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Purpose: Cognitive decline in Down syndrome generally shows neurodegenerative aspects similar to what is observed in Alzheimer’s disease. Few studies reported information on white matter integrity. The aim of this study was to evaluate white matter alterations in a cohort of young Down subjects, without dementia, by means of DTI technique, compared to a normal control group.Methods: The study group consisted of 17 right-handed subjects with DS and many control subjects. All individuals participating in this study were examined by MR exam including DTI acquisition (32 non-coplanar directions); image processing and analysis were performed using FMRIB Software Library (FSL version 4.1.9, http://www.fmrib.ox.ac.uk/fsl)) software package. Finally, the diffusion tensor was estimated voxel by voxel and the FA map derived from the tensor. A two-sample t test was performed to assess differences between DS and control subjects.Results: The FA is decreased in DS subjects, compared to control subjects, in the region of the anterior thalamic radiation, the inferior fronto-occipital fasciculum, the inferior longitudinal fasciculum, and the cortico-spinal tract, bilaterally.Conclusions: The early white matter damage visible in our DS subjects could have great impact in the therapeutic management, in particular in better adapting the timing of therapies to counteract the toxic effect of the deposition of amyloid that leads to oxidative stress. [ABSTRACT FROM AUTHOR]

Objective: To assess what kind of information MR examination in flexed and extended positions provides in Down syndrome subjects with suspected cranio-cervical instability. Methods: Between 2005 and 2008, 35 subjects with DS were recruited in the study. Ethics committee approval was granted and a signed informed consent was obtained from the parents. All the subjects were affected by hypotonic status and ligament laxity established by clinical evaluation, but were asymptomatic about focal neurological symptoms due to medullar damage caused by cranio-cervical instability. Each patient underwent lateral supine radiographs and MR imaging in the neutral, active flexed and extended positions. For evaluating the atlanto-axial and atlanto-occipital joint stability, multiple measurements were calculated. Results: A significant reduction of anterior subarachnoid space in flexed position was evident in DS subjects compared to healthy controls in neutral and flexed positions. Both, space available for cord and ligamentous thickness showed significant differences between DS subjects and healthy controls. In DS subjects with occipito-cervical instability, the anterior subarachnoidal space reduction was significantly reduced in flexed position. Conclusions: In DS subjects with asymptomatic cranio-cervical instability, anterior subarachnoidal evaluation and ligamentous status could add new information about the risk of spinal cord damage. [ABSTRACT FROM AUTHOR]

Introduction: The aim of this study was to determine differences in the characteristic pattern of age-related cortical thinning in men and women with Down's syndrome (DS) by means of MRI and automatic cortical thickness measurements and a cross-sectional design, in a large cohort of young subjects. Methods: Eighty-four subjects with DS, 30 females (11-35 years, mean age ± SD = 22.8 ± 5.9) and 54 males (11-35 years, mean age ± SD = 21.5 ± 6.5), were examined using a 1.5-T scanner. MRI-based quantification of cortical thickness was performed using FreeSurfer software package. For all subjects participating in the study, the Pearson product-moment correlation coefficient between age and mean cortical thickness values has been evaluated. Results: A significant negative correlation between cortical thickness and age was found in female DS subjects, predominantly in frontal and parietal lobes, bilaterally. In male DS subjects, a significant negative correlation between cortical thickness and age was found in the right fronto-temporal lobes and cingulate regions. Whole brain mean cortical thickness values were significantly negative correlated with age only in female DS subjects. Conclusions: Females with Down's syndrome showed a strong correlation between cortical thickness and age, already in early age. We suggest that the cognitive impairment due to hormonal deficit in the postmenopausal period could be emphasized by the early structural decline of gray matter in female DS subjects. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this study was to verify the sensitivity and specificity of the hyperdense middle cerebral artery sign (HMCAS) obtained by multidetector computed tomography (CT) in predicting acute stroke, using diffusion-weighted (DW) magnetic resonance imaging (MRI) as a reference. The location of the HMCAS, the extension of the ischaemic lesion and its prognostic value were also assessed. Materials and methods: The CT examinations of 654 patients with symptoms related to acute cerebral stroke were retrospectively reviewed. DW-MRI confirmed recent stroke in 175 patients. Two expert neuroradiologists analysed the CT examinations of these patients in four phases. Sensitivity, specificity and interobserver reliability was evaluated. Patients were divided into three groups according to the HMCAS site (M1-M2-M3) and the Alberta Stroke Program Early CT Score (ASPECTS) on DW-MRI was calculated. The ASPECTS average score was correlated with the National Institutes of Health Stroke Scale (NIHSS) and modified Rankin scale (mRS) at 3 months. Results: In 41 patients, the presence of HMCAS was confirmed (71 % sensitivity; 100 % specificity; Interobserver reliability k, 84 %). An inverse correlation was found by comparing the ASPECTS and NIHSS scores (Rsq = −0.206). After logistic regression analysis, HMCAS was found to be independently associated with a poor outcome (mRS >2) at 3 months after adjusting for age, NIHSS on admission, risk factors and aetiology of stroke. Conclusions: Our study demonstrated that HMCAS obtained with multidetector CT can be detected in more than 70 % of patients with large acute ischaemic lesion and it is an unfavourable prognostic sign. [ABSTRACT FROM AUTHOR]

