十字征与橄榄脑桥小脑萎缩

十字征(cross sign)即MRI的T2加权像上脑桥的十字形高信号,见于橄榄脑桥小脑萎缩(Olivopontocerebellar atrophy,OP-CA)的患者。我们报道我院收治的1例MRI典型十字征的患者,并结合文献复习分析如下。1临床资料患者,男,60岁,于2004-11-25入院。入院5年前出现行走不稳,动作不灵活,呈进行性加重。近两年来出现口齿不清,吞咽困难,饮水呛咳,不能独自行走。既往家族史无特殊。查体:神清,构音不清,饮水发呛,咽反射消失,蹒跚步态,四肢肌力Ⅴ级,肌张力呈铅管样强直,腱反射(++),双侧巴宾斯基征(-),深浅感觉无障碍,指鼻试验、跟膝胫试验(+),轮替动…     

十字征与橄榄脑桥小脑萎缩

十字征(cross sign)即MRI的T2加权像上脑桥的十字形高信号，见于橄榄脑桥小脑萎缩(Olivopontocerebellar atrophy， OPCA)的患者。我们报道我院收治的1例MRI典型十字征的患者，并结合文献复习分析如下。     

橄榄体脑桥小脑萎缩听觉P300认知电位的研究

目的 观察P300检查在橄榄体脑桥小脑萎缩中的应用价值及影响因素.方法 应用P300检查和认知能力筛选量表(CASI)对34例橄榄体脑桥小脑萎缩患者和与其年龄、性别相匹配的正常对照组进行检测.结果 患者不同年龄组P300潜伏期较对照组明显延长(t=2.682～4.983,P<0.05～0.01),患者不同年龄组CASI评分比正常对照组明显降低(t=2.486～5.241,P<0.01),患者组P300潜伏期与CASI评分之间呈负相关(r=-0.48,P<0.005),病情轻组[(348.68±21.47) ms]与重组[(363.27±30.15) ms]和脑萎缩明显组[(354.46±26.98) ms]与不明显组[(338.74±23.56) ms]之间P300潜伏期均差异有显著意义 (P<0.01).结论 评价橄榄体脑桥小脑萎缩的认知功能状态,P300检查是一项有价值的客观指标,其异常程度与病情轻、重和脑萎缩程度有关.     

橄榄体脑桥小脑萎缩62例临床与MRI诊断研究

橄榄体脑桥小脑萎缩 (ｏｌｉｖｏｐｏｎｔｏｃｅｒｅｂｅｌｌａｒａｔｒｏｐｈｙ,ＯＰＣＡ)是一种原因未明的神经系统变性病。临床影像学的发展 ,大大提高了对该病的诊断率。临床资料 :男 36例 ,女 2 6例。散发型 42例 ,平均发病年龄 5 4 2岁 ,病程 5个月至 10年。遗传型 2 0例 ,平均发病年龄 37 4岁 ,病程 2～ 15年。全部病人均进行头颅ＭＲＩ检查。主要症状均表现为小脑性共济失调 ,并伴有不同程度的脑干损害、锥体束及锥体外系征、智能障碍等症状。遗传学资料 :2 0例遗传型病人属于 13个家系 ,受累总人数为 45例 (包括先证者 )。 11个家…     

类似精神分裂症的橄榄体脑桥小脑萎缩1例

1 病例 患者,男性,33岁,因双手抖动半年,自语,说周围人要害自己2个月而住院.既往体健.其兄患有精神病.     

橄榄脑桥小脑萎缩20例临床分析

目的探讨橄榄脑桥小脑萎缩的临床表现和影像学检查特点,以便早期诊断并改善患者预后。方法回顾性分析经临床诊断的20例橄榄脑桥小脑萎缩患者的相关资料。结果橄榄脑桥小脑萎缩患者临床以小脑性共济失调、言语不清、排尿功能障碍、帕金森症状等最常见。16例患者头颅磁共振检查结果示脑萎缩,主要为小脑和脑干萎缩。结论橄榄脑桥小脑萎缩临床以小脑性共济失调为主要表现,言语障碍较继往报道发生率高,头颅磁共振可表现为小脑、脑干萎缩,并可一定程度上反映病程。     

橄榄脑桥小脑萎缩

多系统萎缩（MSA）是一组原冈不明的散发性成年起病的进行性神经系统多部位变性疾病，主要累及锥体外系、小脑、自主神经、脑干和脊髓。临床表现可归纳为三大综合征：表现为锥体外系功能障碍的黑质一纹状体变性（SND）、表现为自主神经功能障碍的Shy—Drager综合征（SDS）和表现为共济失调的散发性橄榄脯桥小腑萎缩（0PCA）。     

