基底节病变时的认知障碍

基底节不仅是在大脑皮质控制下调节运动功能的一个神经核团,而且也参与情感、学习、记忆、思维、语言等高级神经功能的活动;基底节由于解剖关系,病变时临床表现复杂多样,仅在认知领域就可出现言语障碍、情绪障碍、记忆和注意障碍、视空间障碍等认知障碍,本文仅就基底节的解剖,基底节病变时各项认知障碍的特点及相关机制作一综述.     

Wilson病患者眼区情绪认知障碍的研究

目的探讨Wilson病(Wilson disease,WD)患者是否存在眼区情绪认知障碍,了解基底节有无参与眼区情绪认知加工过程。方法将32例WD患者以及29名与其人口学资料相匹配的健康人(healthy control,HC)作为被试,采用眼区的6种基本情绪(喜、惊、恐、悲、厌、怒)任务,对两组进行测试。结果与HC组比较,WD组在在眼区情绪认知任务对怒(17.53±1.39分,P<0.05),恐(15.88±1.21分,P<0.05)和厌(18.00±1.85分,P<0.001)眼区情绪认知存在明显障碍;而对于喜、惊、悲的眼区情绪认知任务识别却无障碍(均P>0.05)。结论WD患者存在明显的怒、恐及厌眼区情绪认知障碍,基底节可能参与眼区情绪认知任务的加工过程。     

精神分裂症患者海马结构与面孔情绪认知障碍的关联研究

目的:探讨精神分裂症(schizophrenia, SCZ)患者海马结构体积与面孔情绪认知障碍间的关系。方法:纳入41例首发SCZ患者(患者组)与27名健康对照者(对照组)。分别进行阳性和阴性症状量表(positive and negative syndrome scale, PANSS)评定、面孔情绪认知评估、磁共振扫描,应用FreeSurfer 6.0测量海马结构。结果:与对照组相比,患者组面孔情绪测试的拐点、斜率均显著增加。患者组左侧海马总体积、海马角(CA1)、前下托、旁下托、海马杏仁核过渡区(HATA),右侧海马总体积、下托、CA1、前下托、旁下托、分子层(ML)、HATA亚区体积均小于对照组,差异具有统计学意义(P0.05或P0.01)。患者组右侧HATA亚区体积与面孔情绪认知斜率呈负相关(P0.05)。患者组左侧海马下托体积与阴性因子分呈负相关,右侧海马总体积、CA1和ML亚区体积与瓦解因子分呈负相关,左侧海马前下托、旁下托亚区体积与抑郁因子分呈负相关(P0.05或P0.01)。结论:海马体积异常可能与SCZ患者面孔情绪认知障碍有关。     

基底节血管病变记忆障碍的研究

目的 研究基底节血管病变的记忆障碍.方法 采用简易智能状态检查量表(MMSE)对基底节血管病变(梗死与出血)的病人进行智能筛查,评分在20～30分的智能基本正常者91例入选为研究对象.用临床记忆量表进行记忆力测定.将研究组(91例)与正常组(50例)、梗死组(50例)与出血组(41例),梗死组左侧(16例)与右侧(20例)、出血组左侧(19例)与右侧(22例)分别进行记忆力比较.结果 研究组与正常组比较,记忆商数明显下降,差异显著;出血组在人像特点联系回忆和记忆商数(MQ)两项与梗死组比较差异显著;梗死组左侧较右侧在联想记忆和MQ两项损害明显,差异显著.出血组左侧与右侧除无意义图形再认一项外其他各项损害明显,差异显著.结论 基底节区域血流供应阻断或局部出血可引起记忆功能减退.基底节梗死与出血两侧比较,左侧损害更为明显.     

