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Hyperintensities
Kinds of Hyperintensities Selected AbstractsMagnetic resonance images of the globus pallidus in patients with idiopathic portal hypertension: A quantitative analysis of the relationship between signal intensity and the grade of portosystemic shuntJOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY, Issue 5 2006Takeshi Fukuzawa Abstract Background and Aim:, To elucidate a quantitative relationship between hyperintensity of the globus pallidus on T1-weighted magnetic resonance images (MRI) and portosystemic shunt (PSS) in portal hypertension. Methods:, Fifteen patients with idiopathic portal hypertension (IPH) and 44 patients with liver cirrhosis (LC) underwent brain MRI to asses signal intensity at the globus pallidus and Doppler sonography to examine the blood flow volume of PSS. Blood manganese (Mn) levels were examined in 36 patients and neuropsychological tests were performed in 15 patients without overt hepatic encephalopathy. Results:, Pallidal hyperintensity on MRI was more prominent in patients with IPH than in patients with LC. There was no correlation between MRI pallidal hyperintensity and the severity of liver dysfunction or hepatic encephalopathy. The grade of hyperintensity correlated well with the grade of PSS. The correlation was stronger in patients with IPH than in patients with LC. The plasma ammonia level and whole blood Mn level significantly correlated with MRI pallidal hyperintensity, but blood Mn level showed a stronger correlation than plasma ammonia. Conclusion:, Hyperintensity of the globus pallidus on T1-weighted MRI correlated with the development of PSS independent of liver cell function. This brain image should be an index of the grade of PSS rather than a landmark of chronic liver failure. [source] The diagnostic utility of FLAIR imaging in clinically verified amyotrophic lateral sclerosisJOURNAL OF MAGNETIC RESONANCE IMAGING, Issue 5 2003Lijuan Zhang MD Abstract Purpose To explore the overall diagnostic ability of magnetic resonance (MR) fluid-attenuated inversion recovery (FLAIR) imaging for clinically verified amyotrophic lateral sclerosis (ALS). Materials and Methods Abnormal signal intensity in FLAIR images of 18 patients with ALS and 18 age-matched normal controls were scored and compared. Six patients had serial MR imaging scans within 97 days. Mann Whitney U statistics and ANOVA were used for statistical analysis. Results Hyperintensity in the subcortical white matter (SWM) and the dark line along the posterior rim of the precentral gyri were found more frequently in patients with ALS. The scores for these two signs were significantly different from those of normal controls. Hyperintensity in the corticospinal tract (CST) was found in both ALS and normal controls, but the difference was not statistically significant. ANOVA on the serial FLAIR studies revealed no significant difference in the scores among the series. The hyperintensity in SWM had a sensitivity of 56% and specificity of 94%, while the dark line in the motor cortex had a 74% sensitivity and 67% specificity. Conclusion Hyperintensity in SWM and the dark line along the posterior rim of the precentral gyri appeared more frequently in the patients with ALS. SWM hyperintensity has a better specificity in association with clinically verified ALS, while the motor dark line has a better sensitivity. No correlation was found between the FLAIR findings and progression of the disease. J. Magn. Reson. Imaging 2003;17:521,527. © 2003 Wiley-Liss, Inc. [source] White matter lesions in euthymic patients with bipolar disorderACTA PSYCHIATRICA SCANDINAVICA, Issue 6 2009A. J. Lloyd Objective:, We aimed to quantify both load and regional distributions of hyperintensities on magnetic resonance imaging (MRI) in prospectively verified euthymic bipolar patients and matched controls. Method:, Cerebral hyperintensities on T2, proton density and fluid-attenuated inversion recovery (FLAIR) MRI were compared between 48 bipolar and 47 control subjects using semi-quantitative rating scales. Results:, Bipolar subjects had more severe frontal deep white matter lesions (DWML). Hyperintensity load was independent of age in bipolar patients but increased with age in controls. Global prevalence and severity of hyperintensities did not differ between groups. Exploratory analysis showed DWML in excess in the left hemisphere in bipolar subjects but not in controls. Conclusion:, Findings are consistent with clinical, particularly some neurocognitive, features of bipolar disorder and implicate fronto-subcortical circuits in its neurobiology. They more probably reflect a trait abnormality or illness scar rather than a mood state-dependent finding. Processes other than ageing and vascular factors may underlie their development. [source] The use of neuroimaging in the diagnosis of mitochondrial diseaseDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 2 2010Seth D. Friedman Abstract Mutations in nuclear and mitochondrial DNA impacting mitochondrial function result in disease manifestations ranging from early death to abnormalities in all major organ systems and to symptoms that can be largely confined to muscle fatigue. The definitive diagnosis of a mitochondrial disorder can be difficult to establish. When the constellation of symptoms is suggestive of mitochondrial disease, neuroimaging features may be diagnostic and suggestive, can help direct further workup, and can help to further characterize the underlying brain abnormalities. Magnetic resonance imaging changes may be nonspecific, such as atrophy (both general and involving specific structures, such as cerebellum), more suggestive of particular disorders such as focal and often bilateral lesions confined to deep brain nuclei, or clearly characteristic of a given disorder such as stroke-like lesions that do not respect vascular boundaries in mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episode (MELAS). White matter hyperintensities with or without associated gray matter involvement may also be observed. Across patients and