橄榄脑桥小脑萎缩与“十字征”

目的:探讨橄榄脑桥小脑萎缩(OPCA)的临床和影像学特点。方法:对1例橄榄脑桥小脑萎缩患者的临床资料进行回顾性分析并文献复习。结果:本病以小脑共济失调为首发表现,部分出现植物神经系统受损、锥体束或锥体外系症状。影像学除脑干变细、小脑体积变小外,可出现特征性的十字征表现。结论:因该病和某些小脑变性疾病表现相重叠,且无有意义的生化标记物,故其影像学十字征的发现对此病的诊断和鉴别诊断意义重大。     

"十字征"和橄榄体脑桥小脑萎缩

“十字征”(cross sign)即MRI的T2加权像上脑桥的十字形异常高信号影，由Savoiardo于1990年首次报道，见于橄榄体脑桥小脑萎缩(OPCA)的患者。我们报道1例MRI上典型的“十字征”表现，并结合文献复习分析如下，以提高广大临床医生对此征的认识。     

脑干听觉诱发电位改变对Binswanger病的评估价值

目的用脑干听觉诱发电位方法检测Binswanger病的脑功能改变。方法对20例BD病患者、20例非痴呆脑血管病患者、20例老年健康人同时进行脑干听觉诱发电位（BAEP）检查。结果BAEP异常：BD病10例（50％），非痴呆脑血管病2例（10％），经统计学检查BD组BAEP与健康组、非痴呆脑血管病组有显著性差异，非痴呆脑血管病组与健康组无显著性差异。结论BAEP异常反应BD病患者有脑干功能受损和弥漫性脑功能障碍，并可作为BD病患者脑功能损害检查敏感指标，以及与非痴呆脑血管病患者鉴别诊断的一个辅助检查方法。     

多系统萎缩的脑干听觉诱发电位

本组对19例多系统萎缩(MSA)患者,21例帕金森氏病(PD)患者,及19例正常对照组个体作了BAEP检查。结果表明在MSA患者组中,84.2％BAEP异常,而在PD组中则几乎均为正常。这种差异在临床的鉴别诊断中是重要的。结合适当的临床特征,异常的BAEP及CT或MR所见,可及早对MSA作出正确诊断。     

Influence of extreme head rotations on brainstem auditory evoked potentials (BAEP)

The influence of extreme head rotations on BAEP was studied in 47 patients who had vertebral artery angiogram. Patients with and without unilateral hypoplasia of the vertebral artery (VA) lumen were identified and all patients were further divided into two groups: with and without hypertension. The subjects underwent a BAEP examination in three head positions: neutral position, extreme right and extreme left rotation. In the subgroup of patients with hypertension and unilateral VA hypoplasia, and following the rotation away from the non-hypoplastic vessel a statistically significant decrease in the PV wave amplitude and prolonged PV wave latency was found. Head maneuvers in subjects with normal blood pressure or in hypertensive patients with symmetrical VA did not cause significant changes in BAEP. These results document the effect of head rotation in subjects with potentially impaired collateral flow. An increase in the PV wave latency and decrease in its amplitude may reflect subtle changes in the brainstem perfusion in hypertensive patients. These changes maybe due to a reduced capacity to compensate for dynamic obstruction of VA following the head rotations.     

脑干听觉诱发电位对癫(癎)患儿检测的意义

目的探讨脑干听觉诱发电位在癫患儿检测中的意义。方法对2000-01~2008-01我院38例癫患儿BAEP的检测结果进行总结。结果BAEP正常15例(39.5%),异常23例(60.5%),其中21例BAEP改变形式多样,异常指标相混出现。结论癫患者存在脑干功能异常。     

Effects of carbamazepine and valproate on brainstem auditory evoked potentials in epileptic children

Brainstem auditory evoked potentials (BAEPs) were recorded in 18 epileptic children receiving carbamazepine and 10 epileptic children receiving valproate. BAEPs were recorded before the administration of antiepileptic drugs (AEDs) and 13 months later during which the children received AEDs. Statistical analysis of peak latencies and interpeak intervals of waves I–III–V were made. Carbamazepine treatment resulted in prolongation of peak latencies of waves I–III–V and interpeak intervals I–III and I–V. Valproate monotherapy, on the other hand, caused no consistent changes on BAEP. On the basis of these results we suggest that chronic carbamazepine therapy exerts a suppressive influence on the auditory pathways, both peripherally at the level of the cochlea and/or auditory nerve, and centrally at the brainstem.     

