皮质下缺血性脑血管病患者额叶白质病变对认知领域的损害研究

目的探讨皮质下缺血性血管病(SIVD)患者的额叶的白质病变(WML)累及的认知功能域。方法将50例SIVD患者,根据额叶WML评分的严重程度,分为严重WML组(视觉评分3分)27例和轻度WML组(视觉评分≤3分)23例。另以7例无SIVD的人群作为对照。对所有受试者进行全套的认知心理学测查并对结果进行对比分析。结果 3组间一般资料的差异无统计学意义。与轻度WML组和对照组相比,严重WML组的非额叶白质评分、额叶腔隙灶数目明显增多(均P=0.000);与轻度WML组相比,严重WML组的蒙特利尔认知评估量表评分明显降低(P=0.047);且执行功能相关的认知评分明显降低(P=0.006),在调整了额叶的腔隙灶数量后,该差异仍有统计学意义(P=0.038)。多元回归分析发现,对注意执行Z分显著影响的因素为额叶WML评分(P=0.000)。结论在SIVD患者中,额叶的WML主要累及执行功能。     

Ideomotor apraxia in Alzheimer's disease

Ideomotor apraxia, tested on verbal command and by imitation, was checked in 23 patients suffering from dementia of Alzheimer's type of different severity and in 17 age-matched controls. A significant deterioration of ideomotor praxis could be shown even in mild dementia. Correlations of ideomotor apraxia and aphasia, tested by the Token test were found to be significant.     

Human Motor Planning, Motor Programming, and Use of New Task-relevant Information with Different Apraxic Syndromes

Patients with ideomotor apraxia ( n = 2) or ideational apraxia ( n = 2) after left brain stroke and patients with constructional apraxia ( n = 2) after cerebrovascular accident of the right hemisphere, as well as 16 non-brain-damaged control subjects, were given a standardized simple motor task: they were asked to make triangular arm movements of specific size, configuration and spatial orientation without visual control. Motion was analysed three-dimensionally in great detail prior to and after kinaesthetic training using a triangular stencil, and 1 day later. The experiment was conceptualized to assess three aspects of motor behaviour: (i) motor planning, operationalized as specification of content parameters of the movement as a whole; (ii) motor programming, the specification of spatiotemporal parameters of movement segments; and (iii) the ability to make use of task-relevant information provided by the training. Patients with ideational apraxia showed signs of impaired motor planning: they had difficulty in selecting the body parts to be moved, and movement concept and configurational aspects were deficient. The kinaesthetic sensorimotor training given seemed not adequate to reduce behavioural deficits. Kinematic peculiarities of patients with ideomotor apraxia can be understood as deficits in programming movement elements. Submovements were more segmented, showed irregularities as well as additional, not-requested elements. Their impairments could be reduced by task-specific sensorimotor training. Patients suffering from visuoconstructive apraxia after right brain damage might have difficulties in making use of new sensorimotor information relevant for spatial-motor aspects, as suggested by training-induced behavioural impairment with a severely constructional apraxic patient.     

Changes in cortical and subcortical levels of monoamines and their metabolites following unilateral ventrolateral cortical lesions in the rat

Suction lesions were made in the anterior, posterior or both halves of the right ventrolateral cortex in rats. Six days later, levels of the monoamine neurotransmitters, norepinephrine (NE), dopamine (DA) and serotonin (5-HT), and their metabolites, 3,4-dihydroxyphenylacetic acid (DOPAC) and 5-hydroxyindoleacetic acid (5-HIAA), were measured in cortical and subcortical regions of lesioned rats and compared to values in sham-operated animals. NE and 5-HT were decreased in sections of ipsilateral (right) cortex including, and posterior to lesions, while 5-HIAA was increased throughout the ipsilateral cortex. Decreases in monoamines and increases in metabolites and metabolite:monoamine ratios (especially 5-HIAA:5-HT) were found in ipsilateral subcortical structures, including striatum, nucleus accumbens, hippocampus, hypothalamus, midbrain and brainstem, depending on the type of lesion. Subacutely, focal ventrolateral cortical lesions may profoundly alter the levels and utilization rates of monoamine neurotransmitters in widespread regions of the ipsilateral hemisphere.     

