脑干梗死后头颈部Holmes震颤1例报告

正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、意向性的震颤或姿势性震颤,通常在发病后1～24个月起病~([1])。Holmes震颤的病因包括头部的出血、缺血、创伤、肿瘤、代谢紊乱和感染,其中脑血管意外最常见,据Raina~([2])等对29例Holmes震颤的回顾性分析发现,脑血管意外占14例(48.3%)。现有的少数病例报告中,Holmes震颤多表现为肢体的震颤,表现为头颈部震颤的极少见,现就对1例近期收治的脑干梗死后头颈部Holmes震颤进行报道,     

临床不同分期帕金森病患者震颤特点分析

目的研究不同临床分期帕金森病(PD)患者震颤的特点。方法收集2014-11—2015-05首都医科大学附属北京天坛医院神内病房临床确诊或临床诊断PD可能性大的75例患者。患者至少具有一侧上肢静止性或姿势性震颤,按照Hoehn-Yahr分期分为1~1.5期、2~2.5期、3期3组,分别检测各组患者静止、姿势及持物1000g震颤的优势频率、振幅及震颤节律形式。结果 (1)震颤优势频率:静止、姿势状态下为4~7 Hz,1~1.5期与2~2.5期、3期组间比较差异无统计学意义(P0.05)。持物1000g状态下,1~1.5期与2~2.5期、3期组间比较差异有统计学意义(P0.01),1~1.5期患者优势频率除了4~7Hz之外,还有7~10Hz、无规律及无震颤。(2)震颤振幅:随病情进展有下降趋势。(3)震颤节律形式:临床不同分期患者静止、姿势及持物1000g时震颤的节律形式均以交替形式为主,但随着病情进展,非交替节律比例有增加趋势。结论 PD患者震颤属于中枢性震颤,静止、姿势震颤优势频率为4~7Hz,不随病情进展而变化。疾病初期,持物1000g状态震颤优势频率可能受外周调节而表现多样。震颤节律不受病情进展及姿势影响。随病情进展,非交替节律比例有增加趋势,震颤振幅有下降趋势。     

多模式MRI对线粒体脑肌病的诊断价值

目的探讨线粒体脑肌病多模式MRI影像学特点。方法对21例线粒体脑肌病患者的脑MRI、MRA、MRS影像资料进行分析。结果 MRI：线粒体脑肌病病灶多位于颞、枕、顶叶,病灶具有不符合脑动脉分布及游走性的特点;4例患者病灶对称性累及豆状核、丘脑和（或）尾状核;2例患者大脑、小脑、脑干萎缩,脑室增大,脑沟增宽。病灶均呈T1低信号、T2高信号,陈旧性病灶T2信号稍高;MRA：10例新发病灶处脑动脉及其远端分支较对侧相应脑动脉增粗,信号增强;MRS：14例新鲜病灶可检出倒置的乳酸双峰波及NAA/Cr降低。结论线粒体脑肌病的多模式MR影像学特点有一定的特异性,对线粒体脑肌病的诊断有一定价值。     

帕金森病患者丘脑腹外侧核团神经元的电活动特点

目的：探讨丘脑腹外侧核（VL）神经元电活动与帕金森性震颤的关系。方法：应用微电极记录和肌电记录技术。对19例帕金森性震颤患者实施立体定向Vim切开术的同时,对Vim的神经元电信号和对侧肢体肌电活动进行记录。电信号的采集和放大器用四通道微电极放大器系统及PolyView软件,采样频率为7.5kHz。电极阻抗在0.1至0.5MΩ,数据分析包括：神经元放电频率,幅度,放电间期,神经元和肌电活动的相关性。结果：在19个针道记录到189个神经元簇,其中簇辨电活动与肢体姿势性震颤一致的有78个,占41%,这78个VL神经元放电活动与肢体震颤（4-6Hz)的相关系数为R^2=0.68。单细胞分析表明78个震颤细胞群放电频率在6-16Hz,平均放电频率8Hz(n=78)。另外101VL神经元族（59%）紧张型放电在6-35Hz之间,其中19个Vim神经元（19）与运动刺激相关,而16个Vc神经元（16%）与触觉相关,结论：VL核团作为皮层-丘脑-基底节环路的重要中继站,接受来自基底节的输入,参与了原发性帕金森震颤的发生发展。     

丘脑出血后Holmes震颤伴偏瘫1例报告

正Holmes震颤(Holmes tremble,HT)也称丘脑震颤、红核震颤,是一种少见的临床综合征,主要原因为脑血管病[1],通常在原发病后0.5~24个月发生。HT临床表现为以病灶对侧上肢为主的低频的静止性震颤、姿位性震颤和意向性震颤中1种或多种组合,亦可伴有脑损伤的其他症状[2]。HT文献报道较少,现报告我院2018年1月确诊的、经盐酸苯海索联合左乙拉西坦治疗后症状稍改善的1例HT病例。     

