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1.
We studied clinical features of 5 patients with subacute encephalitis/encephalopathy with residual cognitive deficit. In all patients, impairment of consciousness was mild at the onset, progressed between 4 and 15 days after the onset, and reached its peak at 6-18 days after the onset. Neuroradiological or electrophysiological examinations at the onset showed normal or mildly abnormal findings. In accordance with the clinical deterioration, brain atrophy was detected on cranial magetic resonance imaging (MRI) in 3 patients, and electroencephalogram revealed slowing of the background activities in all patients. Single photon emission computed tomography (SPECT) showed hypoperfusion in the fronto-temporal areas in all patients at the recovery stage. Three patients had severe mental deficits, and the other two had mild cognitive deficit. Motor impairment was observed in one patient.  相似文献   

2.
Visual-spatial impairment and neuroanatomical abnormalities are considered hallmark features of neurofibromatosis, type I (NF-I). Numerous studies have demonstrated visual-spatial deficits in children with NF-I, but few relations between these deficits and neuroanatomical abnormalities have been identified. We compared the functional neuroanatomy of cerebral regions involved in the spatial transformation of alphanumeric stimuli in individuals with NF-I and healthy control participants using functional magnetic resonance imaging (fMRI). Given the prevalence of visual pathway abnormalities and visual-spatial deficits in NF-I, we hypothesized that less neuronal hemodynamic activity would be found in occipital and parietal cortices in this group compared with controls. However, NF-I participants relied to a greater degree than controls on posterior cortex (including occipital, parietal, and middle temporal cortices) relative to lateral and inferior frontal regions during visual-spatial analysis. This pattern was significantly related to their behavioral performance on the fMRI task, which in turn was also positively correlated with reading scores. These findings support evidence of frontal cortical anomalies in NF-I and may provide a pathophysiological basis for cognitive deficits in NF-I.  相似文献   

3.
The cerebellar cognitive affective syndrome (CCAS) is a neurobehavioral syndrome that may develop after congenital and acquired cerebellar lesions. The syndrome consists of deficits in executive functioning, spatial cognition, visual-spatial memory and language and also involves personality and behavioral changes. We describe a 58-year-old right-handed man who in addition to affective disturbances presented with a unique combination of cognitive and linguistic deficits following an ischemic infarction in the vascular territory of the right superior cerebellar artery (SCA). Neurocognitive and neurolinguistic examinations were performed in the acute phase (10 days post-onset) and lesion phase (four weeks post-onset) of the stroke. A Tc-99m-ECD SPECT study was performed five weeks after the stroke. Acute phase data revealed a generalized cognitive decline and mild transcortical sensory aphasia. In the lesion phase, the neurobehavioral tableau was dominated by executive dysfunctions, disrupted divided attention, disturbed visual-spatial organization and behavioral abnormalities. Neurolinguistic investigations disclosed visual dyslexia and surface dysgraphia. Reading of words and visual lexical decision tasks of words and nonwords were severely defective and predominantly characterized by visual errors. In addition, writing irregular and ambiguous words resulted in regularization errors (phonologically plausible errors based on phoneme-grapheme correspondence rules). In the absence of any structural damage in the supratentorial brain regions, a quantified SPECT study showed a relative hypoperfusion in the right cerebellar hemisphere and the left medial frontal lobe. CCAS is for the first time reported in association with visual dyslexia and surface dysgraphia. We hypothesize that the cognitive and linguistic deficits might result from functional disruption of the cerebellar-encephalic pathways, connecting the cerebellum to the frontal supratentorial areas which subserve attentional and planning processes. This phenomenon of crossed cerebellar-cerebral diaschisis is supported by SPECT findings revealing a hypoperfusion in the anatomoclinically suspected brain regions. The constellation of cognitive, linguistic and behavioral symptoms adds new evidence to the multifaceted area of cerebellar neurocognition and demonstrates that the cerebellum might play a crucial role in cognitive, linguistic, and affective processing.  相似文献   

