同剂量左旋多巴对帕金森病大鼠行为学影响

目的观察不同剂量左旋多巴治疗帕金森病（PD）的效果,并进一步探讨合理使用左旋多巴治疗PD的剂量。方法采用6-OHDA脑立体定向注射术建立大鼠PD模型,采用行为学观察法观察3种不同剂量左旋多巴[按体重10、50、100mg/（kg.d）]作用不同时间（1、3、5、7d）对PD大鼠黑质细胞毒性作用后的行为学变化以及停止左旋多巴治疗后7d各项指标的变化。结果与对照组比较,小剂量组大鼠旋转圈数随左旋多巴使用剂量和时间增加而减少,中、大剂量组大鼠旋转圈数随左旋多巴使用剂量和时间增加而增加;各组大鼠旋转启动时间随左旋多巴使用剂量和时间增加而减少,最高转速及持续时间则随使用剂量增加而增加。结论应尽可能以小剂量、间隔使用左旋多巴治疗PD。     

侧脑室微量注射左旋多巴治疗大鼠帕金森病

目的观察经侧脑室注射左旋多巴对帕金森病（PD）大鼠的影响。方法应用“羟基多巴胺立体定向脑内注射制备偏侧损毁的PD大鼠模型，并用阿扑吗啡（APO）皮下注射诱发大鼠向健侧旋转。将24只PD大鼠随机分为4组（n=6），经侧脑室注入生理盐水组为对照组，余3组分别经侧脑室注射浓度为0．1μg／μl、1μg／μl和5μg／μl的左旋多巴1μl，4μl／d，连续1周；观察在注射后不同时间大鼠旋转行为以及中脑黑质多巴胺能神经元数量的变化。结果经侧脑室注射1μg/μl和5μg/μl的左旋多巴后。与对照组相比，PD大鼠向健侧的旋转圈数明显减少（P〈0．01），左旋多巴效果在2h左右达到高峰，且中脑黑质多巴胺能神经元的数量也明显增多（P〈0．01）。结论经侧脑室注射适当剂量的左旋多巴可有效地改善PD大鼠的旋转行为，并增加中脑黑质多巴胺能神经元数量。     

联合用药治疗帕金森病40例报告

目的　探索帕金森病 (PD)药物治疗的疗效 ,以充分发挥药物治疗的优势互补性。方法　选用我科自 2 0 0 0 0 1～2 0 0 3 10收治的门诊及住院帕金森病患者 40例 ,采用单一用药 (美多巴或左旋多巴 )及联合用药 (左旋多巴 +溴隐亭 )治疗。结果　联合用药不仅能减少单一用药的剂量 ,对震颤、僵直及运动迟缓的改善均优于单一用药 ,对单一用药所致的并发症有延缓作用。结论　联合用药对PD患者仍不失为一种合理的治疗方法 ,对于延缓致残时间、提高生存质量都具有积极的意义     

帕金森病合并不宁腿综合征26例临床分析

目的探讨帕金森病（Parkinson’s disease,PD）患者合并不宁腿综合征（restless leg syndrome,RLS）的临床特点,且进一步研究PD患者合并RLS的相关因素。方法选取确诊的119例PD患者,根据是否合并RLS分为伴RLS组和单纯PD组,分别比较2组的一般状况、病史、临床表现、严重程度、合并症、治疗情况等临床资料。结果 119例PD患者中26例合并RLS,发生率为20.8%,且RLS均出现在PD症状后。2组在UPDRS、HAMD、HAMA、PSQI上有统计学差异（P〈0.05）,而在一般状况、主要症状、左旋多巴治疗等方面均未见统计学差异（P〉0.05）。另外,发现左旋多巴的日剂量与RLS严重度之间存在正相关关系,即服用左旋多巴剂量越大,RLS程度越重。结论 RLS可能是PD病程中出现的一种并发症。PD运动症状较严重、并发症较多时合并RLS的可能性相对较大,随着多巴胺能药物剂量的加大,RLS的病情可能加重。     

