Science.gov

Sample records for normal neurological development

  1. Neurology in the developing world.

    PubMed

    Singhal, B S; Khadilkar, Satish V

    2014-01-01

    The social and economic impact of neurologic disorders is being increasingly recognized in the developing world. Demographic transition, especially in large Asian populations, has resulted in a significant increase in the elderly population, bringing to the fore neurologic illnesses such as strokes, Alzheimer's disease, and Parkinson's disease. CNS infections such as retroviral diseases, tuberculosis, and malaria still account for high mortality and morbidity. Traumatic brain injury due to traffic accidents takes a high toll of life. Epilepsy continues to be a major health concern with large segments of the developing world's population receiving no treatment. A significant mismatch between the provision of specialized neurologic services and the requirement for them exists, especially in rural areas. Also, health insurance is not available for the majority, with patients having bear the costs themselves, thus limiting the procurement of available healthcare facilities. Neurologic training centers are few and the availability of laboratory facilities and equipment is largely limited to the metropolitan areas. Cultural practices, superstitious beliefs, ignorance, and social stigma may also impede the delivery of neurologic care. Optimizing available human resources, integrating primary, secondary, and tertiary healthcare tiers and making medical treatment more affordable will improve the neurologic care in the developing world.

  2. Normal development.

    PubMed

    Girard, Nadine; Koob, Meriam; Brunel, Herv

    2016-01-01

    Numerous events are involved in brain development, some of which are detected by neuroimaging. Major changes in brain morphology are depicted by brain imaging during the fetal period while changes in brain composition can be demonstrated in both pre- and postnatal periods. Although ultrasonography and computed tomography can show changes in brain morphology, these techniques are insensitive to myelination that is one of the most important events occurring during brain maturation. Magnetic resonance imaging (MRI) is therefore the method of choice to evaluate brain maturation. MRI also gives insight into the microstructure of brain tissue through diffusion-weighted imaging and diffusion tensor imaging. Metabolic changes are also part of brain maturation and are assessed by proton magnetic resonance spectroscopy. Understanding and knowledge of the different steps in brain development are required to be able to detect morphologic and structural changes on neuroimaging. Consequently alterations in normal development can be depicted. PMID:27430460

  3. Human Neurological Development: Past, Present and Future

    NASA Technical Reports Server (NTRS)

    Pelligra, R. (Editor)

    1978-01-01

    Neurological development is considered as the major human potential. Vision, vestibular function, intelligence, and nutrition are discussed as well as the treatment of neurological disfunctions, coma, and convulsive seizures.

  4. Cerebrospinal fluid neurotransmitter metabolites in neurologically normal infants and children.

    PubMed

    Langlais, P J; Walsh, F X; Bird, E D; Levy, H L

    1985-03-01

    Significant inverse correlations with age were observed for free 3-methoxy-4-hydroxyphenylglycol (MHPG), 5-hydroxyindoleacetic acid (5-HIAA), and homovanillic acid (HVA) concentrations in CSF from 75 neurologically normal newborns, infants, and children aged 1 day to 10 years. The concentration of free MHPG decreased rapidly in early neonatal life and was reduced to near adult levels by 8 to 9 months of age. Adult levels of 5-HIAA were observed at about 4 years of age whereas HVA concentrations were still above adult levels at 10 years of age. Data from 0- to 1-month-old premature (28 to 32 weeks of gestation) and full-term (37 weeks of gestation) infants revealed marked changes in HVA and 5-HIAA concentrations which were related to postconceptional rather than postnatal age. This study demonstrates a previously undetected age effect on CSF MHPG concentration during the neonatal period and provides valuable normal data that are necessary for the interpretation of CSF monoamine metabolites in infants and children with hyperphenylalaninemia and other neurologic disease involving monoamine neurotransmitters.

  5. Neurological findings in Alzheimer's disease and normal aging.

    PubMed

    Galasko, D; Kwo-on-Yuen, P F; Klauber, M R; Thal, L J

    1990-06-01

    To determine the potential value of abnormal neurological findings as markers of Alzheimer's disease (AD) and their relationship to the stage of AD, we compared standardized neurological examinations in 135 community-dwelling patients with AD and 91 nondemented elderly individuals. After correcting for differences in age and education between the two groups, we found that rigidity, stooped posture, graphesthesia, neglect of simultaneous tactile stimuli (face-hand test), and snout, grasp, and glabella reflexes were present significantly more often in patients with AD than in control subjects. These findings increased in prevalence in patients with AD according to the severity of dementia. However, in a multivariate logistic regression model only the grasp reflex, graphesthesia, and the face-hand test were statistically significantly associated with the degree of cognitive impairment. Although abnormal neurological findings occur regularly in AD, they are too infrequent early in the course of AD to serve as diagnostic markers. Prospective studies are needed to determine whether patients with the early onset of extrapyramidal or other findings form a distinct subgroup of AD.

  6. Normal pressure hydrocephalus in the spectrum of neurological complications of systemic lupus erythematosus.

    PubMed

    de Oliveira, Fabricio Ferreira; Cardoso, Tania Aparecida Marchiori; Sampaio-Barros, Percival Degrava; Damasceno, Benito Pereira

    2013-06-01

    Normal pressure hydrocephalus is an unusual manifestation of systemic lupus erythematosus and its pathogenesis is still unclear. We report the case of a 39-year-old white woman with systemic lupus erythematosus who developed magnetic gait, speech difficulties, progressive memory impairment, urinary incontinence and episodes of involuntary closure of the eyelids. Signs and symptoms, associated with ventriculomegaly and normal cerebrospinal fluid pressure, suggested normal pressure hydrocephalus, which as a complication of systemic lupus erythematosus believably develops due to the insidious inflammatory process that occurs in the meningeal tissues or to the vasculitis itself. Normal pressure hydrocephalus tends to develop secondary to trauma, infection or subarachnoid haemorrhage, but in 50 % of patients no aetiology is found. Shunt surgery is the only effective treatment, specifically for the gait disorder, which usually improves more than the cognitive symptoms. Since the tap-test showed a strongly positive result, a medium pressure ventriculoperitoneal shunt was inserted, further replaced by a high pressure one in view of the complications, with less than expected improvement. Subdural hematomas and empyemas developed, requiring surgery and antibiotic therapy. A new tap-test was positive, and the patient improved only after a programmable valve was finally placed. However, pressure regulation shall be continuously required, and shunt dysfunction might still develop in the long term. The few similar case reports in the literature are reviewed, confirming the rarity of this neurological complication of systemic lupus erythematosus.

  7. Normal Psychosexual Development

    ERIC Educational Resources Information Center

    Rutter, Michael

    1971-01-01

    Normal sexual development is reviewed with respect to physical maturation, sexual interests, sex drive", psychosexual competence and maturity, gender role, object choice, children's concepts of sexual differences, sex role preference and standards, and psychosexual stages. Biologic, psychoanalytic and psychosocial theories are briefly considered.…

  8. Development of an oximeter for neurology

    NASA Astrophysics Data System (ADS)

    Aleinik, A.; Serikbekova, Z.; Zhukova, N.; Zhukova, I.; Nikitina, M.

    2016-06-01

    Cerebral desaturation can occur during surgery manipulation, whereas other parameters vary insignificantly. Prolonged intervals of cerebral anoxia can cause serious damage to the nervous system. Commonly used method for measurement of cerebral blood flow uses invasive catheters. Other techniques include single photon emission computed tomography (SPECT), positron emission tomography (PET), magnetic resonance imaging (MRI). Tomographic methods frequently use isotope administration, that may result in anaphylactic reactions to contrast media and associated nerve diseases. Moreover, the high cost and the need for continuous monitoring make it difficult to apply these techniques in clinical practice. Cerebral oximetry is a method for measuring oxygen saturation using infrared spectrometry. Moreover reflection pulse oximetry can detect sudden changes in sympathetic tone. For this purpose the reflectance pulse oximeter for use in neurology is developed. Reflectance oximeter has a definite advantage as it can be used to measure oxygen saturation in any part of the body. Preliminary results indicate that the device has a good resolution and high reliability. Modern applied schematics have improved device characteristics compared with existing ones.

  9. Neurological damage disrupts normal sex differences in psychophysiological responsiveness to music.

    PubMed

    Belfi, Amy M; Chen, Kuan-Hua; Schneider, Brett; Tranel, Daniel

    2016-01-01

    Men and women often display different physiological responses to emotional stimuli, and these responses can be affected by brain damage. Here, we investigated how brain damage differentially affects electrodermal responses based on sex. We studied neurologically normal, healthy adults and a sample of neurological patients. Participants listened to music, an emotional stimulus that reliably elicits skin conductance responses (SCRs). Electrodermal activity was recorded while participants listened to musical clips. When analyzing the data without regard to sex, there were no differences between healthy and brain-damaged participants in their SCRs. However, we found a significant interaction between brain injury status and sex. For men, brain damage significantly reduced SCRs. For women, there were no differences between brain-damaged participants and neurologically healthy participants. These findings illustrate the importance of including demographic variables, such as sex, when investigating brain-behavior relationships with a psychophysiological dependent variable. PMID:26681613

  10. Normal psychomotor development.

    PubMed

    Cioni, Giovanni; Sgandurra, Giuseppina

    2013-01-01

    "Psychomotor" development refers to changes in a child's cognitive, emotional, motor, and social capacities from the beginning of life throughout fetal and neonatal periods, infancy, childhood, and adolescence. It occurs in a variety of domains and a wide range of theories makes understanding children's development a challenging undertaking. Different models have tried to interpret the origins of human behavior, the pattern of developmental changes over time, and the individual and contextual factors that could direct child development. No single theory has been able to account for all aspects of child development, but each of them may contribute an important piece to the child development puzzle. Although theories sometimes disagree, much of their information is complementary rather than contradictory. The knowledge of child typical development and related theories and models is greatly useful for clinical practice, leading to recognition of developmental disorders and the ways in which they can be approached and treated. In this chapter, traditional and more modern concepts around functional development of psychomotor abilities are reported, firstly more in general and then specifically in the motor domain. PMID:23622146

  11. Normal growth and development

    MedlinePlus

    ... and development can be divided into four periods: Infancy Preschool years Middle childhood years Adolescence Soon after ... child's age. Healthy eating habits should begin during infancy. This can help prevent diseases such as high ...

  12. Infant Mental Development and Neurological Status, Family Socio-Economic Status, and Intelligence at Age Four.

    ERIC Educational Resources Information Center

    Ireton, Harold; And Others

    The relationship of infant mental development (Bayley Mental Scale, eight months) to four year Binet IQ was explored in the context of the study sample's neurological and socioeconomic characteristics for a sample of 536 full-term children. The Minnesota sample was approximately normal or average in terms of infant mental scores, infant…

  13. Regional cerebral blood flow and anxiety: a correlation study in neurologically normal patients

    SciTech Connect

    Rodriguez, G.; Cogorno, P.; Gris, A.; Marenco, S.; Mesiti, C.; Nobili, F.; Rosadini, G.

    1989-06-01

    Regional CBF (rCBF) was evaluated by the /sup 133/Xe inhalation method in 60 neurologically normal patients (30 men and 30 women) and hemispheric and regional values were correlated with anxiety measurements collected by a self-rating questionnaire before and after the examination. Statistically significant negative correlations between rCBF and anxiety measures were found. rCBF reduction for high anxiety levels is in line with results previously reported by others and could be related to lower performance levels for moderately high anxiety scores as those reported in the present population. This could perhaps be explained by rearrangement of flow from cortical zones to deeper areas of the brain, classically known to be implicated in the control of emotions. However, these results should be interpreted cautiously, since they were obtained in patients and not in normal subjects.

  14. Clinical NMR imaging of the brain in children: normal and neurologic disease

    SciTech Connect

    Johnson, M.A,; Pennock, J.M.; Bydder, G.M.; Steiner, R.E.; Thomas, D.J.; Hayward, R.; Bryant, D.R.T.; Payne, J.A.; Levene, M.I.; Whitelaw, A.; Dubowitz, L.M.S.; Dubowitz, V.

    1983-11-01

    The results of initial clinical nuclear magnetic resonance imaging of the brain in eight normal and 52 children with a wide variety of neurologic diseases were reviewed. The high level of gray-white matter contrast available with inversion-recovery sequences provided a basis for visualizing normal myelination as well as delays or deficits in this process. The appearances seen in cases of parenchymal hemorrhage, cerebral infarction, and proencephalic cysts are described. Ventricular enlargement was readily identified and marginal edema was demonstrated with spin-echo sequences. Abnormalities were seen in cerebral palsy, congenital malformations, Hallervorden-Spatz disease, aminoaciduria, and meningitis. Space-occupying lesions were identified by virtue of their increased relaxation times and mass effects. Nuclear magnetic resonance imaging has considerable potential in pediatric neuroradiologic practice, in some conditions supplying information not available by computed tomography or sonography.

  15. A comparison of the codeswitching patterns of aphasic and neurologically normal bilingual speakers of English and Spanish.

    PubMed

    Muñoz, M L; Marquardt, T P; Copeland, G

    1999-02-01

    Conversational discourse samples were obtained from four aphasic and four neurologically normal Hispanic bilinguals in monolingual English, monolingual Spanish, and bilingual contexts to identify codeswitching patterns. Analysis of the samples based on the Matrix Language Frame (MLF) Model (Myers-Scotton, 1993a) revealed consistent matching of the language context by the aphasic and normal subjects. The aphasic subjects demonstrated a greater frequency of MLF constituents and codeswitching patterns not evident in the speech samples of the normal subjects. Results suggest an increased dependence on both languages for communication following neurological impairment.

  16. Liposomes and nanotechnology in drug development: focus on neurological targets

    PubMed Central

    Ramos-Cabrer, Pedro; Campos, Francisco

    2013-01-01

    Neurological diseases represent a medical, social, and economic problem of paramount importance in developed countries. Although their etiology is generally known, developing therapeutic interventions for the central nervous system is challenging due to the impermeability of the blood–brain barrier. Thus, the fight against neurological diseases usually struggles “at the gates” of the brain. Flooding the bloodstream with drugs, where only a minor fraction reaches its target therapeutic site, is an inefficient, expensive, and dangerous procedure, because of the risk of side effects at nontargeted sites. Currently, advances in the field of nanotechnology have enabled development of a generation of multifunctional molecular platforms that are capable of transporting drugs across the blood–brain barrier, targeting specific cell types or functional states within the brain, releasing drugs in a controlled manner, and enabling visualization of processes in vivo using conventional imaging systems. The marriage between drug delivery and molecular imaging disciplines has resulted in a relatively new discipline, known as theranostics, which represents the basis of the concept of personalized medicine. In this study, we review the concepts of the blood–brain barrier and the strategies used to traverse/bypass it, the role of nanotechnology in theranostics, the wide range of nanoparticles (with emphasis on liposomes) that can be used as stealth drug carriers, imaging probes and targeting devices for the treatment of neurological diseases, and the targets and targeting strategies envisaged in the treatment of different types of brain pathology. PMID:23486739

  17. Liposomes and nanotechnology in drug development: focus on neurological targets.

    PubMed

    Ramos-Cabrer, Pedro; Campos, Francisco

    2013-01-01

    Neurological diseases represent a medical, social, and economic problem of paramount importance in developed countries. Although their etiology is generally known, developing therapeutic interventions for the central nervous system is challenging due to the impermeability of the blood-brain barrier. Thus, the fight against neurological diseases usually struggles "at the gates" of the brain. Flooding the bloodstream with drugs, where only a minor fraction reaches its target therapeutic site, is an inefficient, expensive, and dangerous procedure, because of the risk of side effects at nontargeted sites. Currently, advances in the field of nanotechnology have enabled development of a generation of multifunctional molecular platforms that are capable of transporting drugs across the blood-brain barrier, targeting specific cell types or functional states within the brain, releasing drugs in a controlled manner, and enabling visualization of processes in vivo using conventional imaging systems. The marriage between drug delivery and molecular imaging disciplines has resulted in a relatively new discipline, known as theranostics, which represents the basis of the concept of personalized medicine. In this study, we review the concepts of the blood-brain barrier and the strategies used to traverse/bypass it, the role of nanotechnology in theranostics, the wide range of nanoparticles (with emphasis on liposomes) that can be used as stealth drug carriers, imaging probes and targeting devices for the treatment of neurological diseases, and the targets and targeting strategies envisaged in the treatment of different types of brain pathology.

  18. Dorsal and ventral stream sensitivity in normal development and hemiplegia.

    PubMed

    Gunn, Alison; Cory, Elizabeth; Atkinson, Janette; Braddick, Oliver; Wattam-Bell, John; Guzzetta, Andrea; Cioni, Giovanni

    2002-05-01

    Form and motion coherence thresholds can provide comparable measures of global visual processing in the ventral and dorsal streams respectively. Normal development of thresholds was tested in 360 normally developing children aged 4-11 and in normal adults. The two tasks showed similar developmental trends, with some greater variability and a slight delay in motion coherence compared to form coherence performance, in reaching adult levels. To examine the proposal of dorsal stream vulnerability related to specific developmental disorders, we compared 24 children with hemiplegic cerebral palsy with the normally developing group. Hemiplegic children performed significantly worse than controls on the motion coherence task for their age, but not on the form coherence task; however, within this group no specific brain area was significantly associated with poor motion compared to form coherence performance. These results suggest that extrastriate mechanisms mediating these thresholds normally develop in parallel, but that the dorsal stream has a greater, general vulnerability to early neurological impairment. PMID:11997698

  19. The Relationship of Family Background Factors and Neurological Status to Hyperactivity in a Normal Class Setting.

    ERIC Educational Resources Information Center

    Clarkson, Frank E.; Hayden, Benjamin S.

    Boys, aged 6-10 in regular classes, who were judged as hyperactive by their teachers were compared with matched non-hyperkinetic boys in terms of family background information and neurological functioning. Parent interview data were obtained on 109 hyperkinetic and 135 control Ss, while 121 hyperkinetic and 142 control Ss underwent pediatric…

  20. Frontiers in therapeutic development of allopregnanolone for Alzheimer’s disease and other neurological disorders

    PubMed Central

    Irwin, Ronald W.; Solinsky, Christine M.; Brinton, Roberta Diaz

    2014-01-01

    Allopregnanolone (Allo), a neurosteroid, has emerged as a promising promoter of endogenous regeneration in brain. In a mouse model of Alzheimer’s disease, Allo induced neurogenesis, oligodendrogenesis, white matter generation and cholesterol homeostasis while simultaneously reducing β-amyloid and neuroinflammatory burden. Allo activates signaling pathways and gene expression required for regeneration of neural stem cells and their differentiation into neurons. In parallel, Allo activates systems to sustain cholesterol homeostasis and reduce β-amyloid generation. To advance Allo into studies for chronic human neurological conditions, we examined translational and clinical parameters: dose, regimen, route, formulation, outcome measures, and safety regulations. A treatment regimen of once per week at sub-sedative doses of Allo was optimal for regeneration and reduction in Alzheimer’s pathology. This regimen had a high safety profile following chronic exposure in aged normal and Alzheimer’s mice. Formulation of Allo for multiple routes of administration has been developed for both preclinical and clinical testing. Preclinical evidence for therapeutic efficacy of Allo spans multiple neurological diseases including Alzheimer’s, Parkinson’s, multiple sclerosis, Niemann-Pick, diabetic neuropathy, status epilepticus, and traumatic brain injury. To successfully translate Allo as a therapeutic for multiple neurological disorders, it will be necessary to tailor dose and regimen to the targeted therapeutic mechanisms and disease etiology. Treatment paradigms conducted in accelerated disease models in young animals have a low probability of successful translation to chronic diseases in adult and aged humans. Gender, genetic risks, stage and burden of disease are critical determinants of efficacy. This review focuses on recent advances in development of Allo for Alzheimer’s disease (AD) that have the potential to accelerate therapeutic translation for multiple unmet

  1. Chapter 8: the development of neurology and the neurological sciences in the 17th century.

    PubMed

    Isler, Hansruedi

    2010-01-01

    Circa 1660 several favorable factors, instrumental to the invention of neurology, converged at the University of Oxford. Animals and men were believed to have a material soul whose functions throughout the nervous system were accessible to research. In 1659 inductive methods were introduced in clinical medicine by Thomas Willis, the founder of English epidemiology and biochemistry. The Vertuosi,who later founded the Royal Society, performed chemical experiments in teams, and Willis, head of their laboratory, gained experience in teamwork. In 1658 J.J. Wepfer published his method of dye injection in cerebral vessels at autopsy, and Christopher Wren had already experimented with intravenous injections. William Petty had performed dissections at Leiden, training with Francis Sylvius's brain and comparative anatomy. Petty came to Oxford in 1650, began to study chemistry with Willis, and instructed him in Sylvius's methods of cerebral and comparative anatomy. Willis continued this work with a team of highly qualified colleagues, Wren included, and published the first monograph on brain anatomy, Cerebri anatome, in 1664. This Latin book, illustrated by Wren, came out in four editions in the first year, and was reprinted up to 1720. It contained a definition of reflex action, the recognition of the general functions of cortex, white matter, and brain tracts, a complete description of the autonomic nervous system, Willis's new term "Neurologia," and his promise to follow up with his "Psychologia." He presented the latter in 1672 as De anima brutorum, a book on the material soul of animals and man as the carrier of all functions of the nervous system. There was a physiological part, a textbook of neurophysiology, and a pathological part, a compendium of neurological and psychiatric syndromes, with early descriptions of myasthenia, restless legs, narcolepsy, dissociative and bipolar disease, and general paralysis of the insane. In 1667 he published a book on convulsive

  2. Vision for Perception and Vision for Action: Normal and Unusual Development

    ERIC Educational Resources Information Center

    Dilks, Daniel D.; Hoffman, James E.; Landau, Barbara

    2008-01-01

    Evidence suggests that visual processing is divided into the dorsal ("how") and ventral ("what") streams. We examined the normal development of these streams and their breakdown under neurological deficit by comparing performance of normally developing children and Williams syndrome individuals on two tasks: a visually guided action ("how") task,…

  3. Development of a neurology rotation for internal medicine residents in Haiti.

    PubMed

    Berkowitz, Aaron L; Martineau, Louine; Morse, Michelle E; Israel, Kerling

    2016-01-15

    In many low-income countries where there are few or no neurologists, patients with neurologic diseases are cared for by primary care physicians who receive no formal training in neurology. Here, we report our experience creating a neurology rotation for internal medicine residents in rural Haiti through a collaboration between a public academic medical center in Haiti and a visiting neurologist. We describe the structure of the rotation and the factors that led to its development.

  4. The Effect of Neurological Dysfunction on the Social and Emotional Development of Young Children.

    ERIC Educational Resources Information Center

    Parette, Howard P., Jr.; Hourcade, Jack J.

    The literature review examines the relationship of neurological impairment in young children with their social and emotional development. It identifies disorders of interaction and/or attachment and disorders of independence/dependence as specific maladaptive social and emotional states associated with neurological impairment. Three theoretical…

  5. Prevalence of lateral ventricle asymmetry in brain MRI studies of neurologically normal dogs and dogs with idiopathic epilepsy.

    PubMed

    Pivetta, Mauro; De Risio, Luisa; Newton, Richard; Dennis, Ruth

    2013-01-01

    Asymmetry of the cerebral lateral ventricles is a common finding in cross-sectional imaging of otherwise normal canine brains and has been assumed to be incidental. The purpose of this retrospective study was to compare the prevalence of ventricular asymmetry in brain MRI studies of normal dogs and dogs with idiopathic epilepsy. Brain MRI archives were searched for 100 neurologically normal dogs (Group 1) and 100 dogs with idiopathic epilepsy (Group 2). For each dog, asymmetry of the lateral ventricles was subjectively classified as absent, mild, moderate, and severe based on a consensus of two observers who were unaware of group status. Ventricular areas were measured from transverse T1W images at the level of the interthalamic adhesion. An asymmetry ratio was calculated as the ratio of the larger to smaller ventricular transverse area. There was excellent agreement between subjective assessments of ventricular asymmetry and quantitative assessments using asymmetry ratios (k = 0.995). The prevalence of asymmetry was 38% in Group 1 dogs and 44% in Group 2 dogs. Assymmetry was scored as mild in the majority of Group 2 dogs. There was no significant association between presence/absence and degree of ventricular asymmetry vs. dog group, age, gender, or skull conformation. Findings from the current study supported previously published assumptions that asymmetry of the lateral cerebral ventricles is an incidental finding in MRI studies of the canine brain.

  6. Development of teaching and tuition in the specialty of neurology in the Netherlands.

    PubMed

    Keyser, A

    2003-07-01

    After a short survey of the early history of neurology and psychiatry in the Netherlands, the development of the specialty of neurology is discussed. During the 20th century the training of neurologists and the certification of specialists evolved from an informal master-fellow organization towards a strongly reglemented and legally based procedure. A nationwide Specialist Registration Commission supervises the quality of the training of specialists. Registered neurologists in the Netherlands are subject to a re-certification programme that controls the requirements to be fulfilled by the specialists such as their active involvement in patient care (for at least 16 h a week), attendance of the annual postgraduate courses in neurology (5-year cycle) and regular participation in international congresses of neurology. The undergraduate training in neurology, the neurology clerkship and the postgraduate training in neurosciences are described. Measures taken in order to maintain the balance between the supply of and the demand for neurological care in the near future are reported.

  7. [The Internet application to neurology: electronic forums of Revista de Neurología. How The Internet, Medicine and Neurology Forum has been developed].

    PubMed

    Botia, E

    1998-04-01

    The new communication techniques and, particularly, Internet, are changing our way of collaborating, relating to each other and searching for information, paving the way for telemedicine. Such initiatives are described in the Revista de Neurologia, as well as the methodology followed to prepare the first Electronic Forum of the Revista de Neurologia on 'Internet, Medicine and Neurology', using the web, IRC and E-mail tools.

  8. Duplication of HEY2 in Cardiac and Neurologic Development

    PubMed Central

    Jordan, Valerie K.; Rosenfeld, Jill A.; Lalani, Seema R.; Scott, Daryl A.

    2015-01-01

    HEY2 is a basic helix-loop-helix (bHLH) transcription factor that plays an important role in the developing mammalian heart and brain. In humans, nonsynonymous mutations in HEY2 have been described in patients with atrial ventricular septal defects, and a subset of individuals with chromosomal deletions involving HEY2 have cardiac defects and cognitive impairment. Less is known about the potential effects of HEY2 overexpression. Here, we describe a female child with tetralogy of Fallot who developed severe right ventricular outflow tract obstruction due to a combination of infundibular and valvular pulmonary stenosis. She was also noted to have hypotonia, lower extremity weakness, fine motor delay and speech delay. A copy number variation (CNV) detection analysis followed by real-time quantitative PCR analysis revealed a single gene duplication of HEY2. This is the only duplication involving HEY2 identified in our database of over 70,000 individuals referred for CNV analysis. In the developing heart, overexpression of HEY2 is predicted to cause decreased expression of the cardiac transcription factor GATA4 which, in turn, has been shown to cause tetralogy of Fallot. In mice, misexpression of Hey2 in the developing brain leads to inhibition of neurogenesis and promotion of gliogenesis. Hence, duplication of HEY2 may be a contributing factor to both the congenital heart defects and the neurodevelopmental problems evident in our patient. These results suggest that individuals with HEY2 duplications should be screened for congenital heart defects and monitored closely for evidence of developmental delay and/or cognitive impairment. PMID:25832314

  9. Neurological channelopathies

    PubMed Central

    Graves, T; Hanna, M

    2005-01-01

    Ion channels are membrane-bound proteins that perform key functions in virtually all human cells. Such channels are critically important for the normal function of the excitable tissues of the nervous system, such as muscle and brain. Until relatively recently it was considered that dysfunction of ion channels in the nervous system would be incompatible with life. However, an increasing number of human diseases associated with dysfunctional ion channels are now recognised. Such neurological channelopathies are frequently genetically determined but may also arise through autoimmune mechanisms. In this article clinical, genetic, immunological, and electrophysiological aspects of this expanding group of neurological disorders are reviewed. Clinical situations in which a neurological channelopathy should enter into the differential diagnosis are highlighted. Some practical guidance on how to investigate and treat this complex group of disorders is also included. PMID:15640425

  10. Maternal Intimate Partner Violence: Relationships with Language and Neurological Development of Infants and Toddlers.

    PubMed

    Udo, Ifeyinwa E; Sharps, Phyllis; Bronner, Yvonne; Hossain, Mian B

    2016-07-01

    Objectives This longitudinal study examined the influence of Intimate Partner Violence (IPV) experience of pregnant women participating in the Domestic Violence Enhanced Home Visitation Program on the language and neurological development of infants and toddlers. Methods A total of 210 infants and toddlers born to women reporting low, moderate, and high levels of IPV were included in the analysis. Logistic regression analysis was used to determine the bivariate association between maternal IPV and risk of language and neurological delay of infants and toddlers and between covariates and language and neurological delay. Generalized estimating equation models with logit link was used to predict the risk of language and neurological delay of infants and toddlers as a result of maternal IPV. Results Infants and toddlers born to women exposed to moderate levels of IPV had increased odds of language delay compared to infants and toddlers of women who experienced low levels of violence (OR 5.31, 95 % CI 2.94, 9.50, p < 0.001). Infants and toddlers born to women who experienced moderate and high levels of IPV were at higher risk of neurological delay respectively, compared to infants and toddlers of women who experienced low levels of IPV (OR 5.42, 95 % CI 2.99, 9.82, p < 0.001 and OR 2.57, 95 % CI 1.11, 5.61, p = 0.026). Conclusions for Practice Maternal IPV is associated with increased risk of language and neurological delay of infants and toddlers. These findings have implications for health care for women and infants exposed to IPV. Clinicians including pediatricians working with pregnant women should screen for IPV throughout pregnancy to identify women and children at risk. Interventions to reduce maternal IPV and early intervention services for infants and toddlers exposed to IPV are necessary for optimal maternal and child health.

  11. Growth associated protein (GAP-43): cloning and the development of a sensitive ELISA for neurological disorders.

    PubMed

    Gnanapavan, Sharmilee; Yousaf, Nasim; Heywood, Wendy; Grant, Donna; Mills, Kevin; Chernajovsky, Yuti; Keir, Geoff; Giovannoni, Gavin

    2014-11-15

    GAP-43 has been studied in the rodent and mammalian brain and shown to be present specifically in areas undergoing axonal elongation and synapse formation. GAP-43 was cloned using the baculovirus expression system and purified. A sandwich ELISA was developed using the recombinant GAP-43 as standard and validated. CSF GAP-43 levels were analysed in benign intracranial hypertension, movement disorders, multiple sclerosis, neuropathy, CNS infections, motor neuron disease, and headache (neurological controls). GAP-43 levels were low in all disorders analysed (in particular motor neuron disease; p=0.001, and movement disorders and multiple sclerosis; p<0.0001) compared to controls, aside from CNS infections. GAP-43 is preferentially reduced in the CSF of neurological disorders associated with neurodegeneration.

  12. Neurologic development of the newborn and young child in relation to maternal thyroid function.

    PubMed

    Smit, B J; Kok, J H; Vulsma, T; Briët, J M; Boer, K; Wiersinga, W M

    2000-03-01

    A prospective observational study was performed in pregnant women with known thyroid disease. We studied the effect of maternal thyroid function in the first half of pregnancy on the neurologic development of the infant in the first 2 y of life. Clinical and thyroid function data were collected from 20 pregnant women with known thyroid disease and their newborn children. Infants were divided into three groups according to their maternal thyroid function within the first half of pregnancy: Group A (n = 7): maternal subclinical hypothyroidism, Group B (n = 6): maternal euthyroidism, and Group C (n = 7): maternal hyperthyroidism or subclinical hyperthyroidism. Neurophysiologic, i.e. motor nerve conduction velocity and somatosensory evoked potentials and neurologic and developmental (Bayley scales) assessments were done. One infant, born to a mother with Graves' disease, developed transient hyperthyroidism. At the age of 6 and 12 mo, the mean mental developmental index (MDI) score was 16 points lower for infants in Group A than for those in Group B (p = 0.03 and 0.02, respectively). At the age of 24 mo, the mean MDI score was 6 points lower, which was not statistically significant. Neurophysiologic and neurologic assessments and the mean Psychomotor Developmental scores did not differ among the three groups. In conclusion, maternal subclinical hypothyroidism in the first half of pregnancy was associated with a lower mean MDI score in their infants during the first year of life.

  13. [Psychomotor development and its disorders: between normal and pathological development].

    PubMed

    Vericat, Agustina; Bibiana Orden, Alicia

    2013-10-01

    This article discusses some aspects of psychomotor development and its disorders, with special emphasis on psychomotor retardation. Diagnostic classifications of psychomotor problems, such as DSM-IV and CIE-10, are referred to and their advantages and disadvantages are analyzed. The concept of normality as a synonym for the statistical mean in the context of psychomotor disorders is also analyzed in order to consider its dynamic and variability, thereby avoiding the normality/pathology opposition, while some issues, such as the social and cultural aspects, are highlighted, making it possible to rethink the universality and relativity of psychomotor development. PMID:24061024

  14. [Psychomotor development and its disorders: between normal and pathological development].

    PubMed

    Vericat, Agustina; Bibiana Orden, Alicia

    2013-10-01

    This article discusses some aspects of psychomotor development and its disorders, with special emphasis on psychomotor retardation. Diagnostic classifications of psychomotor problems, such as DSM-IV and CIE-10, are referred to and their advantages and disadvantages are analyzed. The concept of normality as a synonym for the statistical mean in the context of psychomotor disorders is also analyzed in order to consider its dynamic and variability, thereby avoiding the normality/pathology opposition, while some issues, such as the social and cultural aspects, are highlighted, making it possible to rethink the universality and relativity of psychomotor development.

  15. Does aggressive and expectant management of severe preeclampsia affect the neurologic development of the infant?

    PubMed Central

    Ertekin, Arif Aktuğ; Kapudere, Bilge; Eken, Meryem Kurek; İlhan, Gülşah; Dırman, Şükriye; Sargın, Mehmet Akif; Deniz, Engin; Karatekin, Güner; Çöğendez, Ebru; Api, Murat

    2015-01-01

    Objective: To compare and evaluate the influences of expectant and aggressive management of severe preeclampsia on the first year neurologic development of the infants in pregnancies between 27 and 34 weeks of pregnancy. Methods: Seventy women with severe preeclampsia between 27 and 34 weeks of gestation were included in the study. 37 patients were managed aggressively (Group 1) and 33 patients were managed expectantly (Group 2). Glucocorticoids, magnesium sulfate infusion and antihypertensive drugs were administered to each group. After glucocorticoid administration was completed Group 1 was delivered either by cesarean section or vaginal delivery. In Group 2 magnesium sulfate infusion was stopped after glucocorticoid administration was completed. Antihypertensive drugs were given, bed rest and intensive fetal monitorization were continued in this group. Results: The average weeks of gestation, one minute and five minute apgar scores and hospitalization time in intensive care unit were similar in both groups (P > 0.05). Three neonatal complications in Group 2 and five in Group 1 were detected according to the Denver Developmental Screening Test-II and one pathologic case was detected in both groups following neurologic examination. Neonatal mortality was seen in seven patients in Group 1 and one in Group 2. There were no significant differences between groups in terms of neonatal mortality and morbidity and maternal morbidity (P > 0.05). The average latency period was 3.45 ± 5.48 days in Group 2 and none in Group 1. Conclusion: There was no significant difference in the first year neurological development of infants whose mothers underwent either expectant and aggressive management for severe preeclampsia. PMID:26770571

  16. Physical Development: What's Normal? What's Not?

    MedlinePlus

    ... Normal? What’s Not? Page Content Article Body ​Two boys or girls exactly the same age can start or end ... in Girls: What to Expect . Growth in both boys and girls slows considerably soon after puberty is complete. Having ...

  17. Development of a Kinect-based exergaming system for motor rehabilitation in neurological disorders

    NASA Astrophysics Data System (ADS)

    Estepa, A.; Sponton Piriz, S.; Albornoz, E.; Martínez, C.

    2016-04-01

    The development of videogames for physical therapy, known as exergames, has gained much interest in the last years. In this work, a sytem for rehabilitation and clinical evaluation of neurological patients is presented. The Microsoft Kinect device is used to track the full body of patients, and three games were developed to exercise and assess different aspects of balance and gait rehabilitation. The system provides visual feedback by means of an avatar that follows the movements of the patients, and sound and visual stimuli for giving orders during the experience. Also, the system includes a database and management tools for further analysis and monitoring of therapies. The results obtained show, on the one side, a great reception and interest of patients to use the system. On the other side, the specialists considered very useful the data collected and the quantitative analysis provided by the system, which was then adopted for the clinical routine.

  18. Neurological Sequelae of Lupus

    MedlinePlus

    ... Page Synonym(s): Lupus - Neurological Sequelae, Systemic Lupus Erythematosus Table of Contents (click to jump to sections) What ... health problems and have a normal lifespan with periodic doctor visits and treatments with various drugs. What ...

  19. Annotation: Development of Facial Expression Recognition from Childhood to Adolescence--Behavioural and Neurological Perspectives

    ERIC Educational Resources Information Center

    Herba, Catherine; Phillips, Mary

    2004-01-01

    Background: Intact emotion processing is critical for normal emotional development. Recent advances in neuroimaging have facilitated the examination of brain development, and have allowed for the exploration of the relationships between the development of emotion processing abilities, and that of associated neural systems. Methods: A literature…

  20. Impact of maternal diet on human milk composition and neurological development of infants.

    PubMed

    Innis, Sheila M

    2014-03-01

    Maternal nutrition has little or no effect on many nutrients in human milk; for others, human milk may not be designed as a primary nutritional source for the infant; and for a few, maternal nutrition can lead to substantial variations in human milk quality. Human milk fatty acids are among the nutrients that show extreme sensitivity to maternal nutrition and are implicated in neurological development. Extensive development occurs in the infant brain, with growth from ∼ 350 g at birth to 925 g at 1 y, with this growth including extensive dendritic and axonal arborization. Transfer of n-6 (omega-6) and n-3 (omega-3) fatty acids from the maternal diet into human milk occurs with little interconversion of 18:2n-6 to 20:4n-6 or 18:3n-3 to docosahexaenoic acid (DHA) and little evidence of mammary gland regulation to maintain individual fatty acids constant with varying maternal fatty acid nutrition. DHA has gained attention because of its high concentrations and roles in the brain and retina. Studies addressing DHA intakes by lactating women or human milk amounts of DHA at levels above those typical in the United States and Canada on infant outcomes are inconsistent. However, separating effects of the fatty acid supply in gestation or in the weaning diet from effects on neurodevelopment solely due to human milk fatty acids is complex, particularly when neurodevelopment is assessed after the period of exclusive human milk feeding. Information on infant fatty acid intakes, including milk volume consumed and energy density, will aid in understanding of the human milk fatty acids that best support neurological development.

  1. Normalization.

    ERIC Educational Resources Information Center

    Cuevas, Eduardo J.

    1997-01-01

    Discusses cornerstone of Montessori theory, normalization, which asserts that if a child is placed in an optimum prepared environment where inner impulses match external opportunities, the undeviated self emerges, a being totally in harmony with its surroundings. Makes distinctions regarding normalization, normalized, and normality, indicating how…

  2. Mice with mutations of Dock7 have generalized hypopigmentation and white-spotting but show normal neurological function.

    PubMed

    Blasius, Amanda L; Brandl, Katharina; Crozat, Karine; Xia, Yu; Khovananth, Kevin; Krebs, Philippe; Smart, Nora G; Zampolli, Antonella; Ruggeri, Zaverio M; Beutler, Bruce A

    2009-02-24

    The classical recessive coat color mutation misty (m) arose spontaneously on the DBA/J background and causes generalized hypopigmentation and localized white-spotting in mice, with a lack of pigment on the belly, tail tip, and paws. Here we describe moonlight (mnlt), a second hypopigmentation and white-spotting mutation identified on the C57BL/6J background, which yields a phenotypic copy of m/m coat color traits. We demonstrate that the 2 mutations are allelic. m/m and mnlt/mnlt phenotypes both result from mutations that truncate the dedicator of cytokinesis 7 protein (DOCK7), a widely expressed Rho family guanine nucleotide exchange factor. Although Dock7 is transcribed at high levels in the developing brain and has been implicated in both axon development and myelination by in vitro studies, we find no requirement for DOCK7 in neurobehavioral function in vivo. However, DOCK7 has non-redundant role(s) related to the distribution and function of dermal and follicular melanocytes. PMID:19202056

  3. Normal development of the female reproductive system

    EPA Science Inventory

    The embryonic development of the female reproductive system involves a progression of events that is conserved across vertebrate species. The early gonad progresses from a form that is undifferentiated in both genotypic males and females. Rudimentary male (Wolffian) and female (M...

  4. Neurology and neurologic practice in China.

    PubMed

    Shi, Fu-Dong; Jia, Jian-Ping

    2011-11-29

    In the wake of dramatic economic success during the past 2 decades, the specialized field of neurology has undergone a significant transformation in China. With an increase in life expectancy, the problems of aging and cognition have grown. Lifestyle alterations have been associated with an epidemiologic transition both in the incidence and etiology of stroke. These changes, together with an array of social issues and institution of health care reform, are creating challenges for practicing neurologists throughout China. Notable problems include overcrowded, decrepit facilities, overloaded physician schedules, deteriorating physician-patient relationships, and an insufficient infrastructure to accommodate patients who need specialized neurologic care. Conversely, with the creation of large and sophisticated neurology centers in many cities across the country, tremendous opportunities exist. Developments in neurologic subspecialties enable delivery of high-quality care. Clinical and translational research based on large patient populations as well as highly sophisticated technologies are emerging in many neurologic centers and pharmaceutical companies. Child neurology and neurorehabilitation will be fast-developing subdisciplines. Given China's extensive population, the growth and progress of its neurology complex, and its ever-improving quality control, it is reasonable to anticipate that Chinese neurologists will contribute notably to unraveling the pathogenic factors causing neurologic diseases and to providing new therapeutic solutions. PMID:22123780

  5. Neurology and neurologic practice in China.

    PubMed

    Shi, Fu-Dong; Jia, Jian-Ping

    2011-11-29

    In the wake of dramatic economic success during the past 2 decades, the specialized field of neurology has undergone a significant transformation in China. With an increase in life expectancy, the problems of aging and cognition have grown. Lifestyle alterations have been associated with an epidemiologic transition both in the incidence and etiology of stroke. These changes, together with an array of social issues and institution of health care reform, are creating challenges for practicing neurologists throughout China. Notable problems include overcrowded, decrepit facilities, overloaded physician schedules, deteriorating physician-patient relationships, and an insufficient infrastructure to accommodate patients who need specialized neurologic care. Conversely, with the creation of large and sophisticated neurology centers in many cities across the country, tremendous opportunities exist. Developments in neurologic subspecialties enable delivery of high-quality care. Clinical and translational research based on large patient populations as well as highly sophisticated technologies are emerging in many neurologic centers and pharmaceutical companies. Child neurology and neurorehabilitation will be fast-developing subdisciplines. Given China's extensive population, the growth and progress of its neurology complex, and its ever-improving quality control, it is reasonable to anticipate that Chinese neurologists will contribute notably to unraveling the pathogenic factors causing neurologic diseases and to providing new therapeutic solutions.

  6. Early dietary treatments with Lorenzo's oil and docosahexaenoic acid for neurological development in a case with Zellweger syndrome.

    PubMed

    Tanaka, Kyoko; Shimizu, Toshiaki; Ohtsuka, Yoshikazu; Yamashiro, Yuichiro; Oshida, Kyoichi

    2007-10-01

    We treated a girl with Zellweger syndrome using a special infant formula supplemented with middle chain triglyceride (MCT) milk, docosahexaenoic acid (DHA), Lorenzo's oil, and Lunaria oil, which is rich in nervonic acid (C24:1). We examined the fatty acid contents of the plasma and red blood cell (RBC) membrane. Neurological development was evaluated using Denver developmental screening test and auditory brainstem response (ABR). Her delayed neurological development, liver dysfunction, and cholestasis were all improved 2 weeks after starting the dietary treatment. DHA level in RBC membranes was increased and very long chain fatty acid (VLCFA,C26:0) levels were decreased. Our findings suggest that the dietary treatment with combination of MCT milk, DHA, Lorenzo's oil, and Lunaria oil in the patients with Zellweger syndrome bring some benefits for neurological development.

  7. Adolescent brain development in normality and psychopathology

    PubMed Central

    LUCIANA, MONICA

    2014-01-01

    Since this journal’s inception, the field of adolescent brain development has flourished, as researchers have investigated the underpinnings of adolescent risk-taking behaviors. Explanations based on translational models initially attributed such behaviors to executive control deficiencies and poor frontal lobe function. This conclusion was bolstered by evidence that the prefrontal cortex and its interconnections are among the last brain regions to structurally and functionally mature. As substantial heterogeneity of prefrontal function was revealed, applications of neuroeconomic theory to adolescent development led to dual systems models of behavior. Current epidemiological trends, behavioral observations, and functional magnetic resonance imaging based brain activity patterns suggest a quadratic increase in limbically mediated incentive motivation from childhood to adolescence and a decline thereafter. This elevation occurs in the context of immature prefrontal function, so motivational strivings may be difficult to regulate. Theoretical models explain this patterning through brain-based accounts of subcortical–cortical integration, puberty-based models of adolescent sensation seeking, and neurochemical dynamics. Empirically sound tests of these mechanisms, as well as investigations of biology–context interactions, represent the field’s most challenging future goals, so that applications to psychopathology can be refined and so that developmental cascades that incorporate neurobiological variables can be modeled. PMID:24342843

  8. [The child's brain: normal (unaltered) development and development altered by perinatal injury].

    PubMed

    Marín-Padilla, Miguel

    2013-09-01

    In this study we analyse some of the morphological and functional aspects of normal and altered development (the latter due to perinatal injury) in the child's brain. Both normal and altered development are developmental processes that progressively interconnect the different regions. The neuropathological development of subpial and periventricular haemorrhages, as well as that of white matter infarct, are analysed in detail. Any kind of brain damage causes a local lesion with possible remote repercussions. All the components (neurons, fibres, blood capillaries and neuroglias) of the affected region undergo alterations. Those that are destroyed are eliminated by the inflammatory process and those that survive are transformed. The pyramidal neurons with amputated apical dendrites are transformed and become stellate cells, the axonal terminals and those of the radial glial cells are regenerated and the region involved is reinnervated and revascularised with an altered morphology and function (altered local corticogenesis). The specific microvascular system of the grey matter protects its neurons from infarction of the white matter. Although it survives, the grey matter is left disconnected from the afferent and efferent fibres, amputated by the infarct with alterations affecting its morphology and possibly its functioning (altered local corticogenesis). Any local lesion can modify the morphological and functional development of remote regions that are functionally interconnected with it (altered remote corticogenesis). We suggest that any local brain injury can alter the morphology and functioning of the regions that are morphologically and functionally interconnected with it and thus end up affecting the child's neurological and psychological development. These changes can cross different regions of the brain (epileptic auras) and, if they eventually reach the motor region, will give rise to the motor storm that characterises epilepsy.

  9. Chapter 38: American neurology.

    PubMed

    Freemon, Frank R

    2010-01-01

    The great formative event in the history of North America, the Civil War of 1861 to 1865, was the stimulus for the development of clinical neurology and the neurosciences. The first neurological research center on the continent was the US Army hospital at Turner's Lane, Philadelphia, PA. Silas Weir Mitchell and his colleagues described causalgia (reflex sympathetic dystrophy), phantom limb sensation, and Horner's syndrome (before Horner). The medical leader of the Northern army was William Hammond. After the conclusion of hostilities, he began a huge clinical practice in New York City. In the United States, clinical neurology began in private practice, unlike Europe, where neurology began in institutions. Hammond's textbook, which first used the term athetosis, was used by a generation of physicians who encountered patients with neurological signs and symptoms. Early in the 20th century, neurological institutions were formed around universities; probably the most famous was the Montreal Neurological Institute founded by Wilder Penfield. The US federal government sponsored extensive research into the function and dysfunction of the nervous system through the Neurological Institute of Neurological Diseases and Blindness, later called the National Institute of Neurological Diseases and Stroke. The government officially classified the final 10 years of the 20th century as the Decade of the Brain and provided an even greater level of research funding. PMID:19892141

  10. [Sleep and neurological diseases].

    PubMed

    Mayer, G

    2016-06-01

    Knowledge of the physiology of sleep-wake regulation can contribute to an understanding of the pathophysiology and symptoms of neurological diseases and is helpful for initiating specific therapies for sleep-wake cycle stabilization. Based on historically important observations on the close relationship between sleep and neurological diseases, new insights and developments in selected neurological entities are presented in this review article. PMID:27167889

  11. Current neurology

    SciTech Connect

    Appel, S.H. )

    1988-01-01

    The topics covered in this book include: Duchenne muscular dystrophy: DNA diagnosis in practice; Central nervous system magnetic resonance imaging; and Magnetic resonance spectroscopy of neurologic diseases.

  12. Clinical neurologic indices of toxicity in animals.

    PubMed Central

    O'Donoghue, J L

    1996-01-01

    The fundamental structures and functions of the nervous systems of animals and humans are conserved in many ways across species. These similarities provide a basis for developing common neurologic examinations for a number of species of animals and also provide a basis for developing risk assessments across species for neurologic end points. The neurologic examination requires no expensive equipment and can be conducted in the field or wherever impaired animals are identified. The proper conduct of neurologic examinations in animals assumes that the examiner has a fundamental understanding of the normal structure and function of the nervous system as well as knowledge about the spontaneous disease background of the species being studied. PMID:9182039

  13. The crystal structure of human GlnRS provides basis for the development of neurological disorders

    PubMed Central

    Ognjenović, Jana; Wu, Jiang; Matthies, Doreen; Baxa, Ulrich; Subramaniam, Sriram; Ling, Jiqiang; Simonović, Miljan

    2016-01-01

    Cytosolic glutaminyl-tRNA synthetase (GlnRS) is the singular enzyme responsible for translation of glutamine codons. Compound heterozygous mutations in GlnRS cause severe brain disorders by a poorly understood mechanism. Herein, we present crystal structures of the wild type and two pathological mutants of human GlnRS, which reveal, for the first time, the domain organization of the intact enzyme and the structure of the functionally important N-terminal domain (NTD). Pathological mutations mapping in the NTD alter the domain structure, and decrease catalytic activity and stability of GlnRS, whereas missense mutations in the catalytic domain induce misfolding of the enzyme. Our results suggest that the reduced catalytic efficiency and a propensity of GlnRS mutants to misfold trigger the disease development. This report broadens the spectrum of brain pathologies elicited by protein misfolding and provides a paradigm for understanding the role of mutations in aminoacyl-tRNA synthetases in neurological diseases. PMID:26869582

  14. The crystal structure of human GlnRS provides basis for the development of neurological disorders

    DOE PAGES

    Ognjenovic, Jana; Wu, Jiang; Matthies, Doreen; Baxa, Ulrich; Subramaniam, Sriram; Ling, Jiqiang; Simonovic, Miljan

    2016-02-10

    Cytosolic glutaminyl-tRNA synthetase (GlnRS) is the singular enzyme responsible for translation of glutamine codons. Compound heterozygous mutations in GlnRS cause severe brain disorders by a poorly understood mechanism. Herein, we present crystal structures of the wild type and two pathological mutants of human GlnRS, which reveal, for the first time, the domain organization of the intact enzyme and the structure of the functionally important N-terminal domain (NTD). Pathological mutations mapping in the NTD alter the domain structure, and decrease catalytic activity and stability of GlnRS, whereas missense mutations in the catalytic domain induce misfolding of the enzyme. Our results suggestmore » that the reduced catalytic efficiency and a propensity of GlnRS mutants to misfold trigger the disease development. As a result, this report broadens the spectrum of brain pathologies elicited by protein misfolding and provides a paradigm for understanding the role of mutations in aminoacyl-tRNA synthetases in neurological diseases. Keywords« less

  15. "Differently normal" and "normally different": negotiations of female embodiment in women's accounts of 'atypical' sex development.