4.
BACKGROUND: The importance of hemodynamic factors in the pathophysiology of stroke and transient ischemic attacks in patients with internal carotid artery (ICA) stenosis or occlusion remains controversial. OBJECTIVE: To investigate whether perfusion-weighted imaging (PWI) could identify pathophysiologically meaningful categories in patients with symptomatic and asymptomatic ICA occlusive disease. METHODS: Thirty-eight consecutive patients with occlusion (22 patients) or severe stenosis (16 patients) of the ICA, determined by ultrasonography or magnetic resonance angiography, were identified from the Beth Israel Deaconess Medical Center Stroke Database, Boston, Mass. Thirty-five patients were symptomatic (29 who had strokes and 6 who had transient ischemic attacks) and 3 were asymptomatic. All symptomatic patients underwent PWI within the first 24 hours after symptom onset. The patterns of PWI were analyzed according to the degree of ICA stenosis and the clinical presentation. RESULTS: Three patterns of perfusion abnormalities were identified: extensive hypoperfusion involving the middle cerebral arterial and/or anterior cerebral arterial territories (25 patients), localized perfusion deficits involving predominantly the ipsilateral border zone areas (8 patients), and normal perfusion (5 patients). All 3 patterns were found whether or not the ICA was occluded. Patients who had acute stroke most frequently had extensive perfusion deficits involving 1 or 2 territories while patients who had transient ischemic attacks often had hypoperfusion affecting the border zone regions. All asymptomatic patients had normal perfusion. CONCLUSIONS: In our sample the pattern of PWI related to the clinical presentation but not to the degree of ICA disease (occlusive vs severe stenosis). Our study findings add further support to the hypothesis that hypoperfusion is a major contributing factor in the pathophysiology of carotid artery occlusive disease.  相似文献   

5.
In a 60-year-old woman with the visual variant of Alzheimer's disease, single photon emission computed tomography abnormalities were most marked in the parieto-occipital regions of the brain. After treatment with donepezil, improvement is noted on neuropsychological testing and on brain SPECT, including increased perfusion (metabolism) in the occipital lobes.  相似文献   

6.
Parietal lobe impairment is hypothesized to contribute to the dramatic visual-spatial deficits in Williams syndrome (WS). The authors examined the superior and inferior parietal lobule in 17 patients with WS and 17 control female adults (CNLs). The right and left superior parietal lobule gray matter volumes were significantly smaller in patients with WS than in CNLs, even after controlling for total cerebral gray matter. Impaired superior parietal function could explain WS visual-spatial and visual-motor problems.  相似文献   

7.
The 15-Objects Test (15-OT) provides useful gradation of visuoperceptual impairment from normal aging through Alzheimer's disease (AD) and correlates with temporo-parietal perfusion. The objectives of this study were to analyze progression of 15-OT performance in mild cognitive impairment (MCI) and AD, and its correlates with cognition and single photon emission computerized tomography (SPECT), as well as to examine neuropsychological and SPECT differences between the MCI patients who developed AD and those who did not. From the initial 126 participants (42/group), 38 AD, 39 MCI, and 38 elderly controls (EC) were reassessed (SPECT: 35 AD, 33 MCI, 35 EC) after two years. The progression of cognitive and SPECT scores during this period was compared between groups, and baseline data between converters and non-converters. The 15-OT was the only measure of progression that differed between the three groups; worsening scores on 15-OT were associated with worsening in verbal and visual retention, and decreased perfusion on left postsubicular area. In the MCI patients, cerebral perfusion fell over the two years in medial-posterior cingulate and fronto-temporo-parietal regions; AD showed extensive changes involving almost all cerebral regions. No SPECT changes were detected in controls. At baseline, the MCI patients who developed AD differed from non-converters in verbal recognition memory, but not in SPECT perfusion. In conclusion, SPECT and 15-OT appear to provide a potential measure to differentiate between normal aging, MCI, and AD. Worsening on 15-OT was related to decreased perfusion in postsubicular area; but further longitudinal studies are needed to determine the contribution of 15-OT as a predictor of AD from MCI.  相似文献   