丘脑底核电刺激术治疗帕金森病的疗效观察

目的 探讨丘脑底核（STN）行脑深部电刺激术（DBS）治疗帕金森病（PD）的疗效。方法 回顾性分析2016年1月至2017年9月收治的64例PD的临床资料，均采用STN-DBS治疗。术后均随访3个月，使用统一帕金森病评定量表（UPDRS-Ⅲ）评分评估疗效。结果 64例手术顺利完成，平均用时（4.39±1.01）h。共置入128根刺激电极，术后CT计算移位距离为0~1.89 mm，平均（0.91±0.42）mm。术前检测64例改善率在37.20%~82.54%，平均（55.36±5.62）%。术后抗PD药物的左旋多巴等效剂量明显低于术前（P<0.05）；术后开机状态下UPDRS-Ⅲ评分明显低于术前（P<0.05）。术后出现颅内积气29例、颅内出血2例、延伸导线移位3例、情绪改变、构音障碍2例、异动9例，末次随访时均完全改善或症状消失。结论 STN-DBS治疗PD，能有效改善病人运动功能，减少抗PD药物的使用，但围术期并发症风险高，临床应重视操作技巧。     

盐酸司来吉兰治疗早期帕金森病的疗效观察

目的 观察司来吉兰治疗早期帕金森病（PD）的疗效和安全性。方法 采用随机空白对照法,将60例已用安坦、金刚烷胺、维生素E联合治疗的早期PD患者分为司来吉兰治疗组和空白对照组,前者在原用药物剂量不变的基础上添加盐酸司来吉兰片5mg/d,疗程8周。采用改良Webster评分评价药物疗效并观察不良反应。结果 司来吉兰组治疗后4周及8周时改良Webster评分较治疗前明显改善（均P〈0．01）,且明显低于空白对照组（均P〈0．05）;司来吉兰组总有效率为76．7％,明显优于空白对照组（P〈0．01）;未见明显不良反应。结论 司来吉兰是一种安全有效的早期PD治疗用药。     

P型和C型多系统萎缩的急性多巴反应性研究

目的研究P型和C型多系统萎缩（MSA）对左旋多巴的急性反应性。方法对P型MSA患者18例、C型MSA患者13例和帕金森病（PD）患者23例行急性阶梯式左旋多巴试验，药物剂量依次为左旋多巴／苄丝肼50mg／12．5mg、100mg／25mg、150mg／37．5mg、200mg／50mg和300mg／75mg。以UPDRS运动分量表作为评价标准，计算UPDRS运动评分平均最大改善率并比较各组患者的多巴反应性。结果左旋多巴／苄丝肼剂量为。100mg／25mg、150．mg／37．5mg、200mg／50mg和300mg／75mg时，MSA-P型组和MSA—C型组韵UPDRS运动评分平均最大改善率均显著低于PD组，MSA-P型组高于MSA-C型组。MSA-P型组患者随服用左旋多巴／苄丝肼剂量增加UPDRS评分平均最大改善率呈逐渐增高趋势，而MSA-C型不同剂量间UPDRS评分平均最大改善率差异无统计学意义。结论MSA-P型组具有剂量依赖的急性多巴反应性，而MSA-C型组基本无急性多巴反应性。     

帕金森病与阿尔茨海默病

普遍认为帕金森病和阿尔茨海默病是两个独立的、有着显著判别的疾病。但是,相当数量为证据表明,二者具有相互重叠的临床和神经病理特征、相似的病因和病变发生机制。因此,现在有人认为这两种疾病的部分病例可能是同一种神经元退行性变疾病的不同表现形式。本文拟对此作一介绍。     