    PubMed

    Guntram, Lisa

    2013-12-01

    During recent decades numerous feminist scholars have scrutinized the two-sex model and questioned its status in Western societies and medicine. Along the same line, increased attention has been paid to individuals' experiences of atypical sex development, also known as intersex or 'disorders of sex development' (DSD). Yet research on individuals' experiences of finding out about their atypical sex development in adolescence has been scarce. Against this backdrop, the present article analyses 23 in-depth interviews with women who in their teens found out about their atypical sex development. The interviews were conducted during 2009-2012 and the interviewees were all Swedish. Drawing on feminist research on female embodiment and social scientific studies on diagnosis, I examine how the women make sense of their bodies and situations. First, I aim to explore how the women construe normality as they negotiate female embodiment. Second, I aim to investigate how the divergent manners in which these negotiations are expressed can be further understood via the women's different access to a diagnosis. Through a thematic and interpretative analysis, I outline two negotiation strategies: the "differently normal" and the "normally different" strategy. In the former, the women present themselves as just slightly different from 'normal' women. In the latter, they stress that everyone is different in some manner and thereby claim normalcy. The analysis shows that access to diagnosis corresponds to the ways in which the women present themselves as "differently normal" and "normally different", thus shedding light on the complex role of diagnosis in their negotiations of female embodiment. It also reveals that the women make use of what they do have and how alignments with and work on norms interplay as normality is construed. PMID:24331903

  16. Developing Visualization Support System for Teaching/Learning Database Normalization

    ERIC Educational Resources Information Center

    Folorunso, Olusegun; Akinwale, AdioTaofeek

    2010-01-01

    Purpose: In tertiary institution, some students find it hard to learn database design theory, in particular, database normalization. The purpose of this paper is to develop a visualization tool to give students an interactive hands-on experience in database normalization process. Design/methodology/approach: The model-view-controller architecture…

  17. Impact of endocrine-disrupting chemicals on neural development and the onset of neurological disorders.

    PubMed

    Kajta, Małgorzata; Wójtowicz, Anna K

    2013-01-01

    Even though high doses of organic pollutants are toxic, relatively low concentrations have been reported to cause long-term alterations in functioning of individual organisms, populations and even next generations. Among these pollutants are dioxins, polychlorinated biphenyls, pesticides, brominated flame retardants, plasticizers (bisphenol A, nonylphenol, and phthalates) as well as personal care products and drugs. In addition to toxic effects, they are able to interfere with hormone receptors, hormone synthesis or hormone conversion. Because these chemicals alter hormone-dependent processes and disrupt functioning of the endocrine glands, they have been classified as endocrine-disrupting chemicals (EDCs). Because certain EDCs are able to alter neural transmission and the formation of neural networks, the term neural-disrupting chemicals has been introduced, thus implicating EDCs in the etiology of neurological disorders. Recently, public concern has been focused on the effects of EDCs on brain function, concomitantly with an increase in neuropsychiatric disorders, including autism, attention deficit and hyperactivity disorder as well as learning disabilities and aggressiveness. Several lines of evidence suggest that exposure to EDCs is associated with depression and could result in neural degeneration. EDCs act via several classes of receptors with the best documented mechanisms being reported for nuclear steroid and xenobiotic receptors. Low doses of EDCs have been postulated to cause incomplete methylation of specific gene regions in the young brain and to impair neural development and brain functions across generations. Efforts are needed to develop systematic epidemiological studies and to investigate the mechanisms of action of EDCs in order to fully understand their effects on wildlife and humans.

  18. Achondrogenesis type II with normally developed extremities: a case report.

    PubMed

    Kocakoc, Ercan; Kiris, Adem

    2002-07-01

    We present a case of achondrogenesis type II with normally developed extremities that was confirmed with postmortem ultrasonographic and radiographic examination. The length of the long bones may vary and the diagnosis of achondrogenesis should not be ruled out with normally developed extremities. Intrauterine sonographic examination of the vertebrae is very important and the absence of vertebral body ossification may be the unique finding of achondrogenesis type II. Axial ultrasonographic images and postmortem plain radiographs are useful to clarify the pathology. PMID:12124695

  19. Hypoxic Adaptation during Development: Relation to Pattern of Neurological Presentation and Cognitive Disability

    ERIC Educational Resources Information Center

    Kirkham, Fenella J.; Datta, Avijit K.

    2006-01-01

    Children with acute hypoxic-ischaemic events (e.g. stroke) and chronic neurological conditions associated with hypoxia frequently present to paediatric neurologists. Failure to adapt to hypoxia may be a common pathophysiological pathway linking a number of other conditions of childhood with cognitive deficit. There is evidence that congenital…

  20. The discovery of human auditory-motor entrainment and its role in the development of neurologic music therapy.

    PubMed

    Thaut, Michael H

    2015-01-01

    The discovery of rhythmic auditory-motor entrainment in clinical populations was a historical breakthrough in demonstrating for the first time a neurological mechanism linking music to retraining brain and behavioral functions. Early pilot studies from this research center were followed up by a systematic line of research studying rhythmic auditory stimulation on motor therapies for stroke, Parkinson's disease, traumatic brain injury, cerebral palsy, and other movement disorders. The comprehensive effects on improving multiple aspects of motor control established the first neuroscience-based clinical method in music, which became the bedrock for the later development of neurologic music therapy. The discovery of entrainment fundamentally shifted and extended the view of the therapeutic properties of music from a psychosocially dominated view to a view using the structural elements of music to retrain motor control, speech and language function, and cognitive functions such as attention and memory.

  1. The discovery of human auditory-motor entrainment and its role in the development of neurologic music therapy.

    PubMed

    Thaut, Michael H

    2015-01-01

    The discovery of rhythmic auditory-motor entrainment in clinical populations was a historical breakthrough in demonstrating for the first time a neurological mechanism linking music to retraining brain and behavioral functions. Early pilot studies from this research center were followed up by a systematic line of research studying rhythmic auditory stimulation on motor therapies for stroke, Parkinson's disease, traumatic brain injury, cerebral palsy, and other movement disorders. The comprehensive effects on improving multiple aspects of motor control established the first neuroscience-based clinical method in music, which became the bedrock for the later development of neurologic music therapy. The discovery of entrainment fundamentally shifted and extended the view of the therapeutic properties of music from a psychosocially dominated view to a view using the structural elements of music to retrain motor control, speech and language function, and cognitive functions such as attention and memory. PMID:25725919

  2. Adult neurology training during child neurology residency.

    PubMed

    Schor, Nina F

    2012-08-21

    As it is currently configured, completion of child neurology residency requires performance of 12 months of training in adult neurology. Exploration of whether or not this duration of training in adult neurology is appropriate for what child neurology is today must take into account the initial reasons for this requirement and the goals of adult neurology training during child neurology residency.

  3. Childhood Depression Viewed as Normal Development Gone Awry.

    ERIC Educational Resources Information Center

    Wenar, Charles

    Childhood psychopathology can be viewed as normal development gone awry. The key to the mysteries of masked depression and of depression in the infant/toddler period and in middle childhood lies in the concept of loss. Children who experience the loss of a loved parent or caretaker through that person's death may evidence a variety of behaviors…

  4. The Stigma Scale for Chronic Illnesses 8-item version (SSCI-8): Development, validation, and use across neurological conditions

    PubMed Central

    Molina, Yamile; Choi, Seung W.; Cella, David; Rao, Deepa

    2013-01-01

    Purpose Although the impact of stigma has been highlighted for epileptic populations, the experiences of people living with other neurological conditions have been less studied. In order to promote research on stigma among people with neurological conditions, we sought to develop and psychometrically validate an 8-item questionnaire measuring internalised and enacted stigma experienced by people with epilepsy, multiple sclerosis (MS), Parkinson’s disease (PD), stroke, and amyotrophic lateral sclerosis (ALS). Methods We used Item Response Theory (IRT) methodologies to select items and field-tested our items with 587 participants from 8 academic medical centres across the United States. Results We conducted Exploratory and Confirmatory Factor Analysis as well as examined scale the reliability and validity. In addition, we conducted an analysis of variance (ANOVA) test to examine mean total score differences across the 5 neurological conditions. Data from people across conditions revealed that the shortened instrument conformed to an essentially unidimensional model of multi-faceted stigma as a 1-factor questionnaire with correlated residuals on a pair of items that distinctly measured internalised stigma. Conclusions Preliminary evidence suggests that the Stigma Scale for Chronic Illness 8-item version (SSCI-8) fits a unidimensional model, which assesses enacted and internalised stigma, and has adequate internal consistency/reliability and validity in relation to psychological distress and patient performance. Our results suggest fairly low stigma for neurological populations. In addition, our results suggest that stigma may be more severe for patients with ALS relative to those with MS and PD. Our results suggest that the scale could be used practically in the clinic setting to examine stigma without the patient burden associated with lengthier scales. PMID:22639392

  5. Development of suspended normal-metal-type tunneling junction refrigerator

    NASA Astrophysics Data System (ADS)

    Kashiwaya, Satoshi; Kashiwaya, Hiromi; Koyanagi, Masao; Tanaka, Yukio

    2016-09-01

    We have developed a suspended normal-metal-type superconducting-normal metal-superconductor tunneling junction refrigerator for the cooling of highly sensitive sensors operating at ultralow temperatures. The performance of the refrigerator is evaluated by comparing the experimental conductance with the numerical results of a theoretical formulation. The lowest temperature of 0.093 K at a bath temperature of 0.334 K indicates the successful operation of the refrigerator. The maximum cooling power of the present refrigerator estimated on the basis of the nonequilibrium stationary state model is 213 pW for a junction area of 40 × 7 µm2.

  6. Normal and abnormal spine and thoracic cage development

    PubMed Central

    Canavese, Federico; Dimeglio, Alain

    2013-01-01

    Development of the spine and thoracic cage consists of a complex series of events involving multiple metabolic processes, genes and signaling pathways. During growth, complex phenomena occur in rapid succession. This succession of events, this establishment of elements, is programmed according to a hierarchy. These events are well synchronized to maintain harmonious limb, spine and thoracic cage relationships, as growth in the various body segments does not occur simultaneously at the same magnitude or rate. In most severe cases of untreated progressive early-onset spinal deformities, respiratory insufficiency and pulmonary and cardiac hypertension (cor pulmonale), which characterize thoracic insufficiency syndrome (TIS), can develop, sometimes leading to death. TIS is the inability of the thorax to ensure normal breathing. This clinical condition can be linked to costo-vertebral malformations (e.g., fused ribs, hemivertebrae, congenital bars), neuromuscular diseases (e.g., expiratory congenital hypotonia), Jeune or Jarcho-Levin syndromes or to 50% to 75% fusion of the thoracic spine before seven years of age. Complex spinal deformities alter normal growth plate development, and vertebral bodies become progressively distorted, perpetuating the disorder. Therefore, many scoliotic deformities can become growth plate disorders over time. This review aims to provide a comprehensive review of how spinal deformities can affect normal spine and thoracic cage growth. Previous conceptualizations are integrated with more recent scientific data to provide a better understanding of both normal and abnormal spine and thoracic cage growth. PMID:24147251

  7. Occupational neurology.

    PubMed

    Feldman, R G

    1987-01-01

    The nervous system is vulnerable to the effects of certain chemicals and physical conditions found in the work environment. The activities of an occupational neurologist focus on the evaluation of patients with neurological disorders caused by occupational or environmental conditions. When one is making a differential diagnosis in patients with neurological disorders, the possibility of toxic exposure or encounters with physical factors in the workplace must not be overlooked. Central to an accurate clinical diagnosis is the patient's history. A diagnosis of an occupational or environmental neurological problem requires a careful assessment of the clinical abnormalities and confirmation of these disabilities by objective tests such as nerve conduction velocity, evoked potentials, electroencephalogram, neuropsychological batteries, or nerve biopsy. On the basis of information about hazards in the workplace, safety standards and environmental and biological monitoring can be implemented in the workplace to reduce the risks of undue injury. Clinical manifestations of headache, memory disturbance, and peripheral neuropathy are commonly encountered presentations of the effects of occupational hazards. Physicians in everyday clinical practice must be aware of the signs and symptoms associated with exposure to possible neurotoxins and work methods. Occupational and environmental circumstances must be explored when evaluating patients with neurologic disorders.

  8. Histone turnover and chromatin accessibility: Critical mediators of neurological development, plasticity, and disease

    PubMed Central

    Wenderski, Wendy; Maze, Ian

    2016-01-01

    In postmitotic neurons, nucleosomal turnover was long considered to be a static process that is inconsequential to transcription. However, our recent studies in human and rodent brain indicate that replication-independent (RI) nucleosomal turnover, which requires the histone variant H3.3, is dynamic throughout life and is necessary for activity-dependent gene expression, synaptic connectivity, and cognition. H3.3 turnover also facilitates cellular lineage specification and plays a role in suppressing the expression of heterochromatic repetitive elements, including mutagenic transposable sequences, in mouse embryonic stem cells. In this essay, we review mechanisms and functions for RI nucleosomal turnover in brain and present the hypothesis that defects in histone dynamics may represent a common mechanism underlying neurological aging and disease. PMID:26990528

  9. Neurological complication of dengue infection.

    PubMed

    Murthy, J M K

    2010-01-01

    Dengue infection is endemic in more than 100 countries, mostly in the developing world. Recent observations indicate that the clinical profile of dengue is changing, and that neurological manifestations are being reported more frequently. The exact incidence of various neurological complications is uncertain. The pathogenesis of neurological manifestations is multiple and includes: neurotrophic effect of the dengue virus, related to the systemic effects of dengue infection, and immune mediated. In countries endemic to dengue, it will be prudent to investigate for dengue infection in patients with fever and acute neurological manifestations. There is need for understanding of the pathogenesis of various neurological manifestations.

  10. Vitronectin is not essential for normal mammalian development and fertility.

    PubMed Central

    Zheng, X; Saunders, T L; Camper, S A; Samuelson, L C; Ginsburg, D

    1995-01-01

    Vitronectin (VN) is an abundant glycoprotein present in plasma and the extracellular matrix of most tissues. Though the precise function of VN in vivo is unknown, it has been implicated as a participant in diverse biological processes, including cell attachment and spreading, complement activation, and regulation of hemostasis. The major site of synthesis appears to be the liver, though VN is also found in the brain at an early stage of mouse organogenesis, suggesting that it may play an important role in mouse development. Genetic deficiency of VN has not been reported in humans or in other higher organisms. To examine the biologic function of VN within the context of the intact animal, we have established a murine model for VN deficiency through targeted disruption of the murine VN gene. Southern blot analysis of DNA obtained from homozygous null mice demonstrates deletion of all VN coding sequences, and immunological analysis confirms the complete absence of VN protein expression in plasma. However, heterozygous mice carrying one normal and one null VN allele and homozygous null mice completely deficient in VN demonstrate normal development, fertility, and survival. Sera obtained from VN-deficient mice are completely deficient in "serum spreading factor" and plasminogen activator inhibitor 1 binding activities. These observations demonstrate that VN is not essential for cell adhesion and migration during normal mouse development and suggest that its role in these processes may partially overlap with other adhesive matrix components. Images Fig. 1 Fig. 2 Fig. 3 PMID:8618914

  11. [Neurological rehabilitation].

    PubMed

    Hömberg, V

    2010-10-01

    This article describes state of the art concepts of neurological rehabilitation in Germany. In parallel to enormous growth of knowledge in the neurosciences also neurological rehabilitation has made significant progress. The increasing use of concepts of evidence based medicine and an early translation of knowledge from the neurosciences into clinical rehabilitation practice contribute to therapeutic advances. It is now widely accepted, that rehabilitation should start early and should be organized in a multidisciplinary professional team. Therapeutic procedures selected should be evidence based and have to be modified to find custom tailored solutions for individual patients. General rules derived from neuroscientific knowledge have been shown to be useful to design new therapeutic techniques. Neuromodulatory stimulation and special pharmacological treatments provide further options for enhancing results of rehabilitation.

  12. Neurology in Asia.

    PubMed

    Tan, Chong-Tin

    2015-02-10

    Asia is important as it accounts for more than half of the world population. The majority of Asian countries fall into the middle income category. As for cultural traditions, Asia is highly varied, with many languages spoken. The pattern of neurologic diseases in Asia is largely similar to the West, with some disease features being specific to Asia. Whereas Asia constitutes 60% of the world's population, it contains only 20% of the world's neurologists. This disparity is particularly evident in South and South East Asia. As for neurologic care, it is highly variable depending on whether it is an urban or rural setting, the level of economic development, and the system of health care financing. To help remedy the shortage of neurologists, most counties with larger populations have established training programs in neurology. These programs are diverse, with many areas of concern. There are regional organizations serving as a vehicle for networking in neurology and various subspecialties, as well as an official journal (Neurology Asia). The Asian Epilepsy Academy, with its emphasis on workshops in various locations, EEG certification examination, and fellowships, may provide a template of effective regional networking for improving neurology care in the region.

  13. [Correspondence between Winkler and Von Monakow. 2. Developments in psychiatry and neurology (1900-1930)].

    PubMed

    Koehler, P J; Jagella, C

    2001-12-22

    In this second article Winkler's and Von Monakow's views on the evolution of psychiatry and neurology (1900-1930) are described. They discussed a number of scientific subjects in their correspondence, such as the localisation of functions in the central nervous system and in particular Von Monakow's concept of 'diaschisis'. This concept explained the phenomenon that symptoms during the acute phase of central nervous system disorders are more extensive and of a different nature than those during the chronic phase. The controversy between the neuron theory and the reticular theory was also discussed in the correspondence. One of the reasons for the correspondence and meetings was the plan to compile an atlas of the brain although due to the outbreak of World War I this did not come to fruition. Finally there was an extensive correspondence concerning the psychoanalytic movement within the psychiatry, of which Winkler and Von Monakow, as biologically orientated neuropsychiatrists, were highly critical. German language neuropsychiatry has had a considerable influence in the Netherlands and this is in part due to the relationship Winkler had with German neuropsychiatrists and Von Monakow in particular.

  14. Ire1 supports normal ER differentiation in developing Drosophila photoreceptors

    PubMed Central

    Xu, Zuyuan; Chikka, Madhusudana Rao; Xia, Hongai; Ready, Donald F.

    2016-01-01

    ABSTRACT The endoplasmic reticulum (ER) serves virtually all aspects of cell physiology and, by pathways that are incompletely understood, is dynamically remodeled to meet changing cell needs. Inositol-requiring enzyme 1 (Ire1), a conserved core protein of the unfolded protein response (UPR), participates in ER remodeling and is particularly required during the differentiation of cells devoted to intense secretory activity, so-called ‘professional’ secretory cells. Here, we characterize the role of Ire1 in ER differentiation in the developing Drosophila compound eye photoreceptors (R cells). As part of normal development, R cells take a turn as professional secretory cells with a massive secretory effort that builds the photosensitive membrane organelle, the rhabdomere. We find rough ER sheets proliferate as rhabdomere biogenesis culminates, and Ire1 is required for normal ER differentiation. Ire1 is active early in R cell development and is required in anticipation of peak biosynthesis. Without Ire1, the amount of rough ER sheets is strongly reduced and the extensive cortical ER network at the rhabdomere base, the subrhabdomere cisterna (SRC), fails. Instead, ER proliferates in persistent and ribosome-poor tubular tangles. A phase of Ire1 activity early in R cell development thus shapes dynamic ER. PMID:26787744

  15. Are there more bowel symptoms in children with autism compared to normal children and children with other developmental and neurological disorders?: A case control study.

    PubMed

    Smith, R A; Farnworth, H; Wright, B; Allgar, V

    2009-07-01

    There is considerable controversy as to whether there is an association between bowel disorders and autism. Using a bowel symptom questionnaire we compared 51 children with autism spectrum disorder with control groups of 35 children from special school and 112 from mainstream school. There was a significant difference in the reporting of certain bowel symptoms (constipation, diarrhoea, flatulence) and food faddiness between the autism group and the mainstream school control group. There was no significant difference between the autism group and children in the special schools except for faddiness, which is an autism specific symptom and not a bowel symptom. This study confirms previously reported findings of an increase in bowel symptoms in children with autism. It would appear, however, that this is not specifically associated with autism as bowel symptoms were reported in similar frequency to a comparison group of children with other developmental and neurological disorders.

  16. X Chromosome Abnormalities and Cognitive Development: Implications for Understanding Normal Human Development.

    ERIC Educational Resources Information Center

    Walzer, Stanley

    1985-01-01

    Argues that knowledge from studies of individuals with sex chromosome abnormalities can further understanding of aspects of normal human development. Studies of XO girls, XXY boys, XXX girls, and males with a fragile X chromosome are summarized to demonstrate how results contribute to knowledge about normal cognitive development and about…

  17. Associations Between Prolonged Intubation and Developing Post-extubation Dysphagia and Aspiration Pneumonia in Non-neurologic Critically Ill Patients

    PubMed Central

    Kim, Min Jung; Park, Young Sook; Song, You Hong

    2015-01-01

    Objective To identify the associations between the duration of endotracheal intubation and developing post-extubational supraglottic and infraglottic aspiration (PEA) and subsequent aspiration pneumonia. Methods This was a retrospective observational study from January 2009 to November 2014 of all adult patients who had non-neurologic critical illness, required endotracheal intubation and were referred for videofluoroscopic swallowing study. Demographic information, intensive care unit (ICU) admission diagnosis, severity of critical illness, duration of endotracheal intubation, length of stay in ICU, presence of PEA and severity of dysphagia were reviewed. Results Seventy-four patients were enrolled and their PEA frequency was 59%. Patients with PEA had significantly longer endotracheal intubation durations than did those without (median [interquartile range]: 15 [9-21] vs. 10 [6-15] days; p=0.02). In multivariate logistic regression analysis, the endotracheal intubation duration was significantly associated with PEA (odds ratio, 1.09; 95% confidence interval [CI], 1.01-1.18; p=0.04). Spearman correlation analysis of intubation duration and dysphagia severity showed a positive linear association (r=0.282, p=0.02). The areas under the receiver operating characteristic curves (AUCs) of endotracheal intubation duration for developing PEA and aspiration pneumonia were 0.665 (95% CI, 0.542-0.788; p=0.02) and 0.727 (95% CI, 0.614-0.840; p=0.001), respectively. Conclusion In non-neurologic critically ill patients, the duration of endotracheal intubation was independently associated with PEA development. Additionally, the duration was positively correlated with dysphagia severity and may be helpful for identifying patients who require a swallowing evaluation after extubation. PMID:26605174

  18. [Neurological sleep disorders].

    PubMed

    Khatami, Ramin

    2014-11-01

    Neurological sleep disorders are common in the general population and may have a strong impact on quality of life. General practitioners play a key role in recognizing and managing sleep disorders in the general population. They should therefore be familiar with the most important neurological sleep disorders. This review provides a comprehensive overview of the most prevalent and important neurological sleep disorders, including Restless legs syndrome (with and without periodic limb movements in sleep), narcolepsy, NREM- and REM-sleep parasomnias and the complex relationship between sleep and epilepsies. Although narcolepsy is considered as a rare disease, recent discoveries in narcolepsy research provided insight in the function of brain circuitries involved in sleep wake regulation. REM sleep behavioral parasomnia (RBD) is increasingly recognized to represent an early manifestation of neurodegenerative disorders, in particular evolving synucleinopathies. Early diagnosis may thus open new perspectives for developing novel treatment options by targeting neuroprotective substances.

  19. Genetic Analysis in Neurology

    PubMed Central

    Pittman, Alan; Hardy, John

    2014-01-01

    In recent years, neurogenetics research had made some remarkable advances owing to the advent of genotyping arrays and next-generation sequencing. These improvements to the technology have allowed us to determine the whole-genome structure and its variation and to examine its effect on phenotype in an unprecedented manner. The identification of rare disease-causing mutations has led to the identification of new biochemical pathways and has facilitated a greater understanding of the etiology of many neurological diseases. Furthermore, genome-wide association studies have provided information on how common genetic variability impacts on the risk for the development of various complex neurological diseases. Herein, we review how these technological advances have changed the approaches being used to study the genetic basis of neurological disease and how the research findings will be translated into clinical utility. PMID:23571731

  20. Paraneoplastic neurological syndromes

    PubMed Central

    Leypoldt, F; Wandinger, K-P

    2014-01-01

    Paraneoplastic neurological syndromes are immune-mediated erroneous attacks on the central or peripheral nervous systems, or both, directed originally against the tumour itself. They have been known for more than 40 years, but recently the discovery of new subgroups of paraneoplastic encephalitis syndromes with a remarkably good response to immune therapy has ignited new clinical and scientific interest. Knowledge of these subgroups and their associated autoantibodies is important in therapeutic decision-making. However, the abundance of new autoantibodies and syndromes can be confusing. This review paper summarizes current knowledge and new developments in the field of paraneoplastic neurological syndromes, their classification, pathophysiology and treatment. PMID:23937626

  1. [Development, problems and results of specialty-specific genetic counseling at the Neurology Clinic of the Karl Marx University].

    PubMed

    Bachmann, H

    1987-11-01

    Genetic counselling for inherited neurological diseases has been established at the Clinic for Neurology of Karl Marx University. Comprehensive experiences have been got with the specific and sometimes markedly different problems and aims of counselling in Wilsons disease, X-linked recessive muscular dystrophies, myotonic dystrophy and other neuromuscular disorders, Huntingtons chorea and hereditary ataxias.

  2. Design and development of "biomechatronic gym" for early detection of neurological disorders in infants.

    PubMed

    Cecchi, F; Serio, S M; Del Maestro, M; Laschi, C; Sgandurra, G; Cioni, G; Dario, P

    2010-01-01

    The study and measurement of grasping actions and forces in humans is important in a variety of contexts. In infants, it can give insights on the typical and atypical motor development, while it poses functional and operative requirements that are not fully matched by current sensing technology. Novel approaches for measuring infants' grasping actions are based on sensorized platform usable in natural settings. A new set of instrumented toys has been designed for the assessment/stimulation of upper limbs of infants between 4 and 9 months. A purposive biomechatronic gym has been developed by integrating pressure and force sensors and visual/auditory stimulations to the usual gym structure and hanging toys (cow, flower and ring puppets), so that the infants' actions on the gym can be monitored, measured and stimulated. With the developed system, a longitudinal clinical validation has been carried out with seven healthy infants. From data analysis it is possible to identify a trend in manual forces development and this result confirms the usefulness of the system proposed as a clinical tool for monitoring infants' grasping development.

  3. Human Embryonic Stem Cells: A Model for the Study of Neural Development and Neurological Diseases

    PubMed Central

    Prajumwongs, Piya; Weeranantanapan, Oratai; Jaroonwitchawan, Thiranut; Noisa, Parinya

    2016-01-01

    Although the mechanism of neurogenesis has been well documented in other organisms, there might be fundamental differences between human and those species referring to species-specific context. Based on principles learned from other systems, it is found that the signaling pathways required for neural induction and specification of human embryonic stem cells (hESCs) recapitulated those in the early embryo development in vivo at certain degree. This underscores the usefulness of hESCs in understanding early human neural development and reinforces the need to integrate the principles of developmental biology and hESC biology for an efficient neural differentiation. PMID:27239201

  4. NEUROLOGICAL RESEARCH RELEVANT TO READING--1967.

    ERIC Educational Resources Information Center

    ISOM, JOHN B.

    ASPECTS OF NEUROLOGICAL RESEARCH ARE PRESENTED UNDER THE TOPICS OF NEUROLOGICAL GROWTH AND DEVELOPMENT, CEREBRAL DOMINANCE, "SPLIT-BRAIN" SYNDROME, AND SEQUENCING. THE FIRST TWO AREAS INDICATE THAT ASSESSMENT OF A CHILD'S NEUROLOGICAL DEVELOPMENT MUST TAKE INTO ACCOUNT VARIATION OF RATE AND DEGREE OF DEVELOPMENT, AND THAT THE SIGNIFICANCE OF…

  5. Improving Instruction in Middle Level Schools: Implications of Neurological Data for Curriculum Development.

    ERIC Educational Resources Information Center

    Toepfer, Conrad F., Jr.

    Implications of biophysicist Herman Epstein's research on brain growth periodization, which develop a neurobiological consideration of learning issues for middle school curricula, have created controversy among educators and stimulus for further research. This presentation (1) summarizes the writer's interpretations of these data and implications…

  6. The Influence of Lead Exposure and Toxicity to Children's Neurological Development and School Performance.

    ERIC Educational Resources Information Center

    Kimball, Sarah L.

    This report discusses the effects of lead exposure and toxicity on children's cognitive development and school performance and addresses the role of schools in prevention of lead poisoning. Sources of lead exposure include mining, smelting and refining activities, lead paint, leaded gasoline, and industrial emissions. The results of lead poisoning…

  7. Development of Communicative Gestures in Normally Developing Children between 8 and 18 Months: An Exploratory Study

    ERIC Educational Resources Information Center

    Veena, Kadiyali D; Bellur, Rajashekhar

    2015-01-01

    Children who have not developed speech tend to use gestures to communicate. Since gestures are not encouraged and suppressed in the Indian traditional context while speaking, this study focused on profiling the developing gestures in children to explore whether they use the gestures before development of speech. Eight normally developing…

  8. From planes to brains: parallels between military development of virtual reality environments and virtual neurological surgery.

    PubMed

    Schmitt, Paul J; Agarwal, Nitin; Prestigiacomo, Charles J

    2012-01-01

    Military explorations of the practical role of simulators have served as a driving force for much of the virtual reality technology that we have today. The evolution of 3-dimensional and virtual environments from the early flight simulators used during World War II to the sophisticated training simulators in the modern military followed a path that virtual surgical and neurosurgical devices have already begun to parallel. By understanding the evolution of military simulators as well as comparing and contrasting that evolution with current and future surgical simulators, it may be possible to expedite the development of appropriate devices and establish their validity as effective training tools. As such, this article presents a historical perspective examining the progression of neurosurgical simulators, the establishment of effective and appropriate curricula for using them, and the contributions that the military has made during the ongoing maturation of this exciting treatment and training modality.

  9. The Complexity Signature: Developing a Tool to Communicate Biopsychosocial Severity of Disease for Children with Chronic Neurological Complexity.

    PubMed

    Krieg, Sandro M; Sonanini, Sebastian; Sollmann, Nico; Focke, Axel; Gerstl, Lucia; Heinen, Florian

    2016-08-01

    Aim For children with medical complexity, interdisciplinary treatment approaches are required to address the various aspects defined within the biopsychosocial model. Methods The present study identifies dimensions of the biopsychosocial model to generate a standardized visualized severity score for chronic neurological diseases in children. We demonstrate the score's applicability and usefulness in clinical practice among clinicians with and without pediatric board certification with the aid of illustrative patient cases. The results are compared by Spearman correlation coefficient. Results Nine dimensions were identified as the basis for the development of the score, which consists of five grades of severity for each of the selected neuropediatric subsections. All board-certified pediatricians would recommend the application of the severity score in clinical routine. Furthermore, a good correlation was revealed between direct and indirect (severity score) assessment. Interpretation The severity score developed in this study takes into account biopsychosocial aspects of chronic diseases while being comprehensible and easily applicable in clinical routine-a biopsychosocial signature serving as an excellent, striking communication basis within the interdisciplinary team. However, upcoming studies including more patient cases are needed for further refinement. PMID:27228000

  10. Development of dopamine receptor radiopharmaceuticals for the study of neurological and psychiatric disorders

    SciTech Connect

    Dr. Jogeshwar Mukherjee

    2009-01-02

    Our goals in this grant application are directed towards the development of radiotracers that may allow the study of the high-affinity state (functional state) of the dopamine receptors. There have been numerous reports on the presence of two inter-convertible states of these (G-protein coupled) receptors in vitro. However, there is no report that establishes the presence of these separate affinity states in vivo. We have made efforts in this direction in order to provide such direct in vivo evidence about the presence of the high affinity state. This understanding of the functional state of the receptors is of critical significance in our overall diagnosis and treatment of diseases that implicate the G-protein coupled receptors. Four specific aims have been listed in the grant application: (1). Design and syntheses of agonists (2). Radiosyntheses of agonists (3). In vitro pharmacology of agonists (4). In vivo distribution and pharmacology of labeled derivatives. We have accomplished the syntheses and radiosyntheses of three agonist radiotracers labeled with carbon-11. In vitro and in vivo pharmacological experiments have been accomplished in rats and preliminary PET studies in non-human primates have been carried out. Various accomplishments during the funded years, briefly outlined in this document, have been disseminated by several publications in various journals and presentations in national and international meetings (Society of Nuclear Medicine, Society for Neuroscience and International Symposium on Radiopharmaceutical Chemistry).

  11. Welcome to Neurology: Genetics.

    PubMed

    Pulst, Stefan M

    2015-06-01

    The powers of human genetics and genetic technologies have transformed the complexities of neurology and neuroscience at the basic, translational, and now also the clinical level. We have left an era of black and white views of causative genetic variation and are entering a period of more than 50 shades of grey, fascinated with DNA variants that increase or decrease risk, epigenetic modification, and an unexpectedly large number of variants of unknown or potentially pathogenic significance. Loss-of-function alleles and even complete human gene knockouts for certain genes appear to be compatible with a normal phenotype. PMID:27066541

  12. Neurological complications of infantile osteopetrosis.

    PubMed

    Lehman, R A; Reeves, J D; Wilson, W B; Wesenberg, R L

    1977-11-01

    Seven cases of infantile osteopetrosis are presented. Five of these were available for detailed clinical examination and 2 for retrospective review, including autopsy slides. Neurological deficits in these patients are reviewed. Involvement of the central nervous system parenchyma was suggested by observations of delayed development, ocular abnormalities, and reflex changes as well as radiographic and autopsy findings. Cerebral atrophy was present in several of our patients as well as some reported in the literature and may account for the ventricular enlargement found in many of these patients. Though hydrocephalus may be present, it is unclear that this is frequent or that it can occur without antecedent intracranial hemorrhage. The large head size is not accounted for by calvarial thickening or by hydrocephalus. Despite our patients' small stature, pituitary function appeared to be normal. Surgical decompression may stabilize cranial nerve function, particularly when the optic nerves are involved. PMID:617576

  13. Performance of non-neurological older adults on the Wisconsin Card Sorting Test and the Stroop Color-Word Test: normal variability or cognitive impairment?

    PubMed

    Gunner, Jessica H; Miele, Andrea S; Lynch, Julie K; McCaffrey, Robert J

    2012-06-01

    There is currently no standard criterion for determining abnormal test scores in neuropsychology; thus, a number of different criteria are commonly used. We investigated base rates of abnormal scores in healthy older adults using raw and T-scores from indices of the Wisconsin Card Sorting Test and Stroop Color-Word Test. Abnormal scores were examined cumulatively at seven cutoffs including >1.0, >1.5, >2.0, >2.5, and >3.0 standard deviations (SD) from the mean as well as those below the 10th and 5th percentiles. In addition, the number of abnormal scores at each of the seven cutoffs was also examined. Results showed when considering raw scores, ∼15% of individuals obtained scores>1.0 SD from the mean, around 10% were less than the 10th percentile, and 5% fell >1.5 SD or <5th percentile from the mean. Using T-scores, approximately 15%-20% and 5%-10% of scores were >1.0 and >1.5 SD from the mean, respectively. Roughly 15% and 5% fell at the <10th and <5th percentiles, respectively. Both raw and T-scores>2.0 SD from the mean were infrequent. Although the presence of a single abnormal score at 1.0 and 1.5 SD from the mean or at the 10th and 5th percentiles was not unusual, the presence of ≥2 abnormal scores using any criteria was uncommon. Consideration of base rate data regarding the percentage of healthy individuals scoring in the abnormal range should help avoid classifying normal variability as neuropsychological impairment.

  14. [Vitamin D and neurology].

    PubMed

    Thouvenot, Éric; Camu, William

    2013-10-01

    Vitamin D deficiency is associated with a higher risk of multiple sclerosis and also with a higher relapse rate as well as a higher number of MRI lesions. Elders with vitamin D deficiency have worse cognitive performance. Vitamin D deficiency is a risk factor for developing Alzheimer's disease. Ischemic stroke are more frequent and more severe in patients with low vitamin D levels. Carotid atherosclerosis is more frequent and more severe in patients with vitamin D deficiency. Vitamin D deficiency is associated with a higher risk and worse prognosis of Parkinson's disease. In the different neurological disorders discussed herein, gene polymorphisms that could alter vitamin D metabolism are also associated with a higher incidence or a worse disease prognosis. Despite the links between vitamin D deficiency and the risks of developing neurological disorders, there is, to date, no proof that supplementation could alter the course of these diseases.

  15. Oligopeptidase A is required for normal phage P22 development.

    PubMed Central

    Conlin, C A; Vimr, E R; Miller, C G

    1992-01-01

    The opdA gene of Salmonella typhimurium encodes an endoprotease, oligopeptidase A (OpdA). Strains carrying opdA mutations were deficient as hosts for phage P22. P22 and the closely related phages L and A3 formed tiny plaques on an opdA host. Salmonella phages 9NA, KB1, and ES18.h1 were not affected by opdA mutations. Although opdA strains displayed normal doubling times and were infected by P22 as efficiently as opdA+ strains, the burst size of infectious particles from an opdA host was less than 1/10 of that from an opdA+ host. This decrease resulted from a reduced efficiency of plating of particles from an opdA infection. In the absence of a functional opdA gene, most of the P22 particles are defective. To identify the target of OpdA action, P22 mutants which formed plaques larger than wild-type plaques on an opdA mutant lawn were isolated. Marker rescue experiments using cloned fragments of P22 DNA localized these mutations to a 1-kb fragment. The nucleotide sequence of this fragment and a contiguous region (including all of both P22 gene 7 and gene 14) was determined. The mutations leading to opdA independence affected the region of gene 7 coding for the amino terminus of gp7, a protein required for DNA injection by the phage. Comparison of the nucleotide sequence with the N-terminal amino acid sequence of gp7 suggested that a 20-amino-acid peptide is removed from gp7 during phage development. Further experiments showed that this processing was opdA dependent and rapid (half-life, less than 2 min) and occurred in the absence of other phage proteins. The opdA-independent mutations lead to mutant forms of gp7 which function without processing. Images PMID:1522065

  16. Somatic mutation, genomic variation, and neurological disease.

    PubMed

    Poduri, Annapurna; Evrony, Gilad D; Cai, Xuyu; Walsh, Christopher A

    2013-07-01

    Genetic mutations causing human disease are conventionally thought to be inherited through the germ line from one's parents and present in all somatic (body) cells, except for most cancer mutations, which arise somatically. Increasingly, somatic mutations are being identified in diseases other than cancer, including neurodevelopmental diseases. Somatic mutations can arise during the course of prenatal brain development and cause neurological disease-even when present at low levels of mosaicism, for example-resulting in brain malformations associated with epilepsy and intellectual disability. Novel, highly sensitive technologies will allow more accurate evaluation of somatic mutations in neurodevelopmental disorders and during normal brain development.

  17. Somatic Mutation, Genomic Variation, and Neurological Disease

    PubMed Central

    Poduri, Annapurna; Evrony, Gilad D.; Cai, Xuyu; Walsh, Christopher A.

    2014-01-01

    Genetic mutations causing human disease are conventionally thought to be inherited through the germ line from one’s parents and present in all somatic (body) cells, except for most cancer mutations, which arise somatically. Increasingly, somatic mutations are being identified in diseases other than cancer, including neurodevelopmental diseases. Somatic mutations can arise during the course of prenatal brain development and cause neurological disease—even when present at low levels of mosaicism, for example—resulting in brain malformations associated with epilepsy and intellectual disability. Novel, highly sensitive technologies will allow more accurate evaluation of somatic mutations in neurodevelopmental disorders and during normal brain development. PMID:23828942

  18. Human neurologic function and the aging process.

    PubMed

    Potvin, A R; Syndulko, K; Tourtellotte, W W; Lemmon, J A; Potvin, J H

    1980-01-01

    Sixty-one normal men whose ages ranged from 20 to 80 years were evaluated on two occasions by means of a comprehensive series of 128 instrumented tests of neurologic function. The tests measured cognition, vision, strength, steadiness, reactions, speed, coordination, fatigue, gait, station, sensations, and tasks of daily living. The reliability of each test measured was determined, and any measure found unreliable (r less than or equal to 0.41) was not further analyzed. Significant age-related linear decreases were found for almost all neurologic functions. The declines over the age span varied from less than 10 percent to more than 90 percent for different functions. For the upper extremities, the largest declines (greater than 50 percent) were in hand-force steadiness, speed of hand-arm movements, and vibration sense; for the lower extremities, the largest declines were in one-legged balance with eyes closed and in vibration sense. For 13 of 14 tests in which significant dominant body-side effects were found, larger re-testing 7-10 days later, the subjects improved their scores by more than 5 percent on only 17 tests, 9 of which concerned the activities of daily living. No significant differential learning effects were found across age groups. The results point to the importance of developing a data bank on age-based neurologic function so that therapeutic effects can be evaluated in terms of age- and sex-matched normal functioning.

  19. Experience of families with very low birthweight children with neurologic sequelae.

    PubMed

    Rivers, A; Caron, B; Hack, M

    1987-05-01

    To describe the quality of life for surviving very low birthweight (VLBW, less than 1.5 kg) children and their families, the authors interviewed the parents of 22 neurologically abnormal VLBW children, 3 to 7 years after their birth and compared them to the parents of 15 neurologically normal VLBW children. The infants were born between 1976 and 1979, (mean birthweight, 1.2 kg; mean gestational age, 30 weeks). They included three with spastic quadriplegia, 14 with spastic diplegia, and five with hydrocephalus. Eight children were not walking. Seven families rated their child's neurologic problem as severe, while 15 rated the problem as mild. Neurologically abnormal children tended to display more screaming and crying than the normal children. Cost of ongoing medical care was a problem for families of nine neurologically abnormal as compared with two families of normal children. The abnormal children required a total of 61 postneonatal rehospitalizations versus 11 in the normal group. Families of neurologically abnormal children reported on significant major changes in their personal lives for the care of their child. Both groups of parents reported heightened appreciation of their child. Plans for future children were affected in both. Parents identified an urgent need for better medical information, education of pediatricians regarding preterm development and neurologic sequelae, and better contact and support among parents themselves.

  20. Neurological Aspects of Reading Disability.

    ERIC Educational Resources Information Center

    Nelson, Louis R.

    The author, a neurologist, looks at the nature of reading disabilities. He suggests that many reading disabilities are the result of normal constitutional differences and that the term "minimal brain dysfunction" is rarely appropriate and does not help the remediation process. Noted are various theories which relate neurology and reading ability.…

  1. Paradigm shift in NMDA receptor antagonist drug development: molecular mechanism of uncompetitive inhibition by memantine in the treatment of Alzheimer's disease and other neurologic disorders.

    PubMed

    Lipton, Stuart A

    2004-12-01

    , studies in our laboratory have shown that the adamantane derivative, memantine, preferentially blocks excessive NMDA receptor activity without disrupting normal activity. Memantine does this through its action as an uncompetitive, low-affinity, open-channel blocker; it enters the receptor-associated ion channel preferentially when it is excessively open, and, most importantly, its off-rate is relatively fast so that it does not substantially accumulate in the channel to interfere with normal synaptic transmission. Clinical use has corroborated the prediction that memantine is thus well tolerated. Besides Alzheimer's disease, memantine is currently in trials for additional neurological disorders, including other forms of dementia, depression, glaucoma, and severe neuropathic pain. A series of second-generation memantine derivatives are currently in development and may prove to have even greater neuroprotective properties than memantine. These second-generation drugs take advantage of the fact that the NMDA receptor has other modulatory sites in addition to its ion channel that potentially could also be used for safe but effective clinical intervention.

  2. Neurologic Diseases

    MedlinePlus

    ... muscular dystrophy Problems with the way the nervous system develops, such as spina bifida Degenerative diseases, where nerve cells are damaged or die, such as Parkinson's disease and Alzheimer's disease Diseases of the blood vessels that supply ...

  3. Severe neurologic manifestations in acute intermittent porphyria developed after spine surgery under general anesthesia: a case report

    PubMed Central

    Park, Eun Young; Kim, Yi Seul; Lim, Kyung-Jee; Lee, Hye Kyoung; Lee, Soo Kyung; Choi, Hyun

    2014-01-01

    Porphyrias are inherited metabolic disorders resulting from a specific enzyme defect in the heme biosynthetic pathway. Porphyrias are induced by various precipitants. Clinical features include abdominal pain, neurologic manifestations, autonomic neuropathy, and mental disturbance. Diagnosis may be delayed because of variable symptoms that mimic other diseases and because of the rarity of of porphyrias. Although most patients with known porphyria can complete anesthesia and surgery safely, undiagnosed porphyric patients are in danger of porphyric crisis due to inadvertent exposure to precipitating drugs and environment. We report a case of a patient who experienced delayed emergence with neurological disturbance after general anesthesia, ultimately diagnosed as acute intermittent porphyria. PMID:25302100

  4. Neurological and Biological Foundations of Children's Social and Emotional Development: An Integrated Literature Review

    ERIC Educational Resources Information Center

    Nelson, Helen Jean; Kendall, Garth Edward; Shields, Linda

    2014-01-01

    This article provides an integrated review of the expert literature on developmental processes that combine social, biological, and neurological pathways, and the mechanisms through which these pathways may influence school success and health. It begins with a historical overview of the current understanding of how attachment relationships and…

  5. [THE DEVELOPMENT OF MEDICAL CARE OF POPULATION IN CONDITIONS OF SPECIALIZED DAY-TIME HOSPITALS OF NEUROLOGICAL PROFILE].

    PubMed

    Grishina, N K; Solovieva, N B; Abdulsalamova, Z A

    2015-01-01

    The article considers issues concerning increasing of quality and accessibility of medical care in Moscow neurological profile included at the expense of wide-spread implementation of specialized day-time hospitals in health care practice. The analysis applied was based on average Moscow indicators of functioning of public health institutions and characteristics of clinical course of diseases of the mentioned profile.

  6. [Cat eye syndrome with pituitary dwarfism and normal mental development].

    PubMed

    Pierson, M; Gilgenkrantz, S; Saborio, M

    1975-11-01

    In the so-called "cat-eye" syndrome are associated the following malformations: coloboma iridis, anal atresia, pre-auricular fistullae with an extra 47th chromosome of the G group type. About twenty cases have already been reported. Some are familial cases and some have the complete phenotype but without the extra chromosome. Even if the structure of the material of this element is doubtful, its responsability in the phenotype is likely. This case is reported since the patient has pituitary dwarfism and normal intelligence.

  7. Normal and abnormal neuronal migration in the developing cerebral cortex.

    PubMed

    Sun, Xue-Zhi; Takahashi, Sentaro; Cui, Chun; Zhang, Rui; Sakata-Haga, Hiromi; Sawada, Kazuhiko; Fukui, Yoshihiro

    2002-08-01

    Neuronal migration is the critical cellular process which initiates histogenesis of cerebral cortex. Migration involves a series of complex cell interactions and transformation. After completing their final mitosis, neurons migrate from the ventricular zone into the cortical plate, and then establish neuronal lamina and settle onto the outermost layer, forming an "inside-out" gradient of maturation. This process is guided by radial glial fibers, requires proper receptors, ligands, other unknown extracellular factors, and local signaling to stop neuronal migration. This process is also highly sensitive to various physical, chemical and biological agents as well as to genetic mutations. Any disturbance of the normal process may result in neuronal migration disorder. Such neuronal migration disorder is believed as major cause of both gross brain malformation and more special cerebral structural and functional abnormalities in experimental animals and in humans. An increasing number of instructive studies on experimental models and several genetic model systems of neuronal migration disorder have established the foundation of cortex formation and provided deeper insights into the genetic and molecular mechanisms underlying normal and abnormal neuronal migration.

  8. Simulation in neurology.

    PubMed

    Micieli, Giuseppe; Cavallini, Anna; Santalucia, Paola; Gensini, Gianfranco

    2015-10-01

    Simulation is a frontier for disseminating knowledge in almost all the fields of medicine and it is attracting growing interest because it offers a means of developing new teaching and training models, as well as of verifying what has been learned in a critical setting that simulates clinical practice. The role of simulation in neurology, until now limited by the obvious physical limitations of the dummies used to train students and learners, is now increasing since, today, it allows anamnestic data to be related to the instrumental evidence necessary for diagnosis and therapeutic decision-making, i.e., to the findings of neurophysiological investigations (EEG, carotid and vertebral echography and transcranial Doppler, for example) and neuroradiological investigations (CT, MRI imaging), as well as vital parameter monitoring (ECG, saturimetry, blood pressure, respiratory frequency, etc.). Simulation, by providing learners with opportunities to discuss, with experts, different profiles of biological parameters (both during the simulation itself and in the subsequent debriefing session), is becoming an increasingly important tool for training those involved in evaluation of critical neurological patients (stroke, Guillan Barrè syndrome, myasthenia, status epilepticus, headache, vertigo, confusional status, etc.) and complex cases. In this SIMMED (Italian Society for Simulation in Medicine) position paper, the applications (present and, possibly, future) of simulation in neurology are reported.