discrete disease subtypes (e.g., MELAS, Leigh syndrome, etc.), patterns of these features are helpful for diagnosis. However, it is also true that marked variability in expression occurs in all mitochondrial disease subtypes, illustrative of the complexity of the disease process. The present review summarizes the role of neuroimaging in the diagnosis and characterization of patients with suspected mitochondrial disease. © 2010 Wiley-Liss, Inc. Dev Disabil Res Rev 2010;16:129,135. [source] Diabetes mellitus, hypertension and medial temporal lobe atrophy: the LADIS studyDIABETIC MEDICINE, Issue 2 2007E. S. C. Korf Abstract Hypothesis, Based on recent findings on the association between vascular risk factors and hippocampal atrophy, we hypothesized that hypertension and diabetes mellitus (DM) are associated with medial temporal lobe atrophy (MTA) in subjects without disability, independent of the severity of white matter hyperintensities. Methods, In the Leukoaraiosis And DISability in the elderly (LADIS) study, we investigated the relationships between DM, hypertension, blood pressure and MTA in 582 subjects, stratified by white matter hyperintensity severity, using multinomial logistic regression. MTA was visually scored for the left and right medial temporal lobe (score 0,4), and meaned. Results, Mean age was 73.5 years (sd 5.1), 54% was female. Of the subjects, 15% had DM, and 70% had a history of hypertension. The likelihood of having MTA score 3 was significantly higher in subjects with DM (OR 2.9; 95% CI: 1.1,7.8) compared with an MTA score of 0 (no atrophy). The odds ratio for MTA score 2 was not significantly increased (OR 1.8; CI: 0.9,4). Systolic and diastolic blood pressure and a history of hypertension were not associated with MTA. There was no interaction between DM and hypertension. Stratification on white matter hyperintensities (WMH) did not alter the associations. Conclusion Our study strengthens the observation that MTA is associated with DM, independently of the amount of small vessel disease as reflected by WMH. [source] Ventricular cerebrospinal fluid neurofilament protein levels decrease in parallel with white matter pathology after shunt surgery in normal pressure hydrocephalusEUROPEAN JOURNAL OF NEUROLOGY, Issue 3 2007M. Tullberg Normal pressure hydrocephalus (NPH) is characterized by disturbed cerebrospinal fluid (CSF) dynamics and white matter lesions (WML). Although the morphology of these lesions is described, little is known about the biochemistry. Our aim was to explore the relationship between ventricular CSF markers, periventricular WML and postoperative clinical outcome in patients with NPH. We analysed lumbar and ventricular concentrations of 10 CSF markers, 12 clinical symptoms and signs, magnetic resonance imaging (MRI) periventricular white matter hyperintensities (PVH) and ventricular size before and 3 months after shunt surgery in 35 patients with NPH. Higher ventricular CSF neurofilament protein (NFL), an axonal marker, correlated with more extensive PVH. A larger postoperative reduction in NFL correlated with larger reduction in PVH and a more pronounced overall improvement. Albumin ratio, HMPG, NPY, VIP and GD3 increased postoperatively whereas NFL, tau and HVA decreased. Variations in ventricular size were not associated with CSF concentrations of any marker. We conclude that NPH is characterized by an ongoing periventricular neuronal dysfunction seen on MRI as PVH. Clinical improvement after shunt surgery is associated with CSF changes indicating a restitution of axonal function. Other biochemical effects of shunting may include increased monoaminergic and peptidergic neurotransmission, breakdown of blood brain barrier function, and gliosis. [source] Construction of periventricular white matter hyperintensity maps by spatial normalization of the lateral ventriclesHUMAN BRAIN MAPPING, Issue 7 2009Cynthia Jongen Abstract Subcortical and periventricular white matter hyperintensities (WMHs) may have different associations with cognition and pathophysiology. The aim of the present study is to develop an automated method for construction of periventricular WMH maps that enables the analysis of between-group differences in WMH location and characteristics in the periventricular region without the requirement of prior boundary definition. To avoid influence of WMHs on spatial normalization, a reference image of the lateral ventricles was constructed based on images of 24 subjects. Construction was not biased to a single subject. WMHs were segmented by k-nearest neighbor-based classification of magnetic resonance inversion recovery and fluid attenuated inversion recovery images. Cerebrospinal fluid segmentations of individual subjects were nonrigidly mapped to the reference image of the lateral ventricles. The subject's WMHs were transformed to the reference space accordingly. Spatial normalization accuracy was validated using measures of overlap and of displacement relative to the boundary of the lateral ventricles. After spatial normalization, the boundaries of the lateral ventricles closely matched the reference image and in an area of ,1 cm around the lateral ventricles the relative displacement was less than 1 mm. To illustrate the method, it was applied to 61 patients with Type 2 diabetes and 26 control subjects, whereupon periventricular WMH maps were constructed and compared. The proposed method is particularly suited to analyze WMH distribution differences at the level of the lateral ventricles between large groups of patients. Hum Brain Mapp, 2009. © 2008 Wiley-Liss, Inc. [source] Cognitive correlates of brain MRI subcortical signal hyperintensities in non-demented elderlyINTERNATIONAL JOURNAL OF GERIATRIC PSYCHIATRY, Issue 1 2006Gad A. Marshall Abstract Objective To investigate the relationship between magnetic resonance imaging (MRI) subcortical gray and capsular (SGCH) and white matter hyperintensities (WMH) and cognitive functions in non-demented community dwelling elderly. Methods The severity of SGCH and WMH on proton density and T2 MR images in 16 subjects was scored using the