8.
Down's syndrome (DS) is characterized by moderate mental retardation and a variety of abnormalities involving multiple organ systems. There is a high incidence of Alzheimer's disease (AD) type dementia beyond the age of 35. In this study, single photon emission computed tomography (SPECT) brain perfusion imaging of young Down's syndrome patients was performed to define the perfusion pattern. Tc-99m HMPAO brain perfusion SPECT was performed on 17 young DS patients, aged 3-24 years (mean: 10.9+/-5.9 years). None of the patients had dementia symptoms. Brain perfusion scans were acquired 15 min after i.v. injection of 12 MBq/kg of Tc-99m HMPAO using a single head rotating gamma camera. Images were analyzed visually and semiquantitatively by defining side-to-side asymmetry index. Nine DS cases showed normal brain perfusion. Eight of the 17 cases revealed mostly unilateral parieto-temporal, parieto-occipital and frontal hypoperfusions. The side-to-side asymmetry indices for these visually interpreted regional brain perfusion abnormalities ranged from 6 to 15%. These findings revealing mostly unilateral parieto-temporal and frontal hypoperfusions may not be considered as predictive patterns of dementia related Alzheimer type perfusion deficits in DS. However, such findings may connect to other functional imaging studies related to the higher cortical dysfunction in mental retardation.  相似文献   

9.
The aim of this study was to evaluate cognitive development at the onset of West syndrome (WS) with regard to electroencephalogram (EEG) patterns and visual function. Twenty-five patients (14 males, 11 females) at the onset of spasms (T0) in WS and 2 months later (T1) underwent a full clinical evaluation, including neuroimaging, cognitive assessment, video-EEG, and visual function. Mean age of the patients at spasm onset was 5.9 months (SD 2.5; range 2 to 13mo). Cognitive development, assessed with Griffiths Mental Development Scales (GMDS), was generally impaired at T0 and was significantly related to visual function (p<0.001) at both T0 and T1. In general, there was a specific major impairment in the eye-hand coordination scale of the GMDS which tended to disappear after 2 months in less severe cases. At the onset of spasms, sleep EEG organization seemed to be better related to cognitive abilities than awake hypsarrhythmia. These results support a close link between visual function and cognitive competence in WS and provide additional information to improve the understanding of possible mechanisms underlying cognitive impairment.  相似文献   

10.
BACKGROUND: Previous studies have shown that cocaine abusers have cerebral perfusion deficits that may diminish cognitive functioning. This study examined whether cocaine-dependent patients have perfusion abnormalities associated with poor decision-making ability as measured by the Iowa Gambling Task (IGT). METHODS: Seventeen abstinent cocaine-dependent patients were administered the IGT after completion of resting 99mTc-HMPAO single-photon emission computed tomography (SPECT). RESULTS: Better IGT performance was negatively correlated with perfusion within the anterior cingulate gyrus, middle frontal gyrus, medial frontal gyrus, and superior frontal gyrus. The time to complete card selections was positively correlated with the severity of impairment. CONCLUSIONS: Resting hyperperfusion in brain regions previously implicated in decision making and response inhibition was associated with worse IGT scores. Impaired performance was related to a greater amount of time taken for card selections, suggesting that reduced ability was due to cognitive factors other than an impulsive response pattern.  相似文献   

11.
To further understand the nature of the visual-spatial representations required for successful acquisition of written language skills, we investigated the written language abilities of two individuals with Williams syndrome (WS) a developmental genetic disorder in which the presence of severe visual-spatial developmental delays and deficits has been well established. Using a case study approach, we examined the relationship between reading achievement and general cognitive ability, phonological skills, and visual-spatial skills for the two individuals. We found that, despite the strong similarity between the two individuals in terms of their verbal and non-verbal cognitive abilities and their phonological abilities (as well as chronological age and educational opportunities), their reading and spelling abilities differed by more than 5 grade levels. We present evidence that the difference in written language performance was likely to be due to differences in the severity and nature of their visual-spatial impairment. Moreover, we show that specific difficulty processing the orientation of visual stimuli is related to the reading difficulties of one of the two individuals. These results underscore the contribution of visual-spatial abilities to the reading acquisition process and identify WS as a potential source of valuable information regarding the role of visual-spatial processing in reading development.  相似文献   

