... In general, it is believed the abnormalities in brain development that occur in TSC sometimes interfere with the ... cortical tubers (which develop in earlier stages of brain development) in individuals with TSC involve the region of ...

Serotonergic abnormalities have been reported in both autism and epilepsy. This association may provide insights into underlying mechanisms of these disorders because serotonin plays an important neurotrophic role during brain development--and there is evidence for abnormal cortical development ...

It is unknown whether the abnormalities in brain structure and function observed in dyslexic readers are congenital or arise later in development. Analyzing the 2 components of gray matter volume separately may help in differentiating these possibilities. Gray matter volume is the product of cortical surface area, determined during ...

Neural stem cells are present in the human post-natal brain and are important in the development of brain tumours. However, their contribution to non-neoplastic human disease is less clear. We have tested the hypothesis that malformations of cortical development contain abnormal (pathological) stem cells. Such ...

Malformations of cortical development are a broad family of disorders that are characterized by abnormal cytoarchitecture of the cerebral cortex and a high association with epilepsy. In recent years positional cloning strategies have been implemented to identify several distinct gene mutations that are responsible for developmental ...

Malformations of cortical development (MCD) are a common etiology for epilepsy. Laminar heterotopia, bilateral subependymal heterotopia, and lissencephaly have a genetic basis. No gene mutations have yet been identified in patients with focal cortical dysplasias. The aim of this study was to use quantitative morphometric tools to ...

SUMMARYMalformations of the cerebral cortex known as cortical dysplasia account for the majority of cases of intractable childhood epilepsy. With the exception of the tuberous sclerosis complex, the molecular basis of most types of cortical dysplasia is completely unknown. Currently, there are no good animal models available that recapitulate key features ...

For some time, dystonia has been seen as purely a motor disorder. Relatively novel concepts published approximately 10 years ago also presumed that in the development of dystonic dyskinesias, only motor behaviour was abnormal. Neurophysiological observations of various types of dystonic disorders, which were performed using sophisticated electromyography, ...

Bipolar disorder (BD) is a common affective disorder. In morphometric brain imaging studies, BD subjects have atrophic gray matter, especially in the prefrontal area. These structural abnormalities could involve the change of cortical shape. Gyrification index (GI) is a useful metric to measure the degree of cortical complexity. This ...

Malformations of the cerebral cortex are recognized as a common cause of developmental delay, neurological deficits, mental retardation and epilepsy. Currently, the diagnosis of cerebral cortical malformations is based on a subjective interpretation of neuroimaging characteristics of the cerebral gray matter and underlying white matter.There is no automated system for aiding ...

Magnetic resonance imaging (MRI) scans were performed on 13 high-functioning male autistic subjects and 13 male nonautistic control subjects comparable in age and nonverbal IQ. Scans were rated for the presence of cerebral cortical malformations. Five autistic subjects had polymicrogyria, one had schizencephaly and macrogyria, and one had macrogyria. None of the control ...

Cognitive impairment constitutes a relevant clinical aspect of multiple sclerosis (MS). Depending on the disease phase and type, 40-65% of MS patients develop various degrees of cognitive dysfunction. Pathological and MRI studies have failed to demonstrate the existence of a strict relationship between cognitive impairment and subcortical white matter pathology. The ...

We have generated an experimental 'double-hit' model of chronic epilepsy to recapitulate the co-existence of abnormal cortical structure and frequently recurrent seizures as observed in human focal cortical dysplasia. We induced cortical malformations by exposing rats prenatally to methylazoxymethanol acetate and ...

Non-psychotic individuals at increased risk for schizophrenia show alterations in fronto-striatal dopamine signaling and cortical gray matter maturation reminiscent of those seen in schizophrenia. It remains unclear however if variations in dopamine signaling influence rates of structural cortical maturation in typically developing ...

Detailed clinical and neuropathological studies were made in two fullterm newborn human infants who were exposed to methylmercury in utero as a result of maternal ingestion of methylmercury-contaminated bread in early phases of pregnancy. High levels of mercury were detected in various regions of the brain at autopsy. Study of the brains revealed a disturbance in the ...

In this review, we focus on immature neurons and their regulation by the cholinergic system, both during cortical development as well as during adult neurogenesis. We discuss various studies that indicate roles for acetylcholine in precursor development and neuronal differentiation. Cholinergic neurons projecting from the basal ...

Quantitative magnetic resonance imaging (MRI) studies in children with fetal alcohol spectrum disorders (FASDs) have shown regional patterns of dysmorphology, most prominent in parietal and posterior temporal cortices. Various methods of image analysis have been employed in these studies, but abnormalities in cortical thickness have ...

Studies on human patients and animal models of disease have shown that disruptions in prenatal and early postnatal brain development are a root cause of mental retardation. Since proper brain development is achieved by a strict spatiotemporal control of neurogenesis, cell migration, and patterning of synapses, abnormalities in one or ...

Studies on human patients and animal models of disease have shown that disruptions in prenatal and early postnatal brain development are a root cause of mental retardation. Since proper brain development is achieved by a strict spatiotemporal control of neurogenesis, cell migration, and patterning of synapses, abnormalities in one or ...

Schizophrenia is a disorder of cognitive neurodevelopment with characteristic abnormalities in working memory attributed, at least in part, to alterations in the circuitry of the dorsolateral prefrontal cortex. Various environmental exposures from conception through adolescence increase risk for the illness, possibly by altering the developmental trajectories of prefrontal ...