儿童流行性乙型脑炎MRI及扩散加权成像早期诊断的优势

目的探讨儿童乙脑MRI影像特点及早期扩散加权成像价值,以提高对乙脑影像征象认识及早期检出。方法回顾性分析2006-2013年经我院临床诊治的27例乙脑患者,全部病例均为血清IgM乙脑抗体阳性。磁共振扫描仪采用GE 1.5T超导磁共振成像扫描仪。其中20例在发病至神经系统出现症状7d内应用DWI。结果 27例儿童乙脑中24例累及丘脑,13例累及中脑黑质,6例累及皮层或皮层下白质,4例累及基底节,2例累及海马,2例累及胼胝体压部。20例在急性期(7d内)应用DWI检查:8例为细胞毒性水肿,DWI显示病灶范围、信号强度均优于T2WI、FLAIR,其中DWI单独显示3例。7例血管源性水肿,T2WI、FLAIR优于DWI。3例脑内多发病灶内两种水肿共存,DWI与T2WI、FLAIR在显示病灶能力中互有优势。结论儿童乙脑好累及双侧丘脑、中脑黑质,影像学具有一定特征,早期DWI比T2WI、FLAIR更易发现病灶。     

基底节波谱成像对帕金森病及帕金森综合征的鉴别诊断价值

<正>帕金森病(PD)为中老年常见的神经系统变性疾病,临床表现以静止性震颤、肌强直、运动型迟缓、姿势步态异常为主要特征,临床诊断主要靠病史、体检。因脑退行性变而影响到锥体外系并引起震颤麻痹症状或类似震颤麻痹症状称为帕金森综合征(PDS)。由于很多疾病都可以引起震颤,因此常易误诊。本文通过对有震颤症状的40例患者行双侧基底节区单体素H-MRS扫描,以探讨其对帕金森病、帕金森     

误诊为帕金森综合征的肌张力障碍25型1例报告

正原发性肌张力障碍是一种主动肌与拮抗肌收缩不协调或过度收缩引起的以异常姿势和动作为特征的锥体外系疾病。多数为散发病例,少数有家族史,原发性肌张力障碍的病因可能与遗传、环境等因素有关。目前关于基因突变对该病的致病作用逐渐被发现和证实。肌张力障碍的临床表现主要包括姿势异常、重复动作等,与特发性震颤、舞     

基底动脉尖综合征的早期临床与影像研究

目的 探讨基底动脉尖综合征(TOBS)的早期临床和影像特点.方法 回顾性分析34例早期(发病72 h内)TOBS的临床资料和影像特点.结果 TOBS常有脑部多个区域梗死的临床表现.影像学特点为病灶具有多发性,常累及幕上、幕下或丘脑双侧,病灶多位于丘脑、中脑、颞叶、枕叶、小脑等.本组34例中,治愈12例,好转16例,死亡...     

帕金森性震颤和与震颤相关的电活动

目的对PD患者行STN和GPi切开术术中应用微电极记录技术采集神经元的电活动,术后分析其与震颤的关系和特点,为手术选择最佳的毁损位置提供客观的电生理指标.方法40个PD患者,其中21例PD患者接受了立体定向GPi切开术和19例PD患者接受立体定向STN切开术.病人要求清醒合作且处于“关”状态.术中应用微电极和肌电(EMG)记录技术,采集GPi和STN神经元和手术对侧肢体震颤的生物电活动.术后应用分析软件甄别单细胞及其电活动特点,分析其与震颤症状的关系,并进行相关性检验.结果在21个针道共记录到184 GPi个神经元单位,其簇状放电的节律与肢体震颤的节律高度一致(4～6Hz),R2=0.78(P＜0.01).在20个针道共记录到161个STN神经元单位,其放电频率在42～88Hz之间.STN的簇状放电的节律与肢体震颤的节律一致(4～6Hz),R2=0.64(P＜0.01).毁损这些震颤细胞导致震颤症状的消失.结论震颤型PD患者的GPi和STN存在与肢体震颤节律一致的震颤细胞,且震颤和震颤细胞有着内在的关系.对于指导手术毁损的部位和范围提供了可靠的依据.     

Clinical and magnetic resonance imaging manifestations of Holmes tremor

Holmes tremor is a rare symptomatic slow tremor in the proximal parts of the limbs. It may be present at rest or maintenance of a posture, or during the movement of the affected limb. We describe herein three patients of Holmes tremor with possible etiologies of brainstem infarction and head injury. The intervals between the causal events and the appearance of tremor range from 1 month to 12 months. Magnetic resonance imaging studies reveal hypertrophy of the inferior olivary nucleus in all of the three patients, although only one of them has palatal myoclonus. The surface electromyographic recordings reveal characteristic slow oscillation with frequencies of 3.5 to 4.2 Hz. These features suggest that perturbation of the dentato-rubral-olivary circuitry may play a pivotal role for the generation of Holmes tremor. However, no tight correlation is observed between the presence of inferior olivary nuclear hypertrophy and the appearance of symptomatic palatal myoclonus in the current report.     