  9. Pregnancy-induced acute neurologic emergencies and neurologic conditions encountered in pregnancy.

    PubMed

    Alvis, Jeffrey S; Hicks, Richard J

    2012-02-01

    Neurologic complications and conditions associated with pregnancy are rare. Frequently, presenting symptoms of neurologic conditions are nonspecific and can overlap with normal symptoms of pregnancy. As a result, clinical assessment can be insufficient to differentiate symptoms of a normal pregnancy from a neurologic disorder. It is imperative that the radiologist have a basic familiarity with the most common neurologic conditions encountered in pregnancy. The most commonly imaged acute and nonemergent disorders will be described, including eclampsia, cerebrovascular disease including cerebral venous thrombosis, postpartum cerebral angiopathy, multiple sclerosis, tumors, Bell palsy, Guillain-Barré syndrome, and pituitary disorders. PMID:22264902

  10. Understanding normal development of adolescent sexuality: A bumpy ride.

    PubMed

    Kar, Sujita Kumar; Choudhury, Ananya; Singh, Abhishek Pratap

    2015-01-01

    Adolescence, derived from the Latin word "adolescere" meaning "to grow up" is a critical developmental period. During adolescence, major biological as well as psychological developments take place. Development of sexuality is an important bio-psycho-social development, which takes an adult shape during this period. During adolescence, an individual's thought, perception as well as response gets colored sexually. Puberty is an important landmark of sexuality development that occurs in the adolescence. The myriad of changes that occurs in adolescents puts them under enormous stress, which may have adverse physical, as well as psychological consequences. Understanding adolescent sexuality has important clinical, legal, social, cultural, as well as educational implications.

  11. Normal and Abnormal Development in the Arabidopsis Vegetative Shoot Apex.

    PubMed Central

    Medford, JI; Behringer, FJ; Callos, JD; Feldmann, KA

    1992-01-01

    Vegetative development in the Arabidopsis shoot apex follows both sequential and repetitive steps. Early in development, the young vegetative meristem is flat and has a rectangular shape with bilateral symmetry. The first pair of leaf primordia is radially symmetrical and is initiated on opposite sides of the meristem. As development proceeds, the meristem changes first to a bilaterally symmetrical trapezoid and then to a radially symmetrical dome. Vegetative development from the domed meristem continues as leaves are initiated in a repetitive manner. Abnormal development of the vegetative shoot apex is described for a number of mutants. The mutants we describe fall into at least three classes: (1) lesions in the shoot apex that do not show an apparent alteration in the shoot apical meristem, (2) lesions in the apical meristem that also (directly or indirectly) alter leaf primordia, and (3) lesions in the apical meristem that alter meristem size and leaf number but not leaf morphology. These mutations provide tools both to genetically analyze vegetative development of the shoot apex and to learn how vegetative development influences floral development. PMID:12297656

  12. Improving the normalization of complex interventions: measure development based on normalization process theory (NoMAD): study protocol

    PubMed Central

    2013-01-01

    Background Understanding implementation processes is key to ensuring that complex interventions in healthcare are taken up in practice and thus maximize intended benefits for service provision and (ultimately) care to patients. Normalization Process Theory (NPT) provides a framework for understanding how a new intervention becomes part of normal practice. This study aims to develop and validate simple generic tools derived from NPT, to be used to improve the implementation of complex healthcare interventions. Objectives The objectives of this study are to: develop a set of NPT-based measures and formatively evaluate their use for identifying implementation problems and monitoring progress; conduct preliminary evaluation of these measures across a range of interventions and contexts, and identify factors that affect this process; explore the utility of these measures for predicting outcomes; and develop an online users’ manual for the measures. Methods A combination of qualitative (workshops, item development, user feedback, cognitive interviews) and quantitative (survey) methods will be used to develop NPT measures, and test the utility of the measures in six healthcare intervention settings. Discussion The measures developed in the study will be available for use by those involved in planning, implementing, and evaluating complex interventions in healthcare and have the potential to enhance the chances of their implementation, leading to sustained changes in working practices. PMID:23578304

  13. Understanding normal development of adolescent sexuality: A bumpy ride

    PubMed Central

    Kar, Sujita Kumar; Choudhury, Ananya; Singh, Abhishek Pratap

    2015-01-01

    Adolescence, derived from the Latin word “adolescere” meaning “to grow up” is a critical developmental period. During adolescence, major biological as well as psychological developments take place. Development of sexuality is an important bio-psycho-social development, which takes an adult shape during this period. During adolescence, an individual's thought, perception as well as response gets colored sexually. Puberty is an important landmark of sexuality development that occurs in the adolescence. The myriad of changes that occurs in adolescents puts them under enormous stress, which may have adverse physical, as well as psychological consequences. Understanding adolescent sexuality has important clinical, legal, social, cultural, as well as educational implications. PMID:26157296

  14. Complex Neurological Phenotype in Mutant Mice Lacking Tsc2 in Excitatory Neurons of the Developing Forebrain123

    PubMed Central

    Crowell, Beth; Hwa Lee, Gum; Nikolaeva, Ina; Dal Pozzo, Valentina

    2015-01-01

    Abstract Mutations in the TSC1 and TSC2 genes cause tuberous sclerosis complex (TSC), a genetic disease often associated with epilepsy, intellectual disability, and autism, and characterized by the presence of anatomical malformations in the brain as well as tumors in other organs. The TSC1 and TSC2 proteins form a complex that inhibits mammalian target of rapamycin complex 1 (mTORC1) signaling. Previous animal studies demonstrated that Tsc1 or Tsc2 loss of function in the developing brain affects the intrinsic development of neural progenitor cells, neurons, or glia. However, the interplay between different cellular elements during brain development was not previously investigated. In this study, we generated a novel mutant mouse line (NEX-Tsc2) in which the Tsc2 gene is deleted specifically in postmitotic excitatory neurons of the developing forebrain. Homozygous mutant mice failed to thrive and died prematurely, whereas heterozygous mice appeared normal. Mutant mice exhibited distinct neuroanatomical abnormalities, including malpositioning of selected neuronal populations, neuronal hypertrophy, and cortical astrogliosis. Intrinsic neuronal defects correlated with increased mTORC1 signaling, whereas astrogliosis did not result from altered intrinsic signaling, since these cells were not directly affected by the gene knockout strategy. All neuronal and non-neuronal abnormalities were suppressed by continuous postnatal treatment with the mTORC1 inhibitor RAD001. The data suggest that the loss of Tsc2 and mTORC1 signaling activation in excitatory neurons not only disrupts their intrinsic development, but also disrupts the development of cortical astrocytes, likely through the mTORC1-dependent expression of abnormal signaling proteins. This work thus provides new insights into cell-autonomous and non-cell-autonomous functions of Tsc2 in brain development. PMID:26693177

  15. Neuroimaging Studies of Normal Brain Development and Their Relevance for Understanding Childhood Neuropsychiatric Disorders

    ERIC Educational Resources Information Center

    Marsh, Rachel; Gerber, Andrew J.; Peterson, Bradley S.

    2008-01-01

    Neuroimaging findings which identify normal brain development trajectories are presented. Results show that early brain development begins with the neural tube formation and ends with myelintation. How disturbances in brain development patterns are related to childhood psychiatric disorders is examined.

  16. The anatomy and development of normal and abnormal coronary arteries.

    PubMed

    Spicer, Diane E; Henderson, Deborah J; Chaudhry, Bill; Mohun, Timothy J; Anderson, Robert H

    2015-12-01

    At present, there is significant interest in the morphology of the coronary arteries, not least due to the increasingly well-recognised association between anomalous origin of the arteries and sudden cardiac death. Much has also been learnt over the last decade regarding the embryology of the arteries. In this review, therefore, we provide a brief introduction into the recent findings regarding their development. In particular, we emphasise that new evidence, derived using the developing murine heart, points to the arterial stems growing out from the adjacent sinuses of the aortic root, rather than the arteries growing in, as is currently assumed. As we show, the concept of outgrowth provides an excellent explanation for several of the abnormal arrangements encountered in the clinical setting. Before summarising these abnormal features, we draw attention to the need to describe the heart in an attitudinally appropriate manner, following the basic rule of human anatomy, rather than describing the cardiac components with the heart in the "Valentine" orientation. We then show how the major abnormalities involving the coronary arteries in humans can be summarised in terms of abnormal origin from the pulmonary circulation, abnormal aortic origin, or fistulous communications between the coronary arteries and the cardiac cavities. In the case of abnormal aortic origin, we highlight those malformations known to be associated with sudden cardiac death.

  17. GABAA Receptors in Normal Development and Seizures: Friends or Foes?

    PubMed Central

    Galanopoulou, Aristea S

    2008-01-01

    GABAA receptors have an age-adapted function in the brain. During early development, they mediate excitatory effects resulting in activation of calcium sensitive signaling processes that are important for the differentiation of the brain. In more mature stages of development and in adults, GABAA receptors transmit inhibitory signals. The maturation of GABAA signaling follows sex-specific patterns, which appear to also be important for the sexual differentiation of the brain. The inhibitory effects of GABAA receptor activation have been widely exploited in the treatment of conditions where neuronal silencing is necessary. For instance, drugs that target GABAA receptors are the mainstay of treatment of seizures. Recent evidence suggests however that the physiology and function of GABAA receptors changes in the brain of a subject that has epilepsy or status epilepticus. This review will summarize the physiology of and the developmental factors regulating the signaling and function of GABAA receptors; how these may change in the brain that has experienced prior seizures; what are the implications for the age and sex specific treatment of seizures and status epilepticus. Finally, the implications of these changes for the treatment of certain forms of medically refractory epilepsies and status epilepticus will be discussed. PMID:19305785

  18. [Present and future of neurology in Spain].

    PubMed

    Illa Sendra, I; García De Yébenes Prous, J; Ramo Tello, C; Polo Esteban, J M; Molinuevo Guix, J L; Robles Bayón, A; Mulas Delgado, F; Alvarez Sabín, J; Aguilar Barbera, M; Berciano Blanco JA, J A; Blesa González, R; Carnero Pardo, C; Castillo Sánchez, J; Del Ser Quijano, T; Ferrer Abizanda, I; García-Albea Ristol, E; Gómez Isla, T; Graus Ribas, F; Jiménez Hernández, M D; Liaño Martínez, H; Matías Guiu-Guia, J; Zarranz Imirizaldu, J J; Paradas López, C; Elena Martínez, G; Maltas Pérez, G; Ponce Rodríguez, M T

    2001-11-01

    This is a document prepared by the Spanish Society of Neurology (SEN), which was given to the President of Spain (Mr. José María Aznar) last September with the main aim of examining the current situation of Neurology in our country. It analyses the present and future of Neurology in clinical assistance, teaching and research. To prepare this document the criteria of patients' associations has been considered, including the Declaration of Madrid which has been subscribed by thirty of these associations. In spite of its relevant development in the previous decades, the current situation of Neurology in Spain is far from the ideal. To reach the recommendable menber of 3 or 4 neurologists per 100,000 inhabitants it is necessary to duplicate the present number of neurologists which has been estimated around 2/100,000; this situation is especially urgent in some Autonomous Communities. The most important problems in neurological assistance are: inadequate follow-up of the chronic outpatients, low numbers of neurological beds and of duties of Neurology, as well as of neurological case of patients with urgent neurological disorders. It is also necessary to increase the number of professors of Neurology to adequately cover pregraduate teaching; again there are important differences in teaching positions among Autonomous Communities. Neurology residence should be prolonged from 4 to 5 years. Finally, it is necessary to support the appearance of superespecialised units and to promote a coordinated research with other close specialities including basic neuroscience. PMID:11742621

  19. Thermography in Neurologic Practice

    PubMed Central

    Neves, Eduardo Borba; Vilaça-Alves, José; Rosa, Claudio; Reis, Victor Machado

    2015-01-01

    One kind of medical images that has been developed in the last decades is thermal images. These images are assessed by infrared cameras and have shown an exponential development in recent years. In this sense, the aim of this study was to describe possibilities of thermography usage in the neurologic practice. It was performed a systematic review in Web of Knowledge (Thompson Reuters), set in all databases which used two combination of keywords as “topic”: “thermography” and “neurology”; and “thermography” and “neurologic”. The chronological period was defined from 2000 to 2014 (the least 15 years). Among the studies included in this review, only seven were with experimental design. It is few to bring thermography as a daily tool in clinical practice. However, these studies have suggested good results. The studies of review and an analyzed patent showed that the authors consider the thermography as a diagnostic tool and they recommend its usage. It can be concluded that thermography is already used as a diagnostic and monitoring tool of patients with neuropathies, particularly in complex regional pain syndrome, and stroke. And yet, this tool has great potential for future research about its application in diagnosis of other diseases of neurological origin. PMID:26191090

  20. Clinical characterization and long-term prognosis of neurological development in preterm infants with late-onset circulatory collapse

    PubMed Central

    Nakanishi, H; Yamanaka, S; Koriyama, T; Shishida, N; Miyagi, N; Kim, T-J; Kusuda, S

    2010-01-01

    Objective: To characterize the risk factors for late-onset circulatory collapse (LCC) in preterm infants responsive to corticosteroid therapy and evaluate the long-term neurological prognosis. Study Design: A retrospective case–control study for preterm infants (⩽32 weeks' gestation) admitted to our neonatal intensive care unit from 1994 through 2002. Result: Sixty-five infants (11%) were diagnosed with LCC. Infants with a shorter gestation and lower birth weight had a higher incidence of LCC. LCC infants had a significantly lower 1-min Apgar score, significantly higher incidence of severe intraventricular hemorrhage, chronic lung disease, and postnatal periventricular leukomalacia, and significantly longer duration of ventilation use, oxygen use, and hospital stay. Somatic growth at 36 weeks' postmenstrual age was poorer in infants with LCC than without LCC (controls). LCC infants were significantly more likely than controls to have cerebral palsy at 3 years. Conclusion: LCC is associated with poor neurodevelopmental outcomes. Prevention of LCC can lead to improved neurological prognoses. PMID:20357811

  1. A three-dimensional mechanical analysis of normal fault evolution and joint development in perturbed stress fields around normal faults

    NASA Astrophysics Data System (ADS)

    Kattenhorn, Simon Allen

    1998-09-01

    This thesis investigates the role of three dimensionality in the evolution of normal fault systems and joint development in perturbed stress fields induced by slipping normal faults. This is accomplished using: (1) 3D numerical models; (2) field observations of fault and joint characteristics; and (3) seismic data interpretations of 3D normal fault geometries. Numerical models that incorporate the contribution of an increasing lithostatic load with increasing depth on fault slip behavior indicate that normal faults are more prone to slip near the top than the bottom. Energy release rates are maximized at the upper tip, suggesting that faults should grow preferentially in an up-dip direction. For the case of laterally segmented faults, mechanical interaction promotes propagation of segments towards each other, which may result in composite fault surfaces that are longer than they are tall, in agreement with documented natural examples. Slipping faults perturb the surrounding stress field. Field relationships in Arches National Park, Utah, suggest that joints grew in the perturbed stress field around the faults. Numerical analyses indicate that joint orientations depend on the location along the fault tipline in 3D, and may range from fault-parallel to fault-perpendicular. In addition, as the ratio of fault-parallel to fault-perpendicular remote stress increases, so too does the distance away from the fault affected by the perturbed field. In relay zones, joints forming at high angles to fault strike are likely to continue propagating away from the fault if the fault-parallel stress approaches, or slightly exceeds, the fault-perpendicular remote stress during fault slip. Multiple slip maxima at the same stratigraphic level on apparently continuous fault surfaces in 3D seismic data attest to initial lateral segmentation. Mechanical interaction and lateral propagation led to linkage of fault segments and the formation of composite faults that are longer than they are

  2. Occupational Neurological Disorders in Korea

    PubMed Central

    Kang, Seong-Kyu

    2010-01-01

    The purpose of this article was to provide a literature review of occupational neurological disorders and related research in Korea, focusing on chemical hazards. We reviewed occupational neurological disorders investigated by the Occupational Safety and Health Research Institute of Korean Occupational Safety and Health Agency between 1992 and 2009, categorizing them as neurological disorders of the central nervous system (CNS), of the peripheral nervous system (PNS) or as neurodegenerative disorders. We also examined peer-reviewed journal articles related to neurotoxicology, published from 1984 to 2009. Outbreaks of occupational neurological disorder of the CNS due to inorganic mercury and carbon disulfide poisoning had helped prompt the development of the occupational safety and health system of Korea. Other major neurological disorders of the CNS included methyl bromide intoxication and chronic toxic encephalopathy. Most of the PNS disorders were n-hexane-induced peripheral neuritis, reported from the electronics industry. Reports of manganese-induced Parkinsonism resulted in the introduction of neuroimaging techniques to occupational medicine. Since the late 1990s, the direction of research has been moving toward degenerative disorder and early effect of neurotoxicity. To understand the early effects of neurotoxic chemicals in the preclinical stage, more follow-up studies of a longer duration are necessary. PMID:21258587

  3. Update on Paraneoplastic Neurologic Disorders

    PubMed Central

    Rosenfeld, Myrna R.

    2010-01-01

    When patients with cancer develop neurologic symptoms, common causes include metastasis, infections, coagulopathy, metabolic or nutritional disturbances, and neurotoxicity from treatments. A thorough clinical history, temporal association with cancer therapies, and results of ancillary tests usually reveal one of these mechanisms as the etiology. When no etiology is identified, the diagnosis considered is often that of a paraneoplastic neurologic disorder (PND). With the recognition that PNDs are more frequent than previously thought, the availability of diagnostic tests, and the fact that, for some PNDs, treatment helps, PNDs should no longer be considered diagnostic zebras, and when appropriate should be included in the differential diagnosis early in the evaluation. PMID:20479279

  4. Sporadic hemiplegic migraine with permanent neurological deficits.

    PubMed

    Schwedt, Todd J; Zhou, Jiying; Dodick, David W

    2014-01-01

    By definition, the neurologic impairments of hemiplegic migraine are reversible. However, a few cases of permanent neurologic deficits associated with hemiplegic migraine have been reported. Herein, we present the case of a patient with permanent impairments because of hemiplegic migraine despite normalization of associated brain magnetic resonance imaging abnormalities. Cases like these suggest the need to consider aggressive prophylactic therapy for patients with recurrent hemiplegic migraine attacks.

  5. Fish oil and mental health: the role of n-3 long-chain polyunsaturated fatty acids in cognitive development and neurological disorders.

    PubMed

    Assisi, Alessandro; Banzi, Rita; Buonocore, Carmela; Capasso, Filippo; Di Muzio, Valeria; Michelacci, Francesca; Renzo, Danila; Tafuri, Giovanni; Trotta, Francesco; Vitocolonna, Maria; Garattini, Silvio

    2006-11-01

    Epidemiological and experimental studies have indicated that consumption of more n-3 long-chain polyunsaturated fatty acids may reduce the risk for a variety of diseases, including cardiovascular, neurological and immunological disorders, diabetes and cancer. This article focuses on the role of marine n-3 long-chain polyunsaturated fatty acids in brain functions, including the development of the central nervous system and neurological disorders. An overview of the major animal studies and clinical trials is provided here, focusing on fatty acid supplementation during pregnancy and infancy, and prevention and management of Alzheimer's disease, schizophrenia, depression and attention deficit hyperactive disorder. Although an optimal balance in n-3/n-6 long-chain polyunsaturated fatty acid ratio is important for proper neurodevelopment and cognitive functions, results from randomized controlled trials are controversial and do not confirm any useful effect of supplementation on development of preterm and term infants. The relationship between fatty acid status and mental disorders is confirmed by reduced levels of n-3 long-chain polyunsaturated fatty acids in erythrocyte membranes of patients with central nervous system disorders. Nevertheless, there are very little data supporting the use of fish oil in those patients. The only way to verify whether n-3 long-chain polyunsaturated fatty acids are a potential therapeutic option in the management and prevention of mental disorders is to conduct a large definitive randomized controlled trials similar to those required for the licensing of any new pharmacological treatment.

  6. Happiness and neurological diseases.

    PubMed

    Barak, Yoram; Achiron, Anat

    2009-04-01

    addition, specific interventions to increase happiness developed by PoP clinicians have demonstrated efficacy and validity. Several measurement scales now exist that reliably measure happiness as a long-term state, rather than as a fluctuating condition resulting from prevailing mood. These enable the use of happiness as an end point in clinical trials. As happiness becomes increasingly accepted as an important consideration in neurological disease, it is likely to be monitored more extensively, with measures to raise happiness levels being taken as an effective means of improving patient outcomes.

  7. The neurology in Shakespeare.

    PubMed

    Fogan, L

    1989-08-01

    William Shakespeare's 37 plays and poetry contain many references of interest for almost all of the medical specialties. To support that the Bard could be considered a Renaissance neurologist, the following important neurological phenomena have been selected from his repertoire for discussion: tremors, paralysis and stroke, sleep disturbances, epilepsy, dementia, encephalopathies, and the neurology of syphilis. PMID:2667505

  8. Cystic fibrosis transmembrane conductance regulator protein (CFTR) expression in the developing human brain: comparative immunohistochemical study between patients with normal and mutated CFTR.

    PubMed

    Marcorelles, Pascale; Friocourt, Gaëlle; Uguen, Arnaud; Ledé, Françoise; Férec, Claude; Laquerrière, Annie

    2014-11-01

    Cystic Fibrosis Transmembrane conductance Regulator (CFTR) protein has recently been shown to be expressed in the human adult central nervous system (CNS). As CFTR expression has also been documented during embryonic development in several organs, such as the respiratory tract, the intestine and the male reproductive system, suggesting a possible role during development we decided to investigate the expression of CFTR in the human developing CNS. In addition, as some, although rare, neurological symptoms have been reported in patients with CF, we compared the expression of normal and mutated CFTR at several fetal stages. Immunohistochemistry was performed on brain and spinal cord samples of foetuses between 13 and 40 weeks of gestation and compared with five patients with cystic fibrosis (CF) of similar ages. We showed in this study that CFTR is only expressed in neurons and has an early and widespread distribution during development. Although we did not observe any cerebral abnormality in patients with CF, we observed a slight delay in the maturation of several brain structures. We also observed different expression and localization of CFTR depending on the brain structure or the cell maturation stage. Our findings, along with a literature review on the neurological phenotypes of patients with CF, suggest that this gene may play previously unsuspected roles in neuronal maturation or function.

  9. Neurologic presentations of AIDS.

    PubMed

    Singer, Elyse J; Valdes-Sueiras, Miguel; Commins, Deborah; Levine, Andrew

    2010-02-01

    The human immunodeficiency virus (HIV), the cause of AIDS, has infected an estimated 33 million individuals worldwide. HIV is associated with immunodeficiency, neoplasia, and neurologic disease. The continuing evolution of the HIV epidemic has spurred an intense interest in a hitherto neglected area of medicine, neuroinfectious diseases and their consequences. This work has broad applications for the study of central nervous system (CNS) tumors, dementias, neuropathies, and CNS disease in other immunosuppressed individuals. HIV is neuroinvasive (can enter the CNS), neurotrophic (can live in neural tissues), and neurovirulent (causes disease of the nervous system). This article reviews the HIV-associated neurologic syndromes, which can be classified as primary HIV neurologic disease (in which HIV is both necessary and sufficient to cause the illness), secondary or opportunistic neurologic disease (in which HIV interacts with other pathogens, resulting in opportunistic infections and tumors), and treatment-related neurologic disease (such as immune reconstitution inflammatory syndrome). PMID:19932385

  10. Neurology issues in schizophrenia.

    PubMed

    Hüfner, Katharina; Frajo-Apor, Beatrice; Hofer, Alex

    2015-05-01

    Schizophrenia ranks among the leading causes of disability worldwide. The presence of neurological signs co-occurring with the psychiatric symptoms is indicative of an organic brain pathology. In the present article, we review the current literature on neurology issues in schizophrenia. Firstly, common neurological signs found in patients with schizophrenia (neurological soft signs and smell abnormalities) and their association with imaging findings are reviewed. Secondly, the significant association of schizophrenia with epilepsy and stroke is described as well as the absent association with other organic brain diseases such as multiple sclerosis. Thirdly, we discuss the potential role of NMDA receptor antibodies in schizophrenia. Fourthly, neurological side effects of antipsychotic drugs and their treatment are reviewed; and lastly, we discuss neurocognitive deficits in patients with schizophrenia and their treatment. The focus of the review remains on articles with relevance to the clinician. PMID:25773225

  11. Neurology and orthopaedics

    PubMed Central

    Houlden, Henry; Charlton, Paul; Singh, Dishan

    2007-01-01

    Neurology encompasses all aspects of medicine and surgery, but is closer to orthopaedic surgery than many other specialities. Both neurological deficits and bone disorders lead to locomotor system abnormalities, joint complications and limb problems. The main neurological conditions that require the attention of an orthopaedic surgeon are disorders that affect the lower motor neurones. The most common disorders in this group include neuromuscular disorders and traumatic peripheral nerve lesions. Upper motor neurone disorders such as cerebral palsy and stroke are also frequently seen and discussed, as are chronic conditions such as poliomyelitis. The management of these neurological problems is often coordinated in the neurology clinic, and this group, probably more than any other, requires a multidisciplinary team approach. PMID:17308288

  12. Profile of neurological disorders in an adult neurology clinic in Kumasi, Ghana

    PubMed Central

    Sarfo, Fred Stephen; Akassi, John; Badu, Elizabeth; Okorozo, Aham; Ovbiagele, Bruce; Akpalu, Albert

    2016-01-01

    Background Although the burden of neurological disorders is highest among populations in developing countries there is a dearth of data on the clinical spectrum of these disorders. Objective To profile the frequency of neurologic disorders and basic demographic data in an adult neurology out-patient service commissioned in 2011 in Kumasi, Ghana. Methods The study was conducted at the neurology clinic of the Komfo Anokye Teaching Hospital in Kumasi, Ghana. Over a three year period, all medical records of patients enrolled at the out-patient neurology clinic was reviewed by a neurologist and neurological diagnoses classified according to ICD-10. Results 1812 adults enrolled for care in the neurology out-patient service between 2011 and 2013. This comprised of 882 males and 930 females (male: female ratio of 1.0: 1.1) with an overall median age of 54 (IQR, 39–69) years. The commonest primary neurological disorders seen were strokes, epilepsy and seizure disorders, and movement disorders at frequencies of 57.1%, 19.8%, and 8.2% respectively. Conclusions Cerebrovascular diseases, epilepsy and movement disorders were among the commonest neurological disorders and the major contributors to neurologic morbidity among Ghanaians in an urban neurology clinic. PMID:27110596

  13. Normal Cerebellar Development by Qualitative and Quantitative MR Imaging: From the Fetus to the Adolescent.

    PubMed

    Brossard-Racine, Marie; Limperopoulos, Catherine

    2016-08-01

    This article presents an overview of published studies using conventional and quantitative MR imaging to describe normal development of the cerebellum prenatally and postnatally through 18 years of age. Normal cerebellar development and maturational processes are described here within the context of MR imaging morphology, microstructure, metabolism, and functional connectivity. In addition, strengths and weaknesses of these reviewed studies are critically appraised and new directions for future cerebellar MR imaging investigation are made. PMID:27423797

  14. The Long and the Short of it: Gene and Environment Interactions During Early Cortical Development and Consequences for Long-Term Neurological Disease.

    PubMed

    Stolp, Helen; Neuhaus, Ain; Sundramoorthi, Rohan; Molnár, Zoltán

    2012-01-01

    Cortical development is a complex amalgamation of proliferation, migration, differentiation, and circuit formation. These processes follow defined timescales and are controlled by a combination of intrinsic and extrinsic factors. It is currently unclear how robust and flexible these processes are and whether the developing brain has the capacity to recover from disruptions. What is clear is that there are a number of cognitive disorders or conditions that are elicited as a result of disrupted cortical development, although it may take a long time for the full pathophysiology of the conditions to be realized clinically. The critical window for the manifestation of a neurodevelopmental disorder is prolonged, and there is the potential for a complex interplay between genes and environment. While there have been extended investigations into the genetic basis of a number of neurological and mental disorders, limited definitive associations have been discovered. Many environmental factors, including inflammation and stress, have been linked to neurodevelopmental disorders, and it may be that a better understanding of the interplay between genes and environment will speed progress in this field. In particular, the development of the brain needs to be considered in the context of the whole materno-fetal unit as the degree of the metabolic, endocrine, or inflammatory responses, for example, will greatly influence the environment in which the brain develops. This review will emphasize the importance of extending neurodevelopmental studies to the contribution of the placenta, vasculature, cerebrospinal fluid, and to maternal and fetal immune response. These combined investigations are more likely to reveal genetic and environmental factors that influence the different stages of neuronal development and potentially lead to the better understanding of the etiology of neurological and mental disorders such as autism, epilepsy, cerebral palsy, and schizophrenia.

  15. The Long and the Short of it: Gene and Environment Interactions During Early Cortical Development and Consequences for Long-Term Neurological Disease

    PubMed Central

    Stolp, Helen; Neuhaus, Ain; Sundramoorthi, Rohan; Molnár, Zoltán

    2012-01-01

    Cortical development is a complex amalgamation of proliferation, migration, differentiation, and circuit formation. These processes follow defined timescales and are controlled by a combination of intrinsic and extrinsic factors. It is currently unclear how robust and flexible these processes are and whether the developing brain has the capacity to recover from disruptions. What is clear is that there are a number of cognitive disorders or conditions that are elicited as a result of disrupted cortical development, although it may take a long time for the full pathophysiology of the conditions to be realized clinically. The critical window for the manifestation of a neurodevelopmental disorder is prolonged, and there is the potential for a complex interplay between genes and environment. While there have been extended investigations into the genetic basis of a number of neurological and mental disorders, limited definitive associations have been discovered. Many environmental factors, including inflammation and stress, have been linked to neurodevelopmental disorders, and it may be that a better understanding of the interplay between genes and environment will speed progress in this field. In particular, the development of the brain needs to be considered in the context of the whole materno-fetal unit as the degree of the metabolic, endocrine, or inflammatory responses, for example, will greatly influence the environment in which the brain develops. This review will emphasize the importance of extending neurodevelopmental studies to the contribution of the placenta, vasculature, cerebrospinal fluid, and to maternal and fetal immune response. These combined investigations are more likely to reveal genetic and environmental factors that influence the different stages of neuronal development and potentially lead to the better understanding of the etiology of neurological and mental disorders such as autism, epilepsy, cerebral palsy, and schizophrenia. PMID:22701439

  16. Child neurology: Past, present, and future: part 1: history.

    PubMed

    Millichap, John J; Millichap, J Gordon

    2009-08-18

    The founding period of child neurology occurred in 3 phases: 1) early individual contributory phase, 2) organized training phase, and 3) expansion phase. In the late 19th and early 20th centuries, individuals in pediatrics, neurology, and psychiatry established clinics and made important contributions to the literature on childhood epilepsy, cerebral palsy, and pediatric neurology. The latter half of the 20th century saw the organization of training programs in pediatric neurology, with fellowships supported by the NIH. This development was followed by a rapid expansion in the number of trainees certified in child neurology and their appointment to divisions of neurology in children's hospitals. In recent years, referrals of children with neurologic disorders have increased, and disorders previously managed by pediatricians are often seen in neurology clinics. The era of subspecialization is embraced by the practicing physician. The present day status of pediatric neurology and suggestions for the future development of the specialty are subjects for further discussion.

  17. Early preventive attachment-oriented psychotherapeutic intervention program with parents of a very low birthweight premature infant: results of attachment and neurological development.

    PubMed

    Brisch, Karl Heinz; Bechinger, Doris; Betzler, Suzanne; Heinemann, Hilde

    2003-06-01

    The birth of a very small preterm infant (< or = 1500 grams) can be a traumatizing experience for many parents. A developmental risk model is presented that is the background to an early attachment-oriented preventive psychotherapeutic intervention. This comprehensive parent-centered intervention program is composed of supportive group psychotherapy, attachment-oriented focal individual psychotherapy, a home visit and video-based sensitivity training. The intervention aims at improving parental coping, the process of attachment and parent-infant interaction. In a prospective longitudinal design mothers were randomly assigned to a control (N = 44) and an intervention group (N = 43) after preterm delivery. Results show that the percentage of secure (control vs. intervention group: 77.8% vs. 59.4%) and insecure (control vs. intervention group: 8.3% vs. 31.3% avoidant, 13.9% vs. 9.4% ambivalent) attachment quality in high-risk preterm infants is comparable to results from studies with term infants. There was no significant statistical difference in terms of quality of attachment of the preterm infants between the control group and the intervention group. However, only in the control group, impaired neurological development corresponded significantly with an insecure quality of attachment, but not in the intervention group, although there were significantly more neurologically impaired infants in the intervention group. This result is discussed as an effect of the intervention program.

  18. A Comparative Study of Pre-Meaningful Vocalizations Produced by Normally Developing and Down's Syndrome Infants.

    ERIC Educational Resources Information Center

    Oller, D. Kimbrough; Smith, Bruce L.

    1981-01-01

    Premeaningful vocalizations produced by nine normally developing and 10 Down's syndrome infants were recorded as part of a longitudinal study of language development. Both groups began to produce canonical, reduplicated babbling at 8 to 8 1/2 months of age, and trends regarding consonantal and vocalic development for both groups were similar…

  19. Neurologic complications of vaccinations.

    PubMed

    Miravalle, Augusto A; Schreiner, Teri

    2014-01-01

    This chapter reviews the most common neurologic disorders associated with common vaccines, evaluates the data linking the disorder with the vaccine, and discusses the potential mechanism of disease. A literature search was conducted in PubMed using a combination of the following terms: vaccines, vaccination, immunization, and neurologic complications. Data were also gathered from publications of the American Academy of Pediatrics Committee on Infectious Diseases, the World Health Organization, the US Centers for Disease Control and Prevention, and the Vaccine Adverse Event Reporting System. Neurologic complications of vaccination are rare. Many associations have been asserted without objective data to support a causal relationship. Rarely, patients with a neurologic complication will have a poor outcome. However, most patients recover fully from the neurologic complication. Vaccinations have altered the landscape of infectious disease. However, perception of risk associated with vaccinations has limited the success of disease eradication measures. Neurologic complications can be severe, and can provoke fear in potential vaccines. Evaluating whether there is causal link between neurologic disorders and vaccinations, not just temporal association, is critical to addressing public misperception of risk of vaccination. Among the vaccines available today, the cost-benefit analysis of vaccinations and complications strongly argues in favor of vaccination.

  20. Neuro-QOL: quality of life item banks for adults with neurological disorders: item development and calibrations based upon clinical and general population testing

    PubMed Central

    Lai, Jin Shei; Bode, Rita; Choi, Seung; Moy, Claudia; Bleck, Tom; Miller, Deborah; Peterman, Amy; Cella, David

    2013-01-01

    Purpose Neuro-QOL provides a clinically relevant and psychometrically robust health-related quality of life (HRQL) assessment tool for both adults and children with common neurological disorders. We now report the psychometric results for the adult tools. Methods An extensive research, survey and consensus process was used to produce a list of 5 priority adult neurological conditions (stroke, multiple sclerosis, Parkinson’s disease, epilepsy and ALS). We identified relevant health related quality of life (HRQL) domains through multiple methods and data sources including a comprehensive review of the literature and literature search, expert interviews and surveys and patient and caregiver focus groups. The final domain framework consisted of 17 domains of Physical, Mental and Social health. There were five phases of item development: (1) identification of 3,482 extant items, (2) item classification and selection, (3) item review and revision, (4) cognitive interviews with 63 patients to assess their understanding of individual items and (5) field testing of 432 representative items. Participants and Procedures Participants were drawn from the US general population and clinical settings, and included both English and Spanish speaking subjects (N = 3,246). Confirmatory factor analysis (CFA) was used to evaluate the dimensionality of unidimensional domains. Where the domain structure was previously unknown, the dataset was split and first analyzed with exploratory factor analysis and then CFA. Samejima’s graded response model (GRM) was used to calculate IRT parameters. We further evaluated differential item functioning (DIF) on gender, education and age. Results Thirteen unidimensional calibrated item banks consisting of 297 items were developed. All of the tested item banks had high reliability and few or no locally dependent items. The range of item slopes and thresholds with good information are reported for each of the item banks. The banks can support CAT and

  1. Neurology and international organizations.

    PubMed

    Mateen, Farrah J

    2013-07-23

    A growing number of international stakeholders are engaged with neurologic diseases. This article provides a brief overview of important international stakeholders in the practice of neurology, including global disease-specific programs, United Nations agencies, governmental agencies with international influence, nongovernmental organizations, international professional organizations, large private donors, private-public partnerships, commercial interests, armed forces, and universities and colleges. The continued engagement of neurologists is essential for the growing number of international organizations that can and should incorporate neurologic disease into their global agendas.

  2. William Shakespeare's neurology.

    PubMed

    Paciaroni, Maurizio; Bogousslavsky, Julien

    2013-01-01

    Many of Shakespeare's plays contain characters who appear to be afflicted by neurological or psychiatric disorders. Shakespeare, in his descriptive analysis of his protagonists, was contributing to the understanding of these disorders. In fact, Charcot frequently used Shakespearean references in his neurological teaching sessions, stressing how acute objective insight is essential to achieving expert clinical diagnosis. Charcot found in Shakespeare the same rigorous observational techniques for which he himself became famous. This chapter describes many of Shakespearean characters suffering from varied neurological disorders, including Parkinsonism, epilepsy, sleeping disturbances, dementia, headache, prion disease, and paralyses. PMID:24290473

  3. [Neurology of hysteria (conversion disorder)].

    PubMed

    Sonoo, Masahiro

    2014-07-01

    Hysteria has served as an important driving force in the development of both neurology and psychiatry. Jean Martin Charcot's devotion to mesmerism for treating hysterical patients evoked the invention of psychoanalysis by Sigmund Freud. Meanwhile, Joseph Babinski took over the challenge to discriminate between organic and hysterical patients from Charcot and found Babinski's sign, the greatest milestone in modern neurological symptomatology. Nowadays, the usage of the term hysteria is avoided. However, new terms and new classifications are complicated and inconsistent between the two representative taxonomies, the DSM-IV and ICD-10. In the ICD-10, even the alternative term conversion disorder, which was becoming familiar to neurologists, has also disappeared as a group name. The diagnosis of hysteria remains important in clinical neurology. Extensive exclusive diagnoses and over investigation, including various imaging studies, should be avoided because they may prolong the disease course and fix their symptoms. Psychological reasons that seem to explain the conversion are not considered reliable. Positive neurological signs suggesting nonorganic etiologies are the most reliable measures for diagnosing hysteria, as Babinski first argued. Hysterical paresis has several characteristics, such as giving-way weakness or peculiar distributions of weakness. Signs to uncover nonorganic paresis utilizing synergy include Hoover's test and the Sonoo abductor test.

  4. Metabolic phenotyping and systems biology approaches to understanding neurological disorders.

    PubMed

    Dumas, Marc-Emmanuel; Davidovic, Laetitia

    2013-01-01

    The development of high-throughput metabolic profiling and the study of the metabolome are particularly important in brain research where small molecules or metabolites play fundamental signalling roles: neurotransmitters, signalling lipids, osmolytes and even ions. Metabolic profiling has shown that metabolic perturbations in the brain go beyond alterations of neurotransmission and that variations in brain metabolic homeostasis are associated with neurological disorders. In this report, we will focus on recent developments in the field of metabolic phenotyping that have contributed to unravelling the pathophysiology of neurological diseases. Also, we will highlight the necessity of implementing systems biology approaches to integrate metabolic data and tackle the structural and functional complexity of the brain in normal and pathological conditions. PMID:23755365

  5. [Child neurology and rehabilitation].

    PubMed

    Kumagai, K

    2000-05-01

    The history of child neurology and the changing pattern of research methods in this field are reviewed with special reference to holoprosencephaly and recent technical advances in sleep research. This is followed by a discussion on the relationship between child neurology and rehabilitation. The majority of child neurologic disorders are developmental disabilities, but acquired child neurological diseases also show chronic progressive course in many cases. Therefore, child neurologist should understand the basis of rehabilitation approach and appreciate the three classes of disabilities; subsequently, a plan needs to be incorporating medical treatment and a program of rehabilitation for the disabled children. It is important that the role of the various rehabilitation specialists (rehabilitation doctor, physiotherapist, occupational therapist, and others) are understood in relation to the work of pediatric neurologist. Finally, a brief discussion is presented on the rehabilitation approach of patients with hypoxic encephalopathy and the information of welfare equipment.

  6. Haematology and neurology

    PubMed Central

    Austin, Steven; Cohen, Hannah; Losseff, Nick

    2007-01-01

    This review aims to update the reader on advances in the understanding of haematological conditions that may arise in neurological practice. Thrombophilia, antiphospholipid antibody syndrome, thrombotic thrombocytopenic purpura, sickle cell and clonal disorders associated with neuropathy are discussed. PMID:17369588

  7. Theory of mind abilities of children with schizophrenia, children with autism, and normally developing children.

    PubMed

    Pilowsky, T; Yirmiya, N; Arbelle, S; Mozes, T

    2000-04-01

    Theory of mind (ToM) abilities of children with schizophrenia, children with high functioning autism, and normally developing children, matched on mental age (MA), verbal MA, and performance MA, were compared. Both clinical groups were matched on chronological age as well, whereas the normally developing children were younger. A fact belief task, a value belief task, a deception task, and a false belief task were administered. The three groups did not differ on the fact belief task. Children with autism performed more poorly than normally developing children on value belief and false belief tasks, and more poorly than individuals with schizophrenia on the deception task. Children with schizophrenia performed more poorly than normally developing children only on the false belief task. Overall, the group with autism passed significantly fewer tasks compared to the normally developing group. ToM abilities correlated with verbal abilities for individuals with autism. The ToM abilities of children with paranoid schizophrenia and children with undifferentiated or disorganized schizophrenia did not differ. Findings strengthen the notion of a limited understanding of ToM in schizophrenia, and support the notion that ToM deficits, although more severe in autism, are not unique to autism.

  8. Apotemnophilia: a neurological disorder.

    PubMed

    Brang, David; McGeoch, Paul D; Ramachandran, Vilayanur S

    2008-08-27

    Apotemnophilia, a disorder that blurs the distinction between neurology and psychiatry, is characterized by the intense and longstanding desire for amputation of a specific limb. Here we present evidence from two individuals suggestive that this condition, long thought to be entirely psychological in origin, actually has a neurological basis. We found heightened skin conductance response to pinprick below the desired line of amputation. We propose apotemnophilia arises from congenital dysfunction of the right superior parietal lobule and its connection with the insula.

  9. Hemispheric Specialization in Normally and Slowly Developing Children: A Tachistoscopic and Dichaptic Evaluation.

    ERIC Educational Resources Information Center

    Williams, H. G.; And Others

    1980-01-01

    Both right-and left-handed normally developing 6-year-olds showed considerable evidence of bilateralization of hemispheric functions for spatial and verbal information processing; the slowly developing children (ages 5-9) exhibited unusual patterns of hemispheric specialization usually opposite those typically expected in children or adults.…

  10. Wikipedia and neurological disorders.

    PubMed

    Brigo, Francesco; Igwe, Stanley C; Nardone, Raffaele; Lochner, Piergiorgio; Tezzon, Frediano; Otte, Willem M

    2015-07-01

    Our aim was to evaluate Wikipedia page visits in relation to the most common neurological disorders by determining which factors are related to peaks in Wikipedia searches for these conditions. Millions of people worldwide use the internet daily as a source of health information. Wikipedia is a popular free online encyclopedia used by patients and physicians to search for health-related information. The following Wikipedia articles were considered: Alzheimer's disease; Amyotrophic lateral sclerosis; Dementia; Epilepsy; Epileptic seizure; Migraine; Multiple sclerosis; Parkinson's disease; Stroke; Traumatic brain injury. We analyzed information regarding the total article views for 90 days and the rank of these articles among all those available in Wikipedia. We determined the highest search volume peaks to identify possible relation with online news headlines. No relation between incidence or prevalence of neurological disorders and the search volume for the related articles was found. Seven out of 10 neurological conditions showed relations in search volume peaks and news headlines. Six out of these seven peaks were related to news about famous people suffering from neurological disorders, especially those from showbusiness. Identification of discrepancies between disease burden and health seeking behavior on Wikipedia is useful in the planning of public health campaigns. Celebrities who publicly announce their neurological diagnosis might effectively promote awareness programs, increase public knowledge and reduce stigma related to diagnoses of neurological disorders.

  11. Dengue: a new challenge for neurology

    PubMed Central

    Puccioni-Sohler, Marzia; Orsini, Marco; Soares, Cristiane N.

    2012-01-01

    Dengue infection is a leading cause of illness and death in tropical and subtropical regions of the world. Forty percent of the world's population currently lives in these areas. The clinical picture resulting from dengue infection can range from relatively minor to catastrophic hemorrhagic fever. Recently, reports have increased of neurological manifestations. Neuropathogenesis seems to be related to direct nervous system viral invasion, autoimmune reaction, metabolic and hemorrhagic disturbance. Neurological manifestations include encephalitis, encephalopathy, meningitis, Guillain-Barré syndrome, myelitis, acute disseminated encephalomyelitis, polyneuropathy, mononeuropathy, and cerebromeningeal hemorrhage. The development of neurological symptoms in patients with positive Immunoglobulin M (IgM) dengue serology suggests a means of diagnosing the neurological complications associated with dengue. Viral antigens, specific IgM antibodies, and the intrathecal synthesis of dengue antibodies have been successfully detected in cerebrospinal fluid. However, despite diagnostic advancements, the treatment of neurological dengue is problematic. The launch of a dengue vaccine is expected to be beneficial. PMID:23355928

  12. State neurologic societies and the AAN

    PubMed Central

    Narayanaswami, Pushpa; Showers, Dave; Levi, Bruce; Showers, Melissa; Jones, Elaine C.; Busis, Neil A.; Comella, Cynthia L.; Pulst, Stefan M.; Hosey, Jonathan P.; Griggs, Robert C.

    2014-01-01

    Summary This report considers the recommendations of the State Society Task Force (SSTF), which evaluated how the relationship between the American Academy of Neurology (AAN) and neurologic societies of individual states can foster the care of patients with neurologic diseases. The task force also evaluated the role of state neurosociety and state medical society interactions in supporting the profession of neurology. The SSTF recommended that the AAN expand current support services to state neurosocieties and foster additional neurosociety development. Specific services to be considered by the AAN include online combined AAN/state neurosociety dues payment and enhanced Web support. The role of the AAN as a liaison between state neurosocieties and state medical societies is important to facilitate state level advocacy for neurology. PMID:25110622

  13. Mammary Gland Growth Factors: Roles in Normal Development and in Cancer

    PubMed Central

    Hynes, Nancy E.; Watson, Christine J.

    2010-01-01

    Normal development of the mammary gland proceeds via interactions between the epithelium and the mesenchyme that start during embryogenesis and continue during pubertal outgrowth and differentiation. The function of specific peptide growth factors that bind members of the receptor tyrosine kinase family and the cytokine receptor family are required at each stage. In many cases the peptides are produced in one compartment and act on receptors in the other compartment. One of the striking differences between normal development and cancer is the loss of this cross-talk. Mammary tumor cells often produce a peptide and express the receptor on the same cell leading to autocrine activation of signaling pathways, a mechanism that is characteristic for cancer cells. We will discuss different peptides in the context of normal development and cancer in this review. PMID:20554705

  14. 75 FR 3475 - National Institute Of Neurological Disorders and Stroke; Notice of Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-01-21

    ... HUMAN SERVICES National Institutes of Health National Institute Of Neurological Disorders and Stroke.... App.), notice is hereby given of meetings of the National Advisory Neurological Disorders and Stroke... Neurological Disorders and Stroke Council; Training, Career Development, and Special Programs...

  15. Neurological manifestations of malaria.

    PubMed

    Román, G C; Senanayake, N

    1992-03-01

    The involvement of the nervous system in malaria is reviewed in this paper. Cerebral malaria, the acute encephalopathy which complicates exclusively the infection by Plasmodium falciparum commonly affects children and adolescents in hyperendemic areas. Plugging of cerebral capillaries and venules by clumped, parasitized red cells causing sludging in the capillary circulation is one hypothesis to explain its pathogenesis. The other is a humoral hypothesis which proposes nonspecific, immune-mediated, inflammatory responses with release of vasoactive substances capable of producing endothelial damage and alterations of permeability. Cerebral malaria has a mortality rate up to 50%, and also a considerable longterm morbidity, particularly in children. Hypoglycemia, largely in patients treated with quinine, may complicate the cerebral symptomatology. Other central nervous manifestations of malaria include intracranial hemorrhage, cerebral arterial occlusion, and transient extrapyramidal and neuropsychiatric manifestations. A self-limiting, isolated cerebellar ataxia, presumably caused by immunological mechanisms, in patients recovering from falciparum malaria has been recognized in Sri Lanka. Malaria is a common cause of febrile seizures in the tropics, and it also contributes to the development of epilepsy in later life. Several reports of spinal cord and peripheral nerve involvement are also available. A transient muscle paralysis resembling periodic paralysis during febrile episodes of malaria has been described in some patients. The pathogenesis of these neurological manifestations remains unexplored, but offers excellent perspectives for research at a clinical as well as experimental level. PMID:1307475

  16. Behaviors and attitudes of normally developing children toward their intellectually disabled siblings.

    PubMed

    Unal, Nazan; Baran, Gülen

    2011-04-01

    This study investigated behaviors of children who have normal development toward their siblings with intellectual disabilities. 9- to 17-year-old normally developing siblings (55 girls, 39 boys) of 94, 5- to 15-year-old mentally disabled children (51 girls, 43 boys) who were attending a special education and rehabilitation center were enrolled in the study. Data were gathered by using a general information form and the Schaeffer Sibling Behavior Rating Scale. Age of the disabled child did not have a significant effect on siblings' behaviors, while knowledge of family about the diagnosis and educational status of their child with intellectual disabilities affected sibling behaviors.

  17. The impact of continuous and ongoing professional development on the nursing process of taking care of neurological patients.

    PubMed

    Kopacević, Lenka; Mihelcić, Vesna Bozan; Antić, Sonja; Demarin, Vida

    2013-03-01

    Nurses distinguish continuous professional development intended for career improvement and personal development from continuous professional development intended for improvement of skills and knowledge. Too many requests are put in front of an individual unacceptably stretching it onto the life outside work. Students have various expectations from their education. Factors that motivate nurses to study are to improve knowledge, patient care and professional relations. Factors connected to personal development include boost of confidence and embracing of other values. Motivation for additional education is also connected to the change of work, practice improvement, self-confidence improvement, nursing career plans, and necessary intellectual stimulation. The reasons for which nurses decide to undertake further education are also desire for maintenance of clinical competence, but also enjoyment in studying itself. An employer who expects from nurses to opt for such a type of education or stimulate it is another common reason. PMID:23837270

  18. Development and application of methods for regional scaling and normalization in life-cycle impact assessment

    SciTech Connect

    Tolle, D.A.

    1995-12-31

    Life-cycle impact assessment (LCIA) is a technical, quantitative and/or qualitative method to classify, characterize, and valuate potential impacts on human health, ecosystems, and natural resources, based on the environmental burdens identified in a life-cycle inventory. Research described here for two LCIAs included development and application of regional scaling methods for the following 5 of 14 relevant impact categories: Suspended (PM{sub 10}) particulate effects, water use, acid deposition, smog creation, and eutrophication. Normalization is recommended after characterization, because aggregated sums per impact category need to be expressed in equivalent terms before assigning valuation weight factors. The normalization approach described here involves determination of factors that represent the total, geographically-relevant impact for a given impact category. The goal for the 14 normalization factors developed and applied to two LCIAS, was to make them scientifically defensible, while utilizing existing data on emission or resource extraction quantities for three spatial perspectives. Data on the total environmental burden for each inventory item under a given impact category were obtained for normalization factors. Since the boundaries of the two LCIAs were primarily in the US, the data for the regional or local impact category perspectives were restricted to appropriate areas in the US. Normalization factors were developed and applied in the two LCIAs for 11 impact categories involving chemical emissions, water use, solid waste volume, and resource extraction/production land use.