Purpose:? Spread of seizure activity outside the frontal lobe due to cortico-cortical connections can result in alteration in the cortex beyond the frontal lobe in children with intractable frontal lobe epilepsy (FLE). The aim of this study was to identify regions of reduced cortical thickness in children with intractable FLE. Methods:? High-resolution ...

The cerebral cortex develops from the dorsal telencephalon, at the anterior end of the neural tube. Neurons are generated by cell division at the inner surface of the telencephalic wall (in the ventricular zone) and migrate towards its outer surface, where they complete their differentiation. Recent studies have suggested that the transcription factor Pax6 is important for ...

Background:?Progressive cortical gray matter (GM) abnormalities are an established feature of schizophrenia and are more pronounced in rare, severe, and treatment refractory childhood-onset schizophrenia (COS) cases. The effect of sex on brain development in schizophrenia is poorly understood and studies to date have produced ...

Radiation can be used to induced series of developmentall malformations in the brains of rats The degree of reproducibility of the patterns has suggested their use in studies relating certain attributes of behavior with cerebral cortical structure. The patterns of brain malformiation induced by giving 150 to 200 r on each of the days of gestation. 12 to 20. are ...

PurposeIn order to improve the objective localization of bilateral cortical abnormalities in positron emission tomography (PET) image volumes, we developed a new three-dimensional image processing technique. The accuracy of this approach with respect to invasive subdural electroencephalography (EEG) data was assessed in a group of ...

Malformations of cortical development are disorders of altered brain anatomy and architecture that arise from abnormalities in the usual processes of cerebral cortical development. Although they often lead to epilepsy, cognitive delay, and motor impairment, little is known about their effect on ...

The current study examined group differences in cortical volume, surface area, and thickness with age, in a group of typically developing children and a group of children with ASD aged 6-15�years. Results showed evidence of age by group interactions, suggesting atypicalities in the relation between these measures and age in the ASD group. Additional ...

... of the patient in preventing the development of septic ... This abnormality occurs ... studies concerning the mechanisms by which abnormalities of the ...

Calcium loss and muscle atrophy are two of the main metabolic changes experienced by astronauts and crew members during exposure to microgravity in space. Calcium and cytoskeletal events were investigated within sea urchin embryos which were cultured in space under both microgravity and 1 g conditions. Embryos were fixed at time-points ranging from 3 h to 8 days after fertilization. Investigative ...

We measured regional cerebral blood flow with the xenon 133 inhalation technique in 41 patients with major depressive disorder and 40 matched, normal controls during an eyes-closed, resting condition. The depressed group had a marked reduction in global cortical blood flow. To examine topographic abnormalities, traditional multivariate analyses were ...

Understanding human brain development in utero and detecting cortical abnormalities related to specific clinical conditions is an important area of research. In this paper, we describe and evaluate methodology for detection and mapping of delays in early cortical folding from population-based studies of fetal brain ...

We report a female patient with ichthyosis, epilepsy, mental retardation, hypergonadotrophic hypogonadism, polyneuropathy, and cranial dysmorphisms. This clinical picture may satisfy the main diagnostic criteria that characterize Rud syndrome (RS), a rare neurocutaneous disease. The patient underwent extensive clinical evaluation, neurophysiological studies (wakefulness and sleep EEG, EMG), ...

Conditional deletion of APC leads to marked disruption of cortical development and to excessive axonal branching of cortical neurons. However, little is known about the cell biological basis of this neuronal morphological regulation. Here we show that APC deficient cortical neuronal growth cones exhibit marked ...

The aetiology of idiopathic scoliosis (IS) remains unknown; however, there is a growing body of evidence suggesting that the spine deformity could be the expression of a subclinical nervous system disorder. A defective sensory input or an anomalous sensorimotor integration may lead to an abnormal postural tone and therefore the development of a spine ...

The aetiology of idiopathic scoliosis (IS) remains unknown; however, there is a growing body of evidence suggesting that the spine deformity could be the expression of a subclinical nervous system disorder. A defective sensory input or an anomalous sensorimotor integration may lead to an abnormal postural tone and therefore the development of a spine ...

Glutamatergic neurotransmission plays important roles in sensory map formation. The absence of the group I metabotropic glutamate receptor 5 (mGluR5) leads to abnormal sensory map formation throughout the mouse somatosensory pathway. To examine the role of cortical mGluR5 expression on barrel map formation, we generated cortex-specific mGluR5 KO mice. ...

We tested for cortical shape abnormalities using surface-based morphometry across a range of autism spectrum disorders (7.5-18 years of age). We generated sulcal depth maps from structural magnetic resonance imaging data and compared typically developing controls to three autism spectrum disorder subgroups: low-functioning autism, ...

The hypothesis that schizophrenia results from a developmental, as opposed to a degenerative, process affecting the connectivity and network plasticity of the cerebral cortex is supported by findings from morphological and molecular postmortem studies. Specifically, abnormalities in the expression of protein markers of GABA neurotransmission and the lamina- and ...

There is evidence that abnormal cerebral development during childhood is a risk factor for various cognitive and psychiatric disorders. There is not, however, sufficient normative data available on large samples of typically developing children, especially within the narrow preadolescent age range. We analyzed high resolution MRI ...

Purpose: Children with medulloblastoma undergo surgery, radiotherapy, and chemotherapy. After treatment, these children have numerous structural abnormalities. Using high-resolution magnetic resonance imaging, we measured the thickness of the cerebral cortex in a group of medulloblastoma patients and a group of normally developing children. Methods and ...

