脑干梗死后头颈部Holmes震颤1例报告

目前脑血管病诊疗关注的重点仍是急性期的诊治,对于急性期后患者的一些迟发性改变有些认识不足.Holmes震颤又名中脑震颤、红核震颤或丘脑震颤,是脑血管病后少见的运动障碍,在 1889 年由 Benedikt 首次报道, 1904 年由Holmes详细描述,是一种<4. 5 Hz的低频震颤,振幅较大,表现为静止性、意向性的...     

特发性震颤的临床和电生理学特点

目的 探讨特发性震颤(EI)的临床和电生理学特点.方法 回顾性分析并比较33例ET患者(ET组)和30例生理性震颤患者(对照组)的震颤类型、程度、幅度和负重对其的影响,以总结ET的临床和电生理学特点.结果 ET组患者动作性震颤(KT)的震颤程度明显高于对照组,震颤幅度明显大于对照组(均P＜0.01);而姿势性震颤(PT...     

特发性震颤和痉挛性斜颈的头部震颤的临床和电生理研究

目的探讨痉挛性斜颈(CD)和特发性震颤(ET)的头部震颤的临床和电生理特点。方法对17例确诊特发性震颤(ET)和15例确诊痉挛性斜颈(CD)并且具有头部震颤的患者行肌电图震颤分析研究,并探讨两种疾病头部震颤的各自特点。结果 ET组平均年龄(59.58±2.78)岁,平均病程(100.94±31.46)个月,男性4例,女性13例,8例有ET家族史,头部抖动摇头型14例,点头型3例,混合型1例,17例ET患者均有手抖,2例下肢抖动,1例下颌抖动。静止震颤未引出,姿势震颤频率(7.45±0.34)Hz,负重震颤频率(7.52±0.39)Hz,头部震颤评分总分(3.39±0.22)分,平卧分数0分,站立分数(1.29±0.11)分,时间分数(2.41±0.19)分;CD组平均年龄(46.2±3.15)岁,平均病程83.2±21.79月,男性7例,女性8例,2例有ET家族史,头部抖动摇头型9例,点头型4例,混合型2例,4例CD患者有手抖,0例下肢抖动,0例下颌抖动。静止震颤频率(5.55±0.18)Hz,姿势震颤频率(5.58±0.19)Hz,负重震颤频率(5.88±0.23)Hz,头部震颤评分总分(5.67±0.33)分,平卧分数(1.6±0.16)分,站立分数(2.0±0.14)分,时间分数(2.07±0.15)分。CD的头部震颤存在感觉诡计和转颈后特定位置的震颤加重。统计学分析提示ET和CD在发病年龄、姿势性震颤和负重震颤频率、震颤评分总分及平卧分数、站立分数差异均有统计学意义。P值分别为0.033,0.000,0.001,0.000,0.000.0.000。结论痉挛性斜颈比较特发性震颤就诊年龄更年轻,存在静止性震颤,姿势性和负重震颤频率更慢,头部震颤评分的总分和平卧分数及站立分数均较高。并且可以存在感觉诡计及转颈后加重。     

磁共振测量特发性震颤和帕金森病患者基底节区核团体积的研究

目的：利用磁共振体积测量技术评价特发性震颤（ET）,帕金森病（PD）患者基底节区核团体积的变化及互相间的差异。方法：采用1．5T磁共振机测量9例ET患者、5例PD患者和8例年龄匹配正常人全脑体积、尾状核和壳核体积。比较各组之间感兴趣区体积的差异。结果：PD组双侧尾状核标化体积之和、双侧壳核标化体积之和较正常人缩小（P〈0．05）。ET组双侧尾状核标化体积,双侧壳核标化体积与正常对照组无差别。结论：PD患者存在尾状核和壳核体积的缩小,而ET患者无明显尾状核和壳核体积变化。     