基底节血管病变记忆障碍的再研究

基底节血管病变(梗塞与出血)较多见。它的损害除涉及躯体的生理功能外,现已发现基底节梗塞还可引起一些高级神经活动的障碍如记忆障碍等。本文目的在于比较两类病人记忆损害的异同,基底节梗塞或出血范围大小与记忆下降的关系。     

情绪稳定剂联用的安全性及有效性研究

多药联合常用于治疗难治性双相情感障碍。本文复习了情绪稳定剂联用的安全性和有效性的文献,指出由于药物间的相互作用很复杂,有时具有潜在危险,所以,使用应当谨慎,目前最安全、有效的情绪稳定剂联用是锂加丙戊酸。     

精神分裂症患者面孔情绪认知特点及其影响因素研究

目的探讨精神分裂症患者面孔情绪认知的特点及其影响因素。方法采用中国人面孔情绪测验(CFET)对121例精神分裂症患者进行测试,与76名正常对照比较,同时作阳性症状量表(SAPS)和阴性症状量表(SANS)评定。结果患者组6种基本情绪认知正确数均显著低于对照组,远隔错误数均显著高于对照组。回归分析显示,患者情绪认知障碍与年龄、用药情况、病程、住院次数及SAPS和SANS总评分无关,而受教育年限与情绪认知正确数和远隔错误数评分及多项不同情绪认知评分相关。SAPS阳性思维形式障碍因子分与CFET正确数总分呈负相关,而与CFET远隔错误数总分呈正相关,同时与惊、悲、怒情绪认知评分呈相关。SANS的注意障碍因子分与CFET正确数总分呈负相关,而与CFET远隔错误数总分呈正相关。结论精神分裂症患者存在广泛的情绪认知障碍并与某些症状相关,提示情绪认知障碍可能与疾病病理生理过程有关。     

Wilson病患者的厌恶情绪加工障碍

目的　探讨Wilson病 (WD)患者是否出现选择性厌恶情绪认知障碍 ,以进一步验证基底节参与情绪加工的假说。方法　测试 32例WD患者 (WD组 )和 2 0名健康人 (对照组 )对 30张含有 6种基本情绪 (喜、惊、怕、悲、厌和怒 )面孔照片的辨认能力。结果　与对照组比较 ,WD组对厌 [(9.1± 4 .7)分 ,P <0 .0 0 1 ) ] ,怒和怕 [(1 4 .9± 4 .3)分 ;(1 2 .8± 3 .6)分 ,P均 <0 .0 5]情绪面孔的辨别有障碍 ,而相对于怕和怒 ,厌的认知障碍更为明显。结论　WD组表现为相对选择性的厌恶加工障碍     

外伤性基底节损害

外伤性基底节区损害是颅脑外伤巾较为少见的类型,主要为颅脑外伤后基底节区域的结构与功能的异常。基底节损伤后的常见病理改变有外伤性基底节出血和外伤性基底节缺血。由于影像学的进展,其正确诊断率近年来有增高的趋势。本文对外伤性基底节损害的流行病学、分类、临床表现、发生机制及治疗、预后等进行文献综述。     

基底节内侧型高血压脑出血患者的手术时机研究

目的：研究基底节内侧型高血压脑出血患者的手术时机。方法回顾性分析2012‐01—2014‐03在我院进行手术治疗的42例基底节内侧型高血压脑出血患者的临床资料,然后将病人分为A、B、C3组,A组手术时机为＜7 h ,B组为7 h＜ T＜24 h ,C组为＞24 h ,研究不同手术时机对患者治疗效果的影响。结果3组脑血肿量、GCS评分相比,差异无统计学意义（P＞0.05）;3组神经功能缺损、ADL评分相比,差异有统计学意义（P＜0.05）;3组的病死率、总有效率与术后并发症发生率相比,差异有统计学意义（P＜0.05）。结论出血后7 h内是基底节内侧型高血压脑出血患者手术治疗的最佳时机,早期手术治疗可显著改善患者的神经功能,降低病死率与术后并发症的发生率,提高日常生活能力。     

Distinct basal ganglia hyperechogenicity in idiopathic basal ganglia calcification

We report a 67‐year‐old patient with idiopathic basal ganglia calcification (IBGC). He presented with progressive cognitive impairment, frontal lobe dysfunction, mild leg spasticity, and levodopa (L ‐dopa)‐responsive parkinsonism. Transcranial sonography (TCS) revealed marked hyperechogenicity of the basal ganglia and periventricular spaces bilaterally. The detected signal alterations showed a fairly symmetric distribution and corresponded to the hyperintense calcifications depicted on the computer tomography brain scan. The combination of symmetric hyperechogenic areas adjacent to the lateral ventricles and of the basal ganglia may serve as an imaging marker characteristic of IBGC. Hyperechogenicity due to extended basal ganglia calcification as presented here is distinct from the pattern of hyperechogenicity caused by heavy metal accumulation, which is described to be less striking. In addition to atypical parkinsonian syndromes such as progressive supranuclear palsy and multiple system atrophy, IBGC is thus another differential diagnosis of parkinsonism with basal ganglia hyperechogenicity. © 2010 Movement Disorder Society.     