Objective:Converging evidence suggests that abnormalities of brain development may play a role in the pathogenesis of temporal lobe epilepsy (TLE). As sulco-gyral patterns are thought to be a footprint of cortical development, we set out to quantitatively map folding complexity across the neocortex in TLE. ...

Cocaine affects sensory perception and attention but little is known about the neural substrates underlying these effects in the human brain. We used functional magnetic resonance imaging (fMRI) and a sustained visuospatial attention task to assess if the visual attention network is dysfunctional in cocaine abusers (n=14) compared to age-, gender-, and education-matched controls (n=14). Compared ...

Accumulating evidence from structural brain imaging studies on individuals with fetal alcohol spectrum disorder (FASD) has supported links between prenatal alcohol exposure and brain morphological deficits. Although global and regional volumetric reductions appear relatively robust, the effects of alcohol exposure on cortical thickness and relationships with facial ...

Down Syndrome (DS), one of the major genetic causes of mental retardation, is characterized by disrupted corticogenesis produced, in part, by an abnormal layering of neurons in cortical laminas II and III. Because defects in the normal migration of neurons during corticogenesis can result in delayed cortical radial expansion and ...

BackgroundPatients suffering from migraine with aura (MWA) and migraine without aura (MWoA) show abnormalities in visual motion perception during and between attacks. Whether this represents the consequences of structural changes in motion-processing networks in migraineurs is unknown. Moreover, the diagnosis of migraine relies on patient's history, and finding differences in ...

Besides focal white matter lesions, multiple sclerosis brain tissue also displays abnormalities in the gray matter and the normal-appearing white matter. Recent advances in magnetic resonance imaging studies of both types of tissue are discussed. Herein, normal-appearing white matter abnormalities are being found in quantitative magnetic resonance ...

Complex interactions and interconnectivity between neurons are hallmarks of normal neuronal differentiation and development. Neurons also interact with other cell types, notably glia, and rely on substances released by glia for their normal function. A deficit in glial response may disturb this critical neuronal-glial-neuronal interaction in Down syndrome (DS), leading to loss ...

from structural magnetic resonance imaging data and compared typically developing controls to three and white matter (Piven et al., 1996; Carper et al., 2002; Hazlett et al., 2005a,b; Hendry et al., 2005; Mc surface (Piven et al., 1990; Levitt et al., 2003; Hardan et al., 2004). Piven et al. (1990) examined

Three cases are described in which extensive livedo reticularis was associated with premature cerebrovascular disease. The patients presented with transient cerebral ischaemic attacks and gradually developed permanent neurological damage with intellectual impairment. The value of detailed neuropsychological testing to identify bilateral cortical ...

Diffusion tensor imaging (DTI) is a technique that non-invasively provides quantitative measures of water translational diffusion, including fractional anisotropy (FA), that are sensitive to the shape and orientation of cellular elements, such as axons, dendrites and cell somas. For several neurodevelopmental disorders, histopathological investigations have identified ...

Cortical dysplasias or malformations due to abnormalities of cortical development are a well-recognized cause of intractable seizures. This study retrospectively examines the clinicopathologic features of 52 cases of extratemporal cortical dysplasia (from 135 total resections performed over a ...

The neocortices of 10 patients with partial seizures and acquired lesions, 14 patients with malformations of cortical development (MCD) and 33 patients with partial seizures and normal conventional MRI were quantitatively evaluated using whole brain double inversion recovery imaging (DIR) and Statistical Parametric Mapping (SPM). Compared to a group of 30 ...

Autism is often described as a disorder of neural synchronization. However, it is unknown how early in development synchronization abnormalities emerge and whether they are related to the development of early autistic behavioral symptoms. Here, we show that disrupted synchronization is evident in the spontaneous ...

Malformations of cortical development have been described in children and families with language-learning impairment. The objective of this study was to assess the auditory processing information in children with language-learning impairment in the presence or absence of a malformation of cortical development in ...

The disrupted cortical lamination phenotype in reeler mice and subsequent identification of the Reelin signaling pathway have strongly informed models of cortical development. We describe here a marker-based phenotyping approach to reexamine the cytoarchitectural consequences of Reelin deficiency, using high-throughput histology and ...

Damage to the nervous system after poisoning with mercurial compounds has been documented clinically and experimentally. However, mercury poisoning of a human fetus due to maternal exposure has rarely been reported, and neuropathological studies have been limited. The report concerns clinical and neuropathologic findings in two fullterm infants who were exposed to mercury in utero by maternal ...

Maternal infection during pregnancy is an environmental risk factor for the development of severe brain disorders in offspring, including schizophrenia and autism. However, little is known about the neurodevelopmental mechanisms underlying the association between prenatal exposure to infection and the emergence of cognitive and behavioral dysfunctions in later life. By ...

The subplate zone is a transient cytoarchitectonic compartment of the fetal telencephalic wall and contains a population of subplate neurons which are the main neurons of the fetal neocortex and play a key role in normal development of cerebral cortical structure and connectivity. While the subplate zone disappears during the perinatal and early postnatal ...

Adolescent idiopathic scoliosis (AIS) is a 3-D spinal deformity with uncertain etiology; abnormalities in brain development represent one of the possible explanatory concepts for its pathogenesis. The objective of this study is to investigate the brain maturation by thickness of cerebral cortex among female adolescents with and without idiopathic ...

In the developing human brain, the cortical sulci formation is a complex process starting from 14 weeks of gestation onward. The potential influence of underlying mechanisms (genetic, epigenetic, mechanical or environmental) is still poorly understood, because reliable quantification in vivo of the early folding is lacking. In this study, we investigate ...