Apraxia of eyelid closure in Huntington's disease

Summary. We report a patient with genetically confirmed Huntington's disease (HD) presenting apraxia of eyelid closure (AEC). She was unable to close her eyes at command but was able to blink. Chorea and AEC ameliorated significantly during treatment with olanzapine and riluzole, an inhibitor of glutamate release. AEC is reported in progressive supranuclear palsy, Creutzfeldt-Jakob's disease, amyotrophic lateral sclerosis, and as post-stroke AEC. No report on HD is available so far, although oculomotor disturbances are quite common in this disease. Received September 5, 2001; accepted December 5, 2001     

Relationship between depressive symptomatology and the subcortical brain syndrome in dementia

OBJECTIVES: the aim of the present study was to elucidate a possible relationship between depressive symptomatology and regional brain symptomatology in demented patients. METHODS: 170 consecutive inpatients were studied. They suffered from Alzheimer's disease (103 patients), vascular dementia (48 patients), or frontotemporal dementia (19 patients). The patients underwent a neuropsychiatric investigation, which included assessments of (1) depression, and (2) regional brain symptomatology. Depressive symptomatology was assessed as presence of (a) depressed mood, and (b) anxiety. In the further statistical analysis, the presence of depressed mood and/or anxiety was also evaluated. The principle of the regional symptom analysis was the successive aggregation of observable symptom variables, leading to the final determination of four possible regional brain syndromes (frontal, subcortical, parietal and global), by way of the Stepwise comparative status analysis (STEP). The possible correlations between regional brain symptomatology and depressive symptomatology were analysed by means of (a) chi(2) statistics, and (b) a logistic regression analysis in which confounding factors were included (age, degree of dementia, duration). RESULTS: the subcortical syndrome correlated with depressed mood (chi(2), p = 0.002; logistic regression, p = 0.002). A negative correlation was noted between the parietal syndrome and depressed mood (p = 0.010 and p = 0.013, respectively). No other significant correlations between presence of regional brain syndrome and presence of depressive symptomatology could be seen. CONCLUSIONS: demented patients with a clinically established subcortical dysfunction appear to be more susceptible to depressive symptomatology. The results of this study also suggest that posterior brain dysfunction in dementia is not correlated with depressive symptomatology.     

Speech disturbances due to left precentral cortical lesions

We encountered two patients with lesions predominantly localized to the left precentral cortex who presented with speech disorders. Patient 1 had a lesion localized to the anterior part of the left precentral cortex along the precentral sulcus, in the middle part of the convexity. Patient 1 predominantly exhibited disturbance of prosody. Patient 2 had a lesion localized to the left precentral cortex, located slightly more posterior and more inferior in the convexity. Patient 2 predominantly exhibited disturbance of articulation, with distortion of sounds without apparent inconsistency. Even lesions localized to the left precentral cortex present with various types of speech disturbance.     

Apraxia of lid opening is alleviated by pallidal stimulation in a patient with Parkinson's disease

Apraxia of lid opening (ALO) is a syndrome characterized by a non-paralytic inability to open the eyes at will in the absence of visible contraction of the orbicularis oculi muscle. Here we report that globus pallidus internus deep brain stimulation on the right side markedly alleviates ALO as well as gait freezing in a patient with Parkinson's disease.     

缺血性脑血管病患者颈动脉病变与糖代谢的关系

目的 探讨缺血性脑血管病(ICVD)患者颈动脉病变与糖代谢的关系.方法 122例ICVD患者根据葡萄糖耐量试验(OGTT)结果分为糖耐量正常(NGT)组(44例)、糖耐量减低(IGT)组(27例)及糖尿病(DM)组(51例),对各组患者进行颈动脉彩色多普勒超声检查,并对3组间颈动脉病变情况进行分析.结果 DM组年龄明显...     

Cognitive profile of subcortical ischaemic vascular disease

OBJECTIVES: Subcortical ischaemic vascular disease (SIVD) is a subtype of vascular cognitive impairment characterised by extensive white matter lesions and multiple lacunar infarcts. Radiologically defined diagnostic criteria for SIVD have been introduced, but only a few studies have presented empirical data on its clinical and cognitive features. The aim of this study is to describe in detail the neuropsychological characteristics of patients with SIVD from a large well defined stroke cohort. METHODS: A sample of 323 consecutive patients with ischaemic stroke, aged 55-85 years, was investigated using neuropsychological examination and magnetic resonance imaging (MRI). Patients fulfilling the MRI criteria of SIVD (n = 85) were compared to the other stroke patients (n = 238) and to normal control subjects (n = 38). RESULTS: Cognitive performance of the SIVD group was inferior to that of the normal control group throughout all domains. As compared to the other stroke patients, the SIVD group performed significantly worse in tests measuring executive functions and delayed memory recall. Adjusting for depression had no effect on these results. Instead, after controlling for medial temporal lobe atrophy, the differences disappeared for delayed memory but remained significant for executive functions. CONCLUSION: Executive deficits are the most prominent cognitive characteristic associated with SIVD. Patients with SIVD also exhibit subtle deficits in delayed memory, which is explained in part by medial temporal lobe atrophy. Cognitive and mood changes seem to be parallel but independent processes related to SIVD. The results support the concept of SIVD as a separate clinical entity.     