12.
目的后部皮质萎缩(posterior cortical atrophy,PCA)是以视觉障碍为突出表现,并伴进行性认知功能受损的神经系统退行性疾病。PCA的临床表现以视觉加工、计算力下降、阅读书写能力受损为主,患者的视觉认知损害突出,而情景记忆、语言能力和自知力相对保留。本文结合我院收治PCA病例,总结PCA的临床及影像学特征,以期为该病的早期诊治提供参考。方法收集2013年至2018年于宣武医院神经内科确诊的后部皮质萎缩8例患者的临床表现、神经心理学检查、影像学资料等各项检查结果并进行回顾性分析。结果8例PCA患者起病均为隐袭起病,病情进展缓慢,其中有5例符合背侧型后部皮质萎缩特点,临床表现为巴林特综合征、视空间功能障碍以及格茨曼综合征特点,头MRI显示顶枕叶皮质萎缩为主;另3例符合腹侧型后部皮质萎缩特点,临床表现主要为面孔失认、视觉忽视,头MRI显示双侧颞枕叶皮质萎缩为主,而内侧颞叶和海马萎缩程度相对较轻。8例患者均行SPECT检查,显示大脑后部包括顶叶、颞枕叶皮质血流低灌注现象。3例患者行18F FDG PET检查显示大脑后部枕叶、顶叶、颞叶皮质葡萄糖代谢降低。结论对于存在视觉障碍和后部皮质相关认知功能损害的患者,临床医生要考虑到PCA的诊断,特别注意进行视觉相关的全面的神经心理学评估及PET检查,以辅助进行PCA的诊断。  相似文献   

13.
The presence of visual hallucinations (VH) is a significant predictor of dementia in Parkinson's disease (PD) and it is associated with a more rapid cognitive decline. Non-demented PD patients with VH present greater neuropsychological impairment than those without VH in domains such as verbal and visual memory, language comprehension, and visuospatial and visuoperceptive functions. Frontal dysfunction has also been described in PD with VH, including deficits in verbal fluency, sustained attention, and inhibition. In PD with VH, structural and functional abnormalities within the primary visual system and visual association areas, including ventral and dorsal pathways, have been reported. Structural MRI studies have shown that non-demented PD patients with VH present grey matter reduction in parieto-occipital areas and the hippocampal head. A follow-up study performed at a mean of 30 months revealed that unlike PD patients without VH, PD patients with VH frequently develop dementia associated with progressive atrophy in limbic, paralimbic and neocortical areas. Functional MRI (fMRI) studies have revealed altered activation in occipito-temporal and frontal areas in response to simple and complex visual stimuli in PD patients with VH, suggesting a marked impairment in bottom-up visual processing, as well as an attentional deficit in the pathophysiology of VH in PD.  相似文献   

14.
Background and Purpose. Cerebral perfusion abnormalities and neuropsychological impairment are common sequelae of chronic cocaine abuse. While perfusion abnormalities have been shown to relate to cognitive deficits in this substance abuse population, the relationship between cognitive performance and the magnitude of perfusion abnormality has yet to be fully determined. Methods. Thirty‐seven abstinent cocaine abusers and 13 normal controls were administered resting 99m‐Tc‐HMPAO single photon emission computed tomography (SPECT) scans followed by a neuropsychological assessment battery tapping executive skills, attention, memory, and motor performance. Statistical parametric mapping (SPM99) techniques were used to analyze the SPECT data to detect significant regional perfusion abnormalities in the cocaine group relative to normal controls, and resulting abnormal SPECT counts were employed for comparison with the assessment measures to examine the relationship between cocaineinduced perfusion abnormalities and cognitive performance. Results. SPECT data analysis revealed significant regional perfusion abnormalities in the cocaine abuse sample relative to controls and significant differences in neuropsychological functioning on measures of executive functioning, complex attention, memory, and manual dexterity. For chronic cocaine abusers, however, within‐group comparisons of the magnitude of abnormal perfusion and neuropsychological performance were largely nonsignificant, with the exception of complex attention and motor speed. Conclusions. Perfusion abnormalities and neuropsychological impairments readily distinguished cocaine abusers from normal controls. However, when the magnitude of cocaine‐induced perfusion abnormalities is examined in relation to cognitive performance, motor speed and complex attention appear to be the best behavioral indicants of the severity of perfusion dysfunction within this substance abuse population.  相似文献   