... a brief outline for the development and implementation of ... data exceptions with the abnormalities will be ... the attributes of the abnormality and search ...

The olfactocentric paralimbic cortex plays a critical role in the regulation of emotional and neurovegetative functions that are disrupted in core features of bipolar disorder. Adolescence is thought to be a critical period in both the maturation of the olfactocentric paralimbic cortex and in the emergence of bipolar disorder pathology. Together, these factors implicate a central role for the ...

The olfactocentric paralimbic cortex plays a critical role in the regulation of emotional and neurovegetative functions that are disrupted in core features of bipolar disorder. Adolescence is thought to be a critical period in both the maturation of the olfactocentric paralimbic cortex and in the emergence of bipolar disorder pathology. Together, these factors implicate a central role for the ...

Cerebral cortical ?-aminobutyric acid (GABA)ergic interneurons originate from the basal forebrain and migrate into the cortex in 2 phases. First, interneurons cross the boundary between the developing striatum and the cortex to migrate tangentially through the cortical primordium. Second, interneurons migrate radially to their correct ...

Cortical dysplasia has a strong clinical association with epilepsy and mental retardation, but the relationship between alterations in cortical structure and function in dysplasia-related disorders is poorly understood. The cerebral cortex of irradiated rats, an experimental model of cortical dysplasia, was studied using cresyl ...

We report the identification of a hypomorphic mouse allele for Foxc1 (Foxc1^hith) that survives into adulthood revealing previously unknown roles for Foxc1 in development of the skull and cerebral cortex. This line of mice was recovered in a forward genetic screen using ENU mutagenesis to identify mutants with cortical defects. In the ...

Abnormal cortical folding patterns within Broca's area in schizophrenia: Evidence from structural compared cortical folding patterns between patients with schizophrenia and demographically-matched healthy and language dysfunction in schizophrenia. � 2007 Elsevier B.V. All rights reserved. Keywords: Schizophrenia

BackgroundPatients with traumatic brain injury (TBI) often present with significant cognitive deficits without corresponding evidence of cortical damage on neuroradiological examinations. One explanation for this puzzling observation is that the diffuse cortical abnormalities that characterize TBI are difficult to detect with standard ...

OBJECTIVE. Auditory neuropathy spectrum disorder (ANSD) affects approximately 10% of patients with sensorineural hearing loss. While many studies report abnormalities at the level of the cochlea, auditory nerve, and brainstem in children with ANSD, much less is known about their cortical development. We examined central auditory ...

In human patients, cortical dysplasia produced by Doublecortin (DCX) mutations lead to mental retardation and intractable infantile epilepsies, but the underlying mechanisms are not known. DCX(-/-) mice have been generated to investigate this issue. However, they display no neocortical abnormality, lessening their impact on the field. In contrast, in utero ...

Rats were irradiated by 200 R of x ray on day 17 of gestation through the body wall of the mother. When they underwent the following electrophysiological tests at the age of 3 to 4 month, the somatosensory cortex showed a lack of layers II, III, IV, and Va. Spike responses to quick whisker deflections were recorded from single cells in the somatosenory cortex of normal and prenatally x-irradiated ...

The manuscript by Tsai et al. (935�945) is a tour de force analysis of a controversial issue in developmental neurobiology, namely the molecular basis of the devastating human brain malformation, type I lissencephaly (Lis1) (Jellinger, K., and A. Rett. 1976. Neuropadiatrie. 7:66�91). For several decades, defects in neuronal migration have been assumed to underlie all defects in ...

Prior studies on healthy children have demonstrated regional variations and a complex and dynamic relationship between intelligence and cerebral tissue. Yet, there is little information regarding the neuroanatomical correlates of general intelligence in children with epilepsy compared to healthy controls. In vivo imaging techniques, combined with methods for advanced image processing and analysis, ...

Regulation of stem cell self-renewal versus differentiation is critical for embryonic development and adult tissue homeostasis. Drosophila larval neuroblasts divide asymmetrically to self-renew, and are a model system for studying stem cell self-renewal. Here we identify three mutations showing increased brain neuroblast numbers that map to the aurora-A gene, which encodes a ...

Gaucher disease (GD) is an autosomal recessive lysosomal storage disorder characterized by the reduced or absent activity of glucocerebrosidase. The disease is split into three types. Type 3, or chronic neuronopathic GD, manifests with heterogeneous clinical presentations. Skeletal manifestations of GD can include abnormal bone remodeling resulting in the characteristic ...

Multiple adjacent, roughly mirror-image topographic maps are commonly observed in the sensory neocortex of many species. The cortical regions occupied by these maps are generally believed to be determined initially by genetically controlled chemical markers during development, with thalamocortical afferent activity subsequently exerting a progressively ...

Abstract Objective: To investigate possible structural abnormalities of the central auditory pathway in infants with non-syndromic cleft lip and/or palate (NSCL/P). Participants: Twenty seven Chinese infants with NSCL/P, aged from 6 to 24 months. Intervention: Morphological magnetic resonance imaging (MRI) measurements of the central auditory nervous system (CANS) in infants ...

This paper describes algorithms that can identify patterns of brain structure and function associated with Alzheimer's disease, schizophrenia, normal aging, and abnormal brain development based on imaging data collected in large human populations. Extraordinary information can be discovered with these techniques: dynamic brain maps reveal how the brain ...