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We report a case of a woman presenting, 7 days after epidural analgesia for a caesarean section, to the emergency room for a worsening of the headache and tonico-clonic seizures. MRI showed alterations suggestive of the presence of intracranial hypotension (IH) as well as evidence of posterior reversible encephalopathy syndrome (PRES). She was treated with a blood patch which leads to the prompt regression of the clinical symptoms and follow-up MRI, after 15 days, showed complete resolution of radiological alterations. The possible pathogenetic relationship between IH, secondary to the inadvertent dural puncture, and PRES is discussed. We suggest that venous stagnation and hydrostatic edema, secondary to intracranial hypotension, probably played a crucial role in the pathogenesis of PRES. [ABSTRACT FROM AUTHOR]

A rapidly progressing dementia, followed by focal neurological signs, and evidence of periodic sharp wave complexes (PSWC) in the EEG may lead to the clinical suspicion of Creutzfeldt-Jakob disease (CJD). Different clinical variants of CJD have been described in the past, with prominent extrapyramidal or occipital lobe involvement, all included in the sporadic form of CJD (sCJD). Familiar and iatrogenic forms of CJD are also known. More recently a new variant has been described, vCJD [1], casually linked to bovine spongiform encephalopathy (BSE) and it has attracted increasing attention toward each form of rapidly progressing dementia; likewise the differential diagnosis between sCJDvs. vCJD is not always easy. Magnetic resonance imaging (MRI) too seems to have a peculiar role in differentiating sCJD from vCJD, even if the role of MRI in the diagnosis of CJD is still debated. Diffusion MRI is expected to play an important role in the clinical setting of CJD, contributing to formulation of an early diagnosis, especially in cases with unusual clinical presentation. In fact, the sensitivity of diffusion MRI is superior to that of conventional MRI (T1, T2, FLAIR) in detecting specific basal ganglia and cortical abnormalities early in the course of CJD [2] and these abnormalities correlate well with areas of the most severe and characteristic neuropathological changes [3]. We describe a case of autopsy-proven sCJD, with an unusual clinical course without dementia as a presenting symptom and discuss the role of diffusion MRI and laboratory tests in making an early diagnosis. [ABSTRACT FROM AUTHOR]

We report a case of Tolosa-Hunt syndrome (THS) in a patient with systemic lupus erythematosus studied with MRI. Magnetic resonance showed enlargement of the cavernous sinus and compression of the carotid syphon by enhancing tissue. In particular, fat-suppressed T1-weighted images before and after contrast agent injection and MR angiography showed extension of the abnormal tissue to the apex of the orbit and narrowing of the internal carotid artery. A presumptive diagnosis of THS was made and steroid treatment was started with rapid relief of symptoms. Follow-up MR study after steroid therapy demonstrated sub-total resolution of the neuroradiological findings. Neuroradiological findings in THS are quite typical but they may be subtle; furthermore, the presence of a systemic disease may suggest secondary involvement of the cavernous sinus. Utilization of the appropriate MR techniques and follow-up exams may contribute to the diagnosis of THS even in the presence of other systemic diseases. [ABSTRACT FROM AUTHOR]