semi-quantitative rating scale of Scheltens et al. (1993). A limited series of cognitive tests selected a priori were then correlated with severity of SGCH and WMH. Results Analysis demonstrated that severity of SGCH was inversely related to performance on the Digit Span (R,=,,0.64, p,<,0.01) and the Stroop Color Word Tests (R,=,,0.64, p,<,0.01). Severity of WMH was related to worsening performance on the Trail Making Test (R,=,0.67, p,<,0.005). Conclusions These findings indicate that severity of WMH is negatively related to more pure executive cognitive functions, specifically set shifting, while severity of SGCH is inversely related to more basic functions of attention and working memory. Copyright © 2005 John Wiley & Sons, Ltd. [source] Carotid intima-media thickness in late-onset major depressive disorderINTERNATIONAL JOURNAL OF GERIATRIC PSYCHIATRY, Issue 1 2006Cheng-Sheng Chen Abstract Background This study explored whether patients with late-onset major depressive disorder showed higher carotid artery intima-medium thickness (IMT) and investigated the relationship between the IMT and white matter hyperintensities on magnetic resonance imaging (MRI) among patients. Methods Fourteen elderly patients with late-onset major depressive disorder from a psychiatric outpatient clinic and 11 non-depressed controls received a comprehensive psychiatric assessment, ultrasound IMT measurements of the carotid arteries, and cerebral MRI. Results The carotid IMT was higher in the patient group vs the control group (1.26,±,0.30 vs 1.00,±,0.20,mm; t,=,2.40, p,<,0.03). The difference was more apparent in the common carotid artery (1.20,±,0.32 vs 0.97,±,0.13,mm; t,=,2.31, p,<,0.04). There was a high correlation (r,=,0.55, p,<,0.05) between the carotid IMT and white matter hyperintensities among patients with late-onset major depressive disorder. Conclusion Results of this study suggest that atherosclerosis represented by the carotid IMT contributes to the development of late-onset major depressive disorder. The findings support the vascular depression hypothesis. Copyright © 2005 John Wiley & Sons, Ltd. [source] Neuropathological evidence for ischemia in the white matter of the dorsolateral prefrontal cortex in late-life depressionINTERNATIONAL JOURNAL OF GERIATRIC PSYCHIATRY, Issue 1 2003Alan J. Thomas Abstract Background Signal hyperintensities on magnetic resonance imaging in late-life depression are associated with treatment resistance and poor outcome. These lesions are probably vascular in origin and proposed sites for vascular damage include the dorsolateral prefrontal cortex (DLPFC) and anterior cingulate cortex (ACC). Methods We therefore examined white matter in these areas for microvascular disease and evidence of ischemia using intercellular adhesion molecule-1 (ICAM-1) and vascular adhesion molecule-1 (VCAM-1). We obtained postmortem tissue from elderly depressed (n,=,20) and control (n,=,20) subjects and blindly rated microvascular disease and ICAM-1 and VCAM-1 amount using quantitative image analysis in sections of the DLPFC, ACC and occipital cortex (OC; control area). Results We found a significant increase in ICAM-1 in the deep white matter of the DLPFC in the depressed group (p,=,0.01) and a trend towards an increase for VCAM-1 (p,=,0.10). In the gyral white matter there was a trend towards significance for both molecules (p,=,0.07 and 0.10). No differences were found in the ACC or OC or for microvascular disease in any area. Conclusions These findings are consistent with white matter ischemia in the DLPFC and lend support to the ,vascular depression' hypothesis. They implicate the DLPFC as an important site in the pathogenesis of late-life depression and have major implications for the understanding and management of late-life depression and raise the possibility of novel treatments being introduced in the future. Copyright © 2002 John Wiley & Sons, Ltd. [source] Estrogen replacement therapy is associated with less progression of subclinical structural brain disease in normal elderly women: a pilot studyINTERNATIONAL JOURNAL OF GERIATRIC PSYCHIATRY, Issue 7 2002Ian A. Cook Abstract Background Cortical atrophy, central atrophy, deep white-matter hyperintensities, and periventricular hyperintensities are reported in normal aging. Objectives We examined the effects of estrogen replacement therapy (ERT) on these forms of ,subclinical structural brain disease' (SSBD) in normal, postmenopausal women in a pilot, naturalistic, longitudinal study of 15 subjects. Methods Two assessments were performed at least two years apart, with volumetric magnetic resonance imaging (MRI) and neuropsychological testing. Results Women receiving open-label ERT showed significantly less progression of SSBD than those who did not. Conclusions The association between reduced SSBD progression and ERT suggests this intervention could help preserve normal brain structure in healthy elderly women. Copyright © 2002 John Wiley & Sons, Ltd. [source] MRI white matter hyperintensities, 1H-MR spectroscopy and cognitive function in geriatric depression: a comparison of early- and late-onset casesINTERNATIONAL JOURNAL OF GERIATRIC PSYCHIATRY, Issue 12 2001Tetsuhito Murata Abstract Background and Objectives Geriatric depression is often thought to differ from that at other times of adulthood. Recently, several studies have shown that the incidence of white matter hyperintense lessions identified by brain MRI is higher in patients with geriatric depression than in healthy elderly subjects, but a consensus has not yet been reached on the relationship between the severity of white matter lesions and either cognitive impairment or depressive symptoms. Method Forty-seven patients aged 50 to 75 years with major depression were divided into two groups based on age at onset of depression: early-onset (<,50 years) group (20 patients; mean age, 62.7,±,6.7) and late-onset (,50 years) group (27 patients; mean age, 65.6,±,5.4). The severity of hyperintense white matter lesions on MRI was classified by region, then a proton magnetic resonance spectroscopy (1H-MRS) focusing on the