Mobius syndrome with basal ganglia calcification

Basal ganglia calcification has not been described in Mobius syndrome. A family with two children with Mobius syndrome are reported. Bilateral basal ganglia calcification was seen on computed tomography in both. This is the first family where cerebral involvement has been clearly documented in this syndrome.     

Are periodic movements in sleep a basal ganglia dysfunction?

Summary Muscle activity during sleep is a new area of interest in sleep research. No precise brain structures are known to be involved in sleep movement. The etiology of periodic movements during sleep is unknown. The present study was dedicated to evaluate involvement of basal ganglia in periodic movements of the legs during sleep (PMS) in Parkinson's diseased patients. Sleep was monitored in 3 patients suffering from Parkinson's disease and PMS (PMS/PD) and in 3 patients suffering from restless legs syndrome and PMS (PMS/non PD). Following treatment, the six patients were monitored again during sleep. It was found that only the PMS/PD group of patients had improved significantly with appropriate treatment. Improved motor function in PD patients is associated with decreased PMS, regardless of wether the patients are treated with dopaminergic or anticholinergic agents. This is consistent with our previous data. It may be suggested that the striopallidal system is involved in periodic sleep movements of Parkinson's diseased patients.     

Time course of VCAM-1 and ICAM-1 in CSF in patients with basal ganglia haemorrhage

OBJECTIVES: In a pilot study we found a correlation of the clinical outcome with adhesion molecule (AM) concentrations in ventricular cerebrospinal fluid (CSF) but not in serum in patients with intracerebral haemorrhage. We now determined the time course of AM concentration in CSF and serum after basal ganglia haemorrhage (BGH) in order to further uncover pathogenetic mechanisms. MATERIALS AND METHODS: We included 11 patients with acute BGH and ventricular tamponade in which an extraventricular drainage had been applied to treat ventricular ballonade. Paired CSF and serum samples were obtained within 8 h after onset of BGH, as well as on the consecutive days 2, 4, 6, and 8, respectively. The concentrations of soluble ICAM-1 (sICAM-1) and VCAM-1 (sVCAM-1) in CSF and serum were measured by enzyme-linked immunosorbent assay. Moreover, we determined blood volume and perifocal oedema by a semi-automated planimetry technique from initial cranial computed tomography scans. RESULTS: sICAM-1 and sVCAM-1 levels in CSF were highest within the first hours after onset of BGH, then decreased significantly (P < 0.005 and <0.05, respectively) on day 2 and slightly increased thereafter. Furthermore, BGH volume was significantly correlated with the concentrations of sICAM-1 (r = 0.63, P < 0.05) and sVCAM-1 (r = 0.66, P < 0.05) in ventricular CSF but not in serum. CONCLUSIONS: Our results might indicate that the local inflammatory reaction is pronounced early after onset of BGH and appears to be restricted to the central nervous system. Moreover, AM concentrations measured early after BGH onset correlated stronger with radiological and clinical data than follow-up measurements.     

Catecholamine alterations in basal ganglia after hippocampal lesions

Rats were given sham, cortical, or hippocampal lesions and sacrificed 7 or 28 days following surgery. Levels of norepinephrine, dopamine, and the major dopamine metabolites, 3, 4-dihydroxyphenylacetic acid and homovanillic acid (HVA), were assayed in 3 brain regions. At day 7 there was a decrease in dopamine utilization and a decrease in norepinephrine levels in the nucleus accumbens after hippocampal damage but both of these measures returned to normal levels by day 28. In the neostriatum HVA levels decreased at day 7 after hippocampal damage. The utilization of dopamine in the neostriatum was decreased at day 28 in animals that received neocortical lesions but this was not observed in animals with hippocampal destruction. No effects of any lesion at any day were found in the olfactory tubercle region, the third brain region analyzed. It is thought that the removal of hippocampal and neocortical input to the basal ganglia influences catecholamine function reflected in the loss and subsequent recovery of dopamine utilization.     