  19. Transient neurologic syndrome after spinal anesthesia with epidural steroid treatment

    PubMed Central

    Cöcelli, L. Pirbudak; Erkutlu, Ibrahim; Karakurum, Gunhan; Avci, Neslihan; Gül, Rauf; Öner, Ünsal

    2009-01-01

    Background: Transient neurologic syndrome (TNS) is a rare complication of spinal and epidural anesthesia. It is defined as paradoxic postoperative back pain radiating to the lower extremities with no neurologic deficits. Because it is a self-limited disease, the treatment is usually symptomatic and consists of NSAIDs and injections of a neuromuscular-blocking drug at the trigger points. The syndrome may be resistant to this treatment regimen and may last for several months, resulting in a long convalescence. Case summary: A 63-year-old Turkish woman (height, 165 cm; weight, 71 kg) underwent hemorrhoidectomy in the jackknife position using spinal anesthesia. No adverse events occurred during puncture or surgery or in the immediate postoperative recovery period. Recovery from the sensory and motor block was normal. Twenty-four hours after surgery, lower limb and plantar pain developed with no sensory or motor deficit. Neurologic examination revealed normal motor and sensory function. Electroneuromyography showed partial denervation potential of muscles innervated by the left sciatic nerve. The symptoms were suggestive of TNS. Combination oral NSAID treatment with amitriptyline (25 mg/d) and gabapentin (1200 mg/d) was initiated. Because the pain still persisted 6 weeks after surgery, epidural steroid injection with triamcinolone acetate (80 mg) with isotonic saline was administered, resulting in definite pain relief (visual analog scale score = 0). Conclusions: Epidural steroid treatment was effective in this patient with TNS resistant to treatment with NSAIDs, amitriptyline, and gabapentin. Future studies are needed to evaluate this treatment. PMID:24683240

  20. Normal table of embryonic development in the four-toed salamander, Hemidactylium scutatum.

    PubMed

    Hurney, C A; Babcock, S K; Shook, D R; Pelletier, T M; Turner, S D; Maturo, J; Cogbill, S; Snow, M C; Kinch, K

    2015-05-01

    We present a complete staging table of normal development for the lungless salamander, Hemidactylium scutatum (Caudata: Plethodontidae). Terrestrial egg clutches from naturally ovipositing females were collected and maintained at 15 °C in the laboratory. Observations, photographs, and time-lapse movies of embryos were taken throughout the 45-day embryonic period. The complete normal table of development for H. scutatum is divided into 28 stages and extends previous analyses of H. scutatum embryonic development (Bishop, 1920; Humphrey, 1928). Early embryonic stage classifications through neurulation reflect criteria described for Xenopus laevis, Ambystoma maculatum and other salamanders. Later embryonic stage assignments are based on unique features of H. scutatum embryos. Additionally, we provide morphological analysis of gastrulation and neurulation, as well as details on external aspects of eye, gill, limb, pigmentation, and tail development to support future research related to phylogeny, comparative embryology, and molecular mechanisms of development.

  1. Normal table of embryonic development in the four-toed salamander, Hemidactylium scutatum.

    PubMed

    Hurney, C A; Babcock, S K; Shook, D R; Pelletier, T M; Turner, S D; Maturo, J; Cogbill, S; Snow, M C; Kinch, K

    2015-05-01

    We present a complete staging table of normal development for the lungless salamander, Hemidactylium scutatum (Caudata: Plethodontidae). Terrestrial egg clutches from naturally ovipositing females were collected and maintained at 15 °C in the laboratory. Observations, photographs, and time-lapse movies of embryos were taken throughout the 45-day embryonic period. The complete normal table of development for H. scutatum is divided into 28 stages and extends previous analyses of H. scutatum embryonic development (Bishop, 1920; Humphrey, 1928). Early embryonic stage classifications through neurulation reflect criteria described for Xenopus laevis, Ambystoma maculatum and other salamanders. Later embryonic stage assignments are based on unique features of H. scutatum embryos. Additionally, we provide morphological analysis of gastrulation and neurulation, as well as details on external aspects of eye, gill, limb, pigmentation, and tail development to support future research related to phylogeny, comparative embryology, and molecular mechanisms of development. PMID:25617760

  2. [Prevention of virus-related neurological diseases by vaccines].

    PubMed

    Takahashi, M

    1997-04-01

    Prevention of virus-related neurological diseases are surveyed. Patients of poliomyelitis has recently been drastically reduced by world-wide administrating live vaccines. In view of rare incidence of paralysis after giving live vaccine, adoption of inactivated vaccine has recently been reconsidered. A live varicella vaccine was developed and has been world-wide used for normal and high-risk children. Incidence of zoster in vaccinated acute leukemic children is several times higher in those who with rash after vaccination as compared with those without rash, and as no or few rash appears after vaccination of normal children, it is expected that vaccination of normal children would lead to reduction of zoster after their aging. Measles encephalitis has rapidly been reduced by world-wide use of live vaccines. Mouse-brain derived vaccine against Japanese encephalitis(JE) has been used in Asian countries. Development of tissue-culture derived JE vaccine is under way. PMID:9103901

  3. Neurological Complications of Transplantation

    PubMed Central

    Pruitt, Amy A.; Graus, Francesc; Rosenfeld, Myrna R.

    2013-01-01

    Hematopoietic cell transplantation (HCT) is the preferred treatment for an expanding range of neoplastic and nonmalignant conditions. Increasing numbers of solid organ transplantations (SOTs) add an additional population of immunosuppressed patients with multiple potential neurological problems. While the spectrum of neurological complications varies with conditioning procedure and hematopoietic cell or solid organ source, major neurological complications occur with all transplantation procedures. This 2 part review emphasizes a practical consultative approach to central and peripheral nervous system problems related to HCT or SOT with clinical and neuroimaging examples from the authors’ institutional experience with the following conditions: the diversity of manifestations of common infections such as varicella zoster virus, Aspergillus, and progressive multifocal leukoencephalopathy (PML), drug therapy-related complications, stroke mechanisms, the spectrum of graft versus host disease (GVHD), and neurologically important syndromes of immune reconstitution inflammatory syndrome (IRIS), posterior reversible encephalopathy syndrome (PRES), and posttransplantation lymphoproliferative disorder (PTLD). These complications preferentially occur at specific intervals after HCT and SOT, and neurological consultants must recognize an extensive spectrum of syndromes in order to effect timely diagnosis and expedite appropriate treatment. PMID:23983885

  4. Neurology and Don Quixote.

    PubMed

    Palma, Jose-Alberto; Palma, Fermin

    2012-01-01

    Don Quixote de la Mancha, which is considered one of the most important and influential works of Western modern prose, contains many references of interest for almost all of the medical specialties. In this regard, numerous references to neurology can be found in Cervantes' immortal work. In this study, we aimed to read Don Quixote from a neurologist's point of view, describing the neurological phenomena scattered throughout the novel, including tremors, sleep disturbances, neuropsychiatric symptoms, dementia, epilepsy, paralysis, stroke, syncope, traumatic head injury, and headache; we relate these symptoms with depictions of those conditions in the medical literature of the time. We also review Cervantes' sources of neurological information, including the works by renowned Spanish authors such as Juan Huarte de San Juan, Dionisio Daza Chacón and Juan Valverde de Amusco, and we hypothesize that Don Quixote's disorder was actually a neurological condition. Although Cervantes wrote it four centuries ago, Don Quixote contains plenty of references to neurology, and many of the ideas and concepts reflected in it are still of interest. PMID:23006630

  5. Development of Spatial Release from Masking in Mandarin-Speaking Children with Normal Hearing

    ERIC Educational Resources Information Center

    Yuen, Kevin C. P.; Yuan, Meng

    2014-01-01

    Purpose: This study investigated the development of spatial release from masking in children using closed-set Mandarin disyllabic words and monosyllabic words carrying lexical tones as test stimuli and speech spectrum-weighted noise as a masker. Method: Twenty-six children ages 4-9 years and 12 adults, all with normal hearing, participated in…

  6. Polyphenol oxidase affects normal nodule development in red clover (Trifolium pratense L.)

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Polyphenol oxidase (PPO) may have multiple functions in tissues, depending on its cellular or tissue localization. We used PPO RNAi transformants of red clover (Trifolium pratense) to determine the role PPO plays in normal development of plants, and especially in nitrogen-fixing nodules. In red clov...

  7. A Longitudinal Study of the Development of Stop Consonant Production in Normal and Down's Syndrome Children.

    ERIC Educational Resources Information Center

    Smith, Bruce L.; Stoel-Gammon, Carol

    1983-01-01

    The longitudinal study of four normal children, 18 to 36 months old, and five Down's syndrome (DS) children, 3 to 6 years old, analyzed the development of stop consonants and stop clusters. Although similar sound patterns were observed for the two groups, the DS children showed considerable performance delay. (DB)

  8. Development of the water window imaging X-ray microscope utilizing normal-incidence multilayer optics

    NASA Technical Reports Server (NTRS)

    Hoover, Richard B.; Shealy, David L.; Brinkley, B. R.; Baker, Phillip C.; Barbee, Troy W., Jr.; Walker, Arthur B. C., Jr.

    1991-01-01

    A water-window imaging X-ray telescope configured with normal-incidence multilayer X-ray mirrors has been developed to obtain images with unprecedented spatial resolution and contrast of carbon-based microstructures within living cells. The narrow bandpass response inherent in multilayer X-ray optics is accurately tuned to wavelengths within the water window.

  9. Normal Development and Psychopathological Impairment of the Concept of the Object on the Rorschach

    ERIC Educational Resources Information Center

    Blatt, Sidney J.; And Others

    1976-01-01

    Human responses to the Rorschach were analyzed according to developmental principles of differentiation, articulation, and integration in a longitudinal study of normal development (ages 11-12, 13-14, 17-18, and 30) and in a sample of adolescent and young adult inpatients. (Editor)

  10. Understanding Emotions from Standardized Facial Expressions in Autism and Normal Development

    ERIC Educational Resources Information Center

    Castelli, Fulvia

    2005-01-01

    The study investigated the recognition of standardized facial expressions of emotion (anger, fear, disgust, happiness, sadness, surprise) at a perceptual level (experiment 1) and at a semantic level (experiments 2 and 3) in children with autism (N= 20) and normally developing children (N= 20). Results revealed that children with autism were as…

  11. The problem of not developing normally and pediatric neuropsychological rehabilitation: theMitchell Rosenthal Lecture.

    PubMed

    Prigatano, George P

    2008-01-01

    Children who suffer moderately severe-to-severe traumatic brain injury often fail to develop normal cognitive and affective functioning necessary for independent adult living. The cognitive and psychiatric consequences of pediatric traumatic brain injury are outlined and 6 barriers to providing neuropsychological rehabilitation for these children are identified. Suggestions are made to help reduce the impact of those barriers.

  12. Extra-cell cycle regulatory functions of cyclin-dependent kinases (CDK) and CDK inhibitor proteins contribute to brain development and neurological disorders

    PubMed Central

    Kawauchi, Takeshi; Shikanai, Mima; Kosodo, Yoichi

    2013-01-01

    In developing brains, neural progenitors exhibit cell cycle-dependent nuclear movement within the ventricular zone [interkinetic nuclear migration (INM)] and actively proliferate to produce daughter progenitors and/or neurons, whereas newly generated neurons exit from the cell cycle and begin pial surface-directed migration and maturation. Dysregulation of the balance between the proliferation and the cell cycle exit in neural progenitors is one of the major causes of microcephaly (small brain). Recent studies indicate that cell cycle machinery influences not only the proliferation but also INM in neural progenitors. Furthermore, several cell cycle-related proteins, including p27kip1, p57kip2, Cdk5, and Rb, regulate the migration of neurons in the postmitotic state, suggesting that the growth arrest confers dual functions on cell cycle regulators. Consistently, several types of microcephaly occur in conjunction with neuronal migration disorders, such as periventricular heterotopia and lissencephaly. However, cell cycle re-entry by disturbance of growth arrest in mature neurons is thought to trigger neuronal cell death in Alzheimer's disease. In this review, we introduce the cell cycle protein-mediated regulation of two types of nuclear movement, INM and neuronal migration, during cerebral cortical development, and discuss the roles of growth arrest in cortical development and neurological disorders. PMID:23294285

  13. Development of AMPA receptor and GABA B receptor-sensitive spinal hyper-reflexia after spinal air embolism in rat: a systematic neurological, electrophysiological and qualitative histopathological study

    PubMed Central

    Kakinohana, Osamu; Scadeng, Miriam; Corleto, Jose A.; Sevc, Juraj; Lukacova, Nadezda; Marsala, Martin

    2012-01-01

    Decompression sickness results from formation of bubbles in the arterial and venous system, resulting in spinal disseminated neurodegenerative changes and may clinically be presented by motor dysfunction, spinal segmental stretch hyper-reflexia (i.e., spasticity) and muscle rigidity. In our current study, we describe a rat model of spinal air embolism characterized by the development of similar spinal disseminated neurodegenerative changes and functional deficit. In addition, the anti-spastic potency of systemic AMPA receptor antagonist (NGX424) or GABA B receptor agonist (baclofen) treatment was studied. To induce spinal air embolism, animals received an intra-aortic injection of air (50–200 μl/kg). After embolism, the development of spasticity was measured using computer-controlled ankle rotation. Animals receiving 150 or 200 μl of intra-aortic air injections displayed motor dysfunction with developed spastic (50–60% of animals) or flaccid (25–35% of animals) paraplegia at 5–7 days. MRI and spinal histopathological analysis showed disseminated spinal cord infarcts in the lower thoracic to sacral spinal segments. Treatment with NGX424 or baclofen provided a potent anti-spasticity effect (i.e., stretch hyper-reflexia inhibition). This model appears to provide a valuable experimental tool to study the pathophysiology of air embolism-induced spinal injury and permits the assessment of new treatment efficacy targeted to modulate neurological symptoms resulting from spinal air embolism. PMID:22721766

  14. Neurological complications of transplantation.

    PubMed

    Pustavoitau, Aliaksei; Bhardwaj, Anish; Stevens, Robert

    2011-01-01

    Recipients of solid organ or hematopoietic cell transplants are at risk of life-threatening neurological disorders including encephalopathy, seizures, infections and tumors of the central nervous system, stroke, central pontine myelinolysis, and neuromuscular disorders-often requiring admission to, or occurring in, the intensive care unit (ICU). Many of these complications are linked directly or indirectly to immunosuppressive therapy. However, neurological disorders may also result from graft versus host disease, or be an expression of the underlying disease which prompted transplantation, as well as injury induced during radiation, chemotherapy, surgery, and ICU stay. In rare cases, neuroinfectious pathogens may be transmitted with the transplanted tissue or organ. Diagnosis may be a challenge because clinical symptoms and findings on neuroimaging lack specificity, and a biological specimen or tissue diagnosis is often needed for definitive diagnosis. Management is centered on preventing further neurological injury, etiology-targeted therapy, and balancing the benefits and toxicities of specific immunosuppressive agents. PMID:21764765

  15. Genomics in Neurological Disorders

    PubMed Central

    Han, Guangchun; Sun, Jiya; Wang, Jiajia; Bai, Zhouxian; Song, Fuhai; Lei, Hongxing

    2014-01-01

    Neurological disorders comprise a variety of complex diseases in the central nervous system, which can be roughly classified as neurodegenerative diseases and psychiatric disorders. The basic and translational research of neurological disorders has been hindered by the difficulty in accessing the pathological center (i.e., the brain) in live patients. The rapid advancement of sequencing and array technologies has made it possible to investigate the disease mechanism and biomarkers from a systems perspective. In this review, recent progresses in the discovery of novel risk genes, treatment targets and peripheral biomarkers employing genomic technologies will be discussed. Our major focus will be on two of the most heavily investigated neurological disorders, namely Alzheimer’s disease and autism spectrum disorder. PMID:25108264

  16. Dependence of normal development of skeletal muscle in neonatal rats on load bearing

    NASA Technical Reports Server (NTRS)

    Ohira, Y.; Tanaka, T.; Yoshinaga, T.; Kawano, F.; Nomura, T.; Nonaka, I.; Allen, D. L.; Roy, R. R.; Edgerton, V. R.

    2000-01-01

    Antigravity function plays an important role in determining the morphological and physiological properties of the neuromuscular system. Inhibition of the normal development of the neuromuscular system is induced by hindlimb unloading during the neonatal period in rats. However, the role of gravitational loading on the development of skeletal muscle in rats is not well understood. It could be hypothesized that during the early postnatal period, i.e. when minimal weight-supporting activity occurs, the activity imposed by gravity would be of little consequence in directing the normal development of the skeletal musculature. We have addressed this issue by limiting the amount of postnatal weight-support activity of the hindlimbs of rats during the lactation period. We have focused on the development of three characteristics of the muscle fibers, i.e. size, myonuclear number and myosin heavy chain expression.

  17. Neurological examination: pioneering authors and their books.

    PubMed

    Maranhão-Filho, Péricles; Vincent, Maurice Borges; Silva, Marcos Martins da

    2015-02-01

    The objective of this article is to highlight some of the most important pioneering books specifically focused on the neurological examination and their authors. During the XIX Century, Alexander Hammond, William Gowers and Charles Mills pioneered the neurological literature, followed in the XX Century by Aloysio de Castro, Monrad-Krohn, Derek Denny-Brown, Robert Wartenberg, Gordon Holmes, and Russel DeJong. With determination and a marked sense of observation and research, they competently developed and spread the technique and art of the neurological exam.

  18. Leech segmental repeats develop normally in the absence of signals from either anterior or posterior segments

    NASA Technical Reports Server (NTRS)

    Seaver, E. C.; Shankland, M.

    2000-01-01

    We have investigated whether the development of segmental repeats is autonomous in the embryo of the leech Helobdella robusta. The segmental tissues of the germinal band arise from progeny of five stem cells called teloblasts. Asymmetric divisions of the teloblasts form chains of segment founder cells (called primary blast cells) that divide in a stereotypical manner to produce differentiated descendants. Using two distinct techniques, we have looked for potential interactions between neighboring blast cell clones along the anterior-posterior axis. In one technique, we prevented the birth of primary blast cells by injection of DNase I into the teloblast, thereby depriving the last blast cell produced before the ablation of its normal posterior neighbors. We also ablated single blast cells with a laser microbeam, which allowed us to assess potential signals acting on either more anterior or more posterior primary blast cell clones. Our results suggest that interactions along the anterior-posterior axis between neighboring primary blast cell clones are not required for development of normal segmental organization within the blast cell clone. We also examined the possibility that blast cells receive redundant signals from both anterior and posterior neighboring clones and that either is sufficient for normal development. Using double blast cell laser ablations to isolate a primary blast cell clone by removal of both its anterior and its posterior neighbor, we found that the isolated clone still develops normally. These results reveal that the fundamental segmental repeat in the leech embryo, the primary blast cell clone, can develop normally in the apparent absence of signals from adjacent repeats along the anterior-posterior axis.

  19. Elevated temperature enhances normal early embryonic development in the coral Platygyra acuta under low salinity conditions

    NASA Astrophysics Data System (ADS)

    Chui, Apple Pui Yi; Ang, Put

    2015-06-01

    To better understand the possible consequences of climate change on reef building scleractinian corals in a marginal environment, laboratory experiments were conducted to examine the interactive effects of changes in salinity and temperature on percent fertilization success and early embryonic development of the coral Platygyra acuta. In the present study, a salinity of 24 psu (ambient 32 psu) reduced fertilization success by 60 %. Normal embryonic development was reduced by >80 % at 26 psu (ambient 33 psu) with 100 % abnormal development at 22 psu under ambient temperature. Elevated temperature (+3 °C) above the ambient spawning temperature did not show any negative effects on fertilization success. However, there was a trend for more abnormal embryos to develop at elevated temperature in the 2 d of the spawning event. The interactive effects between salinity and temperature are statistically significant only on normal embryonic development of P. acuta, but not on its fertilization success. Salinity was revealed to be the main factor affecting both fertilization success and normal embryonic development. Interestingly, the much lower fertilization success (76 %) observed in the second day of spawning (Trial 2) under ambient temperature recovered to 99 % success under elevated (+3 °C) temperature conditions. Moreover, elevated temperature enhanced normal early embryonic development under lowered salinity (26 psu). This antagonistic interactive effect was consistently observed in two successive nights of spawning. Overall, our results indicate that, in terms of its fertilization success and embryonic development, P. acuta is the most tolerant coral species to reduced salinity thus far reported in the literature. Elevated temperature, at least that within the tolerable range of the corals, could apparently alleviate the potential negative effects from salinity stresses. This mitigating role of elevated temperature appears not to have been reported on corals before.

  20. Neurologic effects of alcoholism.

    PubMed Central

    Diamond, I; Messing, R O

    1994-01-01

    Alcoholism, a worldwide disorder, is the cause of a variety of neurologic disorders. In this article we discuss the cellular pathophysiology of ethanol addition and abuse as well as evidence supporting and refuting the role of inheritance in alcoholism. A genetic marker for alcoholism has not been identified, but neurophysiologic studies may be promising. Some neurologic disorders related to longterm alcoholism are due predominantly to inadequate nutrition (the thiamine deficiency that causes Wernicke's encephalopathy), but others appear to involve the neurotoxicity of ethanol on brain (alcohol withdrawal syndrome and dementia) and peripheral nerves (alcoholic neuropathy and myopathy). Images PMID:7975567

  1. Normalizing medicine: between "intersexuals" and individuals with "Disorders of Sex Development".

    PubMed

    Feder, Ellen K

    2009-06-01

    In this paper, I apply Michel Foucault's analysis of normalization to the 2006 announcement by the US and European Endocrinological Societies that variations on the term "hermaphrodite" and "intersex" would be replaced by the term, "Disorders of Sex Development" or DSD. I argue that the change should be understood as normalizing in a positive sense; rather than fighting for the demedicalization of conditions that have significant consequences for individuals' health, this change can promote the transformation of the conceptualization of intersex conditions from "disorders like no other" to "disorders like many others." Understood in these terms, I conclude, medical attention to those with atypical anatomies should be recast from a preoccupation with "normal appearance" to the concern with human flourishing that is the proper object of medical attention. PMID:19234791

  2. Effects of pre- and postnatal exposure to the UV-filter Octyl Methoxycinnamate (OMC) on the reproductive, auditory and neurological development of rat offspring

    SciTech Connect

    Axelstad, Marta; Boberg, Julie; Hougaard, Karin Sorig; Christiansen, Sofie; Jacobsen, Pernille Rosenskjold; Mandrup, Karen Riiber; Nellemann, Christine; Lund, Soren Peter; Hass, Ulla

    2011-02-01

    Octyl Methoxycinnamate (OMC) is a frequently used UV-filter in sunscreens and other cosmetics. The aim of the present study was to address the potential endocrine disrupting properties of OMC, and to investigate how OMC induced changes in thyroid hormone levels would be related to the neurological development of treated offspring. Groups of 14-18 pregnant Wistar rats were dosed with 0, 500, 750 or 1000 mg OMC/kg bw/day during gestation and lactation. Serum thyroxine (T{sub 4}), testosterone, estradiol and progesterone levels were measured in dams and offspring. Anogenital distance, nipple retention, postnatal growth and timing of sexual maturation were assessed. On postnatal day 16, gene expression in prostate and testes, and weight and histopathology of the thyroid gland, liver, adrenals, prostate, testes, epididymis and ovaries were measured. After weaning, offspring were evaluated in a battery of behavioral and neurophysiological tests, including tests of activity, startle response, cognitive and auditory function. In adult animals, reproductive organ weights and semen quality were investigated. Thyroxine (T{sub 4}) levels showed a very marked decrease during the dosing period in all dosed dams, but were less severely affected in the offspring. On postnatal day 16, high dose male offspring showed reduced relative prostate and testis weights, and a dose-dependent decrease in testosterone levels. In OMC exposed female offspring, motor activity levels were decreased, while low and high dose males showed improved spatial learning abilities. The observed behavioral changes were probably not mediated solely by early T{sub 4} deficiencies, as the observed effects differed from those seen in other studies of developmental hypothyroxinemia. At eight months of age, sperm counts were reduced in all three OMC-dosed groups, and prostate weights were reduced in the highest dose group. Taken together, these results indicate that perinatal OMC-exposure can affect both the

  3. Effects of pre- and postnatal exposure to the UV-filter octyl methoxycinnamate (OMC) on the reproductive, auditory and neurological development of rat offspring.

    PubMed

    Axelstad, Marta; Boberg, Julie; Hougaard, Karin Sørig; Christiansen, Sofie; Jacobsen, Pernille Rosenskjold; Mandrup, Karen Riiber; Nellemann, Christine; Lund, Søren Peter; Hass, Ulla

    2011-02-01

    Octyl Methoxycinnamate (OMC) is a frequently used UV-filter in sunscreens and other cosmetics. The aim of the present study was to address the potential endocrine disrupting properties of OMC, and to investigate how OMC induced changes in thyroid hormone levels would be related to the neurological development of treated offspring. Groups of 14-18 pregnant Wistar rats were dosed with 0, 500, 750 or 1000 mg OMC/kg bw/day during gestation and lactation. Serum thyroxine (T(4)), testosterone, estradiol and progesterone levels were measured in dams and offspring. Anogenital distance, nipple retention, postnatal growth and timing of sexual maturation were assessed. On postnatal day 16, gene expression in prostate and testes, and weight and histopathology of the thyroid gland, liver, adrenals, prostate, testes, epididymis and ovaries were measured. After weaning, offspring were evaluated in a battery of behavioral and neurophysiological tests, including tests of activity, startle response, cognitive and auditory function. In adult animals, reproductive organ weights and semen quality were investigated. Thyroxine (T(4)) levels showed a very marked decrease during the dosing period in all dosed dams, but were less severely affected in the offspring. On postnatal day 16, high dose male offspring showed reduced relative prostate and testis weights, and a dose-dependent decrease in testosterone levels. In OMC exposed female offspring, motor activity levels were decreased, while low and high dose males showed improved spatial learning abilities. The observed behavioral changes were probably not mediated solely by early T(4) deficiencies, as the observed effects differed from those seen in other studies of developmental hypothyroxinemia. At eight months of age, sperm counts were reduced in all three OMC-dosed groups, and prostate weights were reduced in the highest dose group. Taken together, these results indicate that perinatal OMC-exposure can affect both the reproductive and

  4. Development of choline acetyltransferase-immunoreactive neurons in normal and intracranially transplanted retinas in rats.

    PubMed

    Guo, Q X; Chau, R M; Yang, S Z; Jen, L S

    1991-10-21

    Retinas from embryonic day 14 (E14) Sprague-Dawley rats were transplanted to the tectum of newborn (P0) recipient rats, and the distribution pattern of choline acetyltransferase immunoreactivity (ChAT-I) in developing transplants was studied and compared with those observed in the retinas of normal developing rats. In normal retinas, ChAT-I cells were first identified in restricted regions in the ganglion cell layer (GCL) at P4, but were found to cover the entire GCL by P6. A second population of ChAT-I cells was detected in the inner nuclear layer (INL) at P8, and they were observed in most parts of the INL on P10 when two immunoreactive sublaminae began to appear in the inner plexiform layer (IPL). The adult pattern of having two distinct populations of ChAT-I cells, organized in mirror symmetrical fashion in the inner retinal layers was basically established by P12. The time course of development and overall distribution pattern of ChAT-I cells in developing retinal transplants on the whole were very similar to those observed in normal retinas. The first identification of these cells and the establishment of their final distribution pattern were made at stages corresponding to P4 and P12 of normal developing retinas respectively. However, ChAT-I somata were located in the INL at a much earlier stage compared with their counterparts in the normal retina, and a transient population of immunoreactive cells with their processes extending to retinal layers other than the IPL was observed in some transplants from P6 to P10. These features were not observed in normal developing retinas. These results suggest that the development of cholinergic neurons, especially the expression of their characteristic antigen and their final distribution pattern is largely determined by programmes which are intrinsic to the original retinal tissue, despite some minor deviation or variation in the developmental process which may occur under certain abnormal conditions. PMID:1769097

  5. Trajectories of cortical surface area and cortical volume maturation in normal brain development

    PubMed Central

    Ducharme, Simon; Albaugh, Matthew D.; Nguyen, Tuong-Vi; Hudziak, James J.; Mateos-Pérez, J.M.; Labbe, Aurelie; Evans, Alan C.; Karama, Sherif

    2015-01-01

    This is a report of developmental trajectories of cortical surface area and cortical volume in the NIH MRI Study of Normal Brain Development. The quality-controlled sample included 384 individual typically-developing subjects with repeated scanning (1–3 per subject, total scans n=753) from 4.9 to 22.3 years of age. The best-fit model (cubic, quadratic, or first-order linear) was identified at each vertex using mixed-effects models, with statistical correction for multiple comparisons using random field theory. Analyses were performed with and without controlling for total brain volume. These data are provided for reference and comparison with other databases. Further discussion and interpretation on cortical developmental trajectories can be found in the associated Ducharme et al.׳s article “Trajectories of cortical thickness maturation in normal brain development – the importance of quality control procedures” (Ducharme et al., 2015) [1]. PMID:26702424

  6. Genetic variants associated with motion sickness point to roles for inner ear development, neurological processes and glucose homeostasis

    PubMed Central

    Hromatka, Bethann S.; Tung, Joyce Y.; Kiefer, Amy K.; Do, Chuong B.; Hinds, David A.; Eriksson, Nicholas

    2015-01-01

    Roughly one in three individuals is highly susceptible to motion sickness and yet the underlying causes of this condition are not well understood. Despite high heritability, no associated genetic factors have been discovered. Here, we conducted the first genome-wide association study on motion sickness in 80 494 individuals from the 23andMe database who were surveyed about car sickness. Thirty-five single-nucleotide polymorphisms (SNPs) were associated with motion sickness at a genome-wide-significant level (P < 5 × 10−8). Many of these SNPs are near genes involved in balance, and eye, ear and cranial development (e.g. PVRL3, TSHZ1, MUTED, HOXB3, HOXD3). Other SNPs may affect motion sickness through nearby genes with roles in the nervous system, glucose homeostasis or hypoxia. We show that several of these SNPs display sex-specific effects, with up to three times stronger effects in women. We searched for comorbid phenotypes with motion sickness, confirming associations with known comorbidities including migraines, postoperative nausea and vomiting (PONV), vertigo and morning sickness and observing new associations with altitude sickness and many gastrointestinal conditions. We also show that two of these related phenotypes (PONV and migraines) share underlying genetic factors with motion sickness. These results point to the importance of the nervous system in motion sickness and suggest a role for glucose levels in motion-induced nausea and vomiting, a finding that may provide insight into other nausea-related phenotypes like PONV. They also highlight personal characteristics (e.g. being a poor sleeper) that correlate with motion sickness, findings that could help identify risk factors or treatments. PMID:25628336

  7. Genetic variants associated with motion sickness point to roles for inner ear development, neurological processes and glucose homeostasis.

    PubMed

    Hromatka, Bethann S; Tung, Joyce Y; Kiefer, Amy K; Do, Chuong B; Hinds, David A; Eriksson, Nicholas

    2015-05-01

    Roughly one in three individuals is highly susceptible to motion sickness and yet the underlying causes of this condition are not well understood. Despite high heritability, no associated genetic factors have been discovered. Here, we conducted the first genome-wide association study on motion sickness in 80 494 individuals from the 23andMe database who were surveyed about car sickness. Thirty-five single-nucleotide polymorphisms (SNPs) were associated with motion sickness at a genome-wide-significant level (P < 5 × 10(-8)). Many of these SNPs are near genes involved in balance, and eye, ear and cranial development (e.g. PVRL3, TSHZ1, MUTED, HOXB3, HOXD3). Other SNPs may affect motion sickness through nearby genes with roles in the nervous system, glucose homeostasis or hypoxia. We show that several of these SNPs display sex-specific effects, with up to three times stronger effects in women. We searched for comorbid phenotypes with motion sickness, confirming associations with known comorbidities including migraines, postoperative nausea and vomiting (PONV), vertigo and morning sickness and observing new associations with altitude sickness and many gastrointestinal conditions. We also show that two of these related phenotypes (PONV and migraines) share underlying genetic factors with motion sickness. These results point to the importance of the nervous system in motion sickness and suggest a role for glucose levels in motion-induced nausea and vomiting, a finding that may provide insight into other nausea-related phenotypes like PONV. They also highlight personal characteristics (e.g. being a poor sleeper) that correlate with motion sickness, findings that could help identify risk factors or treatments. PMID:25628336

  8. Mice lacking glutamate carboxypeptidase II develop normally, but are less susceptible to traumatic brain injury.

    PubMed

    Gao, Yang; Xu, Siyi; Cui, Zhenwen; Zhang, Mingkun; Lin, Yingying; Cai, Lei; Wang, Zhugang; Luo, Xingguang; Zheng, Yan; Wang, Yong; Luo, Qizhong; Jiang, Jiyao; Neale, Joseph H; Zhong, Chunlong

    2015-07-01

    Glutamate carboxypeptidase II (GCPII) is a transmembrane zinc metallopeptidase found mainly in the nervous system, prostate and small intestine. In the nervous system, glia-bound GCPII mediates the hydrolysis of the neurotransmitter N-acetylaspartylglutamate (NAAG) into glutamate and N-acetylaspartate. Inhibition of GCPII has been shown to attenuate excitotoxicity associated with enhanced glutamate transmission under pathological conditions. However, different strains of mice lacking the GCPII gene are reported to exhibit striking phenotypic differences. In this study, a GCPII gene knockout (KO) strategy involved removing exons 3-5 of GCPII. This generated a new GCPII KO mice line with no overt differences in standard neurological behavior compared to their wild-type (WT) littermates. However, GCPII KO mice were significantly less susceptible to moderate traumatic brain injury (TBI). GCPII gene KO significantly lessened neuronal degeneration and astrocyte damage in the CA2 and CA3 regions of the hippocampus 24 h after moderate TBI. In addition, GCPII gene KO reduced TBI-induced deficits in long-term spatial learning/memory tested in the Morris water maze and motor balance tested via beam walking. Knockout of the GCPII gene is not embryonic lethal and affords histopathological protection with improved long-term behavioral outcomes after TBI, a result that further validates GCPII as a target for drug development consistent with results from studies using GCPII peptidase inhibitors. PMID:25872793

  9. Neurological manifestations of filarial infections.

    PubMed

    Bhalla, Devender; Dumas, Michel; Preux, Pierre-Marie

    2013-01-01

    Filarial infections cause a huge public health burden wherever they are endemic. These filaria may locate anywhere in the human body. Their manifestations and pathogenic mechanisms, except the most common ones, are rarely investigated systematically. Their neurological manifestations, however, are being increasingly recognized particularly with onchocerciasis or Loa loa infections, Wuchereria bancrofti, or Mansonella perstans. The risk of developing these manifestations may also increase in cases that harbor multiple filariasis or coinfections, for instance as with Plasmodium. The microfilaria of Onchocerca and Loa loa are seen in cerebrospinal fluid. The pathogenesis of neurological manifestations of these infections is complex; however, pathogenic reactions may be caused by mechanical disruption, e.g., degeneration often followed by granulomas, causing fibrosis or mass effects on other tissues, vascular lesions, e.g., vascular block of cerebral vessels, or disordered inflammatory responses resulting in meningitis, encephalitis or localized inflammatory responses. The chances of having neurological manifestations may also depend upon the frequency and"heaviness"of infection over a lifetime. Hence, this type of infection should no longer be considered a disease of the commonly affected areas but one that may produce systemic effects or other manifestations, and these should be considered in populations where they are endemic. PMID:23829914

  10. Magnetic resonance imaging of iron deposition in neurological disorders.

    PubMed

    Brass, Steven D; Chen, Nan-kuei; Mulkern, Robert V; Bakshi, Rohit

    2006-02-01

    Deposition of iron in the brain is proposed to play a role in the pathophysiology of the normal aging process and neurodegenerative diseases. Whereas iron is required for normal neuronal metabolism, excessive levels can contribute to the formation of free radicals, leading to lipid peroxidation and neurotoxicity. Magnetic resonance imaging (MRI) is a powerful tool to detect excessive iron in the brain and longitudinally monitor changes in iron levels. Iron deposition is associated with a reduction in the T2 relaxation time, leading to hypointensity on spin-echo and gradient-echo T2-weighted images. The MRI changes associated with iron deposition have been observed both in normal aging and in various chronic neurological diseases, including multiple sclerosis, Alzheimer disease, and Parkinson disease. Magnetic resonance imaging metrics providing information about iron concentrations include R2, R2', and R2*. The purpose of this review is to discuss the role of iron and its detection by MRI in various neurological disorders. We will review the basic biochemical properties of iron and its influence on MRI signal. We will also summarize the sensitivity and specificity of MRI techniques in detecting iron. The MRI and pathological findings pertaining to brain iron will be reviewed with respect to normal aging and a variety of neurological disorders. Finally, the biochemistry and pathophysiology surrounding iron, oxidative stress, free radicals, and lipid peroxidation in the brain will be discussed, including therapeutic implications. The potential role of iron deposition and its assessment by MRI provides exciting potential applications to the diagnosis, longitudinal monitoring, and therapeutic development for disorders of the brain.

  11. Infant neurologic assessment.

    PubMed

    Hobdell, E

    2001-08-01

    Infant neurologic assessment reflects the ongoing maturation of the central nervous system. Traditional approaches to assessment cannot be used. Key factors are accurate observation and flexibility in obtaining the data. A case example using a 4-month-old infant illustrates specific approaches to assessment. PMID:11497071

  12. Ravel's neurological illness.

    PubMed

    Alonso, R J; Pascuzzi, R M

    1999-01-01

    In the last 10 years of his life, Maurice Ravel (1875-1937) experienced a gradually progressive decline in neurological function. Dr. Alajouanine examined Ravel, noting the presence of aphasia and apraxia with relative preservation of comprehension and memory. The exact diagnosis remains unclear, but the likelihood of a progressive degenerative disorder, such as frontotemporal dementia, is herein discussed. PMID:10718529

  13. Roles of Circular RNAs in Neurologic Disease

    PubMed Central

    Shao, Yiye; Chen, Yinghui

    2016-01-01

    Circular RNAs (circRNAs) are a novel type of endogenous noncoding RNA receiving increasing attention. They have been shown to act as a natural microRNA sponges that repress the activity of corresponding miRNAs by binding with them, thus regulating target genes. Numerous studies have shown that miRNAs are involved in the pathogenesis of neurological diseases. Therefore, circRNAs may act as important regulatory factors in the occurrence and development processes of neurological disease. PMID:27147959

  14. Neurological diseases and pain

    PubMed Central

    2012-01-01

    Chronic pain is a frequent component of many neurological disorders, affecting 20–40% of patients for many primary neurological diseases. These diseases result from a wide range of pathophysiologies including traumatic injury to the central nervous system, neurodegeneration and neuroinflammation, and exploring the aetiology of pain in these disorders is an opportunity to achieve new insight into pain processing. Whether pain originates in the central or peripheral nervous system, it frequently becomes centralized through maladaptive responses within the central nervous system that can profoundly alter brain systems and thereby behaviour (e.g. depression). Chronic pain should thus be considered a brain disease in which alterations in neural networks affect multiple aspects of brain function, structure and chemistry. The study and treatment of this disease is greatly complicated by the lack of objective measures for either the symptoms or the underlying mechanisms of chronic pain. In pain associated with neurological disease, it is sometimes difficult to obtain even a subjective evaluation of pain, as is the case for patients in a vegetative state or end-stage Alzheimer's disease. It is critical that neurologists become more involved in chronic pain treatment and research (already significant in the fields of migraine and peripheral neuropathies). To achieve this goal, greater efforts are needed to enhance training for neurologists in pain treatment and promote greater interest in the field. This review describes examples of pain in different neurological diseases including primary neurological pain conditions, discusses the therapeutic potential of brain-targeted therapies and highlights the need for objective measures of pain. PMID:22067541

  15. [Certification for specialists on neurology by Japanese Society of Neurology].

    PubMed

    Suzuki, Norihiro

    2009-11-01

    In accordance with recent ever increasing numbers in elder population in Japan, number of patients of age-related neurological diseases such as stroke, dementia and neurodegenerative diseases, etc., also remarkably increasing. Naturally, social needs for medical intervention in neurological fields inevitably become indispensable. The role of the neurologists, especially specialists of neurology, must be substantially important in the near future. The Japanese Society of Neurology has already launched the system for quality-certified specialists for neurology in 1970's. The aim of the system to educate and certify specialists for neurology has been announced as follows; the specialist of neurology must widely experienced and practiced in clinical fields, and must properly diagnose and judge the neurological illness even they are so complicate and difficult to manage (Rinsho Shinkeigaku (Clinical Neurology) 38 (6): 593-619, 1998). However, in future, the specialists for neurology must cultivate and keep the minds of "Professionalism" of physicians as well as their skills for clinical neurology. The Professionalism consists of altruism, accountability, excellence, duty, honor and integrity, respect and a personal commitment to life-long learning (ABIM: American Board of Internal Medicine, Project Professionalism, 1990-). The specialists of neurology with recent privilege in clinical insurance system for their special ability and techniques for neurological examination, should not only share their clinical specialty but also provide their opinion based upon Professionalism to all over the world. PMID:20030199

  16. Development and Growth of the Normal Cranial Vault : An Embryologic Review

    PubMed Central

    Jin, Sung-Won; Kim, Sang-Dae

    2016-01-01

    Understanding the development of a skull deformity requires an understanding of the normal morphogenesis of the cranium. Craniosynostosis is the premature, pathologic ossification of one or more cranial sutures leading to skull deformities. A review of the English medical literature using textbooks and standard search engines was performed to gather information about the prenatal development and growth of the cranial vault of the neurocranium. A process of morphogenic sequencing begins during prenatal development and growth, continues postnatally, and contributes to the basis for the differential manner of growth of cranial vault bones. This improved knowledge might facilitate comprehension of the pathophysiology of craniosynostosis. PMID:27226848

  17. Neurology outside Paris following Charcot.

    PubMed

    Moulin, Thierry; Clarac, François; Petit, Henri; Broussolle, Emmanuel

    2011-01-01

    The Middle Ages saw the development of numerous universities in the different provinces that later became the kingdom of France. In 1794, Napoleon I established 3 medical schools in Paris, Montpellier and Strasbourg, which were transformed into medical faculties in 1808. France had always been a highly centralized country, but during the 19th century, this trend started to change with the creation of medical faculties in Nancy (1872), Lille (1877), Lyon (1878), Bordeaux (1879), Toulouse (1891), Algiers (1910) and Marseille (1930). Following the creation of the 12 foundation courses, specialized chairs were progressively established in Paris, but for a long time this remained restricted to the French capital. However, with the emergence of medicine as an academic discipline in several towns outside Paris, came the development of neurology. This was greatly influenced by former students of Jean-Martin Charcot, local personalities, and the interactions between the two. Leading figures included Albert Pitres in Bordeaux, Léon Ingelrans in Lille, Eugène Devic and Jules Froment in Lyon, Lucien Cornil in Marseille, Joseph Grasset in Montpellier, and Marcel Riser in Toulouse. The interaction between French and Germanic medical communities also developed at this turbulent time under the influence of several great physicians such as Wilhelm Waldeyer, Adolf Kussmaul, and later Jean Alexandre Barré in Strasbourg, and Hippolyte Bernheim in Nancy. There are a number of other university towns outside Paris in which the development of neurology was probably influenced by the same interactions with psychiatry. It would be worth carrying out a thorough analysis of these towns in order to present an exhaustive overview of the development of neurology in France.

  18. The development of a normalization method for comparing nerve regeneration effectiveness among different graft types.

    PubMed

    Chang, Wei; DeVince, Jeffrey; Green, Gabriella; Shah, Munish Bhupendra; Johns, Michael S; Meng, Yan; Yu, Xiaojun

    2013-12-01

    The inability to compare directly different nerve grafts has been a significant factor hindering the advance of nerve graft development. Due to the abundance of variables that exist in nerve graft construction and multiple assessment types, there has been limited success in comparing nerve graft effectiveness among experiments. Using mathematical techniques on nerve conduction velocity (NCV) autograft data, a normalization function was empirically derived that normalizes differences in gap lengths. Further analysis allowed for the development of the relative regeneration ratio (RRR). The RRR function allows researchers to directly compare nerve graft results based on the NCV data from their respective studies as long as the data was collected at the same post-operation time. This function also allows for comparisons between grafts tested at different gap lengths. Initial testing of this RRR function provided confidence that the function is accurate for a continuum of gap lengths and different nerve graft types. PMID:24118184

  19. Nuclear Medicine Imaging in Pediatric Neurology

    PubMed Central

    Akdemir, Ümit Özgür; Atay Kapucu, Lütfiye Özlem

    2016-01-01

    Nuclear medicine imaging can provide important complementary information in the management of pediatric patients with neurological diseases. Pre-surgical localization of the epileptogenic focus in medically refractory epilepsy patients is the most common indication for nuclear medicine imaging in pediatric neurology. In patients with temporal lobe epilepsy, nuclear medicine imaging is particularly useful when magnetic resonance imaging findings are normal or its findings are discordant with electroencephalogram findings. In pediatric patients with brain tumors, nuclear medicine imaging can be clinically helpful in the diagnosis, directing biopsy, planning therapy, differentiating tumor recurrence from post-treatment sequelae, and assessment of response to therapy. Among other neurological diseases in which nuclear medicine has proved to be useful are patients with head trauma, inflammatory-infectious diseases and hypoxic-ischemic encephalopathy. PMID:27299282

  20. Astrocytes: The missing link in neurological disease?

    PubMed Central

    Lin, Chia-Ching John; Deneen, Benjamin

    2013-01-01

    The central nervous system (CNS) is comprised of numerous cell types that work in concert to facilitate proper function and homeostasis. Disruption of these carefully orchestrated networks results in neuronal dysfunction, manifesting itself in a variety of neurological disorders. While neuronal dysregulation is causative of symptoms manifest in the clinic, the etiology of these disorders is often more complex than simply a loss of neurons or intrinsic dysregulation of their function. In the adult brain, astrocytes comprise the most abundant cell type and play key roles in CNS physiology, therefore it stands to reason that dysregulation of normal astrocyte function contributes to the etiology and progression of varied neurological disorders. We review here some neurological disorders associated with an astrocyte factor and discuss how the related astrocyte dysfunction contributes to the etiology and/or progression of these disorders. PMID:24365571

  1. Nuclear Medicine Imaging in Pediatric Neurology.

    PubMed

    Akdemir, Ümit Özgür; Atay Kapucu, Lütfiye Özlem

    2016-02-01

    Nuclear medicine imaging can provide important complementary information in the management of pediatric patients with neurological diseases. Pre-surgical localization of the epileptogenic focus in medically refractory epilepsy patients is the most common indication for nuclear medicine imaging in pediatric neurology. In patients with temporal lobe epilepsy, nuclear medicine imaging is particularly useful when magnetic resonance imaging findings are normal or its findings are discordant with electroencephalogram findings. In pediatric patients with brain tumors, nuclear medicine imaging can be clinically helpful in the diagnosis, directing biopsy, planning therapy, differentiating tumor recurrence from post-treatment sequelae, and assessment of response to therapy. Among other neurological diseases in which nuclear medicine has proved to be useful are patients with head trauma, inflammatory-infectious diseases and hypoxic-ischemic encephalopathy.

  2. Ultrasonography of the infant hip. Part IV: Normal development in the newborn and preterm neonate.

    PubMed

    Zieger, M; Hilpert, S

    1987-01-01

    Based on preterm and term infants studied by ultrasonography, and on anatomical sections of various gestational ages the physiological maturation of the hip joint is analysed. Current concepts of a linear growth pattern with an arrest immediately after delivery are confirmed. A more rapid growth and ossification of the acetabular edge than of the femoral head postpartum is suggested. To avoid overtreatment, knowledge of the normal range of development as seen in ultrasonography is mandatory.

  3. Setting the agenda for neurological nursing: strategic directions.

    PubMed

    Smith, Lorraine N

    2006-11-01

    This paper explores a range of issues related to neurological care. The scope and scale of neurological conditions is described in order to illustrate disparities in research funding and care delivery as compared with cancer and cardiovascular disease. Financial implications, ethical issues and health service development are outlined as a context for the state of the art of neurological nursing. Areas for potential neurological nursing research are identified. Finally, it is argued that policy and research must be linked if neurological care, research and education are to receive greater resource allocation.

  4. Connective Tissue Growth Factor Is Required for Normal Follicle Development and Ovulation

    PubMed Central

    Nagashima, Takashi; Kim, Jaeyeon; Li, Qinglei; Lydon, John P.; DeMayo, Francesco J.; Lyons, Karen M.

    2011-01-01

    Connective tissue growth factor (CTGF) is a cysteine-rich protein the synthesis and secretion of which are hypothesized to be selectively regulated by activins and other members of the TGF-β superfamily. To investigate the in vivo roles of CTGF in female reproduction, we generated Ctgf ovarian and uterine conditional knockout (cKO) mice. Ctgf cKO mice exhibit severe subfertility and multiple reproductive defects including disrupted follicle development, decreased ovulation rates, increased numbers of corpus luteum, and smaller but functionally normal uterine horns. Steroidogenesis is disrupted in the Ctgf cKO mice, leading to increased levels of serum progesterone. We show that disrupted follicle development is accompanied by a significant increase in granulosa cell apoptosis. Moreover, despite normal cumulus expansion, Ctgf cKO mice exhibit a significant decrease in oocytes ovulated, likely due to impaired ovulatory process. During analyses of mRNA expression, we discovered that Ctgf cKO granulosa cells show gene expression changes similar to our previously reported granulosa cell-specific knockouts of activin and Smad4, the common TGF-β family intracellular signaling protein. We also discovered a significant down-regulation of Adamts1, a progesterone-regulated gene that is critical for the remodeling of extracellular matrix surrounding granulosa cells of preovulatory follicles. These findings demonstrate that CTGF is a downstream mediator in TGF-β and progesterone signaling cascades and is necessary for normal follicle development and ovulation. PMID:21868453

  5. Self-regulation during pretend play in children with intellectual disability and in normally developing children.

    PubMed

    Vieillevoye, Sandrine; Nader-Grosbois, Nathalie

    2008-01-01

    This study investigated the symbolic behavior and the self-regulation in dyads of children with intellectual disability and of normally developing children. Specifically, these processes were studied in link with the children's characteristics (mental age, linguistic level, individual pretend play level). The sample included 80 participants, 40 children with intellectual disability and 40 normally developing children, matched according to their mental age, ranged from 3 to 6 years old. First, a developmental assessment was performed (about cognitive, language and pretend play dimensions); then, in peers dyads, the children were elicited to pretend play by means of four kinds of material referring to four types of scripts (tea party, doctor, transportation, substitute objects eliciting creativity). The average symbolic behavior in individual and dyadic play contexts did not differ in both groups, but the average self-regulation in the group with intellectual disability was lower than in the normally developing group. Some positive partial correlations were obtained between mental age, language abilities, individual pretend play, dyadic pretend play and several self-regulatory strategies in both groups although they varied in importance between groups. Clusters analyses showed that individual and dyadic pretend play explained self-regulation in children of both groups. Specifically, in both groups, the higher was symbolic behavior in creativity context, the higher was self-regulation.