white matter of the frontal lobes, multidimensional neuropsychological tests and evaluation of depressive symptoms were conducted. Results The severity of the deep white matter lesions, the deterioration of cognitive function related to subcortical/frontal brain system and clinician-rated depressive symptoms were all more pronounced in the late-onset group compared with those in the early-onset group. It was further observed that the more severe the deep white matter lesions, the lower the levels of N-acetylaspartate/creatine. With the age of onset as the covariate, the patients with moderate deep white matter lesions had more pronounced cognitive impairment and clinician-rated depressive symptoms than those with none and/or mild lesions. Conclusion These results suggest that subcortical/frontal type cognitive impairment and the persistence of depressive symptoms in geriatric depression is related to moderate deep white matter lesions more often complicated in the late-onset group. The 1H-MRS findings were suggested to be a useful indicator of neuronal/axonal loss in the white matter of the frontal lobes which precedes cognitive impairment. Copyright © 2001 John Wiley & Sons, Ltd. [source] Lower Cardiac Output Is Associated with Greater White Matter Hyperintensities in Older Adults with Cardiovascular DiseaseJOURNAL OF AMERICAN GERIATRICS SOCIETY, Issue 7 2007Angela L. Jefferson PhD OBJECTIVES: To preliminarily examine the association between cardiac output, a measure of systemic blood flow, and structural brain magnetic resonance imaging indices of white matter hyperintensities (WMHs). DESIGN: Cross-sectional. SETTING: University medical setting. PARTICIPANTS: Thirty-six older adults without dementia with prevalent cardiovascular disease (aged 56,85). MEASUREMENTS: Cardiac output, WMHs. RESULTS: Partial correlations, adjusting for age and history of hypertension, yielded an inverse relationship between WMHs adjacent to subcortical nuclei and cardiac output (correlation coefficient=,0.48, P=.03); as cardiac output decreased, WMHs increased significantly. No significant associations were found between cardiac output and total WMHs or periventricular WMHs. CONCLUSION: These preliminary data suggest that systemic blood flow, measured according to cardiac output, is inversely associated with WMHs adjacent to the subcortical nuclei. Cerebrovascular degeneration and the chronicity of hypoperfusion may exacerbate the susceptibility of white matter integrity to alterations in blood flow in older adults. [source] Increased White Matter Signal Hyperintensities in Long-Term Abstinent Alcoholics Compared with Nonalcoholic ControlsALCOHOLISM, Issue 1 2009George Fein Background:, The harmful effects of alcohol dependence on brain structure and function have been well documented, with many resolving with sufficient abstinence. White matter signal hyperintensities (WMSH) are thought to most likely be consequences secondary to the vascular (i.e., hypertension and atherosclerosis) effects of AD. We hypothesized that such effects would persist into long-term abstinence, and evaluated them in middle-aged long-term abstinent alcoholics (LTAA) compared with age and gender comparable nonalcoholic controls (NAC). Methods:, Ninety-seven participants (51 LTAA and 46 NAC) underwent cognitive, psychiatric, and structural brain magnetic resonance image evaluations. WMSH were identified and labeled as deep or periventricular by an automated algorithm developed in-house. WMSH volumes were compared between groups, and the associations of WMSH measures with demographic, alcohol use, psychiatric, and cognitive measures were examined within group. Results:, Long-term abstinent alcoholics had more WMSH than NAC. There was a significant group by age interaction, with WMSH increasing with age in LTAA, but not in NAC. Within LTAA, WMSH load was independently positively associated with alcohol burden and with age. No associations were evident between WMSH volumes and abstinence duration, family drinking history, years of education, or psychiatric or cognitive variables. Conclusion:, The magnitude of alcohol abuse was related to increased WMSH volume. The presence of an age effect in the LTAA but not the controls indicates a synergistic effect wherein alcohol advances the onset of aging-related WMSH formation. The increased WMSH load did not appear to have any significant clinical correlates, indicating that the white matter lesions in our sample may not have been severe enough to manifest as cognitive deficits. A limitation of the study is that we did not have data on the presence or severity of lifetime or current indices of vascular risk factors such as hypertension, smoking, or diabetes. [source] Peripheral nervous system involvement as presenting symptom of systemic B-cell lymphomaJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 2 2004C Casellato Peripheral nervous system involvement has been reported in systemic B or T cell lymphoma and may result from intraneural localization of lymphoma resulting in meningo-radiculopathy or mononeuropathies, or manifest as a sensory-motor polyneuropathy sometimes mimicking chronic inflammatory demyelinating polyneuropathy. We report two patients with a previously unknown NHL presenting in both with a stepwise progressive asymmetric multiradiculoneuropathy initially misdiagnosed as inflammatory radiculopathy. A 58-year-old man presented with a 2 year history of stepwise progressive peroneal sensory loss, impotence, and lower limb painful asymmetric neuropathy. Lumbosacral MRI was normal. Electrophysiological studies were consistent with an axonal multiradiculoneuropathy while CSF examinations repeatedly showed increased protein levels (80,91 mg/dl) with slightly increased white cells (<10 mm3) but no malignant cell. The patient repeatedly failed to respond to steroids although he consistently deteriorated at their suspension. An MRI performed 2 years later when multiple cranial nerve palsies appeared showed bilateral T1 and T2 hyperintensities in the brain and cervical spinal cord. An extensive investigation for neoplasm