Effects of thalamic stimulation frequency on intention and postural tremor

Deep brain stimulation (DBS) of the ventral intermediate nucleus (VIM) of the thalamus improves essential tremor. Suppression of the amplitude of the postural tremor component with VIM DBS depends on stimulation frequency. The purpose of this study was to determine the effect of DBS frequency on the intention tremor component, that is, tremor that is enhanced by target-directed movement, and to compare it to the effect of DBS frequency on postural tremor in people with essential tremor. We measured tremor frequency and amplitude during trials of postural holding and voluntary reaching between two targets at 10 different stimulation frequency settings between 0 and 185 Hz. Tremor frequency did not change with changes in stimulation frequency. Amplitude suppression of both intention and postural tremor depended on stimulation frequency. Maximal tremor reduction occurred at approximately 130 Hz for both forms of tremor. However, at optimal frequencies, the percent reduction in tremor amplitude relative to the DBS OFF condition was greater for postural than for intention tremor. These results suggest that VIM DBS stimulation frequencies near 130 Hz may provide maximal control of intention and postural tremor. Identification of optimal stimulation settings should consider assessment of intention tremor, not just postural tremor, as intention tremor may not be as well controlled as postural tremor but may be a better gauge for functional benefit.     

Clinical and electromyographic examinations of Parkinsonian tremor

Background: The clinical presentations of postural Parkinsonian tremor are variable and different types of tremors have been described. The aim of this study was to re-evaluate the clinical and electromyographic (EMG) pattern of different tremors in Parkinsonian patients.

Methods: One hundred and ten patients with Parkinsonian tremor were included in the study. Patients were subdivided into four groups according to the presence or absence of postural tremor, in addition to a resting tremor and its EMG pattern. The first group consisted of patients without postural tremor. The second group consisted of patients with fast postural tremor (>7 Hz). The third group consisted of patients with slow postural tremor with alternating EMG activity. Patients with slow postural tremor with synchronous EMG activity were included in the fourth group. In each limb position, the tremor of the most involved body part was graded on the Webster Tremor Scale. Surface EMG recordings of the most involved limb in all positions were performed.

Results: Postural tremor in addition to the rest one was found in 84% of the patients. The postural tremor was with lower amplitude than the rest one. The frequencies and EMG patterns of the postural tremors were different and correlated with some specific clinical symptoms. Patients with alternating postural tremor had a kinetic and intention tremor in addition.

Conclusions: Four different subtypes of Parkinsonian tremor were found according to the presence and type of postural tremor. These subtypes had some differing clinical characteristics and probably different relationships to essential tremor.     

Holmes’ tremor as a delayed complication of thalamic stroke

Movement disorders are not commonly associated with stroke. Accordingly, thalamic strokes have rarely been associated with tremor, pseudo-athetosis and dystonic postures. We present a 75-year-old man who developed a disabling tremor 1 year after a posterolateral thalamic stroke. This tremor had low frequency (3–4 Hz), did not disappear on focus and was exacerbated by maintaining a static posture and on target pursuit, which made it very difficult to perform basic functions. MRI demonstrated an old ischemic lesion at the left posterolateral thalamus. Treatment with levodopa led to symptom control. Lesions in the midbrain, cerebellum and thalamus may cause Holmes’ tremor. Delayed onset of symptoms is usually seen, sometimes appearing 2 years after the original injury. This may be due to maturation of a complex neuronal network, leading to slow dopaminergic denervation. Further studies are needed to improve our understanding of this unique disconnection syndrome.     

Irregular jerky tremor, myoclonus, and thalamus: a study using low-frequency stimulation.

High-frequency thalamic stimulation alleviates tremor in Parkinson's disease (PD) and essential tremor (ET). The origin of thalamic myoclonus is unexplained and the effects of low-frequency thalamic stimulation on movement control are still unknown. We studied the effects of stimulation at a low frequency of 15 Hz in five drug-free patients (3 PD, 2 ET) 6 months after thalamic implantation of quadripolar electrodes (unilateral in four patients, bilateral in one patient). Clinical, electrophysiological, and videotaped assessment, using a monopolar 15 Hz frequency (3 V, 90 micros) stimulation current applied simultaneously through two adjacent contacts of the electrode, was performed. We observed myoclonus and irregular jerky tremor in the upper limb contralateral to the site of stimulation. The jerks lasted less than 200 ms, were irregular and not synchronous with stimulation, were superimposed on rest or postural tremor, and increased in response to tactile, proprioceptive, or vibratory stimuli. The fact that this complex movement disorder can be induced by low-frequency stimulation in the ventral intermediate nucleus (Vim) of the thalamus suggests that it results, at least partly, from dysfunction of the Vim and possibly adjacent nuclei of the thalamus.     