  6. Self-regulation during pretend play in children with intellectual disability and in normally developing children.

    PubMed

    Vieillevoye, Sandrine; Nader-Grosbois, Nathalie

    2008-01-01

    This study investigated the symbolic behavior and the self-regulation in dyads of children with intellectual disability and of normally developing children. Specifically, these processes were studied in link with the children's characteristics (mental age, linguistic level, individual pretend play level). The sample included 80 participants, 40 children with intellectual disability and 40 normally developing children, matched according to their mental age, ranged from 3 to 6 years old. First, a developmental assessment was performed (about cognitive, language and pretend play dimensions); then, in peers dyads, the children were elicited to pretend play by means of four kinds of material referring to four types of scripts (tea party, doctor, transportation, substitute objects eliciting creativity). The average symbolic behavior in individual and dyadic play contexts did not differ in both groups, but the average self-regulation in the group with intellectual disability was lower than in the normally developing group. Some positive partial correlations were obtained between mental age, language abilities, individual pretend play, dyadic pretend play and several self-regulatory strategies in both groups although they varied in importance between groups. Clusters analyses showed that individual and dyadic pretend play explained self-regulation in children of both groups. Specifically, in both groups, the higher was symbolic behavior in creativity context, the higher was self-regulation. PMID:17576048

  7. Learning with sublexical information from emerging reading vocabularies in exceptionally early and normal reading development.

    PubMed

    Thompson, G Brian; Fletcher-Flinn, Claire M; Wilson, Kathryn J; McKay, Michael F; Margrain, Valerie G

    2015-03-01

    Predictions from theories of the processes of word reading acquisition have rarely been tested against evidence from exceptionally early readers. The theories of Ehri, Share, and Byrne, and an alternative, Knowledge Sources theory, were so tested. The former three theories postulate that full development of context-free letter sounds and awareness of phonemes are required for normal acquisition, while the claim of the alternative is that with or without such, children can use sublexical information from their emerging reading vocabularies to acquire word reading. Results from two independent samples of children aged 3-5, and 5 years, with mean word reading levels of 7 and 9 years respectively, showed underdevelopment of their context-free letter sounds and phoneme awareness, relative to their word reading levels and normal comparison samples. Despite such underdevelopment, these exceptional readers engaged in a form of phonological recoding that enabled pseudoword reading, at the level of older-age normal controls matched on word reading level. Moreover, in the 5-year-old sample further experiments showed that, relative to normal controls, they had a bias toward use of sublexical information from their reading vocabularies for phonological recoding of heterophonic pseudowords with irregular consistent spelling, and were superior in accessing word meanings independently of phonology, although only if the readers were without exposure to explicit phonics. The three theories were less satisfactory than the alternative theory in accounting for the learning of the exceptionally early readers.

  8. Expression and activity of L-Myc in normal mouse development.

    PubMed Central

    Hatton, K S; Mahon, K; Chin, L; Chiu, F C; Lee, H W; Peng, D; Morgenbesser, S D; Horner, J; DePinho, R A

    1996-01-01

    To determine the role of L-Myc in normal mammalian development and its functional relationship to other members of the Myc family, we determined the normal patterns of L-myc gene expression in the developing mouse by RNA in situ hybridization and assessed the phenotypic impact of L-Myc deficiency produced through standard gene targeting methodology. L-myc transcripts were detected in the developing kidney and lung as well as in both the proliferative and the differentiative zones of the brain and neural tube. Despite significant expression of L-myc in developing mouse tissue, homozygous null L-myc mice were found to be viable, reproductively competent, and represented in expected frequencies from heterozygous matings. A detailed histological survey of embryonic and adult tissues, characterization of an embryonic neuronal marker, and measurement of cellular proliferation in situ did not reveal any congenital abnormalities. The lack of an apparent phenotype associated with L-Myc deficiency indicates that L-Myc is dispensable for gross morphological development and argues against a unique role for L-Myc in early central nervous system development as had been previously suggested. Although overlapping expression patterns among myc family members raise the possibility of complementation of L-Myc deficiency by other Myc oncoproteins, compensatory changes in the levels of c- and/or N-myc transcripts were not detected in homozygous null L-myc mice. PMID:8657155

  9. Neurology goes global

    PubMed Central

    Mateen, Farrah J.

    2014-01-01

    Summary In recent years, the need for additional neurologists and neurologic expertise in many low- and middle-income countries (LMIC) has become more apparent. Many organizations are committed to this unmet need, but the scope of the problem remains mostly underappreciated. Neurologists may be skeptical about their value in resource-limited settings, yet we are critically needed and can have a marked effect. International experiences, however, must be carried out in ethical, informed, and sustainable ways in tandem with local health care providers when possible. We present a brief overview of critical issues in global neurology, the importance of focusing on benefits to the LMIC, and options for volunteer opportunities in clinical service, education, research, and disaster relief. Finally, we offer practical pointers and resources for planning these experiences. PMID:25110621

  10. Insomnia in neurological diseases.

    PubMed

    Provini, Federica; Lombardi, Carolina; Lugaresi, Elio

    2005-03-01

    Insomnia is the most common sleep complaint. Insomnia is not a disease itself but mostly a clinical sign of an underlying disease. Degenerative and vascular diseases involving the central nervous system (CNS) may impair sleep either as a result of the brain lesion or because of illness-related discomfort (motor immobility, social and familial impairment, depression, drugs). Some neurological conditions characterized by movement disorders that start or persist during sleep hinder sleep onset and/or sleep continuity, causing a poor sleep complaint. CNS lesions and/or dysfunction in three specific neurological conditions (fatal familial insomnia, Morvan's chorea, and delirium tremens) impair the basic mechanisms of sleep generation inducing a syndrome in which the inability to sleep is consistently associated with motor and sympathergic overactivation. Agrypnia excitata is the term that aptly defines this generalized overactivation syndrome.

  11. Key sleep neurologic disorders

    PubMed Central

    St. Louis, Erik K.

    2014-01-01

    Summary Sleep disorders are frequent comorbidities in neurologic patients. This review focuses on clinical aspects and prognosis of 3 neurologic sleep disorders: narcolepsy, restless legs syndrome/Willis-Ekbom disease (RLS/WED), and REM sleep behavior disorder (RBD). Narcolepsy causes pervasive, enduring excessive daytime sleepiness, adversely affecting patients' daily functioning. RLS/WED is characterized by an uncomfortable urge to move the legs before sleep, often evolving toward augmentation and resulting in daylong bothersome symptoms. RBD causes potentially injurious dream enactment behaviors that often signify future evolution of overt synucleinopathy neurodegeneration in as many as 81% of patients. Timely recognition, referral for polysomnography, and longitudinal follow-up of narcolepsy, RLS/WED, and RBD patients are imperatives for neurologists in providing quality comprehensive patient care. PMID:24605270

  12. Cranial index of children with normal and abnormal brain development in Sokoto, Nigeria: A comparative study

    PubMed Central

    Musa, Muhammad Awwal; Zagga, Abdullahi Daudu; Danfulani, Mohammed; Tadros, Aziz Abdo; Ahmed, Hamid

    2014-01-01

    Background: Abnormal brain development due to neurodevelopmental disorders in children has always been an important concern, but yet has to be considered as a significant public health problem, especially in the low- and middle-income countries including Nigeria. Aims: The aim of this study is to determine whether abnormal brain development in the form of neurodevelopmental disorders causes any deviation in the cranial index of affected children. Materials and Methods: This is a comparative study on the head length, head width, and cranial index of 112 children (72 males and 40 females) diagnosed with at least one abnormal problem in brain development, in the form of a neurodevelopmental disorder (NDD), in comparison with that of 218 normal growing children without any form of NDD (121 males and 97 females), aged 0-18 years old seen at the Usmanu Danfodiyo University Teaching Hospital, Sokoto, over a period of six months, June to December, 2012. The head length and head width of the children was measured using standard anatomical landmarks and cranial index calculated. The data obtained was entered into the Microsoft excel worksheet and analyzed using SPSS version 17. Results: The mean Cephalic Index for normal growing children with normal brain development was 79.82 ± 3.35 and that of the children with abnormal brain development was 77.78 ± 2.95 and the difference between the two groups was not statistically significant (P > 0.05). Conclusion: It can be deduced from this present study that the cranial index does not change in children with neurodevelopmental disorders. PMID:24966551

  13. [Neurological Disorders and Pregnancy].

    PubMed

    Berlit, P

    2016-02-01

    Neurological disorders caused by pregnancy and puerperium include the posterior reversible encephalopathy syndrome, the amniotic fluid embolism syndrome (AFES), the postpartum angiopathy due to reversible vasoconstriction syndrome, and the Sheehan syndrome. Hypertension and proteinuria are the hallmarks of preeclampsia, seizures define eclampsia. Hemolysis, elevated liver enzymes and low platelets constitute the HELLP syndrome. Vision disturbances including cortical blindness occur in the posterior reversible encephalopathy syndrome (PRES). The Sheehan syndrome presents with panhypopituitarism post partum due to apoplexia of the pituitary gland in severe peripartal blood loss leading to longstanding hypotension. Some neurological disorders occur during pregnancy and puerperium with an increased frequency. These include stroke, sinus thrombosis, the restless legs syndrome and peripheral nerve syndromes, especially the carpal tunnel syndrome. Chronic neurologic diseases need an interdisciplinary approach during pregnancy. Some anticonvulsants double the risk of birth defects. The highest risk exists for valproic acid, the lowest for lamotrigine and levetiracetam. For MS interval treatment, glatiramer acetate and interferones seem to be safe during pregnancy. All other drugs should be avoided. PMID:26953551

  14. Prenatal diagnosis of trisomy 12 mosaicism: normal development of a 3 years old female child.

    PubMed

    Staals, J E A; Schrander-Stumpel, C T R M; Hamers, G; Fryns, J P

    2003-01-01

    Trisomy 12 mosaicism is a rare chromosomal mosaicism in prenatal diagnosis by amniocentesis. In the literature we found at least 27 cases. 13 Pregnancies were terminated, with multiple congenital anomalies (MCA) in 2 out of 13. Of the 12 liveborns with follow-up ranging from 0 to 5 years, 5 presented MCA and died within the first weeks. 2 Fetus died during pregnancy and further data are lacking. A normal outcome, with limited follow up however, was reported in 7/12 liveborns without congenital anomalies and is well demonstrated in the presently reported girl. We describe the 3-years follow up in a girl with trisomy 12 mosaicism, detected by amniocentesis for advanced maternal age. She is a healthy girl with normal physical and psychomotor development.

  15. Rett syndrome: disruption of epigenetic control of postnatal neurological functions.

    PubMed

    Pohodich, Amy E; Zoghbi, Huda Y

    2015-10-15

    Loss-of-function mutations in the X-linked gene Methyl-CpG-binding protein 2 (MECP2) cause a devastating pediatric neurological disorder called Rett syndrome. In males, these mutations typically result in severe neonatal encephalopathy and early lethality. On the other hand, owing to expression of the normal allele in ∼50% of cells, females do not suffer encephalopathy but instead develop Rett syndrome. Typically females with Rett syndrome exhibit a delayed onset of neurologic dysfunction that manifests around the child's first birthday and progresses over the next few years. Features of this disorder include loss of acquired language and motor skills, intellectual impairment and hand stereotypies. The developmental regression observed in patients with Rett syndrome arises from altered neuronal function and is not the result of neurodegeneration. Maintenance of an appropriate level of MeCP2 appears integral to the function of healthy neurons as patients with increased levels of MeCP2, owing to duplication of the Xq28 region encompassing the MECP2 locus, also present with intellectual disability and progressive neurologic symptoms. Despite major efforts over the past two decades to elucidate the molecular functions of MeCP2, the mechanisms underlying the delayed appearance of symptoms remain unclear. In this review, we will highlight recent findings that have expanded our knowledge of MeCP2's functions, and we will discuss how epigenetic regulation, chromatin organization and circuit dynamics may contribute to the postnatal onset of Rett syndrome.

  16. Postpartum cerebral angiopathy presenting with non-aneurysmal subarachnoid hemorrhage and interval development of neurological deficits: a case report and review of literature.

    PubMed

    Yang, Li; Bai, Harrison X; Zhao, Xin; Xiao, Yanqiao; Tan, Liming

    2013-01-01

    Postpartum cerebral angiopathy (PCA) is a cerebrovascular disease that occurs during the postpartum period. It is characterized by reversible multifocal vasoconstriction of the cerebral arteries. We report a patient with PCA proven by cerebral angiography that revealed multifocal, segmental narrowing of the cerebral arteries and non-aneurysmal subarachnoid hemorrhage. The patient suddenly deteriorated with focal neurological deficits on the 5 th day of hospitalization. She was treated with calcium-channel blockers and monitored with daily transcranial Doppler ultrasound. Her symptoms gradually improved and she was discharged on the 11 th day of hospitalization. At 1-month follow-up, patient was completely symptom-free with no neurological deficits.

  17. Flower development in normal tomato and a gibberellin-deficient (ga-2) mutant

    SciTech Connect

    Vester, J.E.; Zeevaart, J.A.D. )

    1988-01-01

    Flower buds of a tomato (Lycopersicon esculentum Mill.) gibberellin-deficient mutant (ga-2/ga-2) were initiated, but did not develop to maturity and eventually aborted. If GA{sub 1} was applied to a developing inflorescence or stem tip, completion of flower bud development and fruit set occurred. In development of the ga-2 flowers, the corolla and stamens did not elongate and the style was misshapen or extended past the tip of the anthers. Light microscope observation indicated that meiosis of both microsporocytes and megasporocytes did not occur. Cells of the sporogenous layer were initiated, but growth was arrested and they eventually degenerated. The ovary was normal in appearance. However, the megasporocytes degenerated, giving rise to a cavity in the ovule. Thus, although GA is not required for flower initiation in tomato, it is essential for meiosis of the microsporocytes and megasporocytes and elongation of the corolla and stamens.

  18. Apical Secretion of FSTL1 in the Respiratory Epithelium for Normal Lung Development

    PubMed Central

    Li, Xue; Liang, Jiurong; Jiang, Dianhua; Geng, Yan; Ning, Wen

    2016-01-01

    Follistatin-like 1 (FSTL1) is a secreted bone morphogenetic protein (BMP) antagonist, and it plays a crucial role in normal lung development. Deletion of Fstl1 leads to postnatal death in mice due to respiratory failure. To further explore the role of FSTL1 in mouse lung development, we created a transgene SFTPC-Fstl1 allele mouse displaying significant epithelial overexpression of Fstl1 in all stages of lung development. However, epithelial overexpression of Fstl1 did not alter lung morphogenesis, epithelial differentiation and lung function. Moreover, we found that FSTL1 function was blocked by the epithelial polarization, which was reflected by the remarkable apical secretion of FSTL1 and the basolateral BMP signaling. Taken together, this study demonstrates that tightly spatial interaction of FSTL1 and BMP signaling plays an essential role in lung development. PMID:27355685

  19. New animal models to study the role of tyrosinase in normal retinal development.

    PubMed

    Lavado, Alfonso; Montoliu, Lluis

    2006-01-01

    Albino animals display a hypopigmented phenotype associated with several visual abnormalities, including rod photoreceptor cell deficits, abnormal patterns of connections between the eye and the brain and a general underdevelopment of central retina. Oculocutaneous albinism type I, a common form of albinism, is caused by mutations in the tyrosinase gene. In mice, the albino phenotype can be corrected by functional tyrosinase transgenes. Tyrosinase transgenic animals not only show normal pigmentation but the correction of all visual abnormalities associated with albinism, confirming a role of tyrosinase, a key enzyme in melanin biosynthesis, in normal retinal development. Here, we will discuss recent work carried out with new tyrosinase transgenic mouse models, to further analyse the role of tyrosinase in retinal development. We will first report a transgenic model with inducible tyrosinase expression that has been used to address the regulated activation of this gene and its associated effects on the development of the visual system. Second, we will comment on an interesting yeast artificial chromosome (YAC)-tyrosinase transgene, lacking important regulatory elements, that has highlighted the significance of local interactions between the retinal pigment epithelium (RPE) and developing neural retina.

  20. Cdk4 deficiency inhibits skin tumor development but does not affect normal keratinocyte proliferation.

    PubMed

    Rodriguez-Puebla, Marcelo L; Miliani de Marval, Paula L; LaCava, Margaret; Moons, David S; Kiyokawa, Hiroaki; Conti, Claudio J

    2002-08-01

    Most human tumors have mutations that result in deregulation of the cdk4/cyclin-Ink4-Rb pathway. Overexpression of D-type cyclins or cdk4 and inactivation of Ink4 inhibitors are common in human tumors. Conversely, lack of cyclin D1 expression results in significant reduction in mouse skin and mammary tumor development. However, complete elimination of tumor development was not observed in these models, suggesting that other cyclin/cdk complexes play an important role in tumorigenesis. Here we described the effects of cdk4 deficiency on mouse skin proliferation and tumor development. Cdk4 deficiency resulted in a 98% reduction in the number of tumors generated through the two-stage carcinogenesis model. The absence of cdk4 did not affect normal keratinocyte proliferation and both wild-type and cdk4 knockout epidermis are equally affected after topical treatment with the tumor promoter 12-O-tetradecanoylphorbol-13-acetate (TPA), resulting in epidermal hyperplasia. In similar fashion, cdk4 knockout keratinocytes proliferated well in an in vivo model of wound-induced proliferation. Biochemical studies in mouse epidermis showed that cdk6 activity increased twofold in cdk4-deficient mice compared to wild-type siblings. These results suggest that therapeutic approaches to inhibit cdk4 activity could provide a target to inhibit tumor development with minimal or no effect in normal tissue.

  1. cdk4 Deficiency Inhibits Skin Tumor Development but Does Not Affect Normal Keratinocyte Proliferation

    PubMed Central

    Rodriguez-Puebla, Marcelo L.; Miliani de Marval, Paula L.; LaCava, Margaret; Moons, David S.; Kiyokawa, Hiroaki; Conti, Claudio J.

    2002-01-01

    Most human tumors have mutations that result in deregulation of the cdk4/cyclin-Ink4-Rb pathway. Overexpression of D-type cyclins or cdk4 and inactivation of Ink4 inhibitors are common in human tumors. Conversely, lack of cyclin D1 expression results in significant reduction in mouse skin and mammary tumor development. However, complete elimination of tumor development was not observed in these models, suggesting that other cyclin/cdk complexes play an important role in tumorigenesis. Here we described the effects of cdk4 deficiency on mouse skin proliferation and tumor development. Cdk4 deficiency resulted in a 98% reduction in the number of tumors generated through the two-stage carcinogenesis model. The absence of cdk4 did not affect normal keratinocyte proliferation and both wild-type and cdk4 knockout epidermis are equally affected after topical treatment with the tumor promoter 12-O-tetradecanoylphorbol-13-acetate (TPA), resulting in epidermal hyperplasia. In similar fashion, cdk4 knockout keratinocytes proliferated well in an in vivo model of wound-induced proliferation. Biochemical studies in mouse epidermis showed that cdk6 activity increased twofold in cdk4-deficient mice compared to wild-type siblings. These results suggest that therapeutic approaches to inhibit cdk4 activity could provide a target to inhibit tumor development with minimal or no effect in normal tissue. PMID:12163365

  2. mTOR signaling and its roles in normal and abnormal brain development

    PubMed Central

    Takei, Nobuyuki; Nawa, Hiroyuki

    2014-01-01

    Target of rapamycin (TOR) was first identified in yeast as a target molecule of rapamycin, an anti-fugal and immunosuppressant macrolide compound. In mammals, its orthologue is called mammalian TOR (mTOR). mTOR is a serine/threonine kinase that converges different extracellular stimuli, such as nutrients and growth factors, and diverges into several biochemical reactions, including translation, autophagy, transcription, and lipid synthesis among others. These biochemical reactions govern cell growth and cause cells to attain an anabolic state. Thus, the disruption of mTOR signaling is implicated in a wide array of diseases such as cancer, diabetes, and obesity. In the central nervous system, the mTOR signaling cascade is activated by nutrients, neurotrophic factors, and neurotransmitters that enhances protein (and possibly lipid) synthesis and suppresses autophagy. These processes contribute to normal neuronal growth by promoting their differentiation, neurite elongation and branching, and synaptic formation during development. Therefore, disruption of mTOR signaling may cause neuronal degeneration and abnormal neural development. While reduced mTOR signaling is associated with neurodegeneration, excess activation of mTOR signaling causes abnormal development of neurons and glia, leading to brain malformation. In this review, we first introduce the current state of molecular knowledge of mTOR complexes and signaling in general. We then describe mTOR activation in neurons, which leads to translational enhancement, and finally discuss the link between mTOR and normal/abnormal neuronal growth during development. PMID:24795562

  3. The beginnings of the Southern Child/Pediatric Neurology Society.

    PubMed

    Dyken, Paul Richard; Bodensteiner, John B

    2015-04-01

    The founding and early development of the Southern Pediatric Neurology Society was in many ways parallel to that of the Child Neurology Society. The organization started out as the Southern Child Neurology Society but the name was changed at the time of incorporation so as to avoid confusion of identity and purpose with the larger Child Neurology Society. Although there are archives of early days and the later development of the Southern Pediatric Neurology Society, the details have never been set down in a narrative explaining the events that led to the development of the organization. In this paper, we try to produce a written record of the history of the founding and early development of the Southern Pediatric Neurology Society.

  4. The human brain and face: mechanisms of cranial, neurological and facial development revealed through malformations of holoprosencephaly, cyclopia and aberrations in chromosome 18.

    PubMed

    Gondré-Lewis, Marjorie C; Gboluaje, Temitayo; Reid, Shaina N; Lin, Stephen; Wang, Paul; Green, William; Diogo, Rui; Fidélia-Lambert, Marie N; Herman, Mary M

    2015-09-01

    The study of inborn genetic errors can lend insight into mechanisms of normal human development and congenital malformations. Here, we present the first detailed comparison of cranial and neuro pathology in two exceedingly rare human individuals with cyclopia and alobar holoprosencephaly (HPE) in the presence and absence of aberrant chromosome 18 (aCh18). The aCh18 fetus contained one normal Ch18 and one with a pseudo-isodicentric duplication of chromosome 18q and partial deletion of 18p from 18p11.31 where the HPE gene, TGIF, resides, to the p terminus. In addition to synophthalmia, the aCh18 cyclopic malformations included a failure of induction of most of the telencephalon - closely approximating anencephaly, unchecked development of brain stem structures, near absence of the sphenoid bone and a malformed neurocranium and viscerocranium that constitute the median face. Although there was complete erasure of the olfactory and superior nasal structures, rudiments of nasal structures derived from the maxillary bone were evident, but with absent pharyngeal structures. The second non-aCh18 cyclopic fetus was initially classified as a true Cyclops, as it appeared to have a proboscis and one median eye with a single iris, but further analysis revealed two eye globes as expected for synophthalmic cyclopia. Furthermore, the proboscis was associated with the medial ethmoid ridge, consistent with an incomplete induction of these nasal structures, even as the nasal septum and paranasal sinuses were apparently developed. An important conclusion of this study is that it is the brain that predicts the overall configuration of the face, due to its influence on the development of surrounding skeletal structures. The present data using a combination of macroscopic, computed tomography (CT) and magnetic resonance imaging (MRI) techniques provide an unparalleled analysis on the extent of the effects of median defects, and insight into normal development and patterning of the brain

  5. The human brain and face: mechanisms of cranial, neurological and facial development revealed through malformations of holoprosencephaly, cyclopia and aberrations in chromosome 18

    PubMed Central

    Gondré-Lewis, Marjorie C.; Gboluaje, Temitayo; Reid, Shaina N.; Lin, Stephen; Wang, Paul; Green, William; Diogo, Rui; Fidélia-Lambert, Marie N.; Herman, Mary M.

    2016-01-01

    The study of inborn genetic errors can lend insight into mechanisms of normal human development and congenital malformations. Here, we present the first detailed comparison of cranial and neuro pathology in two exceedingly rare human individuals with cyclopia and alobar holoprosencephaly (HPE) in the presence and absence of aberrant chromosome 18 (aCh18). The aCh18 fetus contained one normal Ch18 and one with a pseudo-isodicentric duplication of chromosome 18q and partial deletion of 18p from 18p11.31 where the HPE gene, TGIF, resides, to the p terminus. In addition to synophthalmia, the aCh18 cyclopic malformations included a failure of induction of most of the telencephalon – closely approximating anencephaly, unchecked development of brain stem structures, near absence of the sphenoid bone and a malformed neurocranium and viscerocranium that constitute the median face. Although there was complete erasure of the olfactory and superior nasal structures, rudiments of nasal structures derived from the maxillary bone were evident, but with absent pharyngeal structures. The second non-aCh18 cyclopic fetus was initially classified as a true Cyclops, as it appeared to have a proboscis and one median eye with a single iris, but further analysis revealed two eye globes as expected for synophthalmic cyclopia. Furthermore, the proboscis was associated with the medial ethmoid ridge, consistent with an incomplete induction of these nasal structures, even as the nasal septum and paranasal sinuses were apparently developed. An important conclusion of this study is that it is the brain that predicts the overall configuration of the face, due to its influence on the development of surrounding skeletal structures. The present data using a combination of macroscopic, computed tomography (CT) and magnetic resonance imaging (MRI) techniques provide an unparalleled analysis on the extent of the effects of median defects, and insight into normal development and patterning of the

  6. Development of planning abilities in normal aging: differential effects of specific cognitive demands.

    PubMed

    Köstering, Lena; Stahl, Christoph; Leonhart, Rainer; Weiller, Cornelius; Kaller, Christoph P

    2014-01-01

    In line with the frontal hypothesis of aging, the ability to plan ahead undergoes substantial change during normal aging. Although impairments on the Tower of London planning task were reported earlier, associations between age-related declines and specific cognitive demands on planning have not been studied. Here we investigated the impact of search depth and goal ambiguity on planning, which impose demands on the depth and breadth of look-ahead processes, respectively. Besides an overall age-related decline in planning accuracy of 106 healthy older adults, differential search depth effects were found: Whereas planning accuracy of subjects in the early 60s was not affected by variations in search depth, between the ages of 65 and 76 years, accuracy was significantly decreased for high versus low levels of search depth. For subjects older than 76, different search depth levels did not further impact on accuracy, which was lowest overall. This nonlinear pattern may reflect differential impairments in fluid abilities and working memory capacity across various stages of older age. As no age-related effects of goal ambiguity were found, normal aging seems to be specifically sensitive to planning demands on the depth but not the breadth of anticipatory search processes. Hence, cognitive functions subserved by the prefrontal cortex experience differential development over the course of normal aging.

  7. Regulation of Differentiation by Calcium-Sensing Receptor in Normal and Tumoral Developing Nervous System

    PubMed Central

    Mateo-Lozano, Silvia; García, Marta; Rodríguez-Hernández, Carlos J.; de Torres, Carmen

    2016-01-01

    During normal development of the nervous system (NS), neural progenitor cells (NPCs) produce specialized populations of neurons and glial cells upon cell fate restriction and terminal differentiation. These sequential processes require the dynamic regulation of thousands of genes. The calcium-sensing receptor (CaSR) is temporally and spatially regulated in both neurons and glial cells during development of the NS. In particular, CaSR expression and function have been shown to play a significant role during differentiation of NPCs toward the oligodendrocyte lineage and also in maturation of cerebellar granule cell precursors (GCPs). Moreover, CaSR regulates axonal and dendritic growth in both central and peripheral nervous systems (PNSs), a process necessary for proper construction of mature neuronal networks. On the other hand, several lines of evidence support a role for CaSR in promotion of cell differentiation and inhibition of proliferation in neuroblastoma, a tumor arising from precursor cells of developing PNS. Thus, among the variety of NS functions in which the CaSR participates, this mini-review focuses on its role in differentiation of normal and tumoral cells. Current knowledge of the mechanisms responsible for CaSR regulation and function in these contexts is also discussed, together with the therapeutic opportunities provided by CaSR allosteric modulators. PMID:27242543

  8. TrkB is necessary for the normal development of the lung.

    PubMed

    García-Suárez, O; Pérez-Pinera, P; Laurà, R; Germana, A; Esteban, I; Cabo, R; Silos-Santiago, I; Cobo, J L; Vega, J A

    2009-07-31

    Normal development of the lung requires coordinated activation of cascades of signaling pathways initiated by growth factors signaling through their receptors. TrkB and its ligands, brain-derived neurotrophic factor (BDNF) and neurotrophin-4, belong to the neurotrophin family of growth factors, which are expressed in a large variety of non-neuronal tissues including the lung. Aberrant neurotrophin signaling underlies the pathogenesis of several lung-related pathologies, including asthma and lung cancer, however, little is known about the role of neurotrophins in the embryonic development of the lung. To fill this gap in knowledge, we analyzed the pattern of TrkB expression in the murine lung and we observed that TrkB is expressed in alveolar macrophages, type II pneumocytes, neuroepithelial bodies and nerves. Analysis of the structure of lung from mice deficient in TrkB revealed that absence of TrkB signaling results in thinner bronchial epithelium and apparent larger air space, and, more importantly, lack of neuroepithelial bodies, an important reduction in the density of nerve fibres in the bronchial smooth muscle, submucous plexus in bronchioles, and pulmonary artery walls. These findings suggest TrkB is essential for the normal development of the lung and the nervous system in the lung.

  9. Differential roles of ERα and ERβ in normal and neoplastic development in the mouse mammary gland.

    PubMed

    Mehta, Rajendra G; Hawthorne, Michael; Mehta, Rajeshwari R; Torres, Karen E O; Peng, Xinjian; McCormick, David L; Kopelovich, Levy

    2014-01-01

    The present experiments were performed to determine the roles of estrogen receptors α and β (ERα and ERβ) in normal and neoplastic development in the mouse mammary gland. In wild-type mice, in vivo administration of estradiol (E) + progesterone (P) stimulated mammary ductal growth and alveolar differentiation. Mammary glands from mice in which the ERβ gene has been deleted (βERKO mice) demonstrated normal ductal growth and differentiation in response to E + P. By contrast, mammary glands from mice in which the ERα gene has been deleted (αERKO mice) demonstrated only rudimentary ductal structures that did not differentiate in response to E + P. EGF demonstrates estrogen-like activity in the mammary glands of αERKO mice: treatment of αERKO mice with EGF + P (without E) supported normal mammary gland development, induced expression of progesterone receptor (PR), and increased levels of G-protein-coupled receptor (GPR30) protein. Mammary gland development in βERKO mice treated with EGF + P was comparable to that of wild-type mice receiving EGF + P; EGF had no statistically significant effects on the induction of PR or expression of GPR30 in mammary glands harvested from either wild-type mice or βERKO mice. In vitro exposure of mammary glands to 7,12-dimethylbenz[a]anthracene (DMBA) induced preneoplastic mammary alveolar lesions (MAL) in glands from wild-type mice and βERKO mice, but failed to induce MAL in mammary glands from αERKO mice. Microarray analysis of DMBA-treated mammary glands identified 28 functional pathways whose expression was significantly different in αERKO mice versus both βERKO and wild-type mice; key functions that were differentially expressed in αERKO mice included cell division, cell proliferation, and apoptosis. The data demonstrate distinct roles for ERα and ERβ in normal and neoplastic development in the mouse mammary gland, and suggest that EGF can mimic the ERα-mediated effects of E in this organ.

  10. Demonology, neurology, and medicine in Edwardian Britain.

    PubMed

    Hayward, Rhodri

    2004-01-01

    The idea of a conflict between demonology and psychiatry has been a foundational myth in the history of medicine. Nineteenth-century alienists such as J.-M. Charcot and Henry Maudsley developed critiques of supernatural phenomena in an attempt to pathologize religious experience. Modern historians have reanalyzed these critiques, representing them as strategies in medical professionalization. These accounts all maintain an oddly bifurcated approach to the perceived conflict, treating demonology, as a passive and unchanging set of practices, while medicine is depicted as an active and aggressive agent. An examination of early twentieth-century demonological literature reveals a very different story. Fundamentalists and Pentecostalists engaged with the problems of conversion and possession, developing sophisticated models of the normal and the pathological in spiritual experience. Their writings drew upon medical and psychiatric sources ranging widely from Pastorian germ theory to Jacksonian neurology. This article explores the points of contact between the medical and demonological communities in order to demonstrate the contested nature of biomedical innovation. PMID:15161086

  11. Teaching normal birth, normally.

    PubMed

    Hotelling, Barbara A

    2009-01-01

    Teaching normal-birth Lamaze classes normally involves considering the qualities that make birth normal and structuring classes to embrace those qualities. In this column, teaching strategies are suggested for classes that unfold naturally, free from unnecessary interventions. PMID:19436595

  12. MicroRNAs: Key Regulators in the Central Nervous System and Their Implication in Neurological Diseases

    PubMed Central

    Cao, Dan-Dan; Li, Lu; Chan, Wai-Yee

    2016-01-01

    MicroRNAs (miRNAs) are a class of small, well-conserved noncoding RNAs that regulate gene expression post-transcriptionally. They have been demonstrated to regulate a lot of biological pathways and cellular functions. Many miRNAs are dynamically regulated during central nervous system (CNS) development and are spatially expressed in adult brain indicating their essential roles in neural development and function. In addition, accumulating evidence strongly suggests that dysfunction of miRNAs contributes to neurological diseases. These observations, together with their gene regulation property, implicated miRNAs to be the key regulators in the complex genetic network of the CNS. In this review, we first focus on the ways through which miRNAs exert the regulatory function and how miRNAs are regulated in the CNS. We then summarize recent findings that highlight the versatile roles of miRNAs in normal CNS physiology and their association with several types of neurological diseases. Subsequently we discuss the limitations of miRNAs research based on current studies as well as the potential therapeutic applications and challenges of miRNAs in neurological disorders. We endeavor to provide an updated description of the regulatory roles of miRNAs in normal CNS functions and pathogenesis of neurological diseases. PMID:27240359

  13. [Pediatric neurology in rehabilitation].

    PubMed

    Kurihara, Mana

    2007-07-01

    There are some criteria for administering pediatric rehabilitation, such as (1) plasticity of the child's brain sometimes plays an enormous roles in recovery, (2) on the other hand, break-down of the child's brain is sometimes worse than expected, (3) rehabilitation should be continued with the prospect that child grows and develops everyday, (4) family members should join their child's rehabilitation. The team approach is very effective for pediatric rehabilitation. The pediatric neurologist will be one of the best members of the team because he/she can manage a disabled child and his/her family members well, and is familiar with a normal child's development. Rehabilitation should be performed appropriates, which means that the child is evaluated first, a rehabilitation program is developed and rehabilitation is implemented. The World Health Organization published the International Classification of Functioning, Disability and Health (ICF), which moves away from being a "consequence of disease" classification to "components of health". The concept of disability is changing.

  14. Neurological theory of hypertension.

    PubMed

    Eggers, A E

    2003-06-01

    Review of the older literature on the relationship between migraine and hypertension, written in the era before either condition could be treated, discloses a high rate of co-morbidity. A neurological theory of essential hypertension is proposed in which the two diseases are brought together into one entity. It is hypothesized that abnormally functioning serotonergic pacemaker cells in the dorsal raphe nucleus, as part of a chronic stress response, inappropriately activate and inhibit parts of the central and autonomic nervous systems, so as to cause the two conditions. This theory builds on a previously published neural theory of migraine.

  15. Neurologic injury in snowmobiling

    PubMed Central

    Plog, Benjamin A.; Pierre, Clifford A.; Srinivasan, Vasisht; Srinivasan, Kaushik; Petraglia, Anthony L.; Huang, Jason H.

    2014-01-01

    Background: Snowmobiles are increasingly popular recreational, all-terrain utility vehicles that require skill and physical strength to operate given their inherent maneuverability, acceleration, and top speed capabilities. These same characteristics increase the risk of injury with the operation of these vehicles, particularly neurological injury. We characterize our series of 107 patients involved in snowmobiling accidents. Methods: From January 2004 to January 2012, all snowmobiling-related injuries referred to our regional trauma center were reviewed. Information had been recorded in the hospital's trauma registry and medical records were retrospectively reviewed for data pertaining to the injuries, with particular emphasis on neurological injuries and any associated details. Results: A total of 107 patients were identified. Ninety percent of injured riders were male. The mean age was 34.4 years (range 10-70), with 7% younger than age 16. The mean Injury Severity Score was 12.0 ± 0.69 (range 1-34). Although not documented in all patients, alcohol use was found in 7.5% of the patients and drug use found in one patient. Documentation of helmet use was available for only 31 of the patients; of which 13% were not helmeted. Causes included being thrown, flipped, or roll-over (33%), striking a stationary object (27%), being struck by a snowmobile (9%), striking another snowmobile (5.5%) or a car, train, or truck (5.5%), being injured by the machine itself (9%), other (2%) or unspecified (18%). Head injuries occurred in 35% patients, including concussion, subarachnoid hemorrhage, subdural hematoma, contusion, and facial/skull fracture. Spinal fractures occurred in 21% of the patients. Fractures to the thoracic spine were the most common (50%), followed by the cervical (41%) and lumbar (36%) spine. There were also three brachial plexus injuries, one tibial nerve injury, and one internal carotid artery dissection. Average length of stay was 4.98 ± 0.56 days

  16. The neurology of sleep.

    PubMed

    Swick, Todd J

    2005-11-01

    Neurology, by virtue of its study of the brain, is the primary medical science for the elucidation of the anatomy, physiology, pathology and, ultimately, the function of sleep. There has been nothing short of a revolution in the science of sleep over the past 50 years. From the discovery of REM sleep to the identification of Hypocretin/Orexin the basic science and clinical field of sleep medicine has blossomed. This article will explore the anatomy, physiology, biochemistry and, to a limited extent, pathophysiology of the sleep/wake centers of the brain. The field of chronobiology will also be touched upon.

  17. Dicer1 Is Essential for Female Fertility and Normal Development of the Female Reproductive System

    PubMed Central

    Hong, Xiaoman; Luense, Lacey J.; McGinnis, Lynda K.; Nothnick, Warren B.; Christenson, Lane K.

    2008-01-01

    The ribonuclease III endonuclease, Dicer1 (also known as Dicer), is essential for the synthesis of the 19–25 nucleotide noncoding RNAs known as micro-RNAs (miRNAs). These miRNAs associate with the RNA-induced silencing complex to regulate gene expression posttranscriptionally by base pairing with 3′untranslated regions of complementary mRNA targets. Although it is established that miRNAs are expressed in the reproductive tract, their functional role and effect on reproductive disease remain unknown. The studies herein establish for the first time the reproductive phenotype of mice with loxP insertions in the Dicer1 gene (Dicer1fl/fl) when crossed with mice expressing Cre-recombinase driven by the anti-müllerian hormone receptor 2 promoter (Amhr2Cre/+). Adult female Dicer1fl/fl;Amhr2Cre/+ mice displayed normal mating behavior but failed to produce offspring when exposed to fertile males during a 5-month breeding trial. Morphological and histological assessments of the reproductive tracts of immature and adult mice indicated that the uterus and oviduct were hypotrophic, and the oviduct was highly disorganized. Natural mating of Dicer1fl/fl;Amhr2Cre/+ females resulted in successful fertilization as evidenced by the recovery of fertilized oocytes on d 1 pregnancy, which developed normally to blastocysts in culture. Developmentally delayed embryos were collected from Dicer1fl/fl; Amhr2Cre/+ mice on d 3 pregnancy when compared with controls. Oviductal transport was disrupted in the Dicer1fl/fl;Amhr2Cre/+ mouse as evidenced by the failure of embryos to enter the uterus on d 4 pregnancy. These studies implicate Dicer1/miRNA mediated posttranscriptional gene regulation in reproductive somatic tissues as critical for the normal development and function of these tissues and for female fertility. PMID:18703631

  18. Development of the mammillothalamic tract in normal and Pax-6 mutant mice.

    PubMed

    Valverde, F; García, C; López-Mascaraque, L; De Carlos, J A

    2000-04-17

    The mammillary bodies represent important relay stations for one of the major neuronal circuits in the brain: the limbic circuit. Mammillary projections traveling through the principal mammillary tract are established early during development, forming the mammillotegmental bundle, which appears fully developed by embryonic day 15 (E15). The mammillothalamic tract develops later, around E17-E18, forming a compact system of collateral fibers originating from the principal mammillary tract and reaching the thalamus by E20. The Pax-6 gene is expressed in various regions of the developing brain, among which the border separating the ventral thalamus from the dorsal thalamus, known as the zona limitans intrathalamica, is especially significant. In this report, the development of the efferent mammillary system of fibers was studied in wild type and Pax-6 mutant mice by using carbocyanine tracers and Golgi preparations. In mutant mice, the mammillotegmental bundle developed normally; however, the mammillothalamic tract was missing. By using anti-Pax-6 antibodies in wild type mice, the existence of an immunoreactive cell cluster is described surrounding the bifurcation point of the principal mammillary tract. The results of this study suggest that there is a correlation of these cells with a particular type of Golgi impregnated neuron.

  19. The Normal Psychological Development Of the American Adolescent—A Review

    PubMed Central

    Judd, Lewis L.

    1967-01-01

    Adolescence is a time of life marked by emotional turbulence and turmoil, which creates problems for the adolescent, his family and society in general. The psychological development that occurs during this period can be organized into developmental tasks, which emphasize the purposefulness of adolescence. An awareness of the nine essential tasks of development can serve as a basis for assessing the appropriateness and the developmental level of adolescent behavior. The establishment of a realistic self-concept (identity) is the most basic task of adolescence. Behavioral experimentation, the process through which much of the emotional growth of adolescence occurs, also accounts for the majority of the paradoxical and perplexing actions that typify the adolescent. To be in a better position to understand today's teenagers, the physician should not judge normality or abnormality by adult standards, but should view adolescence in reference to its own processes and purposes. PMID:6078890

  20. Medical marijuana in neurology.

    PubMed

    Benbadis, Selim R; Sanchez-Ramos, Juan; Bozorg, Ali; Giarratano, Melissa; Kalidas, Kavita; Katzin, Lara; Robertson, Derrick; Vu, Tuan; Smith, Amanda; Zesiewicz, Theresa

    2014-12-01

    Constituents of the Cannabis plant, cannabinoids, may be of therapeutic value in neurologic diseases. The most abundant cannabinoids are Δ(9)-tetrahydrocannabinol, which possesses psychoactive properties, and cannabidiol, which has no intrinsic psychoactive effects, but exhibits neuroprotective properties in preclinical studies. A small number of high-quality clinical trials support the safety and efficacy of cannabinoids for treatment of spasticity of multiple sclerosis, pain refractory to opioids, glaucoma, nausea and vomiting. Lower level clinical evidence indicates that cannabinoids may be useful for dystonia, tics, tremors, epilepsy, migraine and weight loss. Data are also limited in regards to adverse events and safety. Common nonspecific adverse events are similar to those of other CNS 'depressants' and include weakness, mood changes and dizziness. Cannabinoids can have cardiovascular adverse events and, when smoked chronically, may affect pulmonary function. Fatalities are rare even with recreational use. There is a concern about psychological dependence, but physical dependence is less well documented. Cannabis preparations may presently offer an option for compassionate use in severe neurologic diseases, but at this point, only when standard-of-care therapy is ineffective. As more high-quality clinical data are gathered, the therapeutic application of cannabinoids will likely expand.

  1. Neurology and diving.

    PubMed

    Massey, E Wayne; Moon, Richard E

    2014-01-01

    Diving exposes a person to the combined effects of increased ambient pressure and immersion. The reduction in pressure when surfacing can precipitate decompression sickness (DCS), caused by bubble formation within tissues due to inert gas supersaturation. Arterial gas embolism (AGE) can also occur due to pulmonary barotrauma as a result of breath holding during ascent or gas trapping due to disease, causing lung hyperexpansion, rupture and direct entry of alveolar gas into the blood. Bubble disease due to either DCS or AGE is collectively known as decompression illness. Tissue and intravascular bubbles can induce a cascade of events resulting in CNS injury. Manifestations of decompression illness can vary in severity, from mild (paresthesias, joint pains, fatigue) to severe (vertigo, hearing loss, paraplegia, quadriplegia). Particularly as these conditions are uncommon, early recognition is essential to provide appropriate management, consisting of first aid oxygen, targeted fluid resuscitation and hyperbaric oxygen, which is the definitive treatment. Less common neurologic conditions that do not require hyperbaric oxygen include rupture of a labyrinthine window due to inadequate equalization of middle ear pressure during descent, which can precipitate vertigo and hearing loss. Sinus and middle ear overpressurization during ascent can compress the trigeminal and facial nerves respectively, causing temporary facial hypesthesia and lower motor neuron facial weakness. Some conditions preclude safe diving, such as seizure disorders, since a convulsion underwater is likely to be fatal. Preventive measures to reduce neurologic complications of diving include exclusion of individuals with specific medical conditions and safe diving procedures, particularly related to descent and ascent.

  2. Medical marijuana in neurology.

    PubMed

    Benbadis, Selim R; Sanchez-Ramos, Juan; Bozorg, Ali; Giarratano, Melissa; Kalidas, Kavita; Katzin, Lara; Robertson, Derrick; Vu, Tuan; Smith, Amanda; Zesiewicz, Theresa

    2014-12-01

    Constituents of the Cannabis plant, cannabinoids, may be of therapeutic value in neurologic diseases. The most abundant cannabinoids are Δ(9)-tetrahydrocannabinol, which possesses psychoactive properties, and cannabidiol, which has no intrinsic psychoactive effects, but exhibits neuroprotective properties in preclinical studies. A small number of high-quality clinical trials support the safety and efficacy of cannabinoids for treatment of spasticity of multiple sclerosis, pain refractory to opioids, glaucoma, nausea and vomiting. Lower level clinical evidence indicates that cannabinoids may be useful for dystonia, tics, tremors, epilepsy, migraine and weight loss. Data are also limited in regards to adverse events and safety. Common nonspecific adverse events are similar to those of other CNS 'depressants' and include weakness, mood changes and dizziness. Cannabinoids can have cardiovascular adverse events and, when smoked chronically, may affect pulmonary function. Fatalities are rare even with recreational use. There is a concern about psychological dependence, but physical dependence is less well documented. Cannabis preparations may presently offer an option for compassionate use in severe neurologic diseases, but at this point, only when standard-of-care therapy is ineffective. As more high-quality clinical data are gathered, the therapeutic application of cannabinoids will likely expand. PMID:25427150

  3. DNA Repair Deficiency and Neurological Disease

    PubMed Central

    McKinnon, Peter J.

    2011-01-01

    Preface Responding to genotoxic stress is a prerequisite for development of the nervous system. Mutations in a variety of DNA repair factors can lead to human diseases that are characterized by pronounced neuropathology. In many of these syndromes the neurological component is amongst the most deleterious aspects of the disease. The nervous system poses a particular challenge in terms of clinical intervention, as the neuropathology often arises during nervous system development, and can be fully penetrant by childhood. Understanding how DNA repair deficiency impacts the nervous system will provide a rationale basis for therapies targeted at ameliorating the neurological problems in these syndromes. PMID:19145234

  4. Visuo-manual coordination in preterm infants without neurological impairments.

    PubMed

    Petkovic, Maja; Chokron, Sylvie; Fagard, Jacqueline

    2016-01-01

    The extent of and reasons for visuo-manual coordination deficits in moderate and late preterm born infants without neurological impairments are not well known. This paper presents a longitudinal study on the visuo-manual development of twelve preterm infants, born after 33-36 weeks of gestation without neurological complications, between the ages of 6 and 12 months. Visuo-manual integration and grasping were assessed using the Peabody Developmental Motor Scales, along with bimanual coordination and handedness tests. Visual function was examined once prior to the beginning of the study. Gross motor development was also evaluated every month. Preterm infants were compared to a control group of ten full-term infants according to corrected age. Compared to full-terms, the visual perception of preterm infants was close to normal, with only a measure of visual fixation lower than in full-terms. In contrast, preterm infants had delayed development of visuo-manual integration, grasping, bimanual coordination, and handedness even when compared using corrected age. Tonicity and gestational age at birth were the main variables associated to the delays. These results are discussed in terms of the possible factors underlying such delays. They need to be confirmed on a larger sample of preterm born children, and to be correlated with later development. This would allow developing markers of future neuropsychological impairments during childhood.

  5. Visuo-manual coordination in preterm infants without neurological impairments.

    PubMed

    Petkovic, Maja; Chokron, Sylvie; Fagard, Jacqueline

    2016-01-01

    The extent of and reasons for visuo-manual coordination deficits in moderate and late preterm born infants without neurological impairments are not well known. This paper presents a longitudinal study on the visuo-manual development of twelve preterm infants, born after 33-36 weeks of gestation without neurological complications, between the ages of 6 and 12 months. Visuo-manual integration and grasping were assessed using the Peabody Developmental Motor Scales, along with bimanual coordination and handedness tests. Visual function was examined once prior to the beginning of the study. Gross motor development was also evaluated every month. Preterm infants were compared to a control group of ten full-term infants according to corrected age. Compared to full-terms, the visual perception of preterm infants was close to normal, with only a measure of visual fixation lower than in full-terms. In contrast, preterm infants had delayed development of visuo-manual integration, grasping, bimanual coordination, and handedness even when compared using corrected age. Tonicity and gestational age at birth were the main variables associated to the delays. These results are discussed in terms of the possible factors underlying such delays. They need to be confirmed on a larger sample of preterm born children, and to be correlated with later development. This would allow developing markers of future neuropsychological impairments during childhood. PMID:26812594

  6. Evidence-based review on the effect of normal dietary consumption of fructose on development of hyperlipidemia and obesity in healthy, normal weight individuals.

    PubMed

    Dolan, Laurie C; Potter, Susan M; Burdock, George A

    2010-01-01

    In recent years, there has been episodic speculation that an increase in consumption of fructose from foods and beverages is an underlying factor responsible for the relatively recent increase in obesity and obesity-related diseases such as diabetes. Reports in support of this hypothesis have been published, showing that concentrations of triglycerides (TG) are higher and concentrations of insulin and hormones associated with satiety are lower in animals following the ingestion of fairly large quantities of fructose, compared to other carbohydrates. However, results from human studies are inconsistent. A possible reason for the inconsistent results is that they are dependent on the particular study population, the design of the studies, and/or the amount of fructose administered. A systematic assessment of the strength and quality of the studies and their relevance for healthy, normal weight humans ingesting fructose in a normal dietary manner has not been performed. The purpose of this review was to critically evaluate the existing database for a causal relationship between the ingestion of fructose in a normal, dietary manner and the development of hyperlipidemia or increased body weight in healthy, normal weight humans, using an evidence-based approach. The results of the analysis indicate that fructose does not cause biologically relevant changes in TG or body weight when consumed at levels approaching 95th percentile estimates of intake.