Apraxia in Parkinson’s disease and multiple system atrophy

Objective: To determine praxis function in patients with Parkinson’s disease (PD) and multiple system atrophy (MSA). Methods: Nineteen patients with PD and 16 patients with probable MSA were recruited into study. Twenty‐five age‐matched, healthy subjects were included as controls. The Mayo Clinic praxis test battery was applied. Pantomime tasks, including oral/facial, trunk, and upper extremity movement, were used to evaluate ideomotor apraxia (IMA). Sequential tasks, including Luria test for ideational apraxia (IDA) and use of actual objects, were also tested. In addition, Standardized Mini Mental Test (MMSE), Hamilton Depression (HAM‐D), and Anxiety (HAM‐A) Scales were used. Results: Mean ages of the study participants were 66 ± 7, 68 ± 5, and 65 ± 7 years in PD, MSA, and control groups, respectively. Mean total praxis score was significantly lower for patients with PD (92.4 ± 4) and MSA (75.9 ± 18) than for controls (97.4 ± 2) (P = 0.000). Transitive performances of upper extremities and sequential tasks were significantly impaired in patients with PD compared to control subjects (P < 0.05). There was no correlation between total praxis scores and sum scores of tremor, bradykinesia, and rigidity of both of the upper limbs of patients with PD. Subgroup praxis scores were substantially worse in MSA group (P < 0.0001). Compared to control subjects, mean scores for MMSE, HAM‐D, and HAM‐A tests were significantly worse in MSA group, but, for PD patient group, only HAM‐A scores were worse. Conclusion: Our results indicate that although not a presenting symptom, IMA and IDA may be features of MSA and, to a lesser degree, of PD. Also, it seems to be unrelated to the motor features of patients with PD.     

Small subcortical infarct and intracranial large artery disease in Chinese

Background: Small subcortical infarct (SSI) occurs more frequently among Chinese than Caucasians. Apart from small vessel disease, SSI is also associated with intracranial large artery disease. We aimed to study the frequency of SSI with and without intracranial large artery disease among Chinese stroke patients. Methods: Magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), and diffusion weighted imaging (DWI) were performed among consecutive Chinese stroke patients admitted to our acute stroke unit over a 6-month period. Results: Among the 257 patients with ischemic stroke, 71 patients (27.6%) had SSI. Twelve patients (16.9%) had relevant intracranial large artery disease and three patients (4.2%) had lone relevant extracranial carotid artery disease. No patient had an identifiable cardiac embolic source or other miscellaneous cause. Excluding patients with relevant intracranial large artery disease and extracranial carotid artery disease, the frequency of SSI associated with presumed small vessel disease among patients with ischemic stroke was 21.7%. Patients with SSI associated with intracranial large artery disease had greater number of acute infarcts and slightly greater stroke severity and cognitive impairment than those with presumed small vessel disease. Conclusion: Our present study suggests that the higher frequency of SSI among Chinese may be in part related to a higher frequency of intracranial large artery disease rather than to a higher frequency of small vessel disease.     

Association between subcortical vascular disease on CT and neuropathological findings

BACKGROUND: Small vessel cerebrovascular disease (svCVD) causes cognitive impairment and predicts poorer outcomes in elderly persons. The demonstration of svCVD by structural imaging such as computed tomography (CT) is central to the clinical diagnosis but its accuracy is uncertain. OBJECTIVE: The aim of this study was to assess the validity of CT to detect pathologically verified svCVD. METHODS: Leukoaraiosis, patchy lesions, and lacunes were assessed separately in different cerebral regions on CT scans of 87 elderly persons with Mini-Mental State Examination scores between 0 and 30 enrolled in the OPTIMA project. We rated small vessel disease on pathology separately in the white matter and basal ganglia as absent/mild, moderate, and severe. The presence of microinfarcts was also noted. RESULTS: The severity of all types of CT lesions was associated with pathological findings. Subjects with absent/mild leukoaraiosis on CT decreased and those with severe leukoaraiosis increased with increasing severity of subcortical small vessel ratings on pathology (31, 18 and 0%; 17, 23 and 50%, respectively; p = 0.028). A similar association was present for patchy lesions (73, 59 and 17%; 7, 18 and 33%, respectively; p = 0.004) and lacune (83, 77 and 50%; 0, 4 and 17%, respectively; p = 0.023). Leukoaraiosis, patchy lesions, and lacunes on CT were associated also with microinfarcts on pathology (p < 0.05). CONCLUSION: The standardized assessment of svCVD on CT films at the time of the diagnosis correlates with small vessel disease on pathology at death.     