Our aim was to relate MRI findings in patients with severe traumatic brain injury (TBI) to clinical severity and long-term outcome. We studied 37 patients with severe TBI, who were submitted to clinical assessment for disability and cognition and to MRI 60–90 days after trauma. Clinical assessment was also performed 3, 6 and 12 months later. The number and volume of lesions in various cerebral structures were calculated semiautomatically from FLAIR and fast field-echo images. Possible correlations between total and regional lesion volume and clinical deficits were then investigated. The frontal and temporal lobes were most frequently involved. Total lesion volume on FLAIR images correlated significantly with clinical outcome, whereas that on FFE images did not. Regional analysis showed that FLAIR lesion volume in the corpus callosum correlated significantly with scores on disability and cognition scales at the first clinical assessment. FLAIR lesion volume in the frontal lobes correlated significantly with clinical scores 1 year later. [ABSTRACT FROM AUTHOR]

We describe a pleomorphic xanthoastrocytoma (PXA) in a young girl whose frontal lobe location, solid structure, dural tail and MRI signal characteristics led to a preoperative diagnosis of meningioma. PXA should be considered in differential diagnosis of tumours affecting young patients with neuroradiological characteristics suggestive of meningioma. [ABSTRACT FROM AUTHOR]

Neuroimaging techniques aimed at studying structural changes of the brain may provide useful information for the diagnosis and the clinical management of patients with dementia. Magnetic resonance imaging (MRI) may show abnormalities amenable to surgical treatment in a significant percentage of patients with cognitive impairment. MRI may also assist the differential diagnosis in dementia associated with metabolic or inflammatory diseases.MRI has the potential to detect focal signal abnormalities which may assist the clinical differentiation between Alzheimer's disease (AD) and vascular dementia (VaD). Severe temporal atrophy, hyperintensities involving the hippocampal or insular cortex, and gyral hypointense bands are more frequently noted in AD. Basal ganglionic/thalamic hyperintense foci, thromboembolic infarctions, confluent white matter and irregular periventricular hyperintensities are more common in VaD.The high sensitivity of MRI in detecting T2 hyperintense lesions and the low specificity off white matter lesions have resulted in a poor correlation between MRI findings and both neuropathological and clinical manifestations. In particular, MRI has disclosed a series of white matter focal changes in the elderly population, which are not necessarily associated with cognitive dysfunction.The recent advent of a new MRI method sensitive to the microstructural changes of white matter, the so-called diffusion tensor imaging, may be helpful in correlating clinical manifestations with white matter abnormalities. [ABSTRACT FROM AUTHOR]

In a previous study, we found that the extent of necrosis was the only radiological feature which correlated significantly with survival in patients with glioblastoma. The aim of this paper was to evaluate the variability and prognostic value of the extent of the necrotic area as seen on contrast-enhanced MRI and CT in a larger series. We studied 72 patients who underwent surgical removal of supratentorial glioblastomas and had CT and/or MRI with contrast medium before surgery; 38, all undergoing the same treatment (surgery plus radiotherapy), were followed clinically. Necrosis within the tumour varied greatly, ranging from none (only 1 case) to involvement of 76 % of the tumour. Survival data in the subgroup suggested that only patients with a small area of necrosis (less than 35 % of the tumour) had a significantly longer survival time. When necrosis involved more than 35 % of the mass, patients had a shorter survival time, without any further correlation with the extent of necrosis. [ABSTRACT FROM AUTHOR]

Few studies have attempted to correlate neuroimaging with outcome in patients with glioblastoma. Our aim was to evaluate the relationship between neuroradiological findings and survival in these patients. We studied 18 consecutive patients with glioblastoma who had undergone surgery and radiotherapy. We assessed the following features, using preoperative CT and/or MRI: tumour size, extent of necrotic area within the mass, extent of perifocal oedema and contrast enhancement. The mean survival was 14.2 ± 5 months (range 6-22). The extent of radiological evidence of necrosis within the mass correlated significantly with survival time, whereas tumour size, perifocal oedema and contrast enhancement did not. [ABSTRACT FROM AUTHOR]