Background:Emotion processing abnormalities are considered among the core deficits in schizophrenia. Subjects at high risk (HR) for schizophrenia also show these deficits. Structural neuroimaging studies examining unaffected relatives at high risk for schizophrenia have demonstrated neuroanatomical abnormalities involving neo-cortical ...

Preterm birth is a leading cause of cognitive impairment in childhood and is associated with cerebral gray and white matter abnormalities. Using multimodal image analysis, we tested the hypothesis that altered thalamic development is an important component of preterm brain injury and is associated with other macro- and microstructural alterations. T(1)- ...

BackgroundWe postulated that during ontogenesis cortical surface area and cerebral volume are related by a scaling law whose exponent gives a quantitative measure of cortical development. We used this approach to investigate the hypothesis that premature termination of the intrauterine environment by preterm birth reduces ...

In the human brain, the morphology of cortical gyri and sulci is complex and variable among individuals, and it may reflect pathological functioning with specific abnormalities observed in certain developmental and neuropsychiatric disorders. Since cortical folding occurs early during brain development, these ...

In the human brain, the morphology of cortical gyri and sulci is complex and variable among individuals, and it may reflect pathological functioning with specific abnormalities observed in certain developmental and neuropsychiatric disorders. Since cortical folding occurs early during brain development, these ...

Pinna abnormalities and low-set ears refer to abnormalities in the shape or position of the outer ear (pinna ... critical organs are developing (such as the kidneys). Abnormalities in the shape or position of the pinna ...

Skeletal fragility is common at metaphyseal regions of long bones. The cortices of this region are derived by coalescence of trabeculae around the periphery of the growth plate, not by periosteal apposition, as occurs in the diaphyses. We therefore hypothesized that trabecular bone in childhood predicted both cortical and trabecular morphology in ...

Fatvg is a localized maternal transcript that translocates to the vegetal cortex of Xenopus laevis oocytes through both the METRO and Late RNA localization pathways. It is a member of a gene family that functions in vesicular trafficking. Depletion of the maternal store of fatvg mRNA results in a dual phenotype in which embryos are ventralized and also lack primordial germ cells. This complex ...

The Zeitgeist favors an interpretation of schizophrenia as a condition of abnormal connectivity of cortical neurons, particularly in the prefrontal and temporal cortex. The available evidence points to reduced connectivity, a possible consequence of excessive synaptic pruning in development. A decreased thalamic input to the cerebral ...

Autism is a complex neurodevelopmental disorder of early onset that is highly variable in its clinical presentation. Although the causes of autism in most patients remain unknown, several lines of research support the view that both genetic and environmental factors influence the development of abnormal cortical circuitry that ...

... departments and agencies for Research Development Test and ... Title : WATER DISTRIBUTION ABNORMALITIES IN HYPOTHYROID DOGS DUE ...

... the military departments and agencies for Research Development Test and ... Title : Vascular Abnormalities Associated with Thermal and Electrical ...

... the military departments and agencies for Research Development Test and ... STUDIES ON THE NATURE OF THE ABNORMALITIES IN SOLUTE ...

... 0703 TITLE: Redox Abnormalities as a Vulnerability Phenotype for Autism and Related Alterations in CNS Development ...

... 0702 TITLE: Redox abnormalities as a vulnerability phenotype for Autism and related alternations in CNS development ...

... and analysis capabilities across the military departments and agencies for Research Development Test and ... Title : Platelet Abnormalities in Malaria. ...

... military departments and agencies for Research Development Test and ... Title : Molecular Mechanisms of Glial Abnormalities in Neurofibromatosis. ...

... capabilities across the military departments and agencies for Research Development Test and ... Title : Detection of Abnormalities in MANETs. ...

... military departments and agencies for Research Development Test and ... Title : DETECTING BURNING ABNORMALITIES IN A SOLID PROPELLANT ...

... military departments and agencies for Research Development Test and ... Title : DETECTING BURNING ABNORMALITIES IN A SOLID PROPELLANT ...

... the military departments and agencies for Research Development Test and ... Title : CHROMOSOME ABNORMALITIES IN LIVER AND MARROW OF ...

Fragile X Syndrome (FXS) is the most common heritable cause of intellectual disability. In vitro electrophysiologic data from mouse models of FXS suggest that loss of fragile X mental retardation protein affects intracortical excitability and synaptic plasticity. Specifically, the cortex appears hyperexcitable, and use-dependent long-term potentiation (LTP) and long-term depression (LTD) of ...

Tuberous Sclerosis Complex (TSC) is a multiorgan genetic disease that prominently features brain malformations (tubers) with many patients suffering from epilepsy and autism. These malformations typically exhibit neuronal as well as glial cell abnormalities and likely underlie much of the neurological morbidity seen in TSC. Tuber pathogenesis remains poorly understood though ...

... cell division described above (Frankel 1964). Such a developmental abnormality was not observed during transient inhibition of cell ... ...

Schimke immuno-osseous dysplasia (OMIM 242900) is an uncommon autosomal-recessive multisystem disease caused by mutations in SMARCAL1 (swi/snf-related, matrix-associated, actin-dependent regulator of chromatin, subfamily a-like 1), a gene encoding a putative chromatin remodeling protein. Neurologic manifestations identified to date relate to enhanced atherosclerosis and cerebrovascular disease. ...

SummaryMice with a null mutation of the presenilin 1 gene (Psen1^-/-)die during late intrauterine life or shortly after birth and exhibit multiple CNS and non-CNS abnormalities, including cerebral hemorrhages and altered cortical development. The ...