  7. Fellowship programs in behavioral neurology.

    PubMed

    Green, R C; Benjamin, S; Cummings, J L

    1995-03-01

    We sent a behavioral neurology fellowship questionnaire to each of the training directors of 160 neurology residency programs throughout the world, seeking information about programs offering advanced training in behavioral neurology (or similar fellowships in cognitive neurology, neurobehavior, or cognitive neuroscience). Response rate was 100%. Thirty-four respondents reported active fellowship programs in behavioral neurology, and 28 additional respondents indicated that a behavioral neurology fellowship was planned. Nine of the 34 programs (26.5%) defined themselves as exclusively or predominantly concerned with dementia and age-related neurobehavioral disorders. Directors of the 34 active fellowship programs estimated that their combined programs had graduated 199 fellows and were currently training fifty. Most fellowships concentrated on outpatient clinical training, with teaching required by 78.1% and research required by 81.8%. Specialty certification for behavioral neurology was favored by over 75% of behavioral neurology fellowship training directors but by only 30% of training directors in residency programs without behavioral neurology fellowships. Behavioral neurology training programs have grown dramatically in response to an increased recognition of the academic interest in and the clinical needs for these services. PMID:7898686

  8. AAV-based gene therapy prevents neuropathology and results in normal cognitive development in the hyperargininemic mouse.

    PubMed

    Lee, E K; Hu, C; Bhargava, R; Ponnusamy, R; Park, H; Novicoff, S; Rozengurt, N; Marescau, B; De Deyn, P; Stout, D; Schlichting, L; Grody, W W; Cederbaum, S D; Lipshutz, G S

    2013-08-01

    Complete arginase I deficiency is the least severe urea cycle disorder, characterized by hyperargininemia and infrequent episodes of hyperammonemia. Patients suffer from neurological impairment with cortical and pyramidal tract deterioration, spasticity, loss of ambulation and seizures, and is associated with intellectual disability. In mice, onset is heralded by weight loss beginning around day 15; gait instability follows progressing to inability to stand and development of tail tremor with seizure-like activity and death. Here we report that hyperargininemic mice treated neonatally with an adeno-associated virus (AAV)-expressing arginase and followed long-term lack any presentation consistent with brain dysfunction. Behavioral and histopathological evaluation demonstrated that treated mice are indistinguishable from littermates, and that putative compounds associated with neurotoxicity are diminished. In addition, treatment results in near complete resolution of metabolic abnormalities early in life; however, there is the development of some derangement later with decline in transgene expression. Ammonium challenging revealed that treated mice are affected by exogenous loading much greater than littermates. These results demonstrate that AAV-based therapy for hyperargininemia is effective and prevents development of neurological abnormalities and cognitive dysfunction in a mouse model of hyperargininemia; however, nitrogen challenging reveals that these mice remain impaired in the handling of waste nitrogen.

  9. The development of hepatic stellate cells in normal and abnormal human fetuses – an immunohistochemical study

    PubMed Central

    Loo, Christine K C; Pereira, Tamara N; Pozniak, Katarzyna N; Ramsing, Mette; Vogel, Ida; Ramm, Grant A

    2015-01-01

    The precise embryological origin and development of hepatic stellate cells is not established. Animal studies and observations on human fetuses suggest that they derive from posterior mesodermal cells that migrate via the septum transversum and developing diaphragm to form submesothelial cells beneath the liver capsule, which give rise to mesenchymal cells including hepatic stellate cells. However, it is unclear if these are similar to hepatic stellate cells in adults or if this is the only source of stellate cells. We have studied hepatic stellate cells by immunohistochemistry, in developing human liver from autopsies of fetuses with and without malformations and growth restriction, using cellular Retinol Binding Protein-1 (cRBP-1), Glial Fibrillary Acidic Protein (GFAP), and α-Smooth Muscle Actin (αSMA) antibodies, to identify factors that influence their development. We found that hepatic stellate cells expressing cRBP-1 are present from the end of the first trimester of gestation and reduce in density throughout gestation. They appear abnormally formed and variably reduced in number in fetuses with abnormal mesothelial Wilms Tumor 1 (WT1) function, diaphragmatic hernia and in ectopic liver nodules without mesothelium. Stellate cells showed similarities to intravascular cells and their presence in a fetus with diaphragm agenesis suggests they may be derived from circulating stem cells. Our observations suggest circulating stem cells as well as mesothelium can give rise to hepatic stellate cells, and that they require normal mesothelial function for their development. PMID:26265759

  10. Status of neurology medical school education

    PubMed Central

    Ali, Imran I.; Isaacson, Richard S.; Safdieh, Joseph E.; Finney, Glen R.; Sowell, Michael K.; Sam, Maria C.; Anderson, Heather S.; Shin, Robert K.; Kraakevik, Jeff A.; Coleman, Mary; Drogan, Oksana

    2014-01-01

    Objective: To survey all US medical school clerkship directors (CDs) in neurology and to compare results from a similar survey in 2005. Methods: A survey was developed by a work group of the American Academy of Neurology Undergraduate Education Subcommittee, and sent to all neurology CDs listed in the American Academy of Neurology database. Comparisons were made to a similar 2005 survey. Results: Survey response rate was 73%. Neurology was required in 93% of responding schools. Duration of clerkships was 4 weeks in 74% and 3 weeks in 11%. Clerkships were taken in the third year in 56%, third or fourth year in 19%, and fourth year in 12%. Clerkship duration in 2012 was slightly shorter than in 2005 (fewer clerkships of ≥4 weeks, p = 0.125), but more clerkships have moved into the third year (fewer neurology clerkships during the fourth year, p = 0.051). Simulation training in lumbar punctures was available at 44% of schools, but only 2% of students attempted lumbar punctures on patients. CDs averaged 20% protected time, but reported that they needed at least 32%. Secretarial full-time equivalent was 0.50 or less in 71% of clerkships. Eighty-five percent of CDs were “very satisfied” or “somewhat satisfied,” but more than half experienced “burnout” and 35% had considered relinquishing their role. Conclusion: Trends in neurology undergraduate education since 2005 include shorter clerkships, migration into the third year, and increasing use of technology. CDs are generally satisfied, but report stressors, including inadequate protected time and departmental support. PMID:25305155

  11. Evaluation of Appropriate Reference Genes for Gene Expression Normalization during Watermelon Fruit Development

    PubMed Central

    Kong, Qiusheng; Yuan, Jingxian; Gao, Lingyun; Zhao, Liqiang; Cheng, Fei; Huang, Yuan; Bie, Zhilong

    2015-01-01

    Gene expression analysis in watermelon (Citrullus lanatus) fruit has drawn considerable attention with the availability of genome sequences to understand the regulatory mechanism of fruit development and to improve its quality. Real-time quantitative reverse-transcription PCR (qRT-PCR) is a routine technique for gene expression analysis. However, appropriate reference genes for transcript normalization in watermelon fruits have not been well characterized. The aim of this study was to evaluate the appropriateness of 12 genes for their potential use as reference genes in watermelon fruits. Expression variations of these genes were measured in 48 samples obtained from 12 successive developmental stages of parthenocarpic and fertilized fruits of two watermelon genotypes by using qRT-PCR analysis. Considering the effects of genotype, fruit setting method, and developmental stage, geNorm determined clathrin adaptor complex subunit (ClCAC), β-actin (ClACT), and alpha tubulin 5 (ClTUA5) as the multiple reference genes in watermelon fruit. Furthermore, ClCAC alone or together with SAND family protein (ClSAND) was ranked as the single or two best reference genes by NormFinder. By using the top-ranked reference genes to normalize the transcript abundance of phytoene synthase (ClPSY1), a good correlation between lycopene accumulation and ClPSY1 expression pattern was observed in ripening watermelon fruit. These validated reference genes will facilitate the accurate measurement of gene expression in the studies on watermelon fruit biology. PMID:26110539

  12. Roles of retinoic acid signaling in normal and abnormal development of the palate and tongue.

    PubMed

    Okano, Junko; Udagawa, Jun; Shiota, Kohei

    2014-05-01

    Palatogenesis involves various developmental events such as growth, elevation, elongation and fusion of opposing palatal shelves. Extrinsic factors such as mouth opening and subsequent tongue withdrawal are also needed for the horizontal elevation of palate shelves. Failure of any of these steps can lead to cleft palate, one of the most common birth defects in humans. It has been shown that retinoic acid (RA) plays important roles during palate development, but excess RA causes cleft palate in fetuses of both rodents and humans. Thus, the coordinated regulation of retinoid metabolism is essential for normal palatogenesis. The endogenous RA level is determined by the balance of RA-synthesizing (retinaldehyde dehydrogenases: RALDHs) and RA-degrading enzymes (CYP26s). Cyp26b1 is a key player in normal palatogenesis. In this review, we discuss recent progress in the study of the pathogenesis of RA-induced cleft palate, with special reference to the regulation of endogenous RA levels by RA-degrading enzymes.

  13. Is the early development of girls with Rett disorder really normal?

    PubMed

    Einspieler, Christa; Kerr, Alison M; Prechtl, Heinz F R

    2005-05-01

    An apparently normal early development was one of the initial criteria for classical Rett syndrome. However, several investigators considered Rett syndrome to be a developmental disorder manifesting very soon after birth. Videos of 22 Rett cases were assessed carefully for movements, posture, and behavior during the first 6 mo of life. All signs that deviated from the normal standard were recorded meticulously. Special attention was paid to the face, the hands, and body movements. A detailed analysis clearly demonstrated an abnormal quality of general movements (100%), tongue protrusion (62%), postural stiffness (58%), asymmetric eye opening and closing (56%), abnormal finger movements (52%), hand stereotypies (42%), bursts of abnormal facial expressions (42%), bizarre smile (32%), tremor (28%), and stereotyped body movements (15%). Our study is the first to apply specific standardized measures of early spontaneous movements to Rett infants, proving conclusively that the disorder is manifested within the first months of life. Although not necessarily specific, the signs that we have observed will be of value in alerting clinicians to the possibility of the diagnosis at an early stage, when intervention is likely to be most effective.

  14. Evaluation of Appropriate Reference Genes for Gene Expression Normalization during Watermelon Fruit Development.

    PubMed

    Kong, Qiusheng; Yuan, Jingxian; Gao, Lingyun; Zhao, Liqiang; Cheng, Fei; Huang, Yuan; Bie, Zhilong

    2015-01-01

    Gene expression analysis in watermelon (Citrullus lanatus) fruit has drawn considerable attention with the availability of genome sequences to understand the regulatory mechanism of fruit development and to improve its quality. Real-time quantitative reverse-transcription PCR (qRT-PCR) is a routine technique for gene expression analysis. However, appropriate reference genes for transcript normalization in watermelon fruits have not been well characterized. The aim of this study was to evaluate the appropriateness of 12 genes for their potential use as reference genes in watermelon fruits. Expression variations of these genes were measured in 48 samples obtained from 12 successive developmental stages of parthenocarpic and fertilized fruits of two watermelon genotypes by using qRT-PCR analysis. Considering the effects of genotype, fruit setting method, and developmental stage, geNorm determined clathrin adaptor complex subunit (ClCAC), β-actin (ClACT), and alpha tubulin 5 (ClTUA5) as the multiple reference genes in watermelon fruit. Furthermore, ClCAC alone or together with SAND family protein (ClSAND) was ranked as the single or two best reference genes by NormFinder. By using the top-ranked reference genes to normalize the transcript abundance of phytoene synthase (ClPSY1), a good correlation between lycopene accumulation and ClPSY1 expression pattern was observed in ripening watermelon fruit. These validated reference genes will facilitate the accurate measurement of gene expression in the studies on watermelon fruit biology. PMID:26110539

  15. Evaluation of Appropriate Reference Genes for Gene Expression Normalization during Watermelon Fruit Development.

    PubMed

    Kong, Qiusheng; Yuan, Jingxian; Gao, Lingyun; Zhao, Liqiang; Cheng, Fei; Huang, Yuan; Bie, Zhilong

    2015-01-01

    Gene expression analysis in watermelon (Citrullus lanatus) fruit has drawn considerable attention with the availability of genome sequences to understand the regulatory mechanism of fruit development and to improve its quality. Real-time quantitative reverse-transcription PCR (qRT-PCR) is a routine technique for gene expression analysis. However, appropriate reference genes for transcript normalization in watermelon fruits have not been well characterized. The aim of this study was to evaluate the appropriateness of 12 genes for their potential use as reference genes in watermelon fruits. Expression variations of these genes were measured in 48 samples obtained from 12 successive developmental stages of parthenocarpic and fertilized fruits of two watermelon genotypes by using qRT-PCR analysis. Considering the effects of genotype, fruit setting method, and developmental stage, geNorm determined clathrin adaptor complex subunit (ClCAC), β-actin (ClACT), and alpha tubulin 5 (ClTUA5) as the multiple reference genes in watermelon fruit. Furthermore, ClCAC alone or together with SAND family protein (ClSAND) was ranked as the single or two best reference genes by NormFinder. By using the top-ranked reference genes to normalize the transcript abundance of phytoene synthase (ClPSY1), a good correlation between lycopene accumulation and ClPSY1 expression pattern was observed in ripening watermelon fruit. These validated reference genes will facilitate the accurate measurement of gene expression in the studies on watermelon fruit biology.

  16. Approach to Neurometabolic Diseases from a Pediatric Neurological Point of View

    PubMed Central

    KARIMZADEH, Parvaneh

    2015-01-01

    Objective Neurometabolic disorders are an important group of diseases that mostly are presented in newborns and infants. Neurological manifestations are the prominent signs and symptoms in this group of diseases. Seizures are a common sign and are often refractory to antiepileptic drugs in untreated neurometabolic patients. The onset of symptoms for neurometabolic disorders appears after an interval of normal or near normal growth and development.Additionally, affected children may fare well until a catabolic crisis occurs. Patients with neurometabolic disorders during metabolic decompensation have severe clinical presentation, which include poor feeding, vomiting, lethargy, seizures, and loss of consciousness. This symptom is often fatal but severe neurological insult and regression in neurodevelopmental milestones can result as a prominent sign in patients who survived. Acute symptoms should be immediately treated regardless of the cause. A number of patients with neurometabolic disorders respond favorably and, in some instances, dramatically respond to treatment. Early detection and early intervention is invaluable in some patients to prevent catabolism and normal or near normal neurodevelopmental milestones. This paper discusses neurometabolic disorders, approaches to this group of diseases (from the view of a pediatric neurologist), clinical and neurological manifestations, neuroimaging and electroencephalography findings, early detection, and early treatment. PMID:25767534

  17. Cdc42 and sec10 Are Required for Normal Retinal Development in Zebrafish

    PubMed Central

    Choi, Soo Young; Baek, Jeong-In; Zuo, Xiaofeng; Kim, Seok-Hyung; Dunaief, Joshua L.; Lipschutz, Joshua H.

    2015-01-01

    Purpose. To characterize the function and mechanisms of cdc42 and sec10 in eye development in zebrafish. Methods. Knockdown of zebrafish cdc42 and sec10 was carried out using antisense morpholino injection. The phenotype of morphants was characterized by histology, immunohistology, and transmission electron microscopy (TEM). To investigate a synergistic genetic interaction between cdc42 and sec10, we titrated suboptimal doses of cdc42 and sec10 morpholinos, and coinjected both morpholinos. To study trafficking, a melanosome transport assay was performed using epinephrine. Results. Cdc42 and sec10 knockdown in zebrafish resulted in both abnormal eye development and increased retinal cell death. Cdc42 morphants had a relatively normal retinal structure, aside from the absence of most connecting cilia and outer segments, whereas in sec10 morphants, much of the outer nuclear layer, which is composed of the photoreceptor nuclei, was missing and RPE cell thickness was markedly irregular. Knockdown of cdc42 and sec10 also resulted in an intracellular transport defect affecting retrograde melanosome transport. Furthermore, there was a synergistic genetic interaction between zebrafish cdc42 and sec10, suggesting that cdc42 and sec10 act in the same pathway in retinal development. Conclusions. We propose a model whereby sec10 and cdc42 play a central role in development of the outer segment of the retinal photoreceptor cell by trafficking proteins necessary for ciliogenesis. PMID:26024121

  18. Long-term influence of normal variation in neonatal characteristics on human brain development

    PubMed Central

    Walhovd, Kristine B.; Fjell, Anders M.; Brown, Timothy T.; Kuperman, Joshua M.; Chung, Yoonho; Hagler, Donald J.; Roddey, J. Cooper; Erhart, Matthew; McCabe, Connor; Akshoomoff, Natacha; Amaral, David G.; Bloss, Cinnamon S.; Libiger, Ondrej; Schork, Nicholas J.; Darst, Burcu F.; Casey, B. J.; Chang, Linda; Ernst, Thomas M.; Frazier, Jean; Gruen, Jeffrey R.; Kaufmann, Walter E.; Murray, Sarah S.; van Zijl, Peter; Mostofsky, Stewart; Dale, Anders M.; Jernigan, Terry L.; McCabe, Connor; Chang, Linda; Akshoomoff, Natacha; Newman, Erik; Dale, Anders M.; Ernst, Thomas; Dale, Anders M.; Van Zijl, Peter; Kuperman, Joshua; Murray, Sarah; Bloss, Cinnamon; Schork, Nicholas J.; Appelbaum, Mark; Gamst, Anthony; Thompson, Wesley; Bartsch, Hauke; Jernigan, Terry L.; Dale, Anders M.; Akshoomoff, Natacha; Chang, Linda; Ernst, Thomas; Keating, Brian; Amaral, David; Sowell, Elizabeth; Kaufmann, Walter; Van Zijl, Peter; Mostofsky, Stewart; Casey, B.J.; Ruberry, Erika J.; Powers, Alisa; Rosen, Bruce; Kenet, Tal; Frazier, Jean; Kennedy, David; Gruen, Jeffrey

    2012-01-01

    It is now recognized that a number of cognitive, behavioral, and mental health outcomes across the lifespan can be traced to fetal development. Although the direct mediation is unknown, the substantial variance in fetal growth, most commonly indexed by birth weight, may affect lifespan brain development. We investigated effects of normal variance in birth weight on MRI-derived measures of brain development in 628 healthy children, adolescents, and young adults in the large-scale multicenter Pediatric Imaging, Neurocognition, and Genetics study. This heterogeneous sample was recruited through geographically dispersed sites in the United States. The influence of birth weight on cortical thickness, surface area, and striatal and total brain volumes was investigated, controlling for variance in age, sex, household income, and genetic ancestry factors. Birth weight was found to exert robust positive effects on regional cortical surface area in multiple regions as well as total brain and caudate volumes. These effects were continuous across birth weight ranges and ages and were not confined to subsets of the sample. The findings show that (i) aspects of later child and adolescent brain development are influenced at birth and (ii) relatively small differences in birth weight across groups and conditions typically compared in neuropsychiatric research (e.g., Attention Deficit Hyperactivity Disorder, schizophrenia, and personality disorders) may influence group differences observed in brain parameters of interest at a later stage in life. These findings should serve to increase our attention to early influences. PMID:23169628

  19. Neurologic complications of cardiac tumors.

    PubMed

    Roeltgen, David; Kidwell, Chelsea S

    2014-01-01

    Cardiac tumors are an uncommon cause for neurologic disease, but if undiagnosed can be associated with devastating neurologic consequences. Primary cardiac tumors, both benign and neoplastic, and metastatic tumors occur. Primary cardiac tumors are more likely to be associated with neurologic embolic complications. Metastatic cardiac tumors are more likely to be associated with valvular distraction, arrhythmia, diminished cardiac output and indirect neurological dysfunction. Primary and metastatic cardiac tumors may result in cerebral metastatic disease. Atrial myxoma, a benign primary cardiac tumor, is the most common cardiac tumor associated with neurologic disease, and most commonly causes cerebral embolization and stroke. The use of thrombolytic therapy for these strokes is controversial. Additionally, delayed manifestations, including aneurysm formation and intracranial hemorrhage, are possible. Aneurysm formation has been described as occurring after removal of the primary tumor. The availability of noninvasive cardiac imaging has significantly helped decrease the neurologic morbidity of cardiac tumors and has led to frequent successful intervention. PMID:24365298

  20. History of neurologic examination books.

    PubMed

    Boes, Christopher J

    2015-04-01

    The objective of this study was to create an annotated list of textbooks dedicated to teaching the neurologic examination. Monographs focused primarily on the complete neurologic examination published prior to 1960 were reviewed. This analysis was limited to books with the word "examination" in the title, with exceptions for the texts of Robert Wartenberg and Gordon Holmes. Ten manuals met the criteria. Works dedicated primarily to the neurologic examination without a major emphasis on disease description or treatment first appeared in the early 1900s. Georg Monrad-Krohn's "Blue Book of Neurology" ("Blue Bible") was the earliest success. These treatises served the important purpose of educating trainees on proper neurologic examination technique. They could make a reputation and be profitable for the author (Monrad-Krohn), highlight how neurology was practiced at individual institutions (McKendree, Denny-Brown, Holmes, DeJong, Mayo Clinic authors), and honor retiring mentors (Mayo Clinic authors).

  1. The Spectrum of Neurological Recovery

    PubMed Central

    Mir, Tanveer P.

    2012-01-01

    The equivalence of brain death with death is largely, although not universally accepted. Patients may have suffered insults such as cardiac arrest, vascular catastrophe, poisoning, or head trauma. Early identification of patients at greatest risk of poor neurologic outcome and management in the appropriate critical care setting is the key to maximizing neurological recovery. Recent technological advances and neuroimaging have made it possible to predict neurological reversibility with great accuracy. Significant improvements in therapy such as hypothermia, will improve outcomes in neurological catastrophies, particularly in anoxic-ischemic encephalopathy. The clinical spectrum and diagnostic criteria of minimally conscious and vegetative states is reviewed. The current understanding of the differences in prognosis and prediction of meaningful cognitive and functional recovery in each neurological state is described. Establishing an understanding of the ethical principles that guide medical decisions in clinical practice related to different neurological states is evolving into a new field called neuroethics. PMID:23610514

  2. History of neurologic examination books.

    PubMed

    Boes, Christopher J

    2015-04-01

    The objective of this study was to create an annotated list of textbooks dedicated to teaching the neurologic examination. Monographs focused primarily on the complete neurologic examination published prior to 1960 were reviewed. This analysis was limited to books with the word "examination" in the title, with exceptions for the texts of Robert Wartenberg and Gordon Holmes. Ten manuals met the criteria. Works dedicated primarily to the neurologic examination without a major emphasis on disease description or treatment first appeared in the early 1900s. Georg Monrad-Krohn's "Blue Book of Neurology" ("Blue Bible") was the earliest success. These treatises served the important purpose of educating trainees on proper neurologic examination technique. They could make a reputation and be profitable for the author (Monrad-Krohn), highlight how neurology was practiced at individual institutions (McKendree, Denny-Brown, Holmes, DeJong, Mayo Clinic authors), and honor retiring mentors (Mayo Clinic authors). PMID:25829645

  3. Neurology of Sepsis.

    PubMed

    Sweis, Rochelle; Ortiz, Jorge; Biller, José

    2016-03-01

    Sepsis is a systemic inflammatory response syndrome occurring secondary to infection and labeled severe when end organ dysfunction or tissue hypoperfusion transpires. Sepsis-associated mortality remains high among critically ill patients, with chronic disease and immunosuppression being the most common risk factors. Studies demonstrate that early recognition and treatment are vital to decreasing mortality. Some of the least understood effects of sepsis are the associated neurologic complications. The peripheral nervous system (PNS) has gained most consideration and thought, largely due to dependence on mechanical ventilation. Central nervous system (CNS) complications related to sepsis have only more recently gained attention but continue to go unnoticed. Aside from the clinical examination, electroencephalography (EEG) is a sensitive tool for prognostication or uncovering non-convulsive seizures in encephalopathic patients. Further studies are needed to further define the urgency of a prevention and treatment plan for the deleterious effects of sepsis on the PNS and CNS. PMID:26820754

  4. Neurology of volition.

    PubMed

    Kranick, Sarah M; Hallett, Mark

    2013-09-01

    Neurological disorders of volition may be characterized by deficits in willing and/or agency. When we move our bodies through space, it is the sense that we intended to move (willing) and that our actions were a consequence of this intention (self-agency) that gives us the sense of voluntariness and a general feeling of being "in control." While it is possible to have movements that share executive machinery ordinarily used for voluntary movement but lack a sense of voluntariness, such as psychogenic movement disorders, it is also possible to claim volition for presumed involuntary movements (early chorea) or even when no movement is produced (anosognosia). The study of such patients should enlighten traditional models of how the percepts of volition are generated in the brain with regard to movement. We discuss volition and its components as multi-leveled processes with feedforward and feedback information flow, and dependence on prior expectations as well as external and internal cues.

  5. Hither neurology: research.

    PubMed Central

    Warlow, C P

    1992-01-01

    Neurological disability may be prevented, or it may be alleviated if prevention is impossible or ineffective. Research into prevention and alleviation can be "laboratory" or "clinical", the latter being no less scientific than the former. All proposed treatments must be properly evaluated to ensure that effective interventions are widely adopted and ineffective ones abandoned. Unless an intervention has a major effect on outcome (which most do not), the most efficient assessment is by random allocation of patients to the new intervention versus the old. Although there were, and still are, forces opposed to the proper evaluation of treatment, there are strong economic clinical arguments in its favour, which will lead to appropriate targeting of scarce health resources. PMID:1564502

  6. The immune system in the normal endometrium and implications for endometrial cancer development.

    PubMed

    Vanderstraeten, A; Tuyaerts, S; Amant, F

    2015-06-01

    Although described for the first time some decades ago, the contribution of the immune system to the establishment of tumors has not been extensively pursued for a long time. Over the last decade, however, more and more evidence has been accumulating concerning the role the immune system plays in tumor development and progression and its possible role in patient prognosis. In addition, interest is growing in preclinical and clinical research concerning the use of the immune system in the treatment of cancer. Immunotherapy for gynecological cancers in general, and for endometrial cancer in particular, is still in its infancy. Only a small number of studies, with varying success rates, have been published. Here, we provide a concise overview of the literature available on the role of the immune system in the normal endometrium and in endometrial cancer, in addition to the possible implications for future immunotherapeutic studies.

  7. Neurology and diving.

    PubMed

    Massey, E Wayne; Moon, Richard E

    2014-01-01

    Diving exposes a person to the combined effects of increased ambient pressure and immersion. The reduction in pressure when surfacing can precipitate decompression sickness (DCS), caused by bubble formation within tissues due to inert gas supersaturation. Arterial gas embolism (AGE) can also occur due to pulmonary barotrauma as a result of breath holding during ascent or gas trapping due to disease, causing lung hyperexpansion, rupture and direct entry of alveolar gas into the blood. Bubble disease due to either DCS or AGE is collectively known as decompression illness. Tissue and intravascular bubbles can induce a cascade of events resulting in CNS injury. Manifestations of decompression illness can vary in severity, from mild (paresthesias, joint pains, fatigue) to severe (vertigo, hearing loss, paraplegia, quadriplegia). Particularly as these conditions are uncommon, early recognition is essential to provide appropriate management, consisting of first aid oxygen, targeted fluid resuscitation and hyperbaric oxygen, which is the definitive treatment. Less common neurologic conditions that do not require hyperbaric oxygen include rupture of a labyrinthine window due to inadequate equalization of middle ear pressure during descent, which can precipitate vertigo and hearing loss. Sinus and middle ear overpressurization during ascent can compress the trigeminal and facial nerves respectively, causing temporary facial hypesthesia and lower motor neuron facial weakness. Some conditions preclude safe diving, such as seizure disorders, since a convulsion underwater is likely to be fatal. Preventive measures to reduce neurologic complications of diving include exclusion of individuals with specific medical conditions and safe diving procedures, particularly related to descent and ascent. PMID:24365363

  8. Characterization of the equine blood-testis barrier during tubular development in normal and cryptorchid stallions.

    PubMed

    Rode, K; Sieme, H; Richterich, P; Brehm, R

    2015-09-15

    The formation of the blood-testis barrier (BTB) is defined as occurring with the first appearance of spermatocytes at around puberty and is vital for normal spermatogenesis. This barrier between two adjacent Sertoli cells (SCs) consists of a cell junctional protein complex, which includes tight junctions (TJs), adherens junctions, and gap junctions. In many mammalian species, BTB composition has already been investigated, whereas little is known about the equine BTB. In the present study, immunohistochemistry and qualitative Western Blot analysis were used to assess the expression and distribution patterns of the junctional proteins claudin-11 (TJ), zonula occludens-1 (TJ associated), N-cadherin (adherens junctions), and connexin 43 (gap junctions) in equine testes during tubular development and in testes of stallions exhibiting unilateral cryptorchidism. Therefore, testes of 21 warmblood stallions (aged 12 months-11 years) were obtained during routine surgical castration. In the normal adult equine testis, the junctional proteins are localized at the basolateral region of the seminiferous tubules forming a circumferential seal corresponding to the known BTB localization. N-cadherin is additionally expressed along the lateral SC surface. In immature seminiferous cords still lacking a lumen, a diffuse distribution pattern of the junctional proteins throughout the SC cytoplasm is visible. As lumen formation advances, the immunolocalization shifts progressively toward the basolateral SC membranes. Additionally, apoptotic germ cells were detected and quantified in prepubertal stallions using terminal deoxynucleotidyl transferase dUTP nick end labeling assay and correlated with junctional protein localization. In the retained testis of cryptorchid stallions, which exhibit an aberrant testicular morphology, a deviating expression of the junctional proteins is visible. The present data show for the first time that (1) the equine SC junctional complex contains claudin-11

  9. Neoplasms with schwannian differentiation express transcription factors known to regulate normal schwann cell development.

    PubMed

    Pytel, Peter; Karrison, Theodore; Can Gong; Tonsgard, James H; Krausz, Thomas; Montag, Anthony G

    2010-12-01

    A number of transcription factors have been identified as important in guiding normal Schwann cell development. This study used immunohistochemistry on tissue arrays to assess the expression of some of these transcription factors (Sox5, Sox9, Sox10, AP-2α, Pax7, and FoxD3) on 76 schwannomas, 105 neurofibromas, and 34 malignant peripheral nerve sheath tumors (MPNSTs). Sox9 and Sox10 were found to be widely expressed in all tumor types. FoxD3 reactivity was stronger and more frequently found in schwannomas and MPNSTs than neurofibromas. AP-2α was positive in 31% to 49% of all tumors, but strong reactivity was limited to MPNSTs and schwannomas. Pax7 and Sox5 expression was restricted to subsets of MPNSTs. Statistical analysis showed significant differences between the 3 tumor types in the expression of these markers. No differences were found in the analyzed tumor subgroups, including schwannomas of different sites, schwannomas with or without NF2 association, neurofibromas of different types, or sporadic versus NF1-associated MPNSTs. These results suggest that the transcription factors that guide normal Schwann cell development also play a role in the biology of neoplastic cells with Schwannian differentiation. FoxD3, AP-2α, Pax7, and Sox5 are upregulated in MPNSTs compared with neurofibromas and may be markers of malignant transformation. Screening the expression of FoxD3, Sox9, and Sox10 on 23 cases of other spindle-cell proliferations that may be considered in the differential diagnosis of MPNST, including synovial sarcoma and spindle cell melanoma, suggests that these 3 are helpful markers of Schwannian differentiation in the context of diagnosing MPNSTs.

  10. Normal intellectual development in children born from women with hypothyroxinemia during their pregnancy.

    PubMed

    Grau, Gema; Aguayo, Anibal; Vela, Amaia; Aniel-Quiroga, Angeles; Espada, Mercedes; Miranda, Gorka; Martinez-Indart, Lorea; Martul, Pedro; Castaño, Luis; Rica, Itxaso

    2015-01-01

    Proper maternal thyroid function is known to be essential for neural differentiation and migration in the fetus during the first half of pregnancy. The objectives of this study were to assess the relationship between thyroxin levels, in pregnant women with no thyroid disease and the intellectual development of their offspring in a non-iodine-deficient area, and to know specifically whether or not isolated hypothyroxinemia during pregnancy was associated with a lower intelligence in the offspring. Previously we had publicated values TSH, FT4, free T3 (FT3), anti-thyroid peroxidase antibodies (TPO Abs) and urinary iodine concentration (UIC) in 1322 pregnant women in our hospital area. Now we presented results of intelligence quotient in children born from these pregnancies. We assessed 455 children at one year of age using Brunet-Lezine scale. Of these, 289 children were evaluated again at 6-8 years of age using the WISC-IV. From the total group of children recruited, we established as control subgroup, children born of rigorously normal pregnancies (women with UIC > 150 μg/L, FT4>10th percentile and TPO-Ab negative in both trimesters). The remaining children were divided into two subgroups: those born to mothers with FT4 below the 10th percentile and the rest. No correlation was found between FT4 maternal levels, in either of trimesters studied, and the intellectual scores of offspring. No differences were found in intellectual scores comparing children born to mothers with hypothyroxinemia and those whose mothers were euthyroxinemic in both trimesters, or with the control subgroup. As conclusions we did not find any association between the levels of maternal FT4 during pregnancy and the subsequent intellectual development the offspring from these pregnancies. We attribute this result to the fact that all the pregnant women included had normal thyroid function. PMID:26004887

  11. Darkness during early postnatal development is required for normal circadian patterns in the adult rat.

    PubMed

    Cambras, T; Canal, M M; Cernuda-Cernuda, R; García-Fernández, J M; Díez-Noguera, A

    2015-03-01

    Early light experience influences the brain during development. Perinatal light exposure has an important effect on the development of the circadian system, although the role of quantity versus quality of light in this process is still unclear. We tested the development of the circadian rhythm of locomotor activity under constant bright light from the day of weaning, of six groups of rats raised under different light conditions during suckling. Results indicated that when rats received daily darkness during suckling (rats reared under constant darkness or light-dark cycles with dim or bright light) became arrhythmic when exposed to continuous bright light after weaning. However, those rats reared in the absence of darkness (constant dim or bright light, or alternating dim and bright light) developed a circadian rhythm, which was stronger and had a shorter period depending on the quantity of light received during suckling. Vasointestinal polypeptide immunoreactivity in the suprachiasmatic nucleus (SCN) was higher in those rats with weaker rhythms. However, no apparent differences among these groups were found in the melanopsin-expressing retinal ganglion cells, which provide the SCN with light input in the photoentrainment process. When bright light was shifted to dim light in three of the groups on day 57 after weaning, all of them generated a circadian rhythm with a longer period in those rats previously arrhythmic. Our results indicate the importance of the amount of light received at the early stages of life in the development of the circadian system and suggest that darkness is needed for the normal development of circadian behaviour.

  12. Jean-Martin Charcot at the birth of Russian neurology.

    PubMed

    Vein, Alla A

    2011-01-01

    Russian neurology was virtually nonexistent in the middle of the 19th century which made a traineeship abroad an absolute necessity. Charcot and his school did not just offer professional training, but created the best minds, which would determine the direction of neurology and psychiatry in Russia for many decades. After returning home, young Russian doctors not only implemented everything they had learned in Western Europe, but proceeded to make their own original contributions. The most talented pupils of Charcot, including such prominent names as Kozhevnikov, Korsakov, Minor, Bekhterev and Darkshevich, became the founders of neurological schools in Russia. They laid the basis for the further development of neurology and psychiatry. Remarkably, though trained by the same teachers, each of these future 'founding fathers' of these neurological and psychiatric schools followed his own individual path which resulted in an undeniable diversity in Russian neurology and psychiatry during the period of their formation.

  13. Development of the method and U.S. normalization database for Life Cycle Impact Assessment and sustainability metrics.

    PubMed

    Bare, Jane; Gloria, Thomas; Norris, Gregory

    2006-08-15

    Normalization is an optional step within Life Cycle Impact Assessment (LCIA) that may be used to assist in the interpretation of life cycle inventory data as well as life cycle impact assessment results. Normalization transforms the magnitude of LCI and LCIA results into relative contribution by substance and life cycle impact category. Normalization thus can significantly influence LCA-based decisions when tradeoffs exist. The U. S. Environmental Protection Agency (EPA) has developed a normalization database based on the spatial scale of the 48 continental U.S. states, Hawaii, Alaska, the District of Columbia, and Puerto Rico with a one-year reference time frame. Data within the normalization database were compiled based on the impact methodologies and lists of stressors used in TRACI-the EPA's Tool for the Reduction and Assessment of Chemical and other environmental Impacts. The new normalization database published within this article may be used for LCIA case studies within the United States, and can be used to assist in the further development of a global normalization database. The underlying data analyzed for the development of this database are included to allow the development of normalization data consistent with other impact assessment methodologies as well. PMID:16955915

  14. Normal Development and Function of T Cells in Proline Rich 7 (Prr7) Deficient Mice

    PubMed Central

    Hrdinka, Matous; Sudan, Kritika; Just, Sissy; Drobek, Ales; Stepanek, Ondrej; Schlüter, Dirk; Reinhold, Dirk; Jordan, Bryen A.; Gintschel, Patricia; Schraven, Burkhart; Kreutz, Michael R.

    2016-01-01

    Transmembrane adaptor proteins (TRAPs) are important organisers for the transduction of immunoreceptor-mediated signals. Prr7 is a TRAP that regulates T cell receptor (TCR) signalling and potently induces cell death when overexpressed in human Jurkat T cells. Whether endogenous Prr7 has a similar functional role is currently unknown. To address this issue, we analysed the development and function of the immune system in Prr7 knockout mice. We found that loss of Prr7 partially impairs development of single positive CD4+ T cells in the thymus but has no effect on the development of other T cell subpopulations, B cells, NK cells, or NKT cells. Moreover, Prr7 does not affect the TCR signalling pathway as T cells derived from Prr7 knockout and wild-type animals and stimulated in vitro express the same levels of the activation marker CD69, and retain their ability to proliferate and activate induced cell death programs. Importantly, Prr7 knockout mice retained the capacity to mount a protective immune response when challenged with Listeria monocytogenes infection in vivo. In addition, T cell effector functions (activation, migration, and reactivation) were normal following induction of experimental autoimmune encephalomyelitis (EAE) in Prr7 knockout mice. Collectively, our work shows that loss of Prr7 does not result in a major immune system phenotype and suggests that Prr7 has a dispensable function for TCR signalling. PMID:27657535

  15. The transcriptional activator ZNF143 is essential for normal development in zebrafish

    PubMed Central

    2012-01-01

    Background ZNF143 is a sequence-specific DNA-binding protein that stimulates transcription of both small RNA genes by RNA polymerase II or III, or protein-coding genes by RNA polymerase II, using separable activating domains. We describe phenotypic effects following knockdown of this protein in developing Danio rerio (zebrafish) embryos by injection of morpholino antisense oligonucleotides that target znf143 mRNA. Results The loss of function phenotype is pleiotropic and includes a broad array of abnormalities including defects in heart, blood, ear and midbrain hindbrain boundary. Defects are rescued by coinjection of synthetic mRNA encoding full-length ZNF143 protein, but not by protein lacking the amino-terminal activation domains. Accordingly, expression of several marker genes is affected following knockdown, including GATA-binding protein 1 (gata1), cardiac myosin light chain 2 (cmlc2) and paired box gene 2a (pax2a). The zebrafish pax2a gene proximal promoter contains two binding sites for ZNF143, and reporter gene transcription driven by this promoter in transfected cells is activated by this protein. Conclusions Normal development of zebrafish embryos requires ZNF143. Furthermore, the pax2a gene is probably one example of many protein-coding gene targets of ZNF143 during zebrafish development. PMID:22268977

  16. Neurological disorders and inflammatory bowel diseases

    PubMed Central

    Casella, Giovanni; Tontini, Gian Eugenio; Bassotti, Gabrio; Pastorelli, Luca; Villanacci, Vincenzo; Spina, Luisa; Baldini, Vittorio; Vecchi, Maurizio

    2014-01-01

    Extraintestinal manifestations occur in about one-third of patients living with inflammatory bowel disease (IBD) and may precede the onset of gastrointestinal symptoms by many years. Neurologic disorders associated with IBD are not frequent, being reported in 3% of patients, but they often represent an important cause of morbidity and a relevant diagnostic issue. In addition, the increasing use of immunosuppressant and biological therapies for IBD may also play a pivotal role in the development of neurological disorders of different type and pathogenesis. Hence, we provide a complete and profound review of the main features of neurological complications associated with IBD, with particular reference to those related to drugs and with a specific focus on their clinical presentation and possible pathophysiological mechanisms. PMID:25083051

  17. Nonlocal neurology: beyond localization to holonomy.

    PubMed

    Globus, G G; O'Carroll, C P

    2010-11-01

    The concept of local pathology has long served neurology admirably. Relevant models include self-organizing nonlinear brain dynamics, global workspace and dynamic core theories. However such models are inconsistent with certain clinical phenomena found in Charles Bonnet syndrome, disjunctive agnosia and schizophrenia, where there is disunity of content within the unity of consciousness. This is contrasted with the split-brain case where there is disunity of content and disunity of consciousnesses. The development of quantum brain theory with it nonlocal mechanisms under the law of the whole ("holonomy") offers new possibilities for explaining disintegration within unity. Dissipative quantum brain dynamics and its approach to the binding problem, memory and consciousness are presented. A nonlocal neurology armed with a holonomic understanding might see more deeply into what clinical neurology has always aspired to: the patient as a whole.

  18. Temporal resolution in individuals with neurological disorders

    PubMed Central

    Rabelo, Camila Maia; Weihing, Jeffrey A; Schochat, Eliane

    2015-01-01

    OBJECTIVE: Temporal processing refers to the ability of the central auditory nervous system to encode and detect subtle changes in acoustic signals. This study aims to investigate the temporal resolution ability of individuals with mesial temporal sclerosis and to determine the sensitivity and specificity of the gaps-in-noise test in identifying this type of lesion. METHOD: This prospective study investigated differences in temporal resolution between 30 individuals with normal hearing and without neurological lesions (G1) and 16 individuals with both normal hearing and mesial temporal sclerosis (G2). Test performances were compared, and the sensitivity and specificity were calculated. RESULTS: There was no difference in gap detection thresholds between the two groups, although G1 revealed better average thresholds than G2 did. The sensitivity and specificity of the gaps-in-noise test for neurological lesions were 68% and 98%, respectively. CONCLUSIONS: Temporal resolution ability is compromised in individuals with neurological lesions caused by mesial temporal sclerosis. The gaps-in-noise test was shown to be a sensitive and specific measure of central auditory dysfunction in these patients. PMID:26375561

  19. Acute intravenous synaptamine complex variant KB220™ "normalizes" neurological dysregulation in patients during protracted abstinence from alcohol and opiates as observed using quantitative electroencephalographic and genetic analysis for reward polymorphisms: part 1, pilot study with 2 case reports.

    PubMed

    Miller, David K; Bowirrat, Abdalla; Manka, Matthew; Miller, Merlene; Stokes, Stanley; Manka, Debra; Allen, Cameron; Gant, Charles; Downs, B William; Smolen, Andrew; Stevens, Emily; Yeldandi, Swetha; Blum, Kenneth

    2010-11-01

    It is well established that in both food- and drug-addicted individuals, there is dopamine resistance due to an association with the DRD2 gene A1 allele. Evidence is emerging whereby the potential of utilizing a natural, nonaddicting, safe, putative D2 agonist may find its place in recovery from reward deficiency syndrome (RDS) in patients addicted to psychoactive chemicals. Utilizing quantitative electroencephalography (qEEG) as an imaging tool, we show the impact of Synaptamine Complex Variant KB220™ as a putative activator of the mesolimbic system. We demonstrate for the first time that its intravenous administration reduces or "normalizes" aberrant electrophysiological parameters of the reward circuitry site. For this pilot study, we report that the qEEGs of an alcoholic and a heroin abuser with existing abnormalities (ie, widespread theta and widespread alpha activity, respectively) during protracted abstinence are significantly normalized by the administration of 1 intravenous dose of Synaptamine Complex Variant KB220™. Both patients were genotyped for a number of neurotransmitter reward genes to determine to what extent they carry putative dopaminergic risk alleles that may predispose them for alcohol or heroin dependence, respectively. The genes tested included the dopamine transporter (DAT1, locus symbol SLC6A3), dopamine D4 receptor exon 3 VNTR (DRD4), DRD2 TaqIA (rs1800497), COMT val158 met SNP (rs4680), monoamine oxidase A upstream VNTR (MAOA-uVNTR), and serotonin transporter-linked polymorphic region (5HTTLPR, locus symbol SLC6A4). We emphasize that these are case studies, and it would be unlikely for all individuals to carry all putative risk alleles. Based on previous research and our qEEG studies (parts 1 and 2 of this study), we cautiously suggest that long-term activation of dopaminergic receptors (ie, DRD2 receptors) will result in their proliferation and lead to enhanced "dopamine sensitivity" and an increased sense of happiness

  20. DSPP Is Essential for Normal Development of the Dental-Craniofacial Complex.

    PubMed

    Chen, Y; Zhang, Y; Ramachandran, A; George, A

    2016-03-01

    The craniofacial skeleton is derived from both neural crest cells and mesodermal cells; however, the majority of the bone, cartilage, and connective tissue is derived from the neural crest. Dentin sialophosphoprotein (DSPP) is a precursor protein that is expressed by the connective tissues of the craniofacial skeleton, namely, bone and dentin with high expression levels in the dentin matrix. Gene ablation studies have shown severe dental defects in DSPP-null mutant mice. Therefore, to elucidate the role of DSPP on the developing dental-craniofacial complex, we evaluated phenotypic changes in the structure of intramembranous bone and dentin mineralization using 3 different age groups of DSPP-null and wild-type mice. Results from micro-computed tomographic, radiographic, and optical microscopic analyses showed defective dentin, alveolar and calvarial bones, and sutures during development. The impaired mineralization of the cranial bone correlated well with low expression levels of Runx2, Col1, and OPN identified using calvarial cells from DSPP-null and wild-type mice in an in vitro culture system. However, the upregulation of MMP9, MMP2, FN, and BSP was observed. Interestingly, the null mice also displayed low serum phosphate levels, while calcium levels remained unchanged. Alizarin red and von Kossa staining confirmed the dysfunction in the terminal differentiation of osteoblasts obtained from the developing calvaria of DSPP-null mice. Immunohistochemical analysis of the developing molars showed changes in Runx2, Gli1, Numb, and Notch expression in the dental pulp cells and odontoblasts of DSPP-null mice when compared with wild-type mice. Overall, these observations provide insight into the role of DSPP in the normal development of the calvaria, alveolar bone, and dentin-pulp complex.

  1. The Neurology Quality of Life Measurement Initiative

    PubMed Central

    Cella, David; Nowinski, Cindy; Peterman, Amy; Victorson, David; Miller, Deborah; Lai, Jin-Shei; Moy, Claudia

    2011-01-01

    Objective The National Institute of Neurological Disorders and Stroke (NINDS) commissioned the Neurology Quality of Life (Neuro-QOL) project to develop a bilingual (English/Spanish), clinically relevant and psychometrically robust HRQL assessment tool. This paper describes the development and calibration of these banks and scales. Design Classical and modern test construction methodologies were used, including input from essential stakeholder groups. Setting An online patient panel testing service and eleven academic medical centers and clinics from across the United States and Puerto Rico that treat major neurological disorders. Participants Adult and pediatric patients representing different neurological disorders specified in this study, proxy respondents for select conditions (stroke and pediatric conditions), and English and Spanish speaking participants from the general population. Main Outcome Measures Multiple generic and condition specific measures used to provide construct validity evidence to new Neuro-QOL tool. Results Neuro-QOL has developed 14 generic item banks and 8 targeted scales to assess HRQL in five adult (stroke, multiple sclerosis, Parkinson’s disease, epilepsy, and amyotrophic lateral sclerosis) and two pediatric conditions (epilepsy and muscular dystrophies). Conclusions The Neuro-QOL system will continue to evolve, with validation efforts in clinical populations, and new bank development in health domains not currently included. The potential for Neuro-QOL measures in rehabilitation research and clinical settings is discussed. PMID:21958920

  2. Prenatal Antecedents of Newborn Neurological Maturation

    ERIC Educational Resources Information Center

    DiPietro, Janet A.; Kivlighan, Katie T.; Costigan, Kathleen A.; Rubin, Suzanne E.; Shiffler, Dorothy E.; Henderson, Janice L.; Pillion, Joseph P.

    2010-01-01

    Fetal neurobehavioral development was modeled longitudinally using data collected at weekly intervals from 24 to 38 weeks gestation in a sample of 112 healthy pregnancies. Predictive associations between 3 measures of fetal neurobehavioral functioning and their developmental trajectories to neurological maturation in the first weeks after birth…

  3. Anaerobic Infections in Children with Neurological Impairments.

    ERIC Educational Resources Information Center

    Brook, Itzhak

    1995-01-01

    Children with neurological impairments are prone to develop serious infection with anaerobic bacteria. The most common anaerobic infections are decubitus ulcers; gastrostomy site wound infections; pulmonary infections (aspiration pneumonia, lung abscesses, and tracheitis); and chronic suppurative otitis media. The unique microbiology of each of…

  4. Recognition and treatment of neurologic Wilson's disease.

    PubMed

    Lorincz, Matthew T

    2012-11-01

    As Wilson's disease is both preventable and treatable, the diagnosis must not be missed. Despite this, it is usually misdiagnosed. Misdiagnosis and delay in treatment are clinically relevant because if left untreated, Wilson's disease progresses to hepatic failure or severe neurologic disability, and death. Those adequately treated have a normal life span. Wilson's disease is an autosomal recessive disease caused by mutations in the ATP7B gene. Mutations in ATP7B result in abnormal copper metabolism and subsequent toxic accumulation of copper. The clinical manifestations of neurologic Wilson's disease include variable combinations of dysarthria, dystonia, tremor, parkinsonism, ataxia, and choreoathetosis. Once the possibility of Wilson's disease is considered, diagnosis is straight forward. Currently available treatments, including zinc acetate and trientine, are generally well tolerated and effective.