was negative. The patient died from an intracranial hemorrhage during anticoagulant therapy for deep vein thrombosis. Autoptic studies revealed a widespread non-Hodgkin's type B lymphoma with massive systemic and neural involvement including cauda equina and spinal cord. A 54-year-old man presented with a 1 year history of impotence, urinary incontinence, progressive asymmetric painful distal sensorimotor impairment at four limbs and prominent weight loss. Four previous CSF examinations revealed increased protein levels (80,100 mg/dl), and slightly but inconsistently increased white cells (1,11/mm3) but no malinant cells. Steroids were repeatedly ineffective although the patient consistently deteriorated whenever steroids were discontinued. On admission electrophysiological studies showed an axonal asymmetric polyradiculoneuropathy. Brain and spinal MRI was normal while bone marrow biopsy and aspiration disclosed a B cell lymphoma. [source] Do MRI features distinguish Wilson's disease from other early onset extrapyramidal disorders?MOVEMENT DISORDERS, Issue 6 2010An analysis of 100 cases Abstract Magnetic resonance imaging (MRI) is frequently used in the evaluation of various extrapyramidal disorders. Among the plethora of MRI features in Wilson's disease (WD), only "face of the giant panda" sign has been recognized to distinguish WD from other early onset extrapyramidal disorders (EOEPD). To ascertain the value of various MRI features in differentiating neuropsychiatric form of WD from other EOEPD. This retrospective analysis included 100 patients (M:F = 56:44) of EOEPD (5,40 years), who had undergone MRI during Jan'03 to Nov'08. Their clinical features were recorded and the following MR sequences were analyzed: T1WI, T2WI, FLAIR. Fifty-six patients had WD (M:F = 28:30, age at onset: 14 ± 6.8 years) and 44 had other EOEPD (M:F = 27:17, age at onset: 19 ± 9.8 years) that included Huntington's disease-4, young-onset Parkinson's disease-7, mitochondrial disorders-2, Hallervorden-Spatz disease-8, non-Wilsonian hepatolenticular degeneration-2, toxic/metabolic disorder-1, and others-20. The duration of illness at the time of MRI was comparable (WD: 3.1 ± 4.9 years; Other EOEPD: 2.8 ± 2.4 years). MR signal characteristics varied in topography and severity in both the groups. All the patients of WD had signal abnormalities in MRI, as against 16/44 of the other EOEPD group. The following MR observations were noted exclusively in WD: "Face of giant panda" sign (14.3%), tectal plate hyperintensity (75%), central pontine myelinolysis (CPM)-like abnormalities (62.5%), and concurrent signal changes in basal ganglia, thalamus, and brainstem (55.3%). Besides "Face of giant panda" sign, hyperintensities in tectal-plate and central pons (CPM-like), and simultaneous involvement of basal ganglia, thalamus, and brainstem are virtually pathognomonic of WD. © 2010 Movement Disorder Society [source] Pyramidal tract imaging in multiple-system atrophyMOVEMENT DISORDERS, Issue 11 2005Nicole Limberg MBBS Abstract A new radiological finding of T2 FLAIR hyperintensities in the pyramidal tracts is described in a patient clinically thought to have idiopathic Parkinson's disease but histologically proven to have multiple-system atrophy. © 2005 Movement Disorder Society [source] Clinical features of adult GM1 gangliosidosis: Report of three Indian patients and review of 40 casesMOVEMENT DISORDERS, Issue 11 2004Uday Muthane MBBS Abstract Deficiency of enzyme acid ,-galactosidase causes GM1 gangliosidosis. Patients with adult GM1 gangliosidosis typically present with generalized dystonia. We describe clinical, bone marrow, and radiological features of adult GM1 gangliosidosis to help improve its recognition. We report 3 Indian patients and review of reports between 1981 and October 2002. The disease frequently is reported in the Japanese literature (75%). Patients are normal at birth and have normal early motor and mental development. Onset is within the first decade with abnormal gait, or worsening of speech is an initial symptom. Dystonia occurs in 97% of patients. Facial dystonia described as "facial grimacing" observed in ,90% could be an important clinical clue. Dysarthria/anarthria (97%) is frequent, and eye movements are normal. Bone marrow examination may show Gaucher-like foam cells (39%). Magnetic resonance imaging (MRI) frequently (90.9%) shows bilateral symmetrical putamenal hyperintensities on T2-weighted and proton density images. Diagnosis is confirmed by demonstrating deficiency of ,-galactosidase. Adult (Type 3) GM1 Gangliosidosis commonly presents with generalized dystonia with prominent facial dystonia, severe speech disturbances, and normal eye movements. Bone marrow frequently shows Gaucher-like foam cells. MRI shows typical lesions in the putamen. Deficiency of ,-galactosidase in fibroblasts confirms the diagnosis. © 2004 Movement Disorder Society [source] Microvascular lesions in the brain and retina: The age, gene/environment susceptibility,Reykjavik study,ANNALS OF NEUROLOGY, Issue 5 2009Chengxuan Qiu MD Objective To investigate whether the severity and location of cerebral white matter hyperintensities (WMHs) and brain infarcts are correlated with the signs of retinal microvascular abnormalities in the elderly. Methods The study included 4,176 men and women (mean age, 76 years) who participated in the Age, Gene/Environment Susceptibility (AGES),Reykjavik Study. Digital retinal images of both dilated eyes were taken and evaluated for the presence of retinal focal arteriolar signs (focal arteriolar narrowing and arteriovenous nicking) and retinopathy lesions (retinal blot hemorrhages and microaneurysms). Brain magnetic resonance imaging scans were acquired and evaluated for the presence and distribution of cerebral infarcts and WMHs. Logistic and multinomial logistic models were constructed to estimate the association of retinal microvascular signs to brain lesions. Results Controlling for demographic and major cardiovascular risk factors, we found that retinal focal arteriolar signs, but not retinopathy lesions, were significantly