Cerebral volume measurements and subcortical white matter lesions and short-term treatment response in late life depression

BACKGROUND: Late life depression is associated with volumetric reductions of gray matter and increased prevalence of subcortical white matter lesions. Previous studies have shown a poorer treatment outcome in those with more severe structural brain abnormalities. In this study, quantitative and semi-quantitative magnetic resonance imaging (MRI) measures were studied in relation to response to a 12-week controlled antidepressant monotherapy trial. METHODS: MRI (1.5 T) brain scans of 42 elderly inpatients with major depression, of which 23 were non-responder to a controlled 12-week antidepressant monotherapy trial, were acquired. In addition, clinical outcome was assessed after a one year period. Measures were volumes of global cerebral and subcortical structures. RESULTS: After controlling for confounding, no differences were found between non-responders and responders after 12 weeks and after one year in volumes of cerebral gray and white matter, orbitofrontal cortex, hippocampus and white matter lesions. CONCLUSIONS: Structural brain measures associated with late life depression may not be related to short-term treatment response.     

后循环缺血患者脑血管病变的特点

目的探讨后循环缺血患者脑血管病变的特点。方法对74例后循环缺血患者进行CT血管成像(CTA)检查,分析血管病变的部位、性质和程度。结果本组74例后循环缺血患者中61例(82.4%)被CTA证实有血管病变。其中,血管动脉粥样硬化病变8例(10.8%),血管狭窄38例(51.4%),血管纤细22例(29.7%),血管发育不良12例(16.2%)。单纯前循环血管病变7例(9.5%),单纯后循环血管病变21例(28.4%),前后循环均有血管病变例33例(44.6%);前循环血管病变以颈内动脉颅外段狭窄为主,后循环血管病变以椎动脉颅外段和大脑后动脉病变最为多见;前、后循环血管病变狭窄程度均以重度为主。结论后循环缺血患者的主要血管病变部位在椎动脉颅外段和大脑后动脉,其次为颈内动脉颅外段;最常见的病变性质是动脉粥样硬化和血管狭窄,且以重度狭窄最为多见。     

CT脑立体定向显微直视手术切除脑功能区及深部病变

采用CT脑立体定向技术引导开颅显微外科手术切除脑重要功能区及脑深部病变18例,其中位于运动区皮质下28例、语言中枢皮质下12例、脑深部8例 临床表现主要为癫痫、偏瘫、失语 病变性质主要为脑囊虫病、慢性炎性组织、结核瘤、肺吸虫病、海绵状血管瘤、脂肪瘤及血吸虫病.术后所有病人症状均逐渐消失,完全恢复健康,无手术并发症及死亡.     

Regional subcortical shape analysis in premanifest Huntington's disease

Huntington's disease (HD) involves preferential and progressive degeneration of striatum and other subcortical regions as well as regional cortical atrophy. It is caused by a CAG repeat expansion in the Huntingtin gene, and the longer the expansion the earlier the age of onset. Atrophy begins prior to manifest clinical signs and symptoms, and brain atrophy in premanifest expansion carriers can be studied. We employed a diffeomorphometric pipeline to contrast subcortical structures’ morphological properties in a control group with three disease groups representing different phases of premanifest HD (far, intermediate, and near to onset) as defined by the length of the CAG expansion and the participant's age (CAG‐Age‐Product). A total of 1,428 magnetic resonance image scans from 694 participants from the PREDICT‐HD cohort were used. We found significant region‐specific atrophies in all subcortical structures studied, with the estimated abnormality onset time varying from structure to structure. Heterogeneous shape abnormalities of caudate nuclei were present in premanifest HD participants estimated furthest from onset and putaminal shape abnormalities were present in participants intermediate to onset. Thalamic, hippocampal, and amygdalar shape abnormalities were present in participants nearest to onset. We assessed whether the estimated progression of subcortical pathology in premanifest HD tracked specific pathways. This is plausible for changes in basal ganglia circuits but probably not for changes in hippocampus and amygdala. The regional shape analyses conducted in this study provide useful insights into the effects of HD pathology in subcortical structures.