多系统萎缩患者的临床、影像及电生理分析

目的 探讨多系统萎缩(multiple system atrophy,MSA)的临床、影像以及电生理改变特点,为临床诊断提供依据.方法 对62例MSA患者的临床资料、神经影像学以及肌电图检查结果进行回顾性分析.结果 我们共收集拟诊MSA患者62例,其中MSA-A型29例(46.8 % ),主要临床特点表现为直立性低血压为主的自主神经系统症状;MSA-C型24例(38.7 % ),主要表现为小脑性共济失调;MSA-P型9例(14.5 % ),则以锥体外系症状为主.头颅MRI显示MSA-A型患者部分出现小脑病灶;MSA-C型患者主要病变在小脑、脑桥和延髓;MSA-P型患者病变主要在壳核.51例患者行肛门括约肌肌电图(external anal sphincter electromyography,EAS-EMG)检查,其中46例示典型神经源性损害;19例(30.6 % )曾被误诊为其他疾病.结论 MSA早期易漏诊或误诊,结合临床表现、神经影像学以及EAS-EMG检查,可提高MSA的诊断率.     

多系统萎缩患者的临床、影像及电生理分析

目的 探讨多系统萎缩(multiple system atrophy,MSA)的临床、影像以及电生理改变特点,为临床诊断提供依据.方法 对62例MSA患者的临床资料、神经影像学以及肌电图检查结果进行回顾性分析.结果 我们共收集拟诊MSA患者62例,其中MSA-A型29例(46.8 % ),主要临床特点表现为直立性低血压为主的自主神经系统症状;MSA-C型24例(38.7 % ),主要表现为小脑性共济失调;MSA-P型9例(14.5 % ),则以锥体外系症状为主.头颅MRI显示MSA-A型患者部分出现小脑病灶;MSA-C型患者主要病变在小脑、脑桥和延髓;MSA-P型患者病变主要在壳核.51例患者行肛门括约肌肌电图(external anal sphincter electromyography,EAS-EMG)检查,其中46例示典型神经源性损害;19例(30.6 % )曾被误诊为其他疾病.结论 MSA早期易漏诊或误诊,结合临床表现、神经影像学以及EAS-EMG检查,可提高MSA的诊断率.     

多系统萎缩患者的临床、影像及电生理分析

目的 探讨多系统萎缩(multiple system atrophy,MSA)的临床、影像以及电生理改变特点,为临床诊断提供依据.方法 对62例MSA患者的临床资料、神经影像学以及肌电图检查结果进行回顾性分析.结果 我们共收集拟诊MSA患者62例,其中MSA-A型29例(46.8 % ),主要临床特点表现为直立性低血压为主的自主神经系统症状;MSA-C型24例(38.7 % ),主要表现为小脑性共济失调;MSA-P型9例(14.5 % ),则以锥体外系症状为主.头颅MRI显示MSA-A型患者部分出现小脑病灶;MSA-C型患者主要病变在小脑、脑桥和延髓;MSA-P型患者病变主要在壳核.51例患者行肛门括约肌肌电图(external anal sphincter electromyography,EAS-EMG)检查,其中46例示典型神经源性损害;19例(30.6 % )曾被误诊为其他疾病.结论 MSA早期易漏诊或误诊,结合临床表现、神经影像学以及EAS-EMG检查,可提高MSA的诊断率.     

多系统萎缩患者的临床、影像及电生理分析

目的 探讨多系统萎缩(multiple system atrophy,MSA)的临床、影像以及电生理改变特点,为临床诊断提供依据.方法 对62例MSA患者的临床资料、神经影像学以及肌电图检查结果进行回顾性分析.结果 我们共收集拟诊MSA患者62例,其中MSA-A型29例(46.8 % ),主要临床特点表现为直立性低血压为主的自主神经系统症状;MSA-C型24例(38.7 % ),主要表现为小脑性共济失调;MSA-P型9例(14.5 % ),则以锥体外系症状为主.头颅MRI显示MSA-A型患者部分出现小脑病灶;MSA-C型患者主要病变在小脑、脑桥和延髓;MSA-P型患者病变主要在壳核.51例患者行肛门括约肌肌电图(external anal sphincter electromyography,EAS-EMG)检查,其中46例示典型神经源性损害;19例(30.6 % )曾被误诊为其他疾病.结论 MSA早期易漏诊或误诊,结合临床表现、神经影像学以及EAS-EMG检查,可提高MSA的诊断率.     