Fragile X Syndrome (FXS), also known as Martin-Bell Syndrome, is a genetic abnormality found on the X chromosome. Individuals suffering from FXS display abnormalities in the expression of FMR1--a protein required for typical, healthy neural development. Recent data has suggested that the loss of this protein can cause the cortex to be ...

It is essential in the analysis of emission computed tomography (ECT) scans of the living human brain to be able to reliably and objectively obtain regional quantitative data. Therefore, the following thesis describes new methods for the analysis of the regional distribution of radioactivity in brain ECT images, which reduces the variability inherent in observer guided region of interest ...

Genes associated with human microcephaly, a condition characterized by a small brain, include critical regulators of proliferation, cell fate and DNA repair. We describe a syndrome of congenital microcephaly and diverse defects in cerebral cortical architecture. Genome-wide linkage analysis in two families identified a 7.5-Mb locus on chromosome 19q13.12 containing 148 genes. ...

Genes associated with human microcephaly, a condition characterized by a small brain, include critical regulators of proliferation, cell fate, and DNA repair. We describe a syndrome of congenital microcephaly and diverse defects in cerebral cortical architecture. Genome-wide linkage analysis in two families identified a 7.5 Mb locus on chromosome 19q13.12 containing 148 genes. ...

The advent of high resolution MRI techniques has revolutionized the imaging of cortical malformations. Today, specific gene defects have been identified to be responsible for several of the developmental cortical malformations. In this article we have discussed the developmental cortical malformations under stages of proliferation, ...

An outstanding problem in psychiatry concerns how to link discoveries about the pharmacological, neurophysiological, and neuroanatomical substrates of mental disorders to the abnormal behaviors that they control. A related problem concerns how to understand abnormal behaviors on a continuum with normal behaviors. During the past few decades, neural models ...

Language development is delayed in most children on the autistic spectrum. The children are dysphasic as well as autistic. Comprehension and pragmatics are invariably affected. Lower level mixed receptive/expressive disorders involve phonological and syntactical processing, whereas higher level processing disorders involve semantics and formulation of discourse. In some ...

Numerous reports of elevated global motion thresholds across a variety of neurodevelopmental disorders have prompted researchers to suggest that abnormalities in global motion perception are a result of a general deficiency in the dorsal visual pathway. To test this hypothesis, we assessed the integrity of the dorsal visual pathway at lower subcortical (sensitivity to flicker ...

Characteristically arising in response to overwhelmingly terrifying events, post-traumatic stress disorder (PTSD) is a disorder of memory: sufferers seemingly relive their trauma in the form of involuntary recollection. Prominent cognitive abnormalities, especially in memory functioning, have motivated research designed to elucidate the mediating mechanisms that produce PTSD ...

This study was designed to rate the clinical value of ({sup 123}I)iodoamphetamine (IMP) or ({sup 99m}Tc) hexamethyl propylene amine oxyme (HM-PAO) brain single photon emission computed tomography (SPECT) in neonates, especially in those likely to develop cerebral palsy. The results showed that SPECT abnormalities were congruent in most cases with ...

This review intends to provide examples how comparative and genetic analyses both contribute to our understanding of the rules for cortical development and evolution. Genetic studies helped to understand evolutionary rules of telencephalic organization in vertebrates. The control of the establishment of conserved telencephalic subdivisions and the ...

Nuclear receptor 2E1 (NR2E1) is expressed in human fetal and adult brains; however, its role in human brain-behavior development is unknown. Previously, we have corrected the cortical hypoplasia and behavioral abnormalities in Nr2e1(-/-) mice using a genomic clone spanning human NR2E1, which bolsters the hypothesis that NR2E1 may ...

Nuclear receptor 2E1 (NR2E1) is expressed in human fetal and adult brains; however, its role in human brain�behavior development is unknown. Previously, we have corrected the cortical hypoplasia and behavioral abnormalities in Nr2e1^?/? mice using a genomic clone spanning human NR2E1, which bolsters the ...

Pregnant female rats were irradiated with 200 r on the 17th, 19th, or 21st day of gestation. Other newborn rats received 200-r head irradiation on the 23rd, 26th, or 29th day after conception. In both groups the effects of irradiation on cortical structure and on the electrical activity of the brain were studied. Structural changes resulting from this treatment ...

Fetal alcohol syndrome (FAS) is a major cause of learning and sensory deficits in children. The visual system in particular is markedly affected, with an elevated prevalence of poor visual perceptual skills. Developmental problems involving the neocortex are likely to make a major contribution to some of these abnormalities. Neuronal selectivity to stimulus orientation, a ...

During human brain development, the cerebral cortex undergoes substantial folding, leading to its characteristic highly convoluted form. Folding is necessary to accommodate the expansion of the cerbral cortex; abnormal cortical folding is linked to various neurological disorders, including schizophrenia, epilepsy, autism and mental ...

This study compared MRI cerebral volumes and Neuronal-Nuclei (NeuN) cell densities in pediatric epilepsy surgery patients with cortical dysplasia (CD; n = 25) and hemimegalencephaly (HME; n = 14). Our purpose was to deduce possible mechanisms of pathogenesis and epileptogenesis based on an understanding of normal developmental corticoneurogenesis. We used MRI to measured ...

The imaging approach to refractory partial epilepsy in children is essentially the same as the one used for any epilepsy at that age, except that surgery being generally proposed, it has to be oriented and complete. Yet there are differences. Sedation introduces a risk. The brain of the young child is still immature, and specially designed sequences are needed for proper imaging. Brain immaturity ...