  5. Trends in normalized difference vegetation index (NDVI) associated with urban development in northern West Siberia

    NASA Astrophysics Data System (ADS)

    Esau, Igor; Miles, Victoria V.; Davy, Richard; Miles, Martin W.; Kurchatova, Anna

    2016-08-01

    Exploration and exploitation of oil and gas reserves of northern West Siberia has promoted rapid industrialization and urban development in the region. This development leaves significant footprints on the sensitive northern environment, which is already stressed by the global warming. This study reports the region-wide changes in the vegetation cover as well as the corresponding changes in and around 28 selected urbanized areas. The study utilizes the normalized difference vegetation index (NDVI) from high-resolution (250 m) MODIS data acquired for summer months (June through August) over 15 years (2000-2014). The results reveal the increase of NDVI (or "greening") over the northern (tundra and tundra-forest) part of the region. Simultaneously, the southern, forested part shows the widespread decrease of NDVI (or "browning"). These region-wide patterns are, however, highly fragmented. The statistically significant NDVI trends occupy only a small fraction of the region. Urbanization destroys the vegetation cover within the developed areas and at about 5-10 km distance around them. The studied urbanized areas have the NDVI values by 15 to 45 % lower than the corresponding areas at 20-40 km distance. The largest NDVI reduction is typical for the newly developed areas, whereas the older areas show recovery of the vegetation cover. The study reveals a robust indication of the accelerated greening near the older urban areas. Many Siberian cities become greener even against the wider browning trends at their background. Literature discussion suggests that the observed urban greening could be associated not only with special tending of the within-city green areas but also with the urban heat islands and succession of more productive shrub and tree species growing on warmer sandy soils.

  6. Eculizumab in Typical Hemolytic Uremic Syndrome (HUS) With Neurological Involvement

    PubMed Central

    Pape, Lars; Hartmann, Hans; Bange, Franz Christoph; Suerbaum, Sebastian; Bueltmann, Eva; Ahlenstiel-Grunow, Thurid

    2015-01-01

    Abstract In typical hemolytic uremic syndrome (HUS) approximately 25% of patients show central nervous system (CNS) involvement often leading to serious long-term disabilities. We used the C5-complement inhibitor Eculizumab as rescue therapy. From 2011 to 2014, 11 children (median age 22 months, range 11–175) with enterohemorrhagic Escherichia coli-positive HUS requiring dialysis who had seizures (11/11) and/or were in a stupor or coma (10/11) were treated with Eculizumab. Two patients enrolled on the Safety and Efficacy Study of Eculizumab in Shiga-Toxin Producing E coli Hemolytic-Uremic Syndrome (STEC-HUS) each received 6 doses of Eculizumab, 3 patients 2 doses, and 6 patients 1 dose. Laboratory diagnostics of blood samples and magnetic resonance imaging (MRI) were performed as per center practice. Data were analyzed retrospectively. Cranial MRI was abnormal in 8 of 10 patients with findings in the basal ganglia and/or white matter. A 2-year-old boy with severe cardiac involvement and status epilepticus needed repeated cardio-pulmonary resuscitation and extracorporeal membrane oxygenation. He died 8 days after start of Eculizumab treatment. Two patients with hemorrhagic colitis and repeated seizures required artificial ventilation for 6 and 16 days, respectively. At the time of discharge, 1 patient showed severe neurological impairment and 1 mild neurological impairment. The 8 surviving patients experienced no further seizures after the first dose of Eculizumab. Three patients showed mild neurological impairment at discharge, whilst the remaining 5 showed no impairment. The platelets normalized 4 days (median) after the first dose of Eculizumab (range 0–20 days). The mean duration of dialysis after the first dose of Eculizumab was 14.1 ± 6.1 days. In children with typical HUS and CNS involvement early use of Eculizumab appears to improve neurological outcome. In severe HUS cases which progress rapidly with multiple organ involvement, late treatment with

  7. Smpd3 Expression in both Chondrocytes and Osteoblasts Is Required for Normal Endochondral Bone Development.

    PubMed

    Li, Jingjing; Manickam, Garthiga; Ray, Seemun; Oh, Chun-do; Yasuda, Hideyo; Moffatt, Pierre; Murshed, Monzur

    2016-09-01

    Sphingomyelin phosphodiesterase 3 (SMPD3), a lipid-metabolizing enzyme present in bone and cartilage, has been identified to be a key regulator of skeletal development. A homozygous loss-of-function mutation called fragilitas ossium (fro) in the Smpd3 gene causes poor bone and cartilage mineralization resulting in severe congenital skeletal deformities. Here we show that Smpd3 expression in ATDC5 chondrogenic cells is downregulated by parathyroid hormone-related peptide through transcription factor SOX9. Furthermore, we show that transgenic expression of Smpd3 in the chondrocytes of fro/fro mice corrects the cartilage but not the bone abnormalities. Additionally, we report the generation of Smpd3(flox/flox) mice for the tissue-specific inactivation of Smpd3 using the Cre-loxP system. We found that the skeletal phenotype in Smpd3(flox/flox); Osx-Cre mice, in which the Smpd3 gene is ablated in both late-stage chondrocytes and osteoblasts, closely mimics the skeletal phenotype in fro/fro mice. On the other hand, Smpd3(flox/flox); Col2a1-Cre mice, in which the Smpd3 gene is knocked out in chondrocytes only, recapitulate the fro/fro mouse cartilage phenotype. This work demonstrates that Smpd3 expression in both chondrocytes and osteoblasts is required for normal endochondral bone development. PMID:27325675

  8. Polyphenol oxidase affects normal nodule development in red clover (Trifolium pratense L.)

    PubMed Central

    Webb, K. Judith; Cookson, Alan; Allison, Gordon; Sullivan, Michael L.; Winters, Ana L.

    2014-01-01

    Polyphenol oxidase (PPO) may have multiple functions in tissues depending on its cellular or tissue localization. Here we use PPO RNAi transformants of red clover (Trifolium pratense) to determine the role PPO plays in normal development of plants, and especially in N2-fixing nodules. In red clover, PPO was not essential for either growth or nodule production, or for nodule function in plants grown under optimal, N-free conditions. However, absence of PPO resulted in a more reduced environment in all tissues, as measured by redox potential, and caused subtle developmental changes in nodules. Leaves and, to a lesser extent nodules, lacking PPO tended to accumulate phenolic compounds. A comparison of nodules of two representative contrasting clones by microscopy revealed that nodules lacking PPO were morphologically and anatomically subtly altered, and that phenolics accumulated in different cells and tissues. Developing nodules lacking PPO were longer, and there were more cell layers within the squashed cell layer (SCL), but the walls of these cells were less thickened and the cells were less squashed. Within the N2-fixing zone, bacteroids appeared more granular and were less tightly packed together, and were similar to developmentally compromised bacteroids elicited by catalase mutant rhizobia reported elsewhere. PMID:25566275

  9. EHD1 mediates vesicle trafficking required for normal muscle growth and transverse tubule development.

    PubMed

    Posey, Avery D; Swanson, Kaitlin E; Alvarez, Manuel G; Krishnan, Swathi; Earley, Judy U; Band, Hamid; Pytel, Peter; McNally, Elizabeth M; Demonbreun, Alexis R

    2014-03-15

    EHD proteins have been implicated in intracellular trafficking, especially endocytic recycling, where they mediate receptor and lipid recycling back to the plasma membrane. Additionally, EHDs help regulate cytoskeletal reorganization and induce tubule formation. It was previously shown that EHD proteins bind directly to the C2 domains in myoferlin, a protein that regulates myoblast fusion. Loss of myoferlin impairs normal myoblast fusion leading to smaller muscles in vivo but the intracellular pathways perturbed by loss of myoferlin function are not well known. We now characterized muscle development in EHD1-null mice. EHD1-null myoblasts display defective receptor recycling and mislocalization of key muscle proteins, including caveolin-3 and Fer1L5, a related ferlin protein homologous to myoferlin. Additionally, EHD1-null myoblast fusion is reduced. We found that loss of EHD1 leads to smaller muscles and myofibers in vivo. In wildtype skeletal muscle EHD1 localizes to the transverse tubule (T-tubule), and loss of EHD1 results in overgrowth of T-tubules with excess vesicle accumulation in skeletal muscle. We provide evidence that tubule formation in myoblasts relies on a functional EHD1 ATPase domain. Moreover, we extended our studies to show EHD1 regulates BIN1 induced tubule formation. These data, taken together and with the known interaction between EHD and ferlin proteins, suggests that the EHD proteins coordinate growth and development likely through mediating vesicle recycling and the ability to reorganize the cytoskeleton.

  10. EHD1 mediates vesicle trafficking required for normal muscle growth and tubule development

    PubMed Central

    Posey, Avery D.; Swanson, Kaitlin E.; Alvarez, Manuel G.; Krishnan, Swathi; Earley, Judy E.; Band, Hamid; Pytel, Peter; McNally, Elizabeth M.; Demonbreun, Alexis R.

    2014-01-01

    EHD proteins have been implicated in intracellular trafficking, especially endocytic recycling, where they mediate receptor and lipid recycling back to the plasma membrane. Additionally, EHDs help regulate cytoskeletal reorganization and induce tubule formation. It was previously shown that EHD proteins bind directly to the C2 domains in myoferlin, a protein that regulates myoblast fusion. Loss of myoferlin impairs normal myoblast fusion leading to smaller muscles in vivo but the intracellular pathways perturbed by loss of myoferlin function are not well known. We now characterized muscle development in EHD1-null mice. EHD1-null myoblasts display defective receptor recycling and mislocalization of key muscle proteins, including caveolin-3 and Fer1L5, a related ferlin protein homologous to myoferlin. Additionally, EHD1-null myoblast fusion is reduced. We found that loss of EHD1 leads to smaller muscles and myofibers in vivo. In wildtype skeletal muscle EHD1 localizes to the transverse tubule (T-tubule), and loss of EHD1 results in overgrowth of T-tubules with excess vesicle accumulation in skeletal muscle. We provide evidence that tubule formation in myoblasts relies on a functional EHD1 ATPase domain. Moreover, we extended our studies to show EHD1 regulates BIN1 induced tubule formation. These data, taken together and with the known interaction between EHD and ferlin proteins, suggests that the EHD proteins coordinate growth and development likely through mediating vesicle recycling and the ability to reorganize the cytoskeleton. PMID:24440153

  11. [Neurological soft signs in schizophrenia: correlations with age, sex, educational status and psychopathology].

    PubMed

    Panagiotidis, P; Kaprinis, G; Iacovides, A; Fountoulakis, K

    2013-01-01

    Though the pathobiology of schizophrenia can be examined in multiple levels, the organic notion of brain disease suggests that neurological features will be present. One straightforward, inexpensive method of investigating brain dysfunction in schizophrenia is thought the bedside assessment of neurological abnormalities with a standard neurological examination. Neurological abnormalities are traditionally classified as "hard signs" (impairments in basic motor, sensory, and reflex behaviors, which do not appear to be affected in schizophrenia) and "soft signs", which refer to more complex phenomena such as abnormalities in motor control, integrative sensory function, sensorimotor integration, and cerebral laterality. Additionally, neurological soft signs (NSS) are minor motor and sensory abnormalities that are considered to be normal in the course of early development but abnormal when elicited in later life or persist beyond childhood. Soft signs also, have no definitive localizing significance but are indicative of subtle brain dysfunction. Most authors believe that they are a reflection not only of deficient integration between the sensory and motor systems, but also of dysfunctional neuronal circuits linking subcortical brain structures such as the basal ganglia, the brain stem, and the limbic system. Throughout the last four decades, studies have consistently shown that NSS are more frequently present in patients with schizophrenia than in normal subjects and non-psychotic psychiatric patients. However, the functional relevance of NSS remains unclear and their specificity has often been challenged, even though there is indication for a relative specificity with regard to diagnosis, or symptomatology. Many studies have considered soft signs as categorical variables thus hampering the evaluation of fluctuation with symptomatology and/or treatment, whereas other studies included insufficient number of assessed signs, or lacked a comprehensive assessment of

  12. [Neurological soft signs in schizophrenia: correlations with age, sex, educational status and psychopathology].

    PubMed

    Panagiotidis, P; Kaprinis, G; Iacovides, A; Fountoulakis, K

    2013-01-01

    Though the pathobiology of schizophrenia can be examined in multiple levels, the organic notion of brain disease suggests that neurological features will be present. One straightforward, inexpensive method of investigating brain dysfunction in schizophrenia is thought the bedside assessment of neurological abnormalities with a standard neurological examination. Neurological abnormalities are traditionally classified as "hard signs" (impairments in basic motor, sensory, and reflex behaviors, which do not appear to be affected in schizophrenia) and "soft signs", which refer to more complex phenomena such as abnormalities in motor control, integrative sensory function, sensorimotor integration, and cerebral laterality. Additionally, neurological soft signs (NSS) are minor motor and sensory abnormalities that are considered to be normal in the course of early development but abnormal when elicited in later life or persist beyond childhood. Soft signs also, have no definitive localizing significance but are indicative of subtle brain dysfunction. Most authors believe that they are a reflection not only of deficient integration between the sensory and motor systems, but also of dysfunctional neuronal circuits linking subcortical brain structures such as the basal ganglia, the brain stem, and the limbic system. Throughout the last four decades, studies have consistently shown that NSS are more frequently present in patients with schizophrenia than in normal subjects and non-psychotic psychiatric patients. However, the functional relevance of NSS remains unclear and their specificity has often been challenged, even though there is indication for a relative specificity with regard to diagnosis, or symptomatology. Many studies have considered soft signs as categorical variables thus hampering the evaluation of fluctuation with symptomatology and/or treatment, whereas other studies included insufficient number of assessed signs, or lacked a comprehensive assessment of

  13. Preliminary models of normal fault development in subduction zones: lithospheric strength and outer rise deformation

    NASA Astrophysics Data System (ADS)

    Naliboff, J. B.; Billen, M. I.

    2010-12-01

    A characteristic feature of global subduction zones is normal faulting in the outer rise region, which reflects flexure of the downgoing plate in response to the slab pull force. Variations in the patterns of outer rise normal faulting between different subduction zones likely reflects both the magnitude of flexural induced topography and the strength of the downgoing plate. In particular, the rheology of the uppermost oceanic lithosphere is likely to strongly control the faulting patterns, which have been well documented recently in both the Middle and South American trenches. These recent observations of outer rise faulting provide a unique opportunity to test different rheological models of the oceanic lithosphere using geodynamic numerical experiments. Here, we develop a new approach for modeling deformation in the outer rise and trench regions of downgoing slabs, and discuss preliminary 2-D numerical models examining the relationship between faulting patterns and the rheology of the oceanic lithosphere. To model viscous and brittle deformation within the oceanic lithosphere we use the CIG (Computational Infrastructure for Geodynamics) finite element code Gale, which is designed to solve long-term tectonic problems. In order to resolve deformation features on geologically realistic scales (< 1 km), we model only the portion of the subduction system seaward of the trench. Horizontal and vertical stress boundary conditions on the side walls drive subduction and reflect, respectively, the ridge-push and slab-pull plate-driving forces. The initial viscosity structure of the oceanic lithosphere and underlying asthenosphere follow a composite viscosity law that takes into account both Newtonian and non-Newtonian deformation. The viscosity structure is consequently governed primarily by the strain rate and thermal structure, which follows a half-space cooling model. Modification of the viscosity structure and development of discrete shear zones occurs during yielding

  14. Characterization of the equine blood-testis barrier during tubular development in normal and cryptorchid stallions.

    PubMed

    Rode, K; Sieme, H; Richterich, P; Brehm, R

    2015-09-15

    The formation of the blood-testis barrier (BTB) is defined as occurring with the first appearance of spermatocytes at around puberty and is vital for normal spermatogenesis. This barrier between two adjacent Sertoli cells (SCs) consists of a cell junctional protein complex, which includes tight junctions (TJs), adherens junctions, and gap junctions. In many mammalian species, BTB composition has already been investigated, whereas little is known about the equine BTB. In the present study, immunohistochemistry and qualitative Western Blot analysis were used to assess the expression and distribution patterns of the junctional proteins claudin-11 (TJ), zonula occludens-1 (TJ associated), N-cadherin (adherens junctions), and connexin 43 (gap junctions) in equine testes during tubular development and in testes of stallions exhibiting unilateral cryptorchidism. Therefore, testes of 21 warmblood stallions (aged 12 months-11 years) were obtained during routine surgical castration. In the normal adult equine testis, the junctional proteins are localized at the basolateral region of the seminiferous tubules forming a circumferential seal corresponding to the known BTB localization. N-cadherin is additionally expressed along the lateral SC surface. In immature seminiferous cords still lacking a lumen, a diffuse distribution pattern of the junctional proteins throughout the SC cytoplasm is visible. As lumen formation advances, the immunolocalization shifts progressively toward the basolateral SC membranes. Additionally, apoptotic germ cells were detected and quantified in prepubertal stallions using terminal deoxynucleotidyl transferase dUTP nick end labeling assay and correlated with junctional protein localization. In the retained testis of cryptorchid stallions, which exhibit an aberrant testicular morphology, a deviating expression of the junctional proteins is visible. The present data show for the first time that (1) the equine SC junctional complex contains claudin-11

  15. History of neurologic examination books

    PubMed Central

    2015-01-01

    The objective of this study was to create an annotated list of textbooks dedicated to teaching the neurologic examination. Monographs focused primarily on the complete neurologic examination published prior to 1960 were reviewed. This analysis was limited to books with the word “examination” in the title, with exceptions for the texts of Robert Wartenberg and Gordon Holmes. Ten manuals met the criteria. Works dedicated primarily to the neurologic examination without a major emphasis on disease description or treatment first appeared in the early 1900s. Georg Monrad-Krohn's “Blue Book of Neurology” (“Blue Bible”) was the earliest success. These treatises served the important purpose of educating trainees on proper neurologic examination technique. They could make a reputation and be profitable for the author (Monrad-Krohn), highlight how neurology was practiced at individual institutions (McKendree, Denny-Brown, Holmes, DeJong, Mayo Clinic authors), and honor retiring mentors (Mayo Clinic authors). PMID:25829645

  16. NMDA receptors on zebrafish Mauthner cells require CaMKII-α for normal development.

    PubMed

    Roy, Birbickram; Ferdous, Jannatul; Ali, Declan W

    2015-02-01

    Calcium/calmodulin dependent protein kinase 2 (CaMKII) is a multifunctional protein that is highly enriched in the synapse. It plays important roles in neuronal functions such as synaptic plasticity, synaptogenesis, and neural development. Gene duplication in zebrafish has resulted in the occurrence of seven CaMKII genes (camk2a, camk2b1, camk2b2, camk2g1, camk2g2, camk2d1, and camk2d2) that are developmentally expressed. In this study, we used single cell, real-time quantitative PCR to investigate the expression of CaMKII genes in individual Mauthner cells (M-cells) of 2 days post fertilization (dpf) zebrafish embryos. We found that out of seven different CaMKII genes, only the mRNA for CaMKII-α was expressed in the M-cell at detectable levels, while all other isoforms were undetectable. Morpholino knockdown of CaMKII-α had no significant effect on AMPA synaptic currents (mEPSCs) but decreased the amplitude of NMDA mEPSCs. NMDA events exhibited a biexponential decay with τfast ≈ 30 ms and τslow ≈ 300 ms. Knockdown of CaMKII-α specifically reduced the amplitude of the slow component of the NMDA-mediated currents (mEPSCs), without affecting the fast component, the frequency, or the kinetics of the mEPSCs. Immunolabelling of the M-cell showed increased dendritic arborizations in the morphants compared with controls, and knockdown of CaMKII-α altered locomotor behaviors of touch responses. These results suggest that CaMKII-α is present in embryonic M-cells and that it plays a role in the normal development of excitatory synapses. Our findings pave the way for determining the function of specific CaMKII isoforms during the early stages of M-cell development.

  17. Hmx1 is required for the normal development of somatosensory neurons in the geniculate ganglion.

    PubMed

    Quina, Lely A; Tempest, Lynne; Hsu, Yun-Wei A; Cox, Timothy C; Turner, Eric E

    2012-05-01

    Hmx1 is a variant homeodomain transcription factor expressed in the developing sensory nervous system, retina, and craniofacial mesenchyme. Recently, mutations at the Hmx1 locus have been linked to craniofacial defects in humans, rats, and mice, but its role in nervous system development is largely unknown. Here we show that Hmx1 is expressed in a subset of sensory neurons in the cranial and dorsal root ganglia which does not correspond to any specific sensory modality. Sensory neurons in the dorsal root and trigeminal ganglia of Hmx1dm/dm mouse embryos have no detectable Hmx1 protein, yet they undergo neurogenesis and express sensory subtype markers normally, demonstrating that Hmx1 is not globally required for the specification of sensory neurons from neural crest precursors. Loss of Hmx1 expression has no obvious effect on the early development of the trigeminal (V), superior (IX/X), or dorsal root ganglia neurons in which it is expressed, but results in marked defects in the geniculate (VII) ganglion. Hmx1dm/dm mouse embryos possess only a vestigial posterior auricular nerve, and general somatosensory neurons in the geniculate ganglion are greatly reduced by mid-gestation. Although Hmx1 is expressed in geniculate neurons prior to cell cycle exit, it does not appear to be required for neurogenesis, and the loss of geniculate neurons is likely to be the result of increased cell death. Fate mapping of neural crest-derived tissues indicates that Hmx1-expressing somatosensory neurons at different axial levels may be derived from either the neural crest or the neurogenic placodes. PMID:22586713

  18. Human pluripotent stem cells as a model of trophoblast differentiation in both normal development and disease.

    PubMed

    Horii, Mariko; Li, Yingchun; Wakeland, Anna K; Pizzo, Donald P; Nelson, Katharine K; Sabatini, Karen; Laurent, Louise Chang; Liu, Ying; Parast, Mana M

    2016-07-01

    Trophoblast is the primary epithelial cell type in the placenta, a transient organ required for proper fetal growth and development. Different trophoblast subtypes are responsible for gas/nutrient exchange (syncytiotrophoblasts, STBs) and invasion and maternal vascular remodeling (extravillous trophoblasts, EVTs). Studies of early human placental development are severely hampered by the lack of a representative trophoblast stem cell (TSC) model with the capacity for self-renewal and the ability to differentiate into both STBs and EVTs. Primary cytotrophoblasts (CTBs) isolated from early-gestation (6-8 wk) human placentas are bipotential, a phenotype that is lost with increasing gestational age. We have identified a CDX2(+)/p63(+) CTB subpopulation in the early postimplantation human placenta that is significantly reduced later in gestation. We describe a reproducible protocol, using defined medium containing bone morphogenetic protein 4 by which human pluripotent stem cells (hPSCs) can be differentiated into CDX2(+)/p63(+) CTB stem-like cells. These cells can be replated and further differentiated into STB- and EVT-like cells, based on marker expression, hormone secretion, and invasive ability. As in primary CTBs, differentiation of hPSC-derived CTBs in low oxygen leads to reduced human chorionic gonadotropin secretion and STB-associated gene expression, instead promoting differentiation into HLA-G(+) EVTs in an hypoxia-inducible, factor-dependent manner. To validate further the utility of hPSC-derived CTBs, we demonstrated that differentiation of trisomy 21 (T21) hPSCs recapitulates the delayed CTB maturation and blunted STB differentiation seen in T21 placentae. Collectively, our data suggest that hPSCs are a valuable model of human placental development, enabling us to recapitulate processes that result in both normal and diseased pregnancies. PMID:27325764

  19. Epidermal growth factor receptor function is necessary for normal craniofacial development and palate closure.

    PubMed

    Miettinen, P J; Chin, J R; Shum, L; Slavkin, H C; Shuler, C F; Derynck, R; Werb, Z

    1999-05-01

    Craniofacial malformations are among the most frequent congenital birth defects in humans; cleft palate, that is inadequate fusion of the palatal shelves, occurs with an annual incidence of 1 in 700 to 1 in 1,000 live births among individuals of European descent. The secondary palate arises as bilateral outgrowths from the maxillary processes, and its formation depends on the coordinated development of craniofacial structures including the Meckel's cartilage and the mandible. Cleft lip and palate syndromes in humans are associated with polymorphisms in the gene (TGFA) encoding transforming growth factor-alpha (TGF-alpha), an epidermal growth factor receptor (EGFR) ligand made by most epithelia. Here we have characterized craniofacial development in Egfr-deficient (Egfr-/-) mice. Newborn Egfr-/- mice have facial mediolateral defects including narrow, elongated snouts, underdeveloped lower jaw and a high incidence of cleft palate. Palatal shelf explants from Egfr-/- mice fused, but frequently had residual epithelium in the midline. In addition, morphogenesis of Meckel's cartilage was deficient in cultured mandibular processes from Egfr-/- embryos. The secretion of matrix metalloproteinases (MMPs) was diminished in Egfr-/- explants, consistent with the ability of EGF to increase MMP secretion and with the decreased MMP expression caused by inhibition of Egfr signalling in wild-type explants. Accordingly, inactivation of MMPs in wild-type explants phenocopied the defective morphology of Meckel's cartilage seen in Egfr-/- explants. Our results indicate that EGFR signalling is necessary for normal craniofacial development and that its role is mediated in part by its downstream targets, the MMPs, and may explain the genetic correlation of human cleft palate with polymorphisms in TGFA.

  20. Neurologic Itch Management.

    PubMed

    Şavk, Ekin

    2016-01-01

    Neurologic itch is defined as pruritus resulting from any dysfunction of the nervous system. Itch arising due to a neuroanatomic pathology is seen to be neuropathic. Causes of neuropathic itch range from localized entrapment of a peripheral nerve to generalized degeneration of small nerve fibers. Antipruritic medications commonly used for other types of itch such as antihistamines and corticosteroids lack efficacy in neuropathic itch. Currently there are no therapeutic options that offer relief in all types of neuropathic pruritus, and treatment strategies vary according to etiology. It is best to decide on the appropriate tests and procedures in collaboration with a neurologist during the initial work-up. Treatment of neuropathic itch includes general antipruritic measures, local or systemic pharmacotherapy, various physical modalities, and surgery. Surgical intervention is the obvious choice of therapy in cases of spinal or cerebral mass, abscess, or hemorrhagic stroke, and may provide decompression in entrapment neuropathies. Symptomatic treatment is needed in the vast majority of patients. General antipruritic measures should be encouraged. Local treatment agents with at least some antipruritic effect include capsaicin, local anesthetics, doxepin, tacrolimus, and botulinum toxin A. Current systemic therapy relies on anticonvulsants such as gabapentin and pregabalin. Phototherapy, transcutaneous electrical nerve stimulation, and physical therapy have also been of value in selected cases. Among the avenues to be explored are transcranial magnetic stimulation of the brain, new topical cannabinoid receptor agonists, various modes of acupuncture, a holistic approach with healing touch, and cell transplantation to the spinal cord. PMID:27578080

  1. [Neurological interpretation of dreams] .

    PubMed

    Pareja, J A; Gil-Nagel, A

    2000-10-01

    Cerebral cortical activity is constant throughout the entire human life, but substantially changes during the different phases of the sleep-wake cycle (wakefulness, non-REM sleep and REM sleep), as well as in relation to available information. In particular, perception of the environment is closely linked to the wake-state, while during sleep perception turns to the internal domain or endogenous cerebral activity. External and internal information are mutually exclusive. During wakefulness a neuronal mechanism allows attention to focus on the environment whereas endogenous cortical activity is ignored. The opposite process is provided during sleep. The function external attention-internal attention is coupled with the two modes of brain function during wakefulness and during sleep, providing two possible cortical status: thinking and dreaming. Several neurological processes may influence the declaration of the three states of being or may modify their orderly oscillation through the sleep-wake cycle. In addition, endogenous information and its perception (dreams) may be modified. Disturbances of dreaming may configurate in different general clinical scenarios: lack of dreaming, excess of dreaming (epic dreaming), paroxysmal dreaming (epileptic), nightmares, violent dreaming, daytime-dreaming (hallucinations), and lucid dreaming. Sensorial deprivation, as well as the emergence of internal perception may be the underlying mechanism of hallucinations. The probable isomorphism between hallucinations and dreaming is postulated, analyzed and discussed. PMID:11143502

  2. [Neurological interpretation of dreams] .

    PubMed

    Pareja, J A; Gil-Nagel, A

    2000-10-01

    Cerebral cortical activity is constant throughout the entire human life, but substantially changes during the different phases of the sleep-wake cycle (wakefulness, non-REM sleep and REM sleep), as well as in relation to available information. In particular, perception of the environment is closely linked to the wake-state, while during sleep perception turns to the internal domain or endogenous cerebral activity. External and internal information are mutually exclusive. During wakefulness a neuronal mechanism allows attention to focus on the environment whereas endogenous cortical activity is ignored. The opposite process is provided during sleep. The function external attention-internal attention is coupled with the two modes of brain function during wakefulness and during sleep, providing two possible cortical status: thinking and dreaming. Several neurological processes may influence the declaration of the three states of being or may modify their orderly oscillation through the sleep-wake cycle. In addition, endogenous information and its perception (dreams) may be modified. Disturbances of dreaming may configurate in different general clinical scenarios: lack of dreaming, excess of dreaming (epic dreaming), paroxysmal dreaming (epileptic), nightmares, violent dreaming, daytime-dreaming (hallucinations), and lucid dreaming. Sensorial deprivation, as well as the emergence of internal perception may be the underlying mechanism of hallucinations. The probable isomorphism between hallucinations and dreaming is postulated, analyzed and discussed.

  3. Neurology of Volition

    PubMed Central

    Kranick, Sarah M.; Hallett, Mark

    2016-01-01

    Neurological disorders of volition may be characterized by deficits in willing and/or agency. When we move our bodies through space, it is the sense that we intended to move (willing) and that our actions were a consequence of this intention (self-agency) that gives us the sense of voluntariness and a general feeling of being “in control.” While it is possible to have movements that share executive machinery ordinarily used for voluntary movement but lack a sense of voluntariness, such as psychogenic movement disorders, it is also possible to claim volition for presumed involuntary movements (early chorea) or even when no movement is produced (anosognosia). The study of such patients should enlighten traditional models of how the percepts of volition are generated in the brain with regards to movement. We discuss volition and its components as multi-leveled processes with feedforward and feedback information flow, and dependence on prior expectations as well as external and internal cues. PMID:23329204

  4. Neurology in ancient faces

    PubMed Central

    Appenzeller, O; Stevens, J; Kruszynski, R; Walker, S

    2001-01-01

    BACKGROUND—Clinical paleoneurology is almost non-existent, but recognition of neurological diseases in ancient people might be possible by scrutinising portraits apparently representing people as they appeared in life.
METHODS—About 200 mummy portraits painted in colour at the beginning of the first millennium were examined. Thirty two skulls excavated at Hawara in the Fayum (northern Egypt), where most of the portraits were found were measured, and nine caliper measures on each side of the skulls were taken. The right/left ratios were statistically analyzed by analysis of variance (ANOVA). One skull was subjected to 3D CT scanning and transilluminated.
RESULTS—Two patients were found with progressive facial hemiatrophy (Parry-Romberg syndrome), three with deviations of the visual axes (tropia) and one with oval pupils (corectopia).
CONCLUSIONS—Clinical paleoneurology is possible in the absence of a living nervous system. The patients probably had focal epilepsy, hemiplegic migraine, and autonomic nervous system dysfunction.

 PMID:11254781

  5. Teaching Clinical Neurology with the PLATO IV Computer System

    ERIC Educational Resources Information Center

    Parker, Alan; Trynda, Richard

    1975-01-01

    A "Neurox" program entitled "Canine Neurological Diagnosis" developed at the University of Illinois College of Veterinary Medicine enables a student to obtain the results of 78 possible neurological tests or associated questions on a single case. A lesson and possible adaptations are described. (LBH)

  6. Marrow hypoplasia associated with congenital neurologic anomalies in two siblings.

    PubMed

    Drachtman, R; Weinblatt, M; Sitarz, A; Gold, A; Kochen, J

    1990-10-01

    Two siblings with congenital neurologic structural anomalies and delayed-onset selective bone marrow hypoplasia in a previously undescribed constellation of symptoms are presented. Differences between these cases and other well known syndromes are discussed. The importance of this association is the implication that children with congenital neurologic abnormalities may be at increased risk for the development of hypoplastic hematopoietic conditions. PMID:2264478

  7. Attentional Bias and the Development of Cerebral Dominance in Normal and Learning Disabled Children.

    ERIC Educational Resources Information Center

    Hynd, George W.; And Others

    The magnitude of the dichotic right ear advantage was assessed in 48 normal and 48 learning disabled (LD) children (mean age 8.3 years). Ss were matched according to age, sex, and handedness. An analysis of results indicated a significant right ear advantage in both the normal and LD children, but revealed no developmental trend for either group.…

  8. Development of Normalization Factors for Canada and the United States and Comparison with European Factors

    EPA Science Inventory

    In Life Cycle Assessment (LCA), normalization calculates the magnitude of an impact (midpoint or endpoint) relative to the total effect of a given reference. Using a country or a continent as a reference system is a first step towards global normalization. The goal of this wor...

  9. Development of Normalization Factors for Canada and the United States and Comparison with European Factors.

    EPA Science Inventory

    In Life Cycle Assessment (LCA), normalization calculates the magnitude of an impact (midpoint or endpoint) relative to the total effect of a given reference. Using a country or a continent as a reference system is a first step towards global normalization. The goal of this work ...

  10. Metacognition and Reading: Comparing Three Forms of Metacognition in Normally Developing Readers and Readers with Dyslexia.

    PubMed

    Furnes, Bjarte; Norman, Elisabeth

    2015-08-01

    Metacognition refers to 'cognition about cognition' and includes metacognitive knowledge, strategies and experiences (Efklides, 2008; Flavell, 1979). Research on reading has shown that better readers demonstrate more metacognitive knowledge than poor readers (Baker & Beall, 2009), and that reading ability improves through strategy instruction (Gersten, Fuchs, Williams, & Baker, 2001). The current study is the first to specifically compare the three forms of metacognition in dyslexic (N = 22) versus normally developing readers (N = 22). Participants read two factual texts, with learning outcome measured by a memory task. Metacognitive knowledge and skills were assessed by self-report. Metacognitive experiences were measured by predictions of performance and judgments of learning. Individuals with dyslexia showed insight into their reading problems, but less general knowledge of how to approach text reading. They more often reported lack of available reading strategies, but groups did not differ in the use of deep and surface strategies. Learning outcome and mean ratings of predictions of performance and judgments of learning were lower in dyslexic readers, but not the accuracy with which metacognitive experiences predicted learning. Overall, the results indicate that dyslexic reading and spelling problems are not generally associated with lower levels of metacognitive knowledge, metacognitive strategies or sensitivity to metacognitive experiences in reading situations.

  11. The Wilms' tumor gene Wt1 is required for normal development of the retina.

    PubMed

    Wagner, Kay-Dietrich; Wagner, Nicole; Vidal, Valerie P I; Schley, Gunnar; Wilhelm, Dagmar; Schedl, Andreas; Englert, Christoph; Scholz, Holger

    2002-03-15

    The Wilms' tumor gene Wt1 is known for its important functions during genitourinary and mesothelial formation. Here we show that Wt1 is necessary for neuronal development in the vertebrate retina. Mouse embryos with targeted disruption of Wt1 exhibit remarkably thinner retinas than age-matched wild-type animals. A large fraction of retinal ganglion cells is lost by apoptosis, and the growth of optic nerve fibers is severely disturbed. Strikingly, expression of the class IV POU-domain transcription factor Pou4f2 (formerly Brn-3b), which is critical for the survival of most retinal ganglion cells, is lost in Wt1(-/-) retinas. Forced expression of Wt1 in cultured cells causes an up-regulation of Pou4f2 mRNA. Moreover, the Wt1(-KTS) splice variant can activate a reporter construct carrying 5'-regulatory sequences of the human POU4F2. The lack of Pou4f2 and the ocular defects in Wt1(-/-) embryos are rescued by transgenic expression of a 280 kb yeast artificial chromosome carrying the human WT1 gene. Taken together, our findings demonstrate a continuous requirement for Wt1 in normal retina formation with a critical role in Pou4f2-dependent ganglion cell differentiation.

  12. Development and evaluation of a new contoured cushion system with an optimized normalization algorithm.

    PubMed

    Li, Sujiao; Zhang, Zhengxiang; Wang, Jue

    2014-01-01

    Prevention of pressure sores remains a significant problem confronting spinal cord injury patients and the elderly with limited mobility. One vital aspect of this subject concerns the development of cushions to decrease pressure ulcers for seated patients, particularly those bound by wheelchairs. Here, we present a novel cushion system that employs interface pressure distribution between the cushion and the buttocks to design custom contoured foam cushion. An optimized normalization algorithm was proposed, with which interface pressure distribution was transformed into the carving depth of foam cushions according to the biomechanical characteristics of the foam. The shape and pressure-relief performance of the custom contoured foam cushions was investigated. The outcomes showed that the contoured shape of personalized cushion matched the buttock contour very well. Moreover, the custom contoured cushion could alleviate pressure under buttocks and increase subjective comfort and stability significantly. Furthermore, the fabricating method not only decreased the unit production cost but also simplified the procedure for manufacturing. All in all, this prototype seat cushion would be an effective and economical way to prevent pressure ulcers. PMID:25227054

  13. Statistical thermodynamics of transcription profiles in normal development and tumorigeneses in cohorts of patients.

    PubMed

    Remacle, F; Levine, R D

    2015-12-01

    Experimental biology is providing the distribution of numerous different biological molecules inside cells and in body fluids of patients. Statistical methods of analysis have very successfully examined these rather large databases. We seek to use a thermodynamic analysis to provide a physical understanding and quantitative characterization of human cancers and other pathologies within a molecule-centered approach. The key technical development is the introduction of a Lagrangian. By imposing constraints the minimal value of the Lagrangian defines a thermodynamically stable state of the cellular system. The minimization also allows using experimental data measured at a number of different conditions to evaluate the steady-state distribution of biomolecules such as messenger RNAs. Thereby the number of effectively accessible quantum states of biomolecules is determined from the experimentally measured expression levels. With the increased resolution provided by the minimization of the Lagrangian one can differentiate between normal and diseased patients and further between disease subtypes. Each such refinement corresponds to imposing an additional constraint of biological origin. The constraints are the unbalanced ongoing biological processes in the system. MicroRNA expression level data for control and diseased lung cancer patients are analyzed as an example.

  14. List intonation in pre-schoolers with normal and disordered language development.

    PubMed

    Snow, David

    2015-01-01

    The principal aim of this study was to evaluate pre-schoolers' expressive intonation in light of current debates about the underlying nature of language impairment (LI). Children with LI typically have deficits in grammar, a component of language that is phonologically represented on the segmental level. The hypothesis is that children with LI do not have deficits of this type when grammar is conveyed by intonation, a pitch-based component of language that is phonologically represented on the suprasegmental level. This study focused on the richly diversified suprasegmental patterns of sentences in which the speaker produces a series of items in a list. To address the hypothesis, list intonation in the speech of 4-year-olds with and without LI was acoustically analysed. Lists produced by children with LI were comparable to those produced by children with normal language development (NL). The results do not support the claim that LI stems from a poor understanding of grammatical principles. Rather, LI reflects an underlying impairment of segmental information processing. The discussion focuses on two characteristics of pitch contours which may account for the resilience of intonation in children with LI. Namely, steady state versus transient signals and universal symbol meanings versus arbitrary relationships between form and function. PMID:26308494

  15. Survival Motor Neuron (SMN) protein is required for normal mouse liver development

    PubMed Central

    Szunyogova, Eva; Zhou, Haiyan; Maxwell, Gillian K.; Powis, Rachael A.; Francesco, Muntoni; Gillingwater, Thomas H.; Parson, Simon H.

    2016-01-01

    Spinal Muscular Atrophy (SMA) is caused by mutation or deletion of the survival motor neuron 1 (SMN1) gene. Decreased levels of, cell-ubiquitous, SMN protein is associated with a range of systemic pathologies reported in severe patients. Despite high levels of SMN protein in normal liver, there is no comprehensive study of liver pathology in SMA. We describe failed liver development in response to reduced SMN levels, in a mouse model of severe SMA. The SMA liver is dark red, small and has: iron deposition; immature sinusoids congested with blood; persistent erythropoietic elements and increased immature red blood cells; increased and persistent megakaryocytes which release high levels of platelets found as clot-like accumulations in the heart. Myelopoiesis in contrast, was unaffected. Further analysis revealed significant molecular changes in SMA liver, consistent with the morphological findings. Antisense treatment from birth with PMO25, increased lifespan and ameliorated all morphological defects in liver by postnatal day 21. Defects in the liver are evident at birth, prior to motor system pathology, and impair essential liver function in SMA. Liver is a key recipient of SMA therapies, and systemically delivered antisense treatment, completely rescued liver pathology. Liver therefore, represents an important therapeutic target in SMA. PMID:27698380

  16. The Effect of Executive Function on Science Achievement Among Normally Developing 10-Year Olds

    NASA Astrophysics Data System (ADS)

    Lederman, Sheri G.

    Executive function (EF) is an umbrella term used to identify a set of discrete but interrelated cognitive abilities that enable individuals to engage in goal-directed, future-oriented action in response to a novel context. Developmental studies indicate that EF is predictive of reading and math achievement in middle childhood. The purpose of this study was to identify the association between EF and science achievement among normally developing 10 year olds. A sample of fifth grade students from a Northeastern suburban community participated in tests of EF, science, and intelligence. Consistent with adult models of EF, principal components analysis identified a three-factor model of EF organization in middle childhood, including cognitive flexibility, working memory, and inhibition. Multiple regression analyses revealed that executive function processes of cognitive flexibility, working memory, and inhibition were all predictive of science performance. Post hoc analyses revealed that high-performing science students differed significantly from low-performing students in both cognitive flexibility and working memory. These findings suggest that complex academic demands specific to science achievement rely on the emergence and maturation of EF components.

  17. Gsh-1, an orphan Hox gene, is required for normal pituitary development.

    PubMed Central

    Li, H; Zeitler, P S; Valerius, M T; Small, K; Potter, S S

    1996-01-01

    The anterior pituitary regulates the function of multiple organ systems as well as body growth, and in turn is controlled by peptides released by the hypothalamus. We find that mutation of the Gsh-1 homeobox gene results in pleiotropic effects on pituitary development and function. Homozygous mutants exhibit extreme dwarfism, sexual infantilism and significant perinatal mortality. The mutant pituitary is small in size and hypocellular, with severely reduced numbers of growth hormone- and prolactin-producing cells. Moreover, the pituitary content of a subset of pituitary hormones, including growth hormone, prolactin and luteinizing hormone, is significantly decreased. The hypothalamus, although morphologically normal, is also perturbed in mutants. The gsh-1 gene is shown to be essential for growth hormone-releasing hormone (GHRH) gene expression in the arcuate nucleus of the hypothalamus. Further, sequence and electrophoretic mobility shift data suggest the Gsh-1 and GHRH genes as potential targets regulated by the Gsh-1-encoded protein. The mutant phenotype indicates a critical role for Gsh-1 in the genetic hierarchy of the formation and function of the hypothalamic-pituitary axis. Images PMID:8631293

  18. Liver transplantation in neurological Wilson's Disease: is there indication? A case report.

    PubMed

    Mocchegiani, F; Gemini, S; Vincenzi, P; Montalti, R; Vecchi, A; Nicolini, D; Federici, A; Coletta, M; Pansini, M; Lanari, J; Svegliati Baroni, G; Risaliti, A; Vivarelli, M

    2014-09-01

    Wilson's disease (WD) is an autosomal recessive disorder characterized by copper overload. In this disease, inadequate hepatic excretion leads to copper accumulation in the liver, brain, kidney, and cornea. Severe neurological symptoms can develop in patients with WD, often in the absence of relevant liver damage: it is unclear whether liver transplantation (LT) could reverse neurological symptoms, and at present LT is not recommended in this setting. We report a case of regression of neurological symptoms in a patient affected by WD with prevalent neurological involvement. A 19-year-old man with disabling neuropsychiatric symptoms from WD that included frontal ataxia, akinesia, dystonia, tremors, and behavioral disorders in the presence of preserved liver function (Model for End-Stage Liver Disease score=7; Child-Turcotte-Pugh score=A5) underwent LT in November 2009. At the time of LT, encephalic magnetic resonance imaging (MRI) indicated diffuse neurodegenerative alterations involving subtentorial and supratentorial structures; bilateral Kayser-Fleischer ring was present. Four years after LT, laboratory tests show normalized copper metabolism and excellent liver function test results. Encephalic MRI shows a substantial improvement of already-known signal alterations at nuclei thalamus and putamen, mesencephalon, and pons. Kayser-Fleischer ring disappeared from the right eye, but a little remnant is still visible in the left eye. At neurological examination, all of the previous symptoms and signs are no longer present and behavioral disorders are no longer present; psychosocial functions are completely restored. The present case provides some evidence that LT may be a valid therapeutic option for WD patients with marked neurological impairment, particularly in those no longer responsive to chelation therapy.

  19. Chapter 17: cognitive assessment in neurology.

    PubMed

    Henderson, Victor W

    2010-01-01

    Modern interests in cognitive assessment began with Franz Gall's early 19th century theory of mental organology and Paul Broca's reports in the 1860s on patients with focal brain injury and aphemia. These workers spurred interest in assessing delimited mental abilities in relation to discrete cerebral areas. With roots in experimental and educational psychology, the intelligence testing movement added assessment tools that could be applied to neurological patients. Early- to mid-20th-century landmarks were Alfred Binet and Theodore Simon's intelligence scale, Howard Knox's nonverbal performance tests, and the intelligence quotient conceived by Lewis Terman and refined by David Wechsler. Also developed during this era were Henry Head's Serial Tests for aphasic patients and Kurt Goldstein's tests for brain-injured patients with impairments in "abstract attitude" and concept formation. Other investigators have contributed procedures for the evaluation of language functions, memory, visuospatial and visuoconstructive skills, praxis, and executive functions. A further milestone was the development of short standardized cognitive instruments for dementia assessment. Within a neurological arena, the historical emphasis has been on a flexible, process-driven approach to the service of neurological diagnosis and syndrome identification. Advances in clinical psychology, neurology, and the cognate clinical neurosciences continue to enrich assessment options.

  20. Development and Decline of Memory Functions in Normal, Pathological and Healthy Successful Aging

    PubMed Central

    Sanfratello, L.; Adair, J. C.; Knoefel, J. E.; Caprihan, A.; Stephen, J. M.

    2011-01-01

    Many neuroimaging studies of age-related memory decline interpret resultant differences in brain activation patterns in the elderly as reflecting a type of compensatory response or regression to a simpler state of brain organization. Here we review a series of our own studies which lead us to an alternative interpretation, and highlights a couple of potential confounds in the aging literature that may act to increase the variability of results within age groups and across laboratories. From our perspective, level of cognitive functioning achieved by a group of elderly is largely determined by the health of individuals within this group. Individuals with a history of hypertension, for example, are likely to have multiple white matter insults which compromise cognitive functioning, independent of aging processes. The health of the elderly group has not been well-documented in most previous studies and elderly participants are rarely excluded, or placed into a separate group, due to health-related problems. In addition, recent results show that white matter tracts within the frontal and temporal lobes, regions critical for higher cognitive functions, continue to mature well into the 4th decade of life. This suggests that a young age group may not be the best control group for understanding aging effects on the brain since development is ongoing within this age range. Therefore, we have added a middle-age group to our studies in order to better understand normal development across the lifespan as well as effects of pathology on cognitive functioning in the aging brain. PMID:21452018

  1. Development of the arterial pattern in the upper limb of staged human embryos: normal development and anatomic variations

    PubMed Central

    RODRÍGUEZ-NIEDENFÜHR, M.; BURTON, G. J.; DEU, J.; SAÑUDO, J. R.

    2001-01-01

    A total of 112 human embryos (224 upper limbs) between stages 12 and 23 of development were examined. It was observed that formation of the arterial system in the upper limb takes place as a dual process. An initial capillary plexus appears from the dorsal aorta during stage 12 and develops at the same rate as the limb. At stage 13, the capillary plexus begins a maturation process involving the enlargement and differentiation of selected parts. This remodelling process starts in the aorta and continues in a proximal to distal sequence. By stage 15 the differentiation has reached the subclavian and axillary arteries, by stage 17 it has reached the brachial artery as far as the elbow, by stage 18 it has reached the forearm arteries except for the distal part of the radial, and finally by stage 21 the whole arterial pattern is present in its definitive morphology. This differentiation process parallels the development of the skeletal system chronologically. A number of arterial variations were observed, and classified as follows: superficial brachial (7.7%), accessory brachial (0.6%), brachioradial (14%), superficial brachioulnar (4.7%), superficial brachioulnoradial (0.7%), palmar pattern of the median (18.7%) and superficial brachiomedian (0.7%) arteries. They were observed in embryos belonging to stages 17–23 and were not related to a specific stage of development. Statistical comparison with the rates of variations reported in adults did not show significant differences. It is suggested that the variations arise through the persistence, enlargement and differentiation of parts of the initial network which would normally remain as capillaries or even regress. PMID:11693301

  2. Multiple ribosomal proteins are expressed at high levels in developing zebrafish endoderm and are required for normal exocrine pancreas development.

    PubMed

    Provost, Elayne; Weier, Christopher A; Leach, Steven D

    2013-06-01

    Ribosomal protein L (rpl) genes are essential for assembly of the 60S subunit of the eukaryotic ribosome and may also carry out additional extra-ribosomal functions. We have identified a common expression pattern for rpl genes in developing zebrafish larvae. After initially widespread expression in early embryos, the expression of multiple rpl genes becomes increasingly restricted to the endoderm. With respect to the pancreas, rpl genes are highly expressed in ptf1a-expressing pancreatic progenitors at 48 hpf, suggesting possible functional roles in pancreatic morphogenesis and/or differentiation. Utilizing two available mutant lines, rpl23a(hi2582) and rpl6(hi3655b), we found that ptf1a-expressing pancreatic progenitors fail to properly expand in embryos homozygous for either of these genes. In addition to these durable homozygous phenotypes, we also demonstrated recoverable delays in ptf1a-expressing pancreatic progenitor expansion in rpl23a(hi2582) and rpl6(hi3655b) heterozygotes. Disruptions in ribosome assembly are generally understood to initiate a p53-dependent cellular stress response. However, concomitant p53 knockdown was unable to rescue normal pancreatic progenitor expansion in either rpl23a(hi2582) or rpl6(hi3655b) mutant embryos, suggesting required and p53-independent roles for rpl23a and rpl6 in pancreas development.

  3. Neurologic course of congenital disorders of glycosylation.

    PubMed

    Pearl, P L; Krasnewich, D

    2001-06-01

    Congenital disorders of glycosylation, formerly called carbohydrate-deficient glycoprotein syndrome, may present in infancy with slowly progressive neurologic deficits including cognitive impairment, ataxia, pigmentary retinal degeneration, and neuropathy. The metabolic defect is in N-linked oligosaccharide synthesis, and diagnosis is made by a serum transferrin isoelectric focusing. We reviewed the neurologic course of 10 children with congenital disorders of glycosylation (ages 13 months to 7 years). All had severe developmental delay and ataxia; none walked unassisted, and the highest level of communication was simple sign language in one patient. Five of 10 children had seizures (absence, complex partial, tonic clonic). Only one patient has had strokelike episodes, despite reports that they are common in this population. The underlying basis of these episodes has been hypothesized to be coagulopathy due to dysfunctional, incorrectly glycosylated coagulation factors. This 5-year-old patient with congenital disorders of glycosylation type Ia had two strokelike episodes, with evolving hemiparesis over 5 to 6 days' duration, followed by focal tonic-clonic seizures. Coagulation studies were normal. Electroencephalography showed transient hemispheric polymorphous delta-range slowing and suppression. Magnetic resonance imaging revealed corresponding cortical swelling. Magnetic resonance angiography was normal. Magnetic resonance spectroscopy revealed a decrease in the N-acetylaspartate peak, suggesting neuronal loss, with normal lactate peak. The neuroradiologic data do not support a thrombotic, embolic, or hemorrhagic basis for strokelike episodes in carbohydrate-deficient glycoprotein syndrome; other mechanisms must be considered.