associated with an increasing load of subcortical and periventricular WMHs. The strongest association was found between retinal arteriolar signs and a heavier WMH load, specifically in the subcortical frontal lobe, and periventricular frontal and parietal caps. There was a tendency toward bilateral retinal focal arteriolar narrowing being more strongly associated with the heavier load of subcortical WMHs. Arteriovenous nicking was significantly associated with subcortical infarcts. Interpretation In older adults, retinal focal arteriolar signs, but not retinopathy lesions, are correlated with the load of diffuse WMHs, particularly those located in the subcortical frontal lobe, and the periventricular frontal and parietal caps of the brain. Ann Neurol 2009;65:569,576 [source] Long-term neurological and functional outcome in Nipah virus infectionANNALS OF NEUROLOGY, Issue 3 2007James J. Sejvar MD Objective Nipah virus (NiV) is an emerging zoonosis. Central nervous system disease frequently results in high case-fatality. Long-term neurological assessments of survivors are limited. We assessed long-term neurologic and functional outcomes of 22 patients surviving NiV illness in Bangladesh. Methods During August 2005 and May 2006, we administered a questionnaire on persistent symptoms and functional difficulties to 22 previously identified NiV infection survivors. We performed neurologic evaluations and brain magnetic resonance imaging (MRI). Results Twelve (55%) subjects were male; median age was 14.5 years (range 6,50). Seventeen (77%) survived encephalitis, and 5 survived febrile illness. All but 1 subject had disabling fatigue, with a median duration of 5 months (range, 8 days,8 months). Seven encephalitis patients (32% overall), but none with febrile illness had persistent neurologic dysfunction, including static encephalopathy (n = 4), ocular motor palsies (2), cervical dystonia (2), focal weakness (2), and facial paralysis (1). Four cases had delayed-onset neurologic abnormalities months after acute illness. Behavioral abnormalities were reported by caregivers of over 50% of subjects under age 16. MRI abnormalities were present in 15, and included multifocal hyperintensities, cerebral atrophy, and confluent cortical and subcortical signal changes. Interpretation Although delayed progression to neurologic illness following Nipah fever was not observed, persistent fatigue and functional impairment was frequent. Neurologic sequelae were frequent following Nipah encephalitis. Neurologic dysfunction may persist for years after acute infection, and new neurologic dysfunction may develop after acute illness. Survivors of NiV infection may experience substantial long-term neurologic and functional morbidity. Ann Neurol 2007 [source] Homocysteine, white matter hyperintensities, and cognition in healthy elderly peopleANNALS OF NEUROLOGY, Issue 2 2003Carole Dufouil PhD Hyperhomocysteinemia is associated with an increased risk of vascular disease, and recent results suggest that it also could increase the risk of dementia. We examined the relationship between homocysteine and cognitive decline in 1,241 subjects aged 61 to 73 years, followed up over 4 years. Plasma homocysteine levels were determined in all participants as well as cardiovascular risk factors, apolipoprotein E genotype, plasma levels of folate, and vitamin B12. Cognitive performances were assessed repeatedly by using Mini-Mental State Examination, Trail Making Test, Digit Symbol Substitution Test, and Finger Tapping Test. At 2-year follow-up, 841 subjects underwent cerebral magnetic resonance imaging, and white matter hyperintensities were rated visually. Analyses were adjusted for all cardiovascular risk factors. Cross-sectional analyses showed that higher concentrations of homocysteine were significantly related to poorer performances at all neuropsychological tests. Longitudinal analyses confirmed this finding. The odds of cognitive decline was 2.8-fold (p < 0.05) higher in subjects with homocysteine levels above 15,mol/L compared with those with homocysteine levels below 10,mol/L. In participants who underwent magnetic resonance imaging, the relationship between homocysteine and cognition was unchanged after taking into account white matter hyperintensities suggesting that white matter hyperintensities do not mediate the association between homocysteine and cognition. Ann Neurol 2003;53:000,000 [source] Low cerebral blood flow velocity and risk of white matter hyperintensitiesANNALS OF NEUROLOGY, Issue 3 2001Christophe Tzourio MD Cerebral blood flow velocity (CBF-V) measured by transcranial doppler was assessed in 628 elderly individuals who had cerebral magnetic resonance imaging performed as part of a population-based study on vascular aging. Cerebral white matter hyperintensities (WMHs) were associated with low CBF-V, such as the adjusted odds ratios of severe WMHs from highest (referent) to lowest quartile of mean CBF-V were 1.0, 1.7, 3.7, and 4.3 (p = 0.001). Further, CBF-V was found to be a stronger risk factor for WMHs than high blood pressure. These findings suggest that the assessment of CBF-V might be a powerful tool in future studies on WMHs. Ann Neurol 2001;49:411,414 [source] Valproate activates the Notch3/c-FLIP signaling cascade: a strategy to attenuate white matter hyperintensities in bipolar disorder in late life?BIPOLAR DISORDERS, Issue 3 2009Peixiong Yuan Objectives:, Increased prevalence of deep white matter hyperintensities (DWMHs) has been consistently observed in patients with geriatric depression and bipolar disorder. DMWHs are associated with chronicity, disability, and poor quality of life. They are thought to be ischemic in their etiology and may be related to the underlying pathophysiology of mood disorders in the elderly. Notably, these lesions strikingly resemble radiological findings related to the cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephelopathy (CADASIL) syndrome. CADASIL arises from mutations in Notch3, resulting in impaired signaling via cellular Fas-associated death domain-like interleukin-1-beta-converting enzyme-inhibitory