多系统萎缩患者的临床、影像及电生理分析

目的 探讨多系统萎缩(multiple system atrophy,MSA)的临床、影像以及电生理改变特点,为临床诊断提供依据.方法 对62例MSA患者的临床资料、神经影像学以及肌电图检查结果进行回顾性分析.结果 我们共收集拟诊MSA患者62例,其中MSA-A型29例(46.8 % ),主要临床特点表现为直立性低血压为主的自主神经系统症状;MSA-C型24例(38.7 % ),主要表现为小脑性共济失调;MSA-P型9例(14.5 % ),则以锥体外系症状为主.头颅MRI显示MSA-A型患者部分出现小脑病灶;MSA-C型患者主要病变在小脑、脑桥和延髓;MSA-P型患者病变主要在壳核.51例患者行肛门括约肌肌电图(external anal sphincter electromyography,EAS-EMG)检查,其中46例示典型神经源性损害;19例(30.6 % )曾被误诊为其他疾病.结论 MSA早期易漏诊或误诊,结合临床表现、神经影像学以及EAS-EMG检查,可提高MSA的诊断率.     

多系统萎缩患者的临床、影像及电生理分析

目的 探讨多系统萎缩(multiple system atrophy,MSA)的临床、影像以及电生理改变特点,为临床诊断提供依据.方法 对62例MSA患者的临床资料、神经影像学以及肌电图检查结果进行回顾性分析.结果 我们共收集拟诊MSA患者62例,其中MSA-A型29例(46.8 % ),主要临床特点表现为直立性低血压为主的自主神经系统症状;MSA-C型24例(38.7 % ),主要表现为小脑性共济失调;MSA-P型9例(14.5 % ),则以锥体外系症状为主.头颅MRI显示MSA-A型患者部分出现小脑病灶;MSA-C型患者主要病变在小脑、脑桥和延髓;MSA-P型患者病变主要在壳核.51例患者行肛门括约肌肌电图(external anal sphincter electromyography,EAS-EMG)检查,其中46例示典型神经源性损害;19例(30.6 % )曾被误诊为其他疾病.结论 MSA早期易漏诊或误诊,结合临床表现、神经影像学以及EAS-EMG检查,可提高MSA的诊断率.     

Therapeutic Advances in the Treatment of Holmes Tremor: Systematic Review

ObjectiveWe aimed to formulate a practical clinical treatment algorithm for Holmes tremor (HT) by reviewing currently published clinical data.Materials and MethodsWe performed a systematic review of articles discussing the management of HT published between January 1990 and December 2018. We examined data from 89 patients published across 58 studies detailing the effects of pharmacological or surgical interventions on HT severity. Clinical outcomes were measured by a continuous 1-10 ranked scale. The majority of studies addressing treatment response were case series or case reports. No randomized control studies were identified.ResultsOur review included 24 studies focusing on pharmacologic treatments of 25 HT patients and 34 studies focusing on the effect of deep brain stimulation (DBS) in 64 patients. In the medical intervention group, the most commonly used drugs were levetiracetam, trihexyphenidyl, and levodopa. In the surgically treated group, the thalamic ventralis intermedius nucleus (VIM) and globus pallidus internus (GPi) were the most common brain targets for neuromodulation. The two targets accounted for 57.8% and 32.8% of total cases, respectively. Overall, compared to the medically treated group, DBS provided greater tremor suppression (p = 0.025) and was more effective for the management of postural tremor in HT. Moreover, GPi DBS displayed greater benefit in the resting tremor component (p = 0.042) and overall tremor reduction (p = 0.022).ConclusionsThere is a highly variable response to different medical treatments in HT without randomized clinical trials available to dictate treatment decisions. A variety of medical and surgical treatment options can be considered for the management of HT. Collaborative research between different institutions and researchers are warranted and needed to improve our understanding of the pathophysiology and management of this condition. In this review, we propose a practical treatment algorithm for HT based on currently available evidence.     