Although it has long been thought that frontal lobe abnormality must play an important part in generating the severe impairment in higher-order social, emotional and cognitive functions in autism, only recently have studies identified developmentally early frontal lobe defects. At the microscopic level, neuroinflammatory reactions involving glial activation, migration defects ...

Functional imaging studies have reported with remarkable consistency hyperactivity in the orbitofrontal cortex (OFC), anterior cingulate cortex (ACC), and caudate nucleus of patients with Obsessive-Compulsive Disorder (OCD). These findings have often been interpreted as evidence that abnormalities in cortico-basal ganglia-thalamo-cortical loops involving ...

Mast syndrome (SPG21) is a childhood-onset, autosomal recessive, complicated form of hereditary spastic paraplegia (HSP) characterized by dementia, thin corpus callosum, white matter abnormalities, and cerebellar and extrapyramidal signs in addition to spastic paraparesis. A nucleotide insertion resulting in premature truncation of the SPG21 gene product maspardin underlies ...

Psychopathy is characterized by abnormal emotional processes, but only recent neuroimaging studies have investigated its cerebral correlates. The study aim was to map local differences of cortical and amygdalar morphology. Cortical pattern matching and radial distance mapping techniques were used to analyze the magnetic resonance ...

Development of the human brain follows a complex trajectory of age-specific anatomical and physiological changes. The application of network analysis provides an illuminating perspective on the dynamic interregional and global properties of this intricate and complex system. Here, we provide a critical synopsis of methods of network analysis with a focus on ...

Cortical GABAergic dysfunction may underlie the pathophysiology of psychiatric disorders, including schizophrenia. Here, we characterized a mouse strain in which the essential NR1 subunit of the NMDA receptor (NMDAR) was selectively eliminated in 40�50% of cortical and hippocampal interneurons in early postnatal development. ...

Current models of speech production in adults emphasize the crucial role played by the left perisylvian cortex, primary and pre-motor cortices, the basal ganglia, and the cerebellum for normal speech production. Whether similar brain-behaviour relationships and leftward cortical dominance are found in childhood remains unclear. Here we reviewed recent ...

The Reelin signaling pathway is essential for proper cortical development, but it is unclear to whether Reelin function is primarily important for cortical layering or neuron migration. It has been proposed that Reelin is perhaps required only for somal translocation but not glial-dependent locomotion. This implies that the location of ...

Introduction Characterizing normal versus abnormal cortical geometry is an important goal of human brain mapping. This task proves difficult because of the convoluted nature of the cortical surface. Difficulties arise in both identifying topological landmarks and obtaining reliable quantitative measurements. A standard measurement ...

Abstract: We present anovel, computerized method of examining cerebral cortical thickness. The normal cortex varies in thickness from 2to 4mm, reflecting the morphology of neuronal sublayers. Cortical pathologies often manifest abnormal variations in thickness, with examples of Alzheimer�s disease and cortical ...

Alzheimer's disease (AD) is associated with neurodegeneration in vulnerable limbic and heteromodal regions of the cerebral cortex, detectable in vivo using magnetic resonance imaging. It is not clear whether abnormalities of cortical anatomy in AD can be reliably measured across different subject samples, how closely they track symptoms, and whether they ...

Recent evidence suggests that a rapid, automatic face detection system is supported by subcortical structures including the amygdala, pulvinar, and superior colliculus. Early-emerging abnormalities in these structures may be related to reduced social orienting in children with autism, and subsequently, to aberrant development of ...

Polyhydramnios, megalencephaly, and symptomatic epilepsy syndrome (PMSE) is a rare human autosomal-recessive disorder characterized by abnormal brain development, cognitive disability, and intractable epilepsy. It is caused by homozygous deletions of STE20-related kinase adaptor alpha (STRADA). The underlying pathogenic mechanisms of PMSE and the role of ...

Polyhydramnios, megalencephaly, and symptomatic epilepsy syndrome (PMSE) is a rare human autosomal-recessive disorder characterized by abnormal brain development, cognitive disability, and intractable epilepsy. It is caused by homozygous deletions of STE20-related kinase adaptor ? (STRADA). The underlying pathogenic mechanisms of PMSE and the role of ...

... Abnormality diameter (mm ... Psychophysical functions for the detectability of abnormalities of various ... to do this by the preliminary development of user ...

... of social experience early in their life, one can produce abnormalities in their ... causation of abnormal and normal behaviour in the development of an ...

in a topographic map that are (ii) orientation selective and form a cortical orientation map. Self�organizing mapsCORTICAL ORIENTATION MAP DEVELOPMENT FROM NATURAL IMAGES: THE ROLE OF CORTICAL RESPONSE in a typical orientation selectivity map. 1. Introduction The representation of visual information at the level

Purpose:? Interictal increase of (11) C-alpha-methyl-l-tryptophan (AMT) on positron emission tomography (PET) can be seen in cortical epileptic foci, and is particularly common in cortical developmental malformations. Therefore, in the present study, we evaluated the clinical and histopathologic correlates of AMT-PET abnormalities in ...

SummaryCortical dysplasia is often associated with intractable seizures. Studies in animal models have described changes in inhibitory and excitatory synaptic function that contribute to hyperexcitability. The role of changes in intrinsic excitability and abnormal dendritic properties has received less attention. Changes in hyperpolarization-activated ...

New York State began screening for Krabbe disease in 2006 to identify infants with Krabbe disease before symptom onset. Because neither galactocerebrosidase activity nor most genotypes reliably predict phenotype, the World Wide Registry was developed to determine whether other clinical/neurodiagnostic data could predict early infantile Krabbe disease in the newborn screening ...