Holmes tremor: Application of modern neuroimaging techniques.

Holmes tremor has a characteristic rest, intention, and postural component. The syndrome arises as a consequence of a lesion in the upper brainstem and cerebral peduncles, which, it is postulated, interrupts the cerebello-rubrothalamic pathway. Ataxia, ophthalmoplegia, and bradykinesia are associated features. We present a case of Holmes tremor secondary to a midbrain cavernoma. Modern neuroimaging techniques in this case confirm that a combination of damage to the cerebello-rubrothalamic pathway and the nigrostriatal pathway is required for the full Holmes tremor syndrome to occur.     

急性幕上脑出血患者不同时期痫性发作与预后的关系研究

目的 分析脑出血后住院期间不同时期痫性发作患者临床特点,评价不同时间段卒中后痫性发作 与预后的关系。 方法 本研究为回顾性研究,入组人群选自中国卒中登记中既往无癫痫病史的2382例的自发性幕 上脑出血患者。根据患者住院期间脑出血后伴发痫性发作的时间将患者分为无痫性发作组,脑出血 发病同时（发病24 h内）出现的痫性发作（seizures at onset,SAO）组与发病后住院期间出现的痫性发作 （seizures during hospitalization,SDH）组。收集入组患者性别、年龄、既往病史、入院时GCS评分和出血 部位等临床特点及1年后是否死亡的随访信息,对不同时间段发生卒中后痫性发作与1年死亡率的关系 进行分析。 结果 入组患者中无痫性发作患者共2271例,SAO组61例,SDH组50例。SAO组（55.7%）及SDH组 （44％）患者入院时低GCS评分（3～8分）患者比例高于无痫性发作患者组（21.1%）,SAO及SDH组患者 出血部位多数集中在单纯脑叶或脑叶合并深部白质,而无痫性发作组患者出血部位多位于底节区或丘 脑的深部位置,差异有统计学差异（P <0.0001）。无痫性发作患者组1年死亡率最低（25.1%）,SDH组 死亡率最高（56.0%）,差异有统计学差异（P <0.0001）。多因素Logistic分析发现,与无痫性发作患者 相比,SDH是患者一年后死亡的独立危险因素（OR 2.145,95%CI 1.084～4.245,P＝0.029）。 结论 与脑出血后无痫性发作患者相比,发病后住院期间出现的痫性发作是影响患者1年死亡的独 立危险因素。     

脑梗死与脑出血早期鉴别的血清标志物概述

脑梗死是因局部脑组织血液供应障碍而导致脑组织坏死；脑出血则是因为各种原因导致 脑实质内血管破裂出血。虽然二者发病时临床症状有一定相似性，但其发病机制及病理生理过程截 然不同，这种差异可能会导致发病早期血清中一些蛋白及基因表达的差异。目前，文献中报道了几种 可用于鉴别脑梗死及脑出血的血清标志物，并分析了这些标志物用于脑梗死及脑出血早期鉴别诊断 的价值。本文将对这些血清标志物进行综述，希望能够为卒中早期鉴别诊断提供新思路。     

Symptomatological rubral tremor caused by vertebral-basilar artery embolism]

Rubral tremor is a distinct clinical entity as described by Gorden Holmes. We have reported here a 44 years old woman with rubral tremor appearing about 2 months after an embolic attack of vertebral-basilar artery. On neurological examination there were left homonymous hemianopsia, dysarthria very mild weakness of left upper and lower limbs with clumsiness of her left lower limb and the tremor of the left upper limb. Muscle tone was increased in her left upper limb with dystonic posturing. The tremor of her left upper limb was present at rest with regular rhythm of 2.8 Hz. This tremor included the reciprocal movements such as radial and ulnar flexion of the left wrist and independent movements of different fingers. It was accentuated by postural adjustment and by guided voluntary movements and disappeared during sleep. Surface EMG demonstrated that the grouping discharge was seen not only alternatingly but also synchronously between agonists and antagonists. A brain MRI image revealed multiple lesions including right thalamus and left cerebellum. No lesions were detected in brain stem. On the basis of MRI, it was questionable whether the lesion involved the dentate nucleus in the left cerebellum although the lesion was located at the medulla near the dentate nucleus extending from the cortex. So-called rubral tremor could be generated in lesions of cerebello-rubro-thalamic system without rubral lesion itself.