Alzheimer''''s病和Binswanger''''s病的临床对照研究

目的 探讨Alzheimer's和Binswanger's病的临床鉴别点。方法 对40例Binswanger's病人和20例AD病人的临床症状和CT资料进行对照分析。结果 Binswanger's病组多有卒中、高血压、头昏或晕厥史,自知力多保留,CT特点为脑室周围白质低密度区(WMLA)和多发腔隙性梗塞(LI);AD组多无上述病史,多有情感改变和精神障碍及失语和失认,多无自知力,无局灶性神经系统体征,CT主要表现为皮层萎缩,侧脑室容积大。结论 鉴别是Binswanger's病还是AD应注意两点:一是神经系体征与精神障碍是否同时发生;二是检查是否有多发性脑梗塞和WMLA。     

Prevalence of radiological and clinical cerebrovascular disease in idiopathic Parkinson's disease

Comorbid cerebrovascular disease (CVD) can occur in idiopathic Parkinson's disease (IPD) but its reported prevalence varies considerably. CVD may alter the clinical presentation, course and prognosis in IPD. We aimed to determine the prevalence of radiological and clinical CVD in a neurology clinic IPD population. We undertook a retrospective case-control study of neurology clinic patients with IPD for whom cerebral imaging was available, and excluding probable vascular parkinsonism. IPD diagnosis was validated against UK PDS brain bank criteria. Age and sex-matched controls were identified from patients attending neurology clinics with headache. The presence of radiological cerebrovascular disease, symptomatic CVD (stroke and TIA), and CVD risk factors was recorded for cases and controls. Radiological findings were validated by an experienced consultant neuroradiologist using a structured proforma. Eighty-five cases and 85 controls were studied, based on the number of cases for whom brain imaging existed (CT in 50, MRI in 35) and the number of cases for whom suitable controls could be identified. Indications for brain imaging amongst cases were varied. Cases and controls comprised 55(65%) males, mean (±SD) age (years) 67.4 ± 10.1 (cases), 66.6 ± 9.9 (controls). Radiological CVD was significantly commoner amongst cases (39%) than controls (22%) (p = 0.02, chi-square; odds ratio 2.2, 95% CI: 1.1-4.6). Cases also had significantly more symptomatic CVD, but not CVD risk factors, than controls. Our findings suggest a higher prevalence of radiological and clinical CVD in patients with IPD compared to controls.     

An epidemiological survey of moyamoya disease,unilateral moyamoya disease and quasi-moyamoya disease in Japan

Objectives

Moyamoya disease (MMD) is a unique occlusive disease of the bilateral internal carotid arteries in which, compensation for occlusion results in an enrichment of collateral arteries at the base of the brain. However, the epidemiology of unilateral MMD (typical angiographic evidence of MMD unilaterally with equivocal contralateral findings), and quasi-MMD (MMD present with inherited or acquired disorders) is poorly known. Here, a nationwide epidemiological survey was conducted to estimate the total numbers of patients, the annual incidence rates and prevalences of MMD, unilateral MMD and quasi-MMD in Japan.

Patients and methods

The neurosurgery, neurology and pediatrics departments that were listed in Japanese resident training programs were recruited to participate in this survey. Questionnaires were directly mailed to 2998 departments in February 2006.

Results

A total of 1183 departments replied to the questionnaire (39.5% response rate). It was estimated that there were 6670.9 MMD patients, 840.5 unilateral MMD patients and 430.4 quasi-MMD patients in Japan. The annual incidence rates of MMD, unilateral MMD and quasi-MMD are 1.13, 0.23 and 0.11/100,000, respectively, and the prevalences are 5.22, 0.66 and 0.34/100,000, respectively. These patients were mainly treated by neurosurgeons. An estimated total of 929.1 surgical interventions are performed in Japan annually.

Conclusion

This nationwide study reports the current epidemiologic status of MMD, unilateral MMD and quasi-MMD in Japan.     