Malformations of cortical development (MCDs) are commonly complicated by intractable focal epilepsy. Epileptogenesis in these disorders is not well understood and may depend on the type of MCD. The cellular mechanisms involved in interictal and ictal events are notably different, and could be influenced independently by the type of pathology. We evaluated ...

Correct cell cycle regulation and terminal mitosis are critical for nervous system development. The retinoblastoma (Rb) protein is a key regulator of these processes, as Rb�/� embryos die by E15.5, exhibiting gross hematopoietic and neurological defects. The extensive apoptosis in Rb�/� embryos has been attributed to aberrant S phase entry resulting in conflicting ...

BackgroundSchizophrenia and related psychoses are associated with brain structural abnormalities. Recent findings in �at risk� populations have identified progressive changes in various brain regions preceding illness onset, while changes especially in prefrontal and superior temporal regions have been demonstrated in first-episode schizophrenia patients. However, the ...

The purpose of this study was to determine the efficacy of pre-treating mature bovine oocytes with Taxol before vitrification by the open pulled Straw method (OPS). We evaluated the effects of pre-treating the oocytes with 1 microM Taxol on chromosome organization, spindle morphology, cortical granule distribution and the ability of fertilized oocytes to ...

Although motor deficits are common in autism, the neural correlates underlying the disruption of even basic motor execution are unknown. Motor deficits may be some of the earliest identifiable signs of abnormal development and increased understanding of their neural underpinnings may provide insight into autism-associated differences in parallel systems ...

Although motor deficits are common in autism, the neural correlates underlying the disruption of even basic motor execution are unknown. Motor deficits may be some of the earliest identifiable signs of abnormal development and increased understanding of their neural underpinnings may provide insight into autism-associated differences in parallel systems ...

In normal-hearing children the latency of the P1 component of the cortical evoked response to sound varies as a function of age and, thus, can be used as a biomarker for maturation of central auditory pathways. We assessed P1 latency in 245 congenitally deaf children fit with cochlear implants following various periods of auditory deprivation. If children experience less than ...

The brain vascular system develops in such a way that it continuously adapts the supply of oxygen and other nutrients to the needs of the parenchyma. To accompany the developing brain vesicles, it evolves in several steps: superficial meningeal network first; intraventricular choroid plexuses which determine the arterial pattern; penetrating capillaries ...

Although schizophrenia is characterized by gray matter (GM) abnormalities, particularly in the prefrontal and temporal cortices, it is unclear whether cerebral cortical GM is abnormal in individuals at ultra-high-risk (UHR) for psychosis. We addressed this issue by studying cortical thickness ...

Brain magnetic resonance imaging (MRI) studies in patients with Dravet syndrome and SCN1A mutations have shown abnormal findings in a small minority of patients. The origin of the structural abnormalities--such focal brain atrophy, cortical dysplasia, and hippocampal sclerosis--observed in some children remains unclear. There seems to ...

Following the discovery of context-dependent synchronization of oscillatory neuronal responses in the visual system, novel methods of time series analysis have been developed for the examination of task- and performance-related oscillatory activity and its synchronization. Studies employing these advanced techniques revealed that synchronization of oscillatory responses in the ...

Although schizophrenia is an illness that has been historically characterized by the presence of positive symptomatology, decades of research highlight the importance of cognitive deficits in this disorder. This review proposes that the theoretical model of cognitive control, which is based on contemporary cognitive neuroscience, provides a unifying theory for the cognitive and neural ...

Although schizophrenia is an illness that has been historically characterized by the presence of positive symptomatology, decades of research highlight the importance of cognitive deficits in this disorder. This review proposes that the theoretical model of cognitive control, which is based on contemporary cognitive neuroscience, provides a unifying theory for the cognitive and neural ...

In childhood mitochondrial encephalopathies the common MRI features are bilateral symmetric abnormalities in basal nuclei and brainstem. The presence of diffuse white matter abnormality has been described only in a few cases. Among a series of 110 children with mitochondrial encephalopathies, 8 patients with MR imaging consistent with a leukoencephalopathy ...

An Inattentive/Overactive (I/O) behavioral phenotype has been reported in a significant percentage of children raised from birth in orphanages. While a number of studies have identified both functional and structural brain abnormalities in children raised from birth in orphanages, no published studies have evaluated potential neural correlates of the I/O phenotype. We applied ...

An Inattentive/Overactive (I/O) behavioral phenotype has been reported in a significant percentage of children raised from birth in orphanages. While a number of studies have identified both functional and structural brain abnormalities in children raised from birth in orphanages, no published studies have evaluated potential neural correlates of the I/O phenotype. We applied ...

Ovaries and testes of chick embryos explanted in vitro were submitted to 1600- to 12,450-r irradiation, transferred to fresh medium, and cultured for 4 to 10 days longer. The irradiated cultures appeared grossly normal but histologic examination clearly revealed abnormalities. In the most abnormal cultures, sexual differentiation of the gonads ...

Trichopoliodystrophy (also known as Menkes' kinky or steely hair disease), a recessive sex-linked syndrome, is characterized by severely retarded mental and physical development, convulsions, a particular phenotype and abnormalities of the hair, bones and arteries. Very low levels of copper and ceruloplasmin in the serum confirm the diagnosis. This rare ...

... 1992) Ethanol effects on cortical circuits during sensory and motor processing. In RR Watson, ed. Alcohol and Neurobiology: Brain Development ...