The early CT finding of an hyperdensity of a portion of the middle cerebral artery Hyperdense Middle Cerebral Artery Sign (HMCAS), in patients with supratentorial stroke, is often indicative of an embolic occlusion. Aim of this study was to verify the incidence and reliability of the HMCAS and its possible correlation with early CT findings and with the extent of late brain damage. We studied 36 patients presenting with symptoms of stroke in the MCA territory, by means of CT and angiography performed respectively within 4 and 6 hours. Follow-up CT scans were then obtained after one week and three months from the ischemic event. The HMCAS was present in 50% of our patients and in this group it always correlated positively with the angiographic finding of occlusion. The same group presented a high incidence of erly CT hypodensity (88%). Finally the presence of HMCAS might be considered a negative prognostic sign for the development of extensive brain damage. [ABSTRACT FROM AUTHOR]

The aim of this study was to obtain an MRI severity-related classification of diffuse astrocytic tumours able to integrate the histological data in the grading of such tumours. We studied presurgical MR images of 91 patients with a histological diagnosis of astrocytoma, anaplastic astrocytoma and glioblastoma. A score ranging from 1 to 3 was assigned by two independent readers to each of the following MR features: oedema, mass effect, contrast enhancement, borders, signal homogeneity, necrosis, haemorrhage and flow void. Statistical analysis showed significant differences in the mean MRI scores between the three histological grades. Contrast enhancement was found to be the best predictor of the histological grade followed by necrosis, signal homogeneity and border scores. This classification represents a simple and reproducible means of carefully evaluating some macroscopic characteristics of these tumours. It could be used to integrate histological data especially in cases in which tissue sampling defects may affect the validity of this examination. [ABSTRACT FROM AUTHOR]

Single-photon emission tomography (SPET) with thallium-201 is used in the assessment of patients with gliomas because the amount ofTl accumulated by the tumoral cells increases in proportion to the degree of tumour malignancy, thus making it possible to differentiate high-grade from low-grade gliomas or recurrences from radiation necrosis. However, in large areas of tissue such as those examined inTl SPET studies, the uptake ofTl may vary considerably even in tumours with the same histological diagnosis, as occurs in glioblastomas (GBMs). In order to evaluate the possible influence of the macroscopic characteristics of tumours onTl uptake, we studied a series of 13 patients with histologically proven GBMs, comparing magnetic resonance imaging (MRI) parameters such as tumour dimensions, perilesional oedema, intratumoral necrosis and contrast enhancement with the degree ofTl uptake. The patients underwent bothTl SPET and MRI before surgery. TheTl index (tumour/contralateral unaffected brain) was calculated using two different region of interest (ROI) methods: the first employed irregular large ROIs (3.2±13.9 cm) including pixels with more than 50% maximum activity; the second employed regular square small ROls (2.7 cm) centered on the maximum activity of the lesion. Of the MRI morphological parameters studied, only necrosis significantly reduced the degree ofTl uptake in GBMs when larger ROIs were used. However, by using small regular ROIs the influence of necrosis onTl uptake was found to be less relevant. Since necrosis is related to tumour proliferative activity and represents a negative prognostic factor in astrocytoma, a possible underestimation ofTl uptake due to intratumoral necrosis must be carefully evaluated. [ABSTRACT FROM AUTHOR]

We describe a case of asymmetric PRES due to the presence of hyperplastic anterior choroidal artery (AChA) in a man affected by sever hypertension. Posterior reversible encephalopathy syndrome (PRES) has become synonymous with a unique pattern of brain vasogenic edema and predominates in the parietal and occipital regions, accompanied by clinical neurological alterations. Sever hypertension is a risk factor that exceeds the limits of brain autoregulation, leading to breakthrough brain edema. In our knowledge this is the first case reported in literature, in which a similar vascular abnormality is linked to a PRES syndrome. [ABSTRACT FROM AUTHOR]

The article focuses on a case of SPG7-Paraplegin mutation in a 43 year old patient, who first experienced severe visual problems since his childhood. Topics discussed include the medical history of the patient including identification of moderate spastic paraparesis at age 43, as well as identification of the same mutation on his father. Also mentioned is the association of optic neuropathy and SPG7 mutations.

A reply by S. Pugliese, V. Finocchi, and M. L. Borgia et al to a letter to the editor about their article "Intracranial Hypotension and PRES: Case Report" in a 2010 issue of the journal is presented.