protein (c-FLIP) through an extracellular signal-regulated kinase (ERK)-dependent pathway. These signaling abnormalities have been postulated to underlie the progressive degeneration of vascular smooth muscle cells (VSMC). This study investigates the possibility that the anticonvulsant valproate (VPA), which robustly activates the ERK mitogen-activated protein kinase (MAPK) cascade, may exert cytoprotective effects on VSMC through the Notch3/c-FLIP pathway. Methods:, Human VSMC were treated with therapeutic concentrations of VPA subchronically. c-FLIP was knocked down via small interfering ribonucleic acid transfection. Cell survival, apoptosis, and protein levels were measured. Results:, VPA increased c-FLIP levels dose- and time-dependently and promoted VSMC survival in response to Fas ligand-induced apoptosis in VSMC. The anti-apoptotic effect of VPA was abolished by c-FLIP knockdown. VPA also produced similar in vivo effects in rat brain. Conclusions:, These results raise the intriguing possibility that VPA may be a novel therapeutic agent for the treatment of CADASIL and related disorders. They also suggest that VPA might decrease the liability of patients with late-life mood disorders to develop DWMHs. [source] Increased rates of white matter hyperintensities in late-onset bipolar disorderBIPOLAR DISORDERS, Issue 7 2008Jaqueline Hatsuko Tamashiro Objectives:, Magnetic resonance imaging (MRI) studies have reported an increased frequency of white matter hyperintensities (WMH) in association with late-onset (LO) depression, and this has supported the notion that vascular-related mechanisms may be implicated in the pathophysiology of LO mood disorders. Recent clinical studies have also suggested a link between LO bipolar disorder (LO-BD) and cerebrovascular risk factors, but this has been little investigated with neuroimaging techniques. In order to ascertain whether there could be a specific association between WMH and LO-BD, we directly compared WMH rates between LO-BD subjects (illness onset , 60 years), early-onset BD subjects (EO-BD, illness onset <60 years), and elderly healthy volunteers. Methods:, T2-weighted MRI data were acquired in LO-BD subjects (n = 10, age = 73.60 ± 4.09), EO-BD patients (n = 49, age = 67.78 ± 4.44), and healthy subjects (n = 24, age = 69.00 ± 7.22). WMH rates were assessed using the Scheltens scale. Results:, There was a greater prevalence of WMH in LO-BD patients relative to the two other groups in the deep parietal region (p = 0.018) and basal ganglia (p < 0.045). When between-group comparisons of mean WMH scores were conducted taking account of age differences (ANCOVA), there were more severe scores in LO-BD patients relative to the two other groups in deep frontal and parietal regions, as well as in the putamen (p < 0.05). Conclusions:, Our results provide empirical support to the proposed link between vascular risk factors and LO-BD. If extended in future studies with larger samples, these findings may help to clarify the pathophysiological distinctions between bipolar disorder emerging at early and late stages of life. [source] Bipolar disorder, homocysteine and white matter hyperintensitiesBIPOLAR DISORDERS, Issue 6 2008Amílcar Dos Santos [source] Brain magnetic resonance imaging abnormalities in neuromyelitis opticaACTA NEUROLOGICA SCANDINAVICA, Issue 4 2008Y. Li Objective,,, Brain abnormalities in neuromyelitis optica (NMO) attracted much attention. Our study was to identify the brain magnetic resonance imaging (MRI) abnormalities in Chinese NMO patients. Methods,,, Patients who fulfilled the latest diagnostic criteria of NMO proposed by Wingerchuk et al. [Neurology 66 (2006) 1485] and whose brain MRI did not meet the multiple sclerosis (MS) criteria of McDonald et al. [Ann Neurol 50 (2001) 121] were selected to perform MRI scanning of the brain, spinal cord and optic nerves. Results,,, Twenty-eight of 33 patients (84.8%) had abnormal MRI findings. Twenty-two patients (66.7%) presented with well-defined brain parenchymal lesions and the other six patients (18.2%) with macroscopic symmetrical diffuse hyperintensities in deep white matter. Fifteen of 22 patients had more than one lesion (,2 lesions) and the other seven patients had single lesion. In the supratentorium, most lesions were punctate or small round dot and non-specific in juxtacortical, subcortical and deep white matter regions, a few were patchy atypical confluent lesions. Brainstem was easily involved (14/33, 42.4%) especially in medulla (7/33, 21.2%). Conclusions,,, This study demonstrates the characteristics of brain MRI abnormalities in Chinese NMO patients, which are helpful to the revision of diagnostic criteria for NMO. [source] Diagnostic value of high signal abnormalities on T2 weighted MRI in the differentiation of Alzheimer's, frontotemporal and vascular dementiasACTA NEUROLOGICA SCANDINAVICA, Issue 5 2002A. R. Varma Objective ,,The occurrence of high signal abnormalities on T2 weighted images is strongly age related. The diagnostic value of these changes in a younger population with dementia is not currently known. We studied the potential of high signal changes on magnetic resonance imaging (MRI) in differentiating Alzheimer's disease (AD), frontotemporal dementia (FTD) and vascular dementia (VaD) in younger patients. Methods ,,High signal abnormalities were rated, using a previously validated scale, from hard copies of T2 weighted axial images of 102 patients with AD (n=49), VaD (n=31), FTD (n=22) (mean ages 63,65 years). Results ,,High signal abnormalities were widespread across AD, VaD and FTD. Although they were most frequent and most severe in the VaD group only lacunes and grade III deep white matter hyperintensities (DWMH) were specific for these patients. Conclusions ,,High signal changes on T2 weighted images on MRI are common across degenerative (AD and FTD) and vascular dementias. Although lacunes and grade III DWMH are specific for VaD, the low sensitivities (sensitivities: for lacunes, 0.32; for grade III DWMH, 0.16) limit their use as diagnostic markers for VaD. High signal changes on MRI should