Subacute hemicorporal parkinsonism in 5 patients with infarcts of the basal ganglia

Summary. In 1929, Critchley introduced the term “vascular parkinsonim” (VP), which has been the subject of considerable controversy in neurology. Parkinsonism does not appear to be a frequent consequence of striatal infarcts, although unilateral parkinsonim has been reported as an acute or subacute onset syndrome following strategic infarcts in the striatum. Previous 123-I ioflupano SPECT (DaTSCAN) studies involving radioisotope labeling of the dopamine transporter protein at presynaptic level in patients with IPD (idiopathic Parkinson’s disease) have found this technique to be highly sensitive in exploring the nigrostriatal pathway. Previous studies of VP with DatSCAN have been inconclusive. The present study correlates clinical data (unilateral parkinsonim following contralateral lenticular infarction), and radiological (CT/MRI) and functional neuroimagin findings (DatSCAN) in 5 patients with CT/MRI criteria for striatal infarcts. Finally, in 2 of these patients a diagnosis of IPD was made because of the follow-up of clinical signs and pathological DaTSCAN findings not concordant with the size and location of the vascular lesion.     

Unsuccessful treatment with levodopa of a parkinsonian patient with calcification of the basal ganglia

Summary A patient aged 67 had suffered from hypoparathyroidism (tetany and cataracts) following thyroidectomy at the age of 20, although treated with AT 10. During the last few years there had been a change of gait to small steps and general retardation. Nocturnal epileptic attacks occurred in the last year which led to the admission to hospital where a well developed parkinsonian syndrome without tremor was observed. X-rays of the skull, including computed tomograms, revealed calcification of the basal ganglia and dentate nuclei. Treatment with levodopa was ineffective. The mechanism for this is discussed.

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Zusammenfassung Es wird über einen Fall mit postoperativem Hypoparathyreoidismus berichtet, der Jahrzehnte nach der Operation zu schwerwiegenden Komplikationen führte. Neben Tetanie, Cataracta tetanica, Epilepsie entwickelte sich ein ausgeprägtes Parkinson-Syndrom infolge Stammganglienverkalkungen. Diese Verkalkung kam im Vergleich zur herkömmlichen Röntgenaufnahme des Schädels im cranialen Computer-Tomogramm weitaus deutlicher zur Darstellung. Ein Parkinson-Syndrom dieser Genese wird unter neueren neurochemischen Vorstellungen als Schädigung der postsynaptischen Dopaminrezeptoren im striatonigralen System angesehen. Folglich erweist sich die Parkinson-Symptomatik — wie in unserem Fall — meistens als L-Dopa-therapieresistent.

     

The syndrome of acute bilateral basal ganglia lesions in diabetic uremic patients

Acute extrapyramidal movement disorders have rarely been reported in uremic patients. We had previously presented three cases of acute movement disorders with bilateral basal ganglia lesions in uremia, and had proposed that it is not a rare condition. The objective of this study is to establish a more accurate clinical profile of this rarely described clinical syndrome, and to call attention to its common occurrence. We prospectively studied six patients we encountered from March 1996 to June 2001. We also reviewed the clinical records of a large population of uremic patients and identified six more cases. The clinical manifestations, laboratory findings, neuroimages, and clinical outcomes of these 12 patients were analyzed. When possible, each patient was followed up to the present time. Twelve patients had acute onset of movement disorders and bilateral basal ganglia lesions. All of the patients were diabetic. They had acute-onset Parkinsonism or dyskinesias, together with various symptoms such as consciousness disturbance, dysarthria, dysphagia, or ataxia. The main laboratory test results of abnormalities consisted of elevated blood urea nitrogen, creatinine, and metabolic acidosis. They had uniform neuroimaging findings of symmetrical bilateral basal ganglion changes. These changes regressed or disappeared during follow-up. The clinical prognoses were diverse. We believe that this group of patients represents a well-demarcated clinical syndrome, which is not uncommon but has previously been rarely addressed. The underlying mechanism of such lesions may be associated with metabolic, as well as vascular factors.