听觉诱发电位及体感诱发电位在颅脑外伤致昏迷患者的应用

严重颅脑损伤昏迷患者脑功能的监测是判断预后和指导治疗的重要手段。听觉诱发电位(BAEP)及体感诱发电位(SSEP)因其方便、无创并能连续实时监测昏迷患者相关神经传导通路电生理的变化,间接反映脑干上行激活系统、大脑皮层结构与功能的完整性,越来越成为临床对严重颅脑损伤昏迷患者预测预后和指导治疗的重要手段。本文就近年来相关研究进行综述,旨在指导临床实践。     

The role of MRI in the diagnosis of olivopontocerebellar atrophy

Under the term of olivopontocerebellar atrophy different nosological pictures are grouped, all characterized by showing clinical signs of deficiency of the structures of the pons and of the cerebellum.The diagnosis of olivopontocerebellar atrophy has been made, until now, by clinical criteria while typical anatomopathological changes are found at the autoptic studies.We describe three patients affected by olivopontocerebellar atrophy, of different types and at different stages of disease.In all cases MRI showed a similar and typical picture of atrophy of the olivary eminences of the medulla oblongata resulting in straightening of the angle usually present on the ventral frontier between pons and medulla oblongata. This diagnostic tool demonstrated thus to be of primary relevance yet in the early phases of the disease.

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Sommario Sotto il termine di atrofia olivo-pontocerebellare si raggruppano quadri nosografici di verosimile diversa origine, caratterizzati tutti dalla presenza di segni clinici deficitari pontini e cerebellari. La diagnosi è stata sino ad ora solo clinica con conferma autoptica del danno pontocerebellare. Sono descritti tre casi di atrofia olivo-pontocerebellare, appartenenti, a tre distinti sottogruppi ed in fasi diverse della malattia. In tutti e tre i casi la MRI ha evidenziato un quadro di atrofia pontina e cerebellare ed una riduzione dimensionale delle olive bulbari che risultava in un allargamento dell'angolo pontobulbare normalmente rilevabile sulla faccia anteriore del tronco in proiezione laterale. L'esame si conferma di importanza diagnostica essenziale nella malattia sin dalle sue fasi iniziali.

     

脑干听觉诱发电位在弥漫性轴索损伤中的应用

目的探讨脑干听觉诱发电位(BAEP)在弥漫性轴索损伤(DAI)的临床诊断及预后评估的价值。方法对52例DAI患者进行。BAEP反复测定,动态观察病情转化过程中BAEP各波的变化。结果①本研究中CT 对DAI的检出率为28．8％,BAEP总异常率为73．1％,经x2检验,两者差别有统计学意义。②在本研究中病例组 BAEP与对照组行t检验比较,两者差别有统计学意义。③BAEP分级与GCS、GOS的相关系数分别为-0．51022、 -0．5539,BAEP分级与GCS及GOS之间呈明显的负相关(P<0．0001)。结论BAEP可从电生理角度评价脑干的功能状态,有助于临床对脑干损伤的诊断、病情监测、疗效评估和预后判断。BAEP是评价DAI患者中脑干损伤后脑干功能和患者预后的客观指标。     

Prognostic evaluation of brainstem hematomas: the role of CT scan and brainstem auditory evoked potentials

Abstract– 6 cases of brainstem hematoma were studied utilizing CT scan and brainstem auditory evoked potential (BAEP) recordings. CT scan did not contribute to an early discrimination between primary and secondary hematomas. Size of the hematoma and the presence of blood in the CSF did not represent evident signs in differentiating benign from unfavourable brainstem hematomas or hemorrhages. BAEP recordings showed the presence of electrophysiological anomalies at the level of the lesion, demonstrating that bleeding as well as tumor in the brainstem can provoke a focal damage.     

橄榄—桥脑—小脑萎缩伴视网膜变性的临床分子生物学研究

目的 探讨橄榄－桥脑－小脑萎缩伴有视网膜变性（ＳＣＡ７）的临床表型与与致病基因突变的关系。方法 对发病表现为共济失调、辨色力异常、视力下降的２个家系共２１名成员（８例患者）进行了系统的临床及辅助检查、通过聚合酶链反应（ＰＣＲ）及聚 烯酰胺凝胶电泳等技术检测家系成员和另５５名健康人的ＳＣＡ７基因内ＣＡＧ重复序列,分析ＳＣＡ７临床表型与ＣＡＧ重复突变的相关。上家系１１个亲代－子代对均存在遗传早现现象,