Moyamoya病的临床表现与影像学特点

目的 回顾分析12例Moyamoya病的临床和影像学特征，探讨Moyamoya病的影像学诊断价值。方法 分析12例Moyamoya病患的临床资料及数字减影血管造影(DSA)、磁共振血管造影(MRA)、磁共振成像(MRI)和CT结果。结果 所有病例均表现有颈内动脉或其分支不同程度的狭窄或闭塞和颅底异常血管网(MMD血管)；其中病变呈双侧8例．单侧4例。12例中头颅CT表现有梗塞灶4例，脑出血5例，其余3例表现正常。结论 除DsA外．MRI和MRA是两种可以很好评价Moyamoya病的影像学方法。若儿童或青壮年发生脑血管病，反复出现脑梗死或出现脑室出血、脑叶出血或蛛网膜下腔出血(SAH)则要考虑Moyamoya病的可能。     

脑血管疾病的磁共振诊断

目的为提高MRI及MRA对脑血管病诊断的准确性。方法 对120例颅脑MRI和MRA资料，包括自旋回波T1WI、T2WI和3D TDF(MOTSA法)MRA及3D重建(包括MIP及Navigator)图像进行回顾性分析，并对正常脑血管、动脉瘤、血管畸形及动脉硬化的MR表现进行比较。结果 3DTOFMRA可显示较细的血管分支，原始图像对末梢血管显示更加清楚。较小动脉瘤常规MR显示困难，MRA的MIP像部分显示，所见动脉瘤均可借助原始图像上作出诊断；Navigator技术可进一步观察动脉瘤颈部开口和瘤腔内壁；对已行GDC栓塞治疗后的动脉瘤，MRA原始图像可提示治疗后瘤体是否完全闭塞或再通。小血管畸形尤其静脉畸形诊断较困难，C,d—DTPA增强后可显示。MRI可显示硬化血管腔狭窄、血管壁增厚或粥样斑块，MRA结合Navigator，可进一步发现血管壁的粥样硬化斑块和血管腔狭窄程度。结论MRI和MRA相结合，尤其对MRA原始图像观察和Navigator技术的应用，对小动脉瘤、血管畸形以及动脉粥样硬化的诊断准确性均可进一步提高。     

Long-Term Outcome and Neuroimaging of Deep Brain Stimulation in Holmes Tremor: A Case Series

BackgroundDifferent deep brain stimulation (DBS) targets have been suggested as treatment for patients with pharmacologically refractory Holmes tremor (HT). We report the clinical and quality of life (QoL) long-term (up to nine years) outcome in four patients with HT treated with DBS (in thalamic ventral intermediate nucleus-VIM or in dentato-rubro-thalamic tract-DRTT).Materials and MethodsThe patients underwent routine clinical evaluations before and after DBS (typically annually). Tremor severity and activities of daily living (ADL) were quantified by the Fahn-Tolosa-Marin Tremor-Rating-Scale (FTMTRS). QoL was assessed using the RAND SF-36-item Health Survey (RAND SF-36). In addition, we computed, in all four patients, the VTA based on the best stimulation settings using heuristic approaches included in the open source toolbox LEAD-DBS.ResultsIn all patients, tremor and ADL improved significantly at one-year post-DBS follow-up (34–61% improvement in FTMTRS total score compared to baseline). In three out of four patients, the improvement of tremor was sustained no longer than two to three years and only in one patient was sustained up to nine years. In this patient, the largest intersection between VTA and DBS target has been observed. Scores for ADL deteriorated over the course of time, reaching worse levels compared to baseline already during the three-year post-DBS follow-up, in three out of four patients. Physical and mental health component scores of RAND SF-36 had very different outcome between patients and follow-ups and were not associated with tremor-related outcomes.ConclusionsThe benefits of DBS in HT might not be always long lasting. Although QoL slightly improved, this change seemed to be independent of the motor outcome following DBS. The estimation of DBS target and VTA proximity could be a useful tool for DBS clinicians in order to facilitate the DBS programming process and optimize DBS treatment.     