15.
The aim of this work is to study the cognition, progressive gait impairment, and neuroimaging findings in two patients over 65?years old of the previously described type 3 familial cortical myoclonic tremor with epilepsy (FCMTE3). We report investigations in two of these five FCMTE3 subjects over 65 and showing progressive gait disorders. They both had a pseudo-Parkinson??s way of walking and visual intolerance to bright light and brightness contrast without EEG abnormalities exacerbating cortical myoclonus or triggering seizure. Case 1 had moderate gait impairment and a severe frontal syndrome. Case 2 had severe gait impairment and diffuse cognitive disorders. Both cases had cortical hypoperfusion (predominantly in the left frontal lobe) and no cerebellar abnormality on cerebral perfusion SPECT. DAT-SPECT showed dopaminergic depletion. These data indicate fronto-striatal dysfunction associated with gait impairment and cognitive disorders appearing after several decades of disease progression. This gives clues to understanding the pathogenesis and evolution of FCMTE3. Permanent myoclonic discharges or long-term valproate treatment may cause significant toxic effects on neurons (dopaminergic and frontal neurons). Further functional and molecular analyses are required in order to better understand this pathology and the consequences of chronic cortical myoclonus.  相似文献   

16.
Diffusion- and perfusion-weighted MRI patterns in borderzone infarcts   总被引:10,自引:0,他引:10  
BACKGROUND AND PURPOSE: The pathophysiology of borderzone infarcts is not well understood. We investigated whether combined diffusion-weighted imaging (DWI) and perfusion-weighted imaging (PWI) could identify pathophysiologically meaningful categories of borderzone infarcts. METHODS: Seventeen patients with borderzone infarcts were identified from the Beth Israel Deaconess Medical Center Stroke Database. All patients had DWI and PWI, the majority of them within the first 24 hours of symptom onset. RESULTS: Three patterns of perfusion abnormalities were associated with the diffusion lesions: 1, normal perfusion (5 patients); 2, localized perfusion deficits matching the area of restricted diffusion (5 patients); and 3, extensive perfusion deficits involving 1 or more vascular territories (7 patients). All but 1 patient with pattern 1 had transient peri-infarct hypotension as the presumed stroke mechanism. Two patients with pattern 2 had cardiac or aortic embolic sources; none had large-artery disease or arterial hypotension. Reperfusion was detected in all patients with this pattern who submitted to a follow-up study. All patients with pattern 3 had severe stenosis or occlusion of a large artery: the internal carotid, anterior cerebral, or middle cerebral. CONCLUSIONS: We postulate that the perfusion abnormality varies according to the mechanism of the borderzone infarction. Transient perfusion deficits occurring with hypotension in the absence of significant large-artery disease may not be revealed by PWI. Embolism may cause some cases of small borderzone perfusion deficits. Critical large-artery disease may cause large territorial perfusion deficits and predispose to borderzone infarction.  相似文献   

17.
BACKGROUND: Patients with objective evidence of memory impairment have been considered to be at risk for developing Alzheimer's disease (AD). However, little is known about patterns of regional cerebral blood flow abnormalities and their prognostic significance in these patients. METHODS: The authors retrospectively studied 28 nondemented subjects with memory loss and investigated patterns of blood flow abnormalities on single photon emission computed tomography (SPECT). RESULTS: The patients were followed up for more than 2 years; during follow-up, 14 patients (50%) developed AD. The onset of memory impairment in patients who progressed to AD was significantly earlier than in those who remained in a nondemented condition. SPECT data from the initial evaluation were analyzed by region of interest analysis and statistical parametric mapping. Interestingly, both groups of patients shared hypoperfusion in the medial temporal regions and the posterior cingulate. In addition to these regions, significant blood flow reduction in the parietal and anterior cingulate cortices was detected in patients who progressed to AD. CONCLUSIONS: These results demonstrate that (1) subjects with an earlier onset of memory loss have an increased risk for developing AD, (2) SPECT can be useful for distinguishing subjects with memory loss who will rapidly progress to AD from those who will not, and (3) perfusion impairment typical of AD was evident even in subjects with memory impairment who remained nondemented.  相似文献   