be interpreted with caution in dementias. Their presence, even in younger patients, should not deter one from diagnosing AD or FTD. [source] The neuropsychology and neuroanatomy of bipolar affective disorder: a critical reviewBIPOLAR DISORDERS, Issue 3 2001Carrie E Bearden Objectives: To present a comprehensive review of the existing neuropsychological and neuroimaging literature on bipolar affective disorder. This review critically evaluates two common conceptions regarding the neuropsychology of bipolar disorder: 1) that, in contrast to schizophrenia, bipolar affective disorder is not associated with general cognitive impairment independent of illness episodes, and 2) relative right hemisphere (RH) dysfunction is implicated in bipolar illness patients, supported by reports of relatively greater impairment in visuospatial functioning, lateralization abnormalities, and mania secondary to RH lesions. Methods: The major computerized databases (Medline and PSYCInfo) were consulted in order to conduct a comprehensive, integrated review of the literature on the neuropsychology and neuroanatomy of bipolar disorder. Articles meeting specified criteria were included in this review. Results: In a critical evaluation of the above notions, this paper determines that: 1) while there is little evidence for selective RH dysfunction, significant cognitive impairment may be present in bipolar illness, particularly in a subgroup of chronic, elderly or multiple-episode patients, suggesting a possible toxic disease process, and 2) the underlying functional correlate of these cognitive deficits may be white matter lesions (,signal hyperintensities') in the frontal lobes and basal ganglia, regions critical for executive function, attention, speeded information processing, learning and memory, and affect regulation. While this hypothesized neural correlate of cognitive impairment in bipolar disorder is speculative, preliminary functional neuroimaging evidence supports the notion of frontal and subcortical hypometabolism in bipolar illness. Conclusions: The etiology of the structural brain abnormalities commonly seen in bipolar illness, and their corresponding functional deficits, remains unknown. It is possible that neurodevelopmental anomalies may play a role, and it remains to be determined whether there is also some pathophysiological progression that occurs with repeated illness episodes. More research is needed on first-episode patients, relatives of bipolar probands, and within prospective longitudinal paradigms in order to isolate disease-specific impairments and genetic markers of neurocognitive function in bipolar disorder. [source] Evaluation of rHA labeled with Gd,DTPA for blood pool imaging and targeted contrast deliveryCONTRAST MEDIA & MOLECULAR IMAGING, Issue 1 2010Jim M. Wild Abstract A new contrast agent was developed by linking Gd,DTPA chelate to recombinant human albumin in the laboratory. The molar relaxivity of the new agent was tested in aqueous solution at B0 1.5,T and temperature 20°C. The soluble compound had a higher molar longitudinal relaxivity and molar transverse relaxivity in water (r1,=,7.2,s,1,mM,1, r2,=,18.4,s,1,mM,1) than those measured for Gd,DTPA solution (r1,=,3.5,s,1,mM,1, r2,=,5.5,s,1,mM,1). The performance of the compound as a blood pool agent was investigated with soluble and microparticulate forms of the compound and comparisons were made with Gd,DTPA and the polymeric blood-pool agent, Gadomer. T1 -weighted imaging experiments show that the soluble compound acts as a highly effective blood pool agent with hyperintensity in the vasculature persisting beyond 2,h post administration, compared with free Gd,DTPA, which was cleared from the blood pool after approximately 10,min. The clearance kinetics of the new agents were examined, due to the incomplete elimination within 14 days post injection; both rHA labeled compounds are probably not suitable for development as routine blood pool contrast media. However, with free sites on the Gd-loaded rHA molecule, there are possibilities for binding the agent to antibodies in the laboratory, which was demonstrated, and thus there exist potential applications for in vivo molecular imaging with this agent. Copyright © 2010 John Wiley & Sons, Ltd. [source] Voxel-based morphometry of sporadic epileptic patients with mesiotemporal sclerosisEPILEPSIA, Issue 4 2010Angelo Labate Summary Purpose:, In refractory temporal lobe epilepsy (rTLE), gray matter (GM) abnormalities are not confined to the hippocampus but also are found in extrahippocampal structures. Very recently we observed in mild TLE (mTLE) with or without mesiotemporal sclerosis (MTS), GM reductions in regions outside the presumed epileptogenic focus. To date, there are no studies that directly investigate whether whole-brain GM volume differs between rTLE and mTLE. Herein, we used optimized voxel-based morphometry (VBM) to identify GM abnormalities beyond the hippocampus in both rTLE and mTLE with evidence of MTS. Methods:, Brain magnetic resonance imaging (MRI) and optimized VBM were performed in 19 unrelated patients with mTLE, 19 patients with rTLE, and 37 healthy controls. MRI diagnosis of MTS was based on the atrophy of the hippocampal formation and/or mesiotemporal hyperintensity on fluid-attenuated inversion recovery (FLAIR) or T2 images, or both. Results:, No patients (rTLE and mTLE) had generalized tonic,clonic or complex partial seizures for at least 3 weeks before scanning. Both mTLE and rTLE patients showed GM volume reduction of the bilateral thalamus, left hippocampus, and sensorimotor cortex compared with controls. No significant GM difference was found between rTLE and mTLE groups. Discussion:, In both rTLE and mTLE, VBM shows GM reductions not confined to the hippocampus involving mainly the thalamus bilaterally. This finding together with the lack of significant GM differences between the two TLE groups supports the hypothesis that mTLE and rTLE might lie along a biologic continuum, suggesting a pathophysiologic role of the thalamus in partial epilepsy. [source] | |||||||||||||||||||||||||||||||