Case of Simple Partial Status Epilepticus in Occipital Lobe Epilepsy Misdiagnosed as Migraine: Clinical, Electrophysiological, and Magnetic Resonance Imaging Characteristics

Summary: A 31–year-old man had a unique form of occipital lobe epilepsy. Since age 13 years, he has had episodes of simple partial status epilepticus (SE) occurring twice a month. These typically consisted of elementary visual hallucinations of flashing lights obscuring his left visual field for a period of 2 days, associated with a severe frontal headache initially diagnosed as migraine. These episoaes of simple partial SE then evolved to a complex partial seizure (CPS) or secondarily eneralized seizure. There were unique EEG features, including: (a) the perception of a flash of light in the left visual field with a single sharpislow wave discharge over the right occipital lobe, (b) right occipital lobe epileptiform activity during the prolonged aura, and (c) an abnormal response to photic stimulation, with occipital lobe discharges during low rates of stimulation (3–5 Hz), time-locked to the stimzlus. High-resolution magnetic resonance imaging (MRI) with quantitative morphometry demonstrated that the right hemisphere and right caudate nucleus were smaller than those on the left. An abnormal gyral pattern was also noted over the right parietal region. Occasionally, distinguishing occipital lobe epilepsy from migraine may be difficult.     

多巴胺转运体显像对特发性震颤与早期帕金森病的鉴别诊断价值

目的:探讨９９ｍＴｃ－ＴＲＯＤＡＴ－１ ＳＰＥＣＴ多巴胺转运体(ＤＡＴ)显像对特发性震颤(ＥＴ)、早期帕金森病(ＰＤ)鉴别诊断的价值。方法:对９例ＥＴ,１４例早期ＰＤ,５例ＥＴ合并ＰＤ(ＥＴ－ＰＤ)患者和２０名健康人进行９９ｍＴｃ－ＴＲＯＤＡＴ－１－ＳＰＥＣＴＤＡＴ断层显像。结果:与年龄、性别相配对的正常人相比,ＥＴ患者纹状体(ＳＴ)与小脑(ＣＢ)ＤＡＴ特异性摄取比值(ＳＴ/ＣＢ)无显著性差异,早期ＰＤ和ＥＴ－ＰＤ患者ＳＴ/ＣＢ显著性降低。结论:９９ｍＴｃ－ＴＲＯＤＡＴ－１ＳＰＥＣＴ ＤＡＴ显像有助于ＥＴ、早期ＰＤ的鉴别诊断。     

可逆性后部白质脑病综合征的临床和影像学特点

目的 探讨可逆性后部白质脑病综合征（reversible posterior leukoencephalopathy syndrome,RPLES）的 临床和影像学特点。 方法 回顾性分析中国人民解放军第306医院4例RPLES的临床症状及影像学特点。 结果 本组病例病因包括肾功能不全,高血压病,妊娠子痫,腰椎手术后。所有病例均有癫痫大发作 及头痛,恶心呕吐,精神行为异常。其中肢体瘫痪1例,失语1例,癫痫持续状态1例。影像学表现为单 侧或双侧以顶枕叶为主白质及灰质病变,病变范围可累及额颞叶。磁共振成像（magnetic resonance imaging,MRI）表观弥散系数（apparent diffusion coefficient,ADC）、T2加权像、平扫液体衰减反转恢复 序列（fluid attenuated inversion recovery,FLAIR）均呈高信号,T1加权像呈略长信号,1例磁敏感成像上 呈微出血。 结论 头痛和癫痫发作是RPLES的主要临床症状,多种病因可引起RPLES,影像学改变以皮质及皮质 下水肿为主。     

烟雾病的临床及影像研究

目的:研究烟雾病(moyamoya disease,MMD)的临床及影像学特征。方法:回顾性分析27例烟雾病,观察其临床及影像学特点。结果:本组病例多为儿童及青壮年起病,女性居多。病程为多发,主要有脑梗死、出血和短暂脑缺血发作等起病形式,临床表现为肢体麻木、无力、头痛、智力减退等症状;DSA在脑底动脉环发现血管闭塞、狭窄及烟雾状毛细血管网形成,MRA对本病有一定阳性发现。结论:对于儿童及青壮年反复发作的脑卒中患者要考虑MMD的可能,目前仍依靠数字减影血管造影确诊,MRA对本病有一定阳性发现。