Intracranial atherosclerotic disease

Atherosclerotic disease of the intracranial arteries is responsible for at least 10% of ischaemic strokes worldwide. Symptomatic disease has been extensively studied in the past few years, using diagnostic methods including multi‐slice computed tomography and high resolution magnetic resonance imaging. A literature search was performed using PubMed and OvidSP between 1984 and May 2013. Variations of the terms ‘intracranial atherosclerosis’ plus ‘ischemic stroke’, ‘plaque’, ‘morphology’, ‘imaging’ were used and a combination of them. The reference lists of identified articles were also consulted for additional references. Amongst symptomatic patients the prevalence of intracranial atherosclerotic disease is around 10%, depending on race ethnicity, and the diagnosis requires the presence of ≥50% stenosis in the territory of the symptomatic vessel in a patient with stroke or transient ischaemic attack. The prognosis of intracranial atherosclerotic disease related stroke is poor. Although risk factor control can lead to a better outcome of intracranial atherosclerotic disease related strokes, the significance of asymptomatic disease is still a matter of debate.     

Downward finger displacement distinguishes Parkinson disease dementia from Alzheimer disease

Purpose/Aim of the study: To study finger displacement in patients with Parkinson disease dementia (PDD) and in patients with Alzheimer disease (AD).

Methods: We examined 56 patients with PDD and 35 with AD. Patients were examined during their regular outpatient clinic visit. Finger displacement was measured by observers not actively involved in the study using a creative grid ruler for all PDD and AD patients. Finger displacement was examined by asking patients to point their index fingers toward the grid ruler with the nails facing upward. Patients were asked to maintain the pointing position for 15 s. After 15 s, patients were asked to close their eyes for another 15 s while maintaining the same position. A positive result was downward index finger displacement of ≥5 cm within the 15-second time window with eyes closed.

Results: Of the 56 PDD patients, 53 had bilateral finger displacement of >5 cm. In comparison, of the 35 AD patients, only 1 patient had minimal displacement.

Conclusions: Results of the non-invasive finger displacement test may provide insight, on an outpatient basis, of the integrity of subcortical–cortical circuits. Downward finger displacement, especially bilateral downward displacement, may signal the extensive disruption of subcortical–cortical circuits that occurs in PDD patients.

Abbreviations: AChE: acetylcholinesterase; AD: Alzheimer disease; DLB: dementia with Lewy bodies; ET: essential tremor; MDS-UPDRS: Movement Disorder Society–sponsored Unified Parkinson's Disease Rating Scale; MMSE: Mini-Mental State Examination; PD: Parkinson disease; PDD: Parkinson disease dementia     

Genetically confirmed Huntington's disease masquerading as motor neuron disease

We describe a patient with Huntington's disease (HD) who showed asymmetrical upper limb amyotrophy as a main manifestation. Chorea and psychiatric symptoms were not prominent. Electromyography revealed generalized active and chronic denervation and fasciculations. A genetic test showed 46 CAG repeats in the huntingtin gene. Asymmetrical amyotrophy restricted to the upper limb has been reported in some patients with progressive chorea and amyotrophy without acanthocytosis, but genetically proven cases of HD have rarely been reported. It is not known why only a few HD patients show the motor neuronal loss; however, certain as‐yet‐unidentified genetic factors combined with some environment factors and the underlying cellular dysfunctions by polyglutamine aggregation could be responsible for the motor neuronal loss similar to that in amyotrophic lateral sclerosis. © 2008 Movement Disorder Society     

平山病误诊运动神经元病二例临床分析

目的探讨平山病诊断要点,提高临床医生对该病认识,降低误诊率。方法报道我院近两年被误诊为运动神经元病的2例平山病病例,结合文献复习就平山病与运动神经元病的临床特点、神经电生理特性以及影像学诊断要点进行总结分析和鉴别。结果 :结合文献报道分析,平山病平均起病年龄16.91±2.47岁,男女比18.6:1,均表现以上肢远端受累为主的肌无力和肌萎缩,其中寒冷麻痹72.3%,手伸展震颤74.3%,肌电图示神经源性异常肌电改变,屈颈位颈椎MR有特征性改变:硬膜外腔见月牙形异常信号或流空血管影。结论 "寒冷麻痹及手平举震颤",是平山病临床表现,也是避免误诊的重要鉴别要点。颈椎MR特征性改变对确诊平山病具有重要价值。