18.
Summary. Due to the increasing importance of early recognition and differential diagnosis of dementias, cerebral perfusion scans using “single photon emission computed tomography” (SPECT) are increasingly integrated into the examination routine. The goal of this study was to check the diagnostic validity of SPECT scans of MCI- and DAT-patients, two subgroups out of 369 persons with etiologically unclear cognitive dysfunction, which underwent an assessment program for probable dementia including cognitive testing, cranial computed tomography, ultrasound, routine laboratory testing including vascular risk factors. After exclusion of patients with no or other forms of dementia we analyzed SPECT data of patients with mild cognitive impairment (MCI; n = 85) and dementia of the Alzheimer type (DAT; n = 78) in comparison with a healthy control group (n = 34). Visual assessment as well as a manual “regions of interest” (ROI) regionalization of the cortex were performed, whereby a ROI/cerebellum ratio was calculated as a semi-quantitative value. Association cortex areas were assessed regarding frontal, temporal, and parietal lobes of both hemispheres. When comparing the ratios of patients with DAT and controls, we found a statistically significant reduction of the cerebral perfusion in all measured cortex areas (p < 0.001). The comparison of patients with MCI with the selected control group also established a statistically significant difference in the cerebral perfusion for the evaluated cortex areas with the exception of the left hemispheric frontal and parietal cortex. A considerable number of the MCI patients showed an MMSE-score within the normal range, but with regard to the perfusion in the right hemispheric association cortex these patients also could be distinguished unambiguously from controls. Sensitivity levels found by visual assessment were at least as high as those found by the ROI method (pathological assessment: visual 49.4% vs. ROI 47.1% for MCI; visual 75.6% vs. ROI 73.1% for DAT). High experienced visual assessment of cerebral perfusion scans using SPECT provides an useful additional tool in diagnosis of cognitive impairment. The used semiquantitative ROI-method is nearly equivalent and does not depend on the experience of the investigator.  相似文献   

19.
Study of Regional Cerebral Blood Flow in West Syndrome   总被引:8,自引:8,他引:0  
Summary: Focal cortical disturbances are frequent sequelae in West syndrome (WS) even though it is a generalized epileptic syndrome. Functional neuroimaging was used to determine whether focal perfusion abnormalities exist at WS onset and change during evolution. We studied regional cerebral blood flow (rCBF) at different stages of WS. Mean CBF (mCBF) and rCBF were measured using SPECT (single photon emission computed tomography) and 133Xe in 13 WS patients: at onset (20 cases), just after steroids (17 cases), and after a mean follow-up of 2 years (26 cases). At WS onset, interictal mCBF was increased as the result of foci of hyper- and hypoperfusion, which were, respectively, mainly located in the frontal and posterior cortex. Just after steroid therapy, mCBF decreased without any focal predominance. During follow-up, hypoperfused foci remained unchanged whereas the frontal hyperperfused foci decreased after spasm control. Our results show that focal abnormalities are present at WS onset. Focal hypoactivity could reflect a cortical lesion responsible for WS and focal hyperactivity could play a role in the persistence of generalized epilepsy.  相似文献   

20.
Diagnosis of probable Alzheimer disease (AD) is made by a combination of characteristic clinical findings, when normal laboratory studies reveal no structural or metabolic cause of the dementia. Definite diagnosis of AD, however, can only be made with brain tissue examination. PET scanning reveals parietotemporal decreases in cerebral blood flow (CBF) and glucose metabolism that differentiate AD from normal elderly and from multi-infarct dementia. Preliminary studies suggest that similar defects in CBF are detectable in single photon emission computed tomography (SPECT) in AD. Utilizing the iodinated ligand [123I] HIPDM ([123I] hydroxyiodobenzylpropanediamine), we studied 19 patients with probable AD of varying severity, with emphasis on mild cases, to assess the utility of SPECT as a diagnostic test in AD. Parietotemporal perfusion on SPECT was decreased unilaterally or bilaterally in 16 of 19 AD patients, similar to the defects reported with PET. The degree and extent of decreased CBF on SPECT correlated with AD severity. Strong correlations were obtained between decreases in computer-generated ratios of parietal to cerebellar activity and the level of cognitive function. SPECT was read as normal (on the radiographic film) by the nuclear medicine physician in all cases with Mini-Mental State (MMS) score greater than 24, and showed bilateral parietal perfusion deficits in only 1 of 4 patients with MMS between 22 and 24. Ten of 12 patients with MMS less than or equal to 21 had bilateral parietal abnormalities; the other 2 had unilateral perfusion defects. All patients with MMS less than 15 were bilaterally abnormal. SPECT is less expensive and more widely available than PET, and may have an adjunctive role in diagnosis of AD and other dementias if utilized under the proper circumstances.  相似文献   

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