  4. Cytokine Therapies in Neurological Disease.

    PubMed

    Azodi, Shila; Jacobson, Steven

    2016-07-01

    Cytokines are a heterogeneous group of glycoproteins that coordinate physiological functions. Cytokine deregulation is observed in many neurological diseases. This article reviews current research focused on human clinical trials of cytokine and anticytokine therapies in the treatment of several neurological disease including stroke, neuromuscular diseases, neuroinfectious diseases, demyelinating diseases, and neurobehavioral diseases. This research suggests that cytokine therapy applications may play an important role in offering new strategies for disease modulation and treatment. Further, this research provides insights into the causal link between cytokine deregulation and neurological diseases. PMID:27388288

  5. Neurological findings of Lyme disease.

    PubMed Central

    Pachner, A. R.; Steere, A. C.

    1984-01-01

    Neurologic involvement of Lyme disease typically consists of meningitis, cranial neuropathy, and radiculoneuritis, alone or in combination, lasting for months. From 1976 to 1983, we studied 38 patients with Lyme meningitis. Headache and mild neck stiffness, which fluctuated in intensity, and lymphocytic pleocytosis were the common findings. Half of the patients also had facial palsies, which were unilateral in 12 and bilateral in seven. In addition, 12 patients had motor and/or sensory radiculoneuropathies; asymmetric weakness of extremities was the most common finding. Although incomplete presentations of neurologic involvement of Lyme disease may be confused with other entities, the typical constellation of neurologic symptoms represents a unique clinical picture. PMID:6516450

  6. [Neurological complications in cancer patients].

    PubMed

    Hundsberger, Thomas; Roth, Patrick; Roelcke, Ulrich

    2014-08-20

    Neurological symptoms in cancer patients have a great impact on quality of life and need an interdisciplinary approach. They lead to significant impairment in activities of daily living (gait disorders, dizziness), a loss of patients independency (vegetative disturbances, wheel-chair dependency) and interfere with social activities (ban of driving in case of epilepsy). In this article we describe three main and serious neurological problems in the context of oncological patients. These are chemotherapy-induced polyneuropathy, malignant spinal cord compression and epileptic seizures. Our aim is to increase the awareness of neurological complications in cancer patients to improve patients care.

  7. Neurological effects of deep diving.

    PubMed

    Grønning, Marit; Aarli, Johan A

    2011-05-15

    Deep diving is defined as diving to depths more than 50 m of seawater (msw), and is mainly used for occupational and military purposes. A deep dive is characterized by the compression phase, the bottom time and the decompression phase. Neurological and neurophysiologic effects are demonstrated in divers during the compression phase and the bottom time. Immediate and transient neurological effects after deep dives have been shown in some divers. However, the results from the epidemiological studies regarding long term neurological effects from deep diving are conflicting and still not conclusive. Prospective clinical studies with sufficient power and sensitivity are needed to solve this very important issue.

  8. A model for the geomorphic development of normal-fault facets

    NASA Astrophysics Data System (ADS)

    Tucker, G. E.; Hobley, D. E. J.; McCoy, S. W.

    2014-12-01

    Triangular facets are among the most striking landforms associated with normal faulting. The genesis of facets is of great interest both for the information facets contain about tectonic motion, and because the progressive emergence of facets makes them potential recorders of both geomorphic and tectonic history. In this report, we present observations of triangular facets in the western United States and in the Italian Central Apennines. Facets in these regions typically form quasi-planar surfaces that are aligned in series along and above the trace of an active fault. Some facet surfaces consist mainly of exposed bedrock, with a thin and highly discontinuous cover of loose regolith. Other facets are mantled by a several-decimeter-thick regolith cover. Over the course of its morphologic development, a facet slope segment may evolve from a steep (~60 degree) bedrock fault scarp, well above the angle of repose for soil, to a gentler (~20-40 degree) slope that can potentially sustain a coherent regolith cover. This evolutionary trajectory across the angle of repose renders nonlinear diffusion theory inapplicable. To formulate an alternative process-based theory for facet evolution, we use a particle-based approach that acknowledges the possibility for both short- and long-range sediment-grain motions, depending on the topography. The processes of rock weathering, grain entrainment, and grain motion are represented as stochastic state-pair transitions with specified transition rates. The model predicts that facet behavior can range smoothly along the spectrum from a weathering-limited mode to a transport-limited mode, depending on the ratio of fault-slip rate to bare-bedrock regolith production rate. The model also implies that facets formed along a fault with pinned tips should show systematic variation in slope angle that correlates with along-fault position and slip rate. Preliminary observations from central Italy and the eastern Basin and Range are consistent

  9. Oral glycopyrrolate for the treatment of chronic severe drooling caused by neurological disorders in children.

    PubMed

    Evatt, Marian L

    2011-01-01

    Excessive drooling may complicate the care of children with chronic neurological conditions by socially isolating both patients and families and by causing secondary dermatitis and infection. Normal control of saliva requires normal integrity of oral structures, normal oropharyngeal sensation, and motor functioning, as well as normal cognitive awareness and rate of salivary production. Glycopyrrolate is an anticholinergic medication with a quaternary structure that recently received Food and Drug Administration approval to treat sialorrhea due to neurological problems in children ages 3-16 years. This review summarizes the few published studies of safety and efficacy of glycopyrrolate for drooling in children with chronic neurological conditions.

  10. Normal-fault development in two-phase experimental models of shortening followed by extension and comparison to natural examples

    NASA Astrophysics Data System (ADS)

    Warrell, K. F.; Withjack, M. O.; Schlische, R. W.

    2014-12-01

    Field- and seismic-reflection-based studies have documented the influence of pre-existing thrust faults on normal-fault development during subsequent extension. Published experimental (analog) models of shortening followed by extension with dry sand as the modeling medium show limited extensional reactivation of moderate-angle thrust faults (dipping > 40º). These dry sand models provide insight into the influence of pre-existing thrusts on normal-fault development, but these models have not reactivated low-angle (< 35º) thrust faults as seen in nature. New experimental (analog) models, using wet clay over silicone polymer to simulate brittle upper crust over ductile lower crust, suggest that low-angle thrust faults from an older shortening phase can reactivate as normal faults. In two-phase models of shortening followed by extension, normal faults nucleate above pre-existing thrust faults and likely link with thrusts at depth to create listric faults, movement on which produces rollover folds. Faults grow and link more rapidly in two-phase than in single-phase (extension-only) models. Fewer faults with higher displacements form in two-phase models, likely because, for a given displacement magnitude, a low-angle normal fault accommodates more horizontal extension than a high-angle normal fault. The resulting rift basins are wider and shallower than those forming along high-angle normal faults. Features in these models are similar to natural examples. Seismic-reflection profiles from the outer Hebrides, offshore Scotland, show listric faults partially reactivating pre-existing thrust faults with a rollover fold in the hanging wall; in crystalline basement, the thrust is reactivated, and in overlying sedimentary strata, a new, high-angle normal fault forms. Profiles from the Chignecto subbasin of the Fundy basin, offshore Canada, show full reactivation of thrust faults as low-angle normal faults where crystalline basement rocks make up the footwall.

  11. From accommodation zones to metamorphic core complexes: Tracking the progressive development of major normal fault systems

    SciTech Connect

    Faulds, J.E. . Dept. of Geology)

    1992-01-01

    The along-strike dimension in rifted continental crust is critical to assessing models of continental extension because individual normal faults or fault systems can potentially be traced from their tips in accommodation zones to their culminations in metamorphic core complexes. Accommodation zones and the linkages between the zones and core complexes have not been thoroughly studied or incorporated extensively into models of continental extension. Regionally extensive, gently dipping normal faults (i.e., detachment faults) that surface in metamorphic core complexes terminate and flip polarity in accommodation zones. Diametrical lateral transport of upper-plate rocks in positively dipping detachment terranes should presumably induce strike-slip faulting on segments of accommodation zones paralleling the extension direction. Most accommodation zones correspond, however, to belts of intermeshing conjugate normal faults with little strike-slip faulting. Normal faults simply terminate along-strike in the zones with little, if any, transfer of slip to strike-slip faults. Decreases in cumulative strain within individual normal fault systems toward some accommodation zones cannot alone account for the lack of strike-slip faulting. These findings pose a serious challenge to generally accepted notions of large-magnitude, lateral motion of parts of detachment terranes. Large-scale lateral translations of rifted continental crust may be governed more by discrete axes of extension than by detachment geometries. The dovetail-like interfingering of conjugate normal fault systems and attendant tilt-block domains observed in some accommodation zones (e.g., Colorado River extensional corridor, US) does suggest, however, that at least some major normal faults projecting into the zones from metamorphic core complexes have listric geometries that flatten out at relatively shallow depths.

  12. Trajectories of cortical thickness maturation in normal brain development--The importance of quality control procedures.

    PubMed

    Ducharme, Simon; Albaugh, Matthew D; Nguyen, Tuong-Vi; Hudziak, James J; Mateos-Pérez, J M; Labbe, Aurelie; Evans, Alan C; Karama, Sherif

    2016-01-15

    Several reports have described cortical thickness (CTh) developmental trajectories, with conflicting results. Some studies have reported inverted-U shape curves with peaks of CTh in late childhood to adolescence, while others suggested predominant monotonic decline after age 6. In this study, we reviewed CTh developmental trajectories in the NIH MRI Study of Normal Brain Development, and in a second step, evaluated the impact of post-processing quality control (QC) procedures on identified trajectories. The quality-controlled sample included 384 individual subjects with repeated scanning (1-3 per subject, total scans n=753) from 4.9 to 22.3years of age. The best-fit model (cubic, quadratic, or first-order linear) was identified at each vertex using mixed-effects models. The majority of brain regions showed linear monotonic decline of CTh. There were few areas of cubic trajectories, mostly in bilateral temporo-parietal areas and the right prefrontal cortex, in which CTh peaks were at, or prior to, age 8. When controlling for total brain volume, CTh trajectories were even more uniformly linear. The only sex difference was faster thinning of occipital areas in boys compared to girls. The best-fit model for whole brain mean thickness was a monotonic decline of 0.027mm per year. QC procedures had a significant impact on identified trajectories, with a clear shift toward more complex trajectories (i.e., quadratic or cubic) when including all scans without QC (n=954). Trajectories were almost exclusively linear when using only scans that passed the most stringent QC (n=598). The impact of QC probably relates to decreasing the inclusion of scans with CTh underestimation secondary to movement artifacts, which are more common in younger subjects. In summary, our results suggest that CTh follows a simple linear decline in most cortical areas by age 5, and all areas by age 8. This study further supports the crucial importance of implementing post-processing QC in CTh studies

  13. Trajectories of cortical thickness maturation in normal brain development--The importance of quality control procedures.

    PubMed

    Ducharme, Simon; Albaugh, Matthew D; Nguyen, Tuong-Vi; Hudziak, James J; Mateos-Pérez, J M; Labbe, Aurelie; Evans, Alan C; Karama, Sherif

    2016-01-15

    Several reports have described cortical thickness (CTh) developmental trajectories, with conflicting results. Some studies have reported inverted-U shape curves with peaks of CTh in late childhood to adolescence, while others suggested predominant monotonic decline after age 6. In this study, we reviewed CTh developmental trajectories in the NIH MRI Study of Normal Brain Development, and in a second step, evaluated the impact of post-processing quality control (QC) procedures on identified trajectories. The quality-controlled sample included 384 individual subjects with repeated scanning (1-3 per subject, total scans n=753) from 4.9 to 22.3years of age. The best-fit model (cubic, quadratic, or first-order linear) was identified at each vertex using mixed-effects models. The majority of brain regions showed linear monotonic decline of CTh. There were few areas of cubic trajectories, mostly in bilateral temporo-parietal areas and the right prefrontal cortex, in which CTh peaks were at, or prior to, age 8. When controlling for total brain volume, CTh trajectories were even more uniformly linear. The only sex difference was faster thinning of occipital areas in boys compared to girls. The best-fit model for whole brain mean thickness was a monotonic decline of 0.027mm per year. QC procedures had a significant impact on identified trajectories, with a clear shift toward more complex trajectories (i.e., quadratic or cubic) when including all scans without QC (n=954). Trajectories were almost exclusively linear when using only scans that passed the most stringent QC (n=598). The impact of QC probably relates to decreasing the inclusion of scans with CTh underestimation secondary to movement artifacts, which are more common in younger subjects. In summary, our results suggest that CTh follows a simple linear decline in most cortical areas by age 5, and all areas by age 8. This study further supports the crucial importance of implementing post-processing QC in CTh studies

  14. A Developmental Study of Static Postural Control and Superimposed Arm Movements in Normal and Slowly Developing Children.

    ERIC Educational Resources Information Center

    Fisher, Janet M.

    Selected electromyographic parameters underlying static postural control in 4, 6, and 8 year old normally and slowly developing children during performance of selected arm movements were studied. Developmental delays in balance control were assessed by the Cashin Test of Motor Development (1974) and/or the Williams Gross Motor Coordination Test…

  15. Syntactic Development in the Oral Language of Learning Disabled and Normal Students at the Intermediate and Secondary Level.

    ERIC Educational Resources Information Center

    Simms, Rochelle B.; Crump, W. Donald

    1983-01-01

    Syntactic development in the oral language of learning disabled and normal students at two intermediate and two secondary-school age levels was compared using two indices, the T-unit and the Syntactic Density Score. It was concluded that no single, sensitive, numerical index exists to quantify syntactic development. (SW)

  16. A Comparison Study of Gross Motor Development Skills of Normal, Hearing-Impaired and Down Syndrome Children.

    ERIC Educational Resources Information Center

    Bilir, Sule; And Others

    This study, conducted in Ankara, Turkey, compared motor development in 48 normal children (ages 3 to 6), 12 children (ages 5 to 7) with Down syndrome, and 33 children (ages 3 to 7) with hearing impairments. The Motor Development Section of the Portage Early Childhood Educational Program checklist was administered to all the children. Results…

  17. Neurological Complications of Bariatric Surgery.

    PubMed

    Goodman, Jerry Clay

    2015-12-01

    Obesity has attained pandemic proportions, and bariatric surgery is increasingly being employed resulting in turn to more neurological complications which must be recognized and managed. Neurological complications may result from mechanical or inflammatory mechanisms but primarily result from micro-nutritional deficiencies. Vitamin B12, thiamine, and copper constitute the most frequent deficiencies. Neurological complications may occur at reasonably predictable times after bariatric surgery and are associated with the type of surgery used. During the early post-operative period, compressive or stretch peripheral nerve injury, rhabdomyolysis, Wernicke's encephalopathy, and inflammatory polyradiculoneuropathy may occur. Late complications ensue after months to years and include combined system degeneration (vitamin B12 deficiency) and hypocupric myelopathy. Bariatric surgery patients require careful nutritional follow-up with routine monitoring of micronutrients at 6 weeks and 3, 6, and 12 months post-operatively and then annually after surgery and multivitamin supplementation for life. Sustained vigilance for common and rare neurological complications is essential.

  18. [Consciousness disorders from neurological view].

    PubMed

    Lange, Rüdiger; Erbguth, Frank

    2016-09-01

    "Disturbances of consciousness of unknown origin" require an interdisciplinary approach due to the broad variety of possibly underlying causes. Primary neurological pathologies account for about half of the cases, which emphasizes the key role of the neurologist in the primary assessment and planning of the diagnostic and therapeutic strategy. The most important goal is to quickly identify patients with extremely time-critical conditions like ischemic stroke, bacterial meningitis or space occupying intracranial hemorrhage. The most important tool to generate a working hypothesis is the clinical neurological examination. However, even in apparently neurological presentations like e.g. first ever epileptic seizure, underlying even non-neurological pathologies have to be considered. PMID:27642737

  19. Neurologic disorder and criminal responsibility.

    PubMed

    Yaffe, Gideon

    2013-01-01

    Sufferers from neurologic and psychiatric disorders are not uncommonly defendants in criminal trials. This chapter surveys a variety of different ways in which neurologic disorder bears on criminal responsibility. It discusses the way in which a neurologic disorder might bear on the questions of whether or not the defendant acted voluntarily; whether or not he or she was in the mental state that is required for guilt for the crime; and whether or not he or she is deserving of an insanity defense. The discussion demonstrates that a just determination of whether a sufferer from a neurologic disorder is diminished in his or her criminal responsibility for harmful conduct requires equal appreciation of the nature of the relevant disorder and its impact on behavior, on the one hand, and of the legal import of facts about the psychologic mechanisms through which behavior is generated, on the other.

  20. Neurologic disorder and criminal responsibility.

    PubMed

    Yaffe, Gideon

    2013-01-01

    Sufferers from neurologic and psychiatric disorders are not uncommonly defendants in criminal trials. This chapter surveys a variety of different ways in which neurologic disorder bears on criminal responsibility. It discusses the way in which a neurologic disorder might bear on the questions of whether or not the defendant acted voluntarily; whether or not he or she was in the mental state that is required for guilt for the crime; and whether or not he or she is deserving of an insanity defense. The discussion demonstrates that a just determination of whether a sufferer from a neurologic disorder is diminished in his or her criminal responsibility for harmful conduct requires equal appreciation of the nature of the relevant disorder and its impact on behavior, on the one hand, and of the legal import of facts about the psychologic mechanisms through which behavior is generated, on the other. PMID:24182391

  1. [Continuing medical education (CME) in neurology. Concept of the German Society of Neurology (DGN) and the Neurology Section of the Professional League of German Neurologic Medicine (BVDN)].

    PubMed

    Reuther, P; Diener, H C; Franz, P; Hacke, W; Hofmann, W; Hopf, H C; Jungmann, F; Wiethölter, H

    1999-10-01

    Continuous medical education in Neurology (CME-Neurology) has been promoted in a concept organized by both the German society of neurology, German association for occupational interests of neurologists and psychiatrists). CME-Neurology has been started in January 1999 and is closely adapted to the CME guidelines of neurology section of UEMS and EFNS. The program shall serve to the maintenance and upgrading of knowledge skills and competence of postgraduate training in neurology.

  2. Historical perspective of Indian neurology

    PubMed Central

    Mishra, Shrikant; Trikamji, Bhavesh; Singh, Sandeep; Singh, Parampreet; Nair, Rajasekharan

    2013-01-01

    Objective: To chronicle the history of medicine and neurology in India with a focus on its establishment and evolution. Background: The history of neurology in India is divided into two periods: ancient and modern. The ancient period dates back to the mid-second millennium Before Christ (B.C.) during the creation of the Ayurvedic Indian system of Medicine, which detailed descriptions of neurological disorders called Vata Vyadhi. The early 20th century witnessed the birth of modern Indian medicine with the onset of formal physician training at the nation's first allopathic medical colleges located in Madras (1835), Calcutta (1835) and Mumbai (1848). Prior to India's independence from Britain in 1947, only 25 medical schools existed in the entire country. Today, there are over 355. In 1951, physicians across the field of neurology and neurosurgery united to create the Neurological Society of India (NSI). Four decades later in 1991, neurologists branched out to establish a separate organization called the Indian Academy of Neurology (IAN). Design/Methods: Information was gathered through literature review using PubMed, MD Consult, OVID, primary texts and research at various academic institutions in India. Results: Neurological disorders were first described in ancient India under Ayurveda. The transition to modern medicine occurred more recently through formal training at medical schools beginning in the 1930's. Early pioneers and founders of the NSI (1951) include Dr. Jacob Chandy, Dr. B Ramamurthi, Dr. S. T. Narasimhan and Dr. Baldev Singh. Later, Dr. J. S. Chopra, a prominent neurologist and visionary, recognized the need for primary centers of collaboration and subsequently established the IAN (1991). The future of Neurology in India is growing rapidly. Currently, there are 1100 practicing neurologists and more than 150 post-graduate trainees who join the ranks every year. As the number of neurologists rises across India, there is an increase in the amount of

  3. Addressing neurological disorders with neuromodulation.

    PubMed

    Oluigbo, Chima O; Rezai, Ali R

    2011-07-01

    Neurological disorders are becoming increasingly common in developed countries as a result of the aging population. In spite of medications, these disorders can result in progressive loss of function as well as chronic physical, cognitive, and emotional disability that ultimately places enormous emotional and economic on the patient, caretakers, and the society in general. Neuromodulation is emerging as a therapeutic option in these patients. Neuromodulation is a field, which involves implantable devices that allow for the reversible adjustable application of electrical, chemical, or biological agents to the central or peripheral nervous system with the objective of altering its functioning with the objective of achieving a therapeutic or clinically beneficial effect. It is a rapidly evolving field that brings together many different specialties in the fields of medicine, materials science, computer science and technology, biomedical, and neural engineering as well as the surgical or interventional specialties. It has multiple current and emerging indications, and an enormous potential for growth. The main challenges before it are in the need for effective collaboration between engineers, basic scientists, and clinicians to develop innovations that address specific problems resulting in new devices and clinical applications. PMID:21193369

  4. Addressing neurological disorders with neuromodulation.

    PubMed

    Oluigbo, Chima O; Rezai, Ali R

    2011-07-01

    Neurological disorders are becoming increasingly common in developed countries as a result of the aging population. In spite of medications, these disorders can result in progressive loss of function as well as chronic physical, cognitive, and emotional disability that ultimately places enormous emotional and economic on the patient, caretakers, and the society in general. Neuromodulation is emerging as a therapeutic option in these patients. Neuromodulation is a field, which involves implantable devices that allow for the reversible adjustable application of electrical, chemical, or biological agents to the central or peripheral nervous system with the objective of altering its functioning with the objective of achieving a therapeutic or clinically beneficial effect. It is a rapidly evolving field that brings together many different specialties in the fields of medicine, materials science, computer science and technology, biomedical, and neural engineering as well as the surgical or interventional specialties. It has multiple current and emerging indications, and an enormous potential for growth. The main challenges before it are in the need for effective collaboration between engineers, basic scientists, and clinicians to develop innovations that address specific problems resulting in new devices and clinical applications.

  5. Development of normalization factors for Canada and the United States and comparison with European factors.

    PubMed

    Lautier, Anne; Rosenbaum, Ralph K; Margni, Manuele; Bare, Jane; Roy, Pierre-Olivier; Deschênes, Louise

    2010-12-01

    In Life Cycle Assessment (LCA), normalization calculates the magnitude of an impact (midpoint or endpoint) relative to the total effect of a given reference. The goal of this work is to calculate normalization factors for Canada and the US and to compare them with existing European normalization factors. The differences between geographical areas were highlighted by identifying and comparing the main contributors to a given impact category in Canada, the US and Europe. This comparison verified that the main contributors in Europe and in the US are also present in the Canadian inventory. It also showed that normalized profiles are highly dependent on the selected reference due to differences in the industrial and economic activities. To meet practitioners' needs, Canadian normalization factors have been calculated using the characterization factors from LUCAS (Canadian), IMPACT 2002+ (European), and TRACI (US) respectively. The main sources of uncertainty related to Canadian NFs are data gaps (pesticides, metals) and aggregated data (metals, VOC), but the uncertainty related to CFs generally remains unknown. A final discussion is proposed based on the comparison of resource extraction and resource consumption and raises the question of the legitimacy of defining a country by its geographical borders.

  6. [Pain - a neglected neurological issue].

    PubMed

    Birklein, F; Baron, R; Gaul, C; Maihöfner, C; Rommel, O; Straube, A; Tölle, T; Wasner, G

    2016-06-01

    Chronic pain represents a great challenge; according to epidemiological data increasing numbers of patients should be expected. Based on recent advances, a better understanding of the pathophysiology of chronic pain has been achieved and neurologists have made a major contribution to this understanding. Chronic pain is accompanied by substantial maladaptive plastic alterations in both the peripheral and central nervous systems; therefore, neurological knowledge is of paramount importance for pain therapists but this contrasts with the current treatment situation of pain patients in Germany. There are basically too few departments and practices undertaking treatment, and neurologists are an exception in most pain centers. Furthermore, due to economic reasons neurological hospitals are currently experiencing a dearth of inpatients suffering from chronic pain. Diagnostic and/or treatment procedures for neurological pain entities (e.g. headaches or neuropathic pain) are insufficiently represented in the German diagnosis-related groups (DRG) reimbursement system and the obstacles for an efficient pain therapy in neurological practices are too high. Finally, there are too few academic positions for pain medicine in neurological hospitals; therefore, career opportunities for motivated young neurologists with an interest in pain are lacking. In order to address the unmet therapeutic needs of patients with chronic pain there is a high demand for (i) establishment of departments for neurological pain medicine, (ii) modification of the German DRG system and (iii) education of young neurologists with expertise in pain. Pain medicine in particular should be especially appealing to neurologists .

  7. Hippocrates: the forefather of neurology.

    PubMed

    Breitenfeld, T; Jurasic, M J; Breitenfeld, D

    2014-09-01

    Hippocrates is one of the most influential medical doctors of all times. He started observing and experimenting in times of mysticism and magic. He carried a holistic and humanitarian approach to the patient with examination as the principal approach-inspection, palpation and auscultation are still the most important tools in diagnosing algorithms of today. He had immense experience with the human body most likely due to numerous wound treatments he had performed; some even believe he performed autopsies despite the negative trend at the time. Hippocrates identified the brain as the analyst of the outside world, the interpreter of consciousness and the center of intelligence and willpower. Interestingly, Hippocrates was aware of many valid concepts in neurology; his treatise On the Sacred Disease was the most important for understanding neurology and epilepsy. His other ideas pioneered modern day neurology mentioning neurological diseases like apoplexy, spondylitis, hemiplegia, and paraplegia. Today, 10 % of neurological Pubmed and 7 % of neuroscience Scopus reviews mention Corpus Hippocraticum as one of the sources. Therefore, Hippocrates may be considered as the forefather of neurology. PMID:25027011

  8. [Pain - a neglected neurological issue].

    PubMed

    Birklein, F; Baron, R; Gaul, C; Maihöfner, C; Rommel, O; Straube, A; Tölle, T; Wasner, G

    2016-06-01

    Chronic pain represents a great challenge; according to epidemiological data increasing numbers of patients should be expected. Based on recent advances, a better understanding of the pathophysiology of chronic pain has been achieved and neurologists have made a major contribution to this understanding. Chronic pain is accompanied by substantial maladaptive plastic alterations in both the peripheral and central nervous systems; therefore, neurological knowledge is of paramount importance for pain therapists but this contrasts with the current treatment situation of pain patients in Germany. There are basically too few departments and practices undertaking treatment, and neurologists are an exception in most pain centers. Furthermore, due to economic reasons neurological hospitals are currently experiencing a dearth of inpatients suffering from chronic pain. Diagnostic and/or treatment procedures for neurological pain entities (e.g. headaches or neuropathic pain) are insufficiently represented in the German diagnosis-related groups (DRG) reimbursement system and the obstacles for an efficient pain therapy in neurological practices are too high. Finally, there are too few academic positions for pain medicine in neurological hospitals; therefore, career opportunities for motivated young neurologists with an interest in pain are lacking. In order to address the unmet therapeutic needs of patients with chronic pain there is a high demand for (i) establishment of departments for neurological pain medicine, (ii) modification of the German DRG system and (iii) education of young neurologists with expertise in pain. Pain medicine in particular should be especially appealing to neurologists . PMID:27167885

  9. Neurologic Disorders in Immunocompetent Patients with Autochthonous Acute Hepatitis E

    PubMed Central

    Perrin, H. Blasco; Cintas, P.; Abravanel, F.; Gérolami, R.; d'Alteroche, L.; Raynal, J.-N.; Alric, L.; Dupuis, E.; Prudhomme, L.; Vaucher, E.; Couzigou, P.; Liversain, J.-M.; Bureau, C.; Vinel, J.-P.; Kamar, N.; Izopet, J.

    2015-01-01

    Neurologic disorders, mainly Guillain-Barré syndrome and Parsonage–Turner syndrome (PTS), have been described in patients with hepatitis E virus (HEV) infection in industrialized and developing countries. We report a wider range of neurologic disorders in nonimmunocompromised patients with acute HEV infection. Data from 15 French immunocompetent patients with acute HEV infection and neurologic disorders were retrospectively recorded from January 2006 through June 2013. The disorders could be divided into 4 main entities: mononeuritis multiplex, PTS, meningoradiculitis, and acute demyelinating neuropathy. HEV infection was treated with ribavirin in 3 patients (for PTS or mononeuritis multiplex). One patient was treated with corticosteroids (for mononeuropathy multiplex), and 5 others received intravenous immunoglobulin (for PTS, meningoradiculitis, Guillain-Barré syndrome, or Miller Fisher syndrome). We conclude that pleiotropic neurologic disorders are seen in HEV-infected immunocompetent patients. Patients with acute neurologic manifestations and aminotransferase abnormalities should be screened for HEV infection. PMID:26490255

  10. The Development of the Distance Education College of East China Normal University: A Case Study

    ERIC Educational Resources Information Center

    Ye, Lixin; Su, Xiaobing; Yan, Hanbing

    2009-01-01

    Purpose: This paper aims to present a picture of the past and the present status of the Distance Education College (DEC) of East China Normal University (ECNU). It describes what the DEC has achieved in each phase, and probes into some essential problems that the DEC has attempted to solve. Design/methodology/approach: This article provides a…

  11. Development of Planning Abilities in Normal Aging: Differential Effects of Specific Cognitive Demands

    ERIC Educational Resources Information Center

    Köstering, Lena; Stahl, Christoph; Leonhart, Rainer; Weiller, Cornelius; Kaller, Christoph P.

    2014-01-01

    In line with the frontal hypothesis of aging, the ability to plan ahead undergoes substantial change during normal aging. Although impairments on the Tower of London planning task were reported earlier, associations between age-related declines and specific cognitive demands on planning have not been studied. Here we investigated the impact of…

  12. Common variable immunodeficiency, impaired neurological development and reduced numbers of T regulatory cells in a 10-year-old boy with a STAT1 gain-of-function mutation.

    PubMed

    Kobbe, Robin; Kolster, Manuela; Fuchs, Sebastian; Schulze-Sturm, Ulf; Jenderny, Jutta; Kochhan, Lothar; Staab, Julia; Tolosa, Eva; Grimbacher, Bodo; Meyer, Thomas

    2016-07-25

    Recently, gain-of-function (GOF) mutations in the gene encoding signal transducer and activator of transcription 1 (STAT1) have been associated with chronic mucocutaneous candidiasis (CMC). This case report describes a 10-year-old boy presenting with signs of common variable immunodeficiency (CVID), failure to thrive, impaired neurological development, and a history of recurrent mucocutaneous Candida infections. Sequencing of the STAT1 gene identified a heterozygous missense mutation in exon 7 encoding the STAT1 coiled-coil domain (c.514T>C, p.Phe172Leu). In addition to hypogammaglobulinemia with B-cell deficiency, and a low percentage of Th17 cells, immunological analysis of the patient revealed a marked depletion of forkhead-box P3(+)-expressing regulatory T cells (Tregs). In vitro stimulation of T cells from the patient with interferon-α (IFNα) and/or IFNɣ resulted in a significantly increased expression of STAT1-regulated target genes such as MIG1, IRF1, MX1, MCP1/CCL2, IFI-56K, and CXCL10 as compared to IFN-treated cells from a healthy control, while no IFNα/ɣ-mediated up-regulation of the FOXP3 gene was found. These data demonstrate that the STAT1 GOF mutation F172L, which results in impaired stability of the antiparallel STAT1 dimer conformation, is associated with inhibited Treg cell development and neurological symptoms.

  13. Common variable immunodeficiency, impaired neurological development and reduced numbers of T regulatory cells in a 10-year-old boy with a STAT1 gain-of-function mutation.

    PubMed

    Kobbe, Robin; Kolster, Manuela; Fuchs, Sebastian; Schulze-Sturm, Ulf; Jenderny, Jutta; Kochhan, Lothar; Staab, Julia; Tolosa, Eva; Grimbacher, Bodo; Meyer, Thomas

    2016-07-25

    Recently, gain-of-function (GOF) mutations in the gene encoding signal transducer and activator of transcription 1 (STAT1) have been associated with chronic mucocutaneous candidiasis (CMC). This case report describes a 10-year-old boy presenting with signs of common variable immunodeficiency (CVID), failure to thrive, impaired neurological development, and a history of recurrent mucocutaneous Candida infections. Sequencing of the STAT1 gene identified a heterozygous missense mutation in exon 7 encoding the STAT1 coiled-coil domain (c.514T>C, p.Phe172Leu). In addition to hypogammaglobulinemia with B-cell deficiency, and a low percentage of Th17 cells, immunological analysis of the patient revealed a marked depletion of forkhead-box P3(+)-expressing regulatory T cells (Tregs). In vitro stimulation of T cells from the patient with interferon-α (IFNα) and/or IFNɣ resulted in a significantly increased expression of STAT1-regulated target genes such as MIG1, IRF1, MX1, MCP1/CCL2, IFI-56K, and CXCL10 as compared to IFN-treated cells from a healthy control, while no IFNα/ɣ-mediated up-regulation of the FOXP3 gene was found. These data demonstrate that the STAT1 GOF mutation F172L, which results in impaired stability of the antiparallel STAT1 dimer conformation, is associated with inhibited Treg cell development and neurological symptoms. PMID:27063510

  14. Toward precision medicine in neurological diseases.

    PubMed

    Tan, Lin; Jiang, Teng; Tan, Lan; Yu, Jin-Tai

    2016-03-01

    Technological development has paved the way for accelerated genomic discovery and is bringing precision medicine into view. The goal of precision medicine is to deliver optimally targeted and timed interventions tailored to an individual's molecular drivers of disease. Neurological diseases are promisingly suited models for precision medicine because of the rapidly expanding genetic knowledge base, phenotypic classification, the development of biomarkers and the potential modifying treatments. Moving forward, it is crucial that through these integrated research platforms to provide analysis both for accurate personal genome analysis and gene and drug discovery. Here we describe our vision of how precision medicine can bring greater clarity to the clinical and biological complexity of neurological diseases. PMID:27127757

  15. Hepatitis E virus and neurological injury.

    PubMed

    Dalton, Harry R; Kamar, Nassim; van Eijk, Jeroen J J; Mclean, Brendan N; Cintas, Pascal; Bendall, Richard P; Jacobs, Bart C

    2016-02-01

    Hepatitis E is hyperendemic in many developing countries in Asia and Africa, and is caused by hepatitis E virus (HEV) genotypes 1 and 2, which are spread via the faecal-oral route by contaminated water. Recent data show that HEV infection is also endemic in developed countries. In such geographical settings, hepatitis E is caused by HEV genotypes 3 and 4, and is mainly a porcine zoonosis. In a minority of cases, HEV causes acute and chronic hepatitis, but infection is commonly asymptomatic or unrecognized. HEV infection is associated with a number of extrahepatic manifestations, including a range of neurological injuries. To date, 91 cases of HEV-associated neurological injury--most commonly, Guillain-Barré syndrome, neuralgic amyotrophy, and encephalitis/myelitis--have been reported. Here, we review the reported cases, discuss possible pathogenic mechanisms, and present our perspectives on future directions and research questions.

  16. Neurology in the Vietnam War.

    PubMed

    Gunderson, Carl H; Daroff, Robert B

    2016-01-01

    Between December 1965 and December 1971, the United States maintained armed forces in Vietnam never less than 180,000 men and women in support of the war. At one time, this commitment exceeded half a million soldiers, sailors, and airmen from both the United States and its allies. Such forces required an extensive medical presence, including 19 neurologists. All but two of the neurologists had been drafted for a 2-year tour of duty after deferment for residency training. They were assigned to Vietnam for one of those 2 years in two Army Medical Units and one Air Force facility providing neurological care for American and allied forces, as well as many civilians. Their practice included exposure to unfamiliar disorders including cerebral malaria, Japanese B encephalitis, sleep deprivation seizures, and toxic encephalitis caused by injection or inhalation of C-4 explosive. They and neurologists at facilities in the United States published studies on all of these entities both during and after the war. These publications spawned the Defense and Veterans Head Injury Study, which was conceived during the Korean War and continues today as the Defense and Veterans Head Injury Center. It initially focused on post-traumatic epilepsy and later on all effects of brain injury. The Agent Orange controversy arose after the war; during the war, it was not perceived as a threat by medical personnel. Although soldiers in previous wars had developed serious psychological impairments, post-traumatic stress disorder was formally recognized in the servicemen returning from Vietnam.

  17. Neurology in the Vietnam War.

    PubMed

    Gunderson, Carl H; Daroff, Robert B

    2016-01-01

    Between December 1965 and December 1971, the United States maintained armed forces in Vietnam never less than 180,000 men and women in support of the war. At one time, this commitment exceeded half a million soldiers, sailors, and airmen from both the United States and its allies. Such forces required an extensive medical presence, including 19 neurologists. All but two of the neurologists had been drafted for a 2-year tour of duty after deferment for residency training. They were assigned to Vietnam for one of those 2 years in two Army Medical Units and one Air Force facility providing neurological care for American and allied forces, as well as many civilians. Their practice included exposure to unfamiliar disorders including cerebral malaria, Japanese B encephalitis, sleep deprivation seizures, and toxic encephalitis caused by injection or inhalation of C-4 explosive. They and neurologists at facilities in the United States published studies on all of these entities both during and after the war. These publications spawned the Defense and Veterans Head Injury Study, which was conceived during the Korean War and continues today as the Defense and Veterans Head Injury Center. It initially focused on post-traumatic epilepsy and later on all effects of brain injury. The Agent Orange controversy arose after the war; during the war, it was not perceived as a threat by medical personnel. Although soldiers in previous wars had developed serious psychological impairments, post-traumatic stress disorder was formally recognized in the servicemen returning from Vietnam. PMID:27035455

  18. The effect of normal development and of severe undernutrition on some minor components of cortical bone

    PubMed Central

    McCance, R. A.; Southgate, D. A. T.; Spencer, P. J.; Weston, P. D.

    1966-01-01

    1. The amounts of calcium, magnesium, sodium and citric acid in the bones of undernourished pigs 1 year old were compared with the amounts in the bones of smaller newborn animals, normal animals of the same weight aged 4 weeks and of the same age weighing 170kg. 2. The differences that were found between 4 weeks and 1 year of age in the normal animals were expected as effects of aging. However, between birth and 4 weeks of age the changes in composition were in the opposite direction to those between 4 weeks and 1 year. 3. Undernutrition produced a bone that resembled chemically that of an animal 1 year old. PMID:5968547

  19. Growth hormone receptor gene expression in the skeletal muscle of normal and double-muscled bovines during foetal development.

    PubMed

    Listrat, Anne; Hocquette, Jean François; Picard, Brigitte; Ménissier, François; Djiane, Jean; Jammes, Hélène

    2005-01-01

    The expression of the growth hormone receptor (GHR) gene was investigated in semitendinosus muscle during bovine foetal development in both normal and double-muscled Charolais foetuses which differ with respect to muscle development. Northern-blot analysis of foetal muscle RNA preparations with a GHR cDNA probe identified the 4.5 kb GHR mRNA as early as 130 days post-conception. In double-muscled animals, the expression of GHR mRNA increased from 130 to 210 days of gestation while it stayed stable in normal ones. It was significantly higher (P < 0.05) in double-muscled foetuses compared to normal ones from the second third of gestation. Northern-blot analysis of foetal muscle RNA preparations from both genotypes with a beta-actin cDNA probe, revealed lower beta-actin gene expression in double-muscled foetuses than in normal ones, suggesting a delay in the differentiation of muscle cells. In situ hybridisation revealed the localisation of specific GHR mRNA in muscle cells at all gestation stages analysed (130, 170, 210 days post-conception) but not in connective tissue surrounding the muscle cells. At the adult stage, the hybridisation signal was also very high and observed in muscle cells only. These results show the ontogeny of GHR mRNA in bovine muscle and demonstrate a difference between normal and double-muscled animals.

  20. Acute Neurological Involvement in Diarrhea-Associated Hemolytic Uremic Syndrome

    PubMed Central

    Kwon, Thérésa; Elmaleh, Monique; Charbit, Marina; Launay, Emma Allain; Harambat, Jérôme; Brun, Muriel; Ranchin, Bruno; Bandin, Flavio; Cloarec, Sylvie; Bourdat-Michel, Guylhene; Piètrement, Christine; Champion, Gérard; Ulinski, Tim; Deschênes, Georges

    2010-01-01

    Background and objectives: Neurologic involvement is the most threatening complication of diarrhea-associated hemolytic uremic syndrome (D+HUS). Design, setting, participants, & measurements: We report a retrospective multicenter series of 52 patients with severe initial neurologic involvement that occurred in the course of D+HUS. Results: Verotoxigenic Escherichia coli infection was documented in 24. All except two patients had acute renal failure that required peritoneal dialysis, hemodialysis, or both techniques. A first group of eight patients remained with normal consciousness; five of them had protracted seizures. A second group of 23 patients had stuporous coma; five of these had protracted severe seizures, and 18 had a neurologic defect including pyramidal syndrome, hemiplegia or hemiparesia, and extrapyramidal syndrome. A third group of 21 patients had severe coma. Plasma exchanges were undertaken in 25 patients, 11 of whom were treated within 24 hours after the first neurologic sign; four died, two survived with severe sequelae, and five were alive without neurologic defect. Magnetic resonance imaging (MRI) for 29 patients showed that (1) every structure of the central nervous system was susceptible to involvement; (2) no correlation seemed to exist between special profile of localization on early MRI and the final prognosis; and (3) MRI did not exhibit any focal lesions in three patients. The overall prognosis of the series was marked by the death of nine patients and severe sequelae in 13. Conclusions: Neurologic involvement is associated with a severe renal disease but does not lead systematically to death or severe disability. PMID:20498239

  1. Coal-seismic, desktop computer programs in BASIC; Part 6, Develop rms velocity functions and apply mute and normal movement

    USGS Publications Warehouse

    Hasbrouck, W.P.

    1983-01-01

    Processing of data taken with the U.S. Geological Survey's coal-seismic system is done with a desktop, stand-alone computer. Programs for this computer are written in the extended BASIC language utilized by the Tektronix 4051 Graphic System. This report presents computer programs used to develop rms velocity functions and apply mute and normal moveout to a 12-trace seismogram.

  2. Implicit Attitudes towards Children with Autism versus Normally Developing Children as Predictors of Professional Burnout and Psychopathology

    ERIC Educational Resources Information Center

    Kelly, Amanda; Barnes-Holmes, Dermot

    2013-01-01

    Tutors trained in applied behaviour analysis (n = 16) and mainstream school teachers (n = 16) were exposed to an Implicit Relational Assessment Procedure (IRAP) designed to assess implicit attitudes towards individuals with autism versus normally developing individuals. Participants also completed a range of explicit measures, including measures…

  3. Liver transplantation for hepatic and neurological Wilson's disease.

    PubMed

    Geissler, I; Heinemann, K; Rohm, S; Hauss, J; Lamesch, P

    2003-06-01

    Wilson's disease (WD) is an autosomal-recessive inherited disorder of copper metabolism characterized by excessive deposition of copper throughout the body. If medical treatment fails in cases of fulminant hepatic failure and progressive hepatic dysfunction due to advanced cirrhosis, liver transplantation (OLTx) has been demonstrated to be a valuable treatment option. Between December 1993 and December 2002, 225 OLTxs in 198 patients were performed in our institution. In this consecutive series six patients (three females and three males) were liver grafted for WD. The follow-up ranged from 3 to 7 years. All patients are alive with well-functioning grafts at present. The ceruloplasmin levels increased after transplantation and remained normal. The Kayser-Fleischer ring disappeared in all patients, and urinary copper excretion normalized. The neurological manifestations in the two patients with severe neurological symptoms showed after 2 to 5 years a downward tendency; in one the ataxic movements disappeared completely. The psychiatric disorder in one patient disappeared as well the mild neurological symptoms in the patient with CHILD A cirrhosis. These two patients are fully recovered and returned to work. OLTx should be considered as a treatment option in patients with severe progressive neurological deficits even in cases with stable liver function since liver grafting definitely cures the underlying biochemical defect. In such cases an early decision for liver transplantation is justified because neurological deficits may become irreversible.

  4. Neuroanatomical foundations of naming impairments across different neurologic conditions

    PubMed Central

    Gleichgerrcht, Ezequiel; Fridriksson, Julius

    2015-01-01

    The ability to name objects or abstract entities is an essential feature of speech and language, being commonly considered a central component of normal neurologic function. For this reason, the bedside testing of naming performance is part of the neurologic examination, especially since naming impairments can signify the early onset of a progressive disease or the occurrence of a more established problem. Modern neuroscience research suggests that naming relies on specific and distributed networks that operate in concert to support various processing stages, spanning from object recognition to spoken words. Likewise, studies evaluating the types of naming impairments in patients with neurologic conditions have contributed to the understanding of acquired forms of naming impairments and the underlying stages during normal language processing. In this article, we review the neurobiological mechanisms supporting naming, with a focus on the clinical application of these concepts. We provide an overview of the stages of cognitive processing that are hypothesized to support naming. For each stage, we explore the evidence revealing its neural basis, drawing parallels to clinical syndromes that commonly disrupt each stage. We review the patterns of naming impairment across various neurologic conditions, including classic language disorders, such as poststroke aphasia or primary progressive aphasia, as well as other diseases where language impairments may be subtle but helpful for the appropriate diagnosis. In this context, we provide a structured and practical guide for the bedside naming assessments rooted in modern neuroscience, aimed at supporting the evaluation and diagnosis of neurologic conditions that affect language. PMID:26115732

  5. Explanation and management of neurological disability.

    PubMed Central

    Critchley, E M; Mitchell, J D

    1987-01-01

    Patients are often told of the diagnosis of a neurological disability in less than ideal circumstances such that they may not fully comprehend it. Clinicians must be able to sense when the patient's understanding is imperfect even if superficially he or she appears to take in what has been said and its implications. A programme of further counselling is often required to follow this up. A critical look at current procedures will show how these problems arise. It is suggested that improvements in the care of neurologically disabled patients would follow from improved communication between the many agencies that the concerned in their care and from providing books and other material explaining disability and its treatment. A logical development would be for disabled living centres to be established adjacent to existing neuroscience units. PMID:2954610

  6. The neurological basis of occupation.

    PubMed

    Gutman, Sharon A; Schindler, Victoria P

    2007-01-01

    The purpose of the present paper was to survey the literature about the neurological basis of human activity and its relationship to occupation and health. Activities related to neurological function were organized into three categories: those that activate the brain's reward system; those that promote the relaxation response; and those that preserve cognitive function into old age. The results from the literature review correlating neurological evidence and activities showed that purposeful and meaningful activities could counter the effects of stress-related diseases and reduce the risk for dementia. Specifically, it was found that music, drawing, meditation, reading, arts and crafts, and home repairs, for example, can stimulate the neurogical system and enhance health and well-being, Prospective research studies are needed to examine the effects of purposeful activities on reducing stress and slowing the rate of cognitive decline.

  7. Neurologic complications of scuba diving.

    PubMed

    Newton, H B

    2001-06-01

    Recreational scuba diving has become a popular sport in the United States, with almost 9 million certified divers. When severe diving injury occurs, the nervous system is frequently involved. In dive-related barotrauma, compressed or expanding gas within the ears, sinuses and lungs causes various forms of neurologic injury. Otic barotrauma often induces pain, vertigo and hearing loss. In pulmonary barotrauma of ascent, lung damage can precipitate arterial gas embolism, causing blockage of cerebral blood vessels and alterations of consciousness, seizures and focal neurologic deficits. In patients with decompression sickness, the vestibular system, spinal cord and brain are affected by the formation of nitrogen bubbles. Common signs and symptoms include vertigo, thoracic myelopathy with leg weakness, confusion, headache and hemiparesis. Other diving-related neurologic complications include headache and oxygen toxicity.

  8. Quality Metrics in Inpatient Neurology.

    PubMed

    Dhand, Amar

    2015-12-01

    Quality of care in the context of inpatient neurology is the standard of performance by neurologists and the hospital system as measured against ideal models of care. There are growing regulatory pressures to define health care value through concrete quantifiable metrics linked to reimbursement. Theoretical models of quality acknowledge its multimodal character with quantitative and qualitative dimensions. For example, the Donabedian model distils quality as a phenomenon of three interconnected domains, structure-process-outcome, with each domain mutually influential. The actual measurement of quality may be implicit, as in peer review in morbidity and mortality rounds, or explicit, in which criteria are prespecified and systemized before assessment. As a practical contribution, in this article a set of candidate quality indicators for inpatient neurology based on an updated review of treatment guidelines is proposed. These quality indicators may serve as an initial blueprint for explicit quality metrics long overdue for inpatient neurology.

  9. The neurological basis of occupation.

    PubMed

    Gutman, Sharon A; Schindler, Victoria P

    2007-01-01

    The purpose of the present paper was to survey the literature about the neurological basis of human activity and its relationship to occupation and health. Activities related to neurological function were organized into three categories: those that activate the brain's reward system; those that promote the relaxation response; and those that preserve cognitive function into old age. The results from the literature review correlating neurological evidence and activities showed that purposeful and meaningful activities could counter the effects of stress-related diseases and reduce the risk for dementia. Specifically, it was found that music, drawing, meditation, reading, arts and crafts, and home repairs, for example, can stimulate the neurogical system and enhance health and well-being, Prospective research studies are needed to examine the effects of purposeful activities on reducing stress and slowing the rate of cognitive decline. PMID:17623380

  10. Progress in gene therapy for neurological disorders

    PubMed Central

    Simonato, Michele; Bennett, Jean; Boulis, Nicholas M.; Castro, Maria G.; Fink, David J.; Goins, William F.; Gray, Steven J.; Lowenstein, Pedro R.; Vandenberghe, Luk H.; Wilson, Thomas J.; Wolfe, John H.; Glorioso, Joseph C.

    2013-01-01

    Diseases of the nervous system have devastating effects and are widely distributed among the population, being especially prevalent in the elderly. These diseases are often caused by inherited genetic mutations that result in abnormal nervous system development, neurodegeneration, or impaired neuronal function. Other causes of neurological diseases include genetic and epigenetic changes induced by environmental insults, injury, disease-related events or inflammatory processes. Standard medical and surgical practice has not proved effective in curing or treating these diseases, and appropriate pharmaceuticals do not exist or are insufficient to slow disease progression. Gene therapy is emerging as a powerful approach with potential to treat and even cure some of the most common diseases of the nervous system. Gene therapy for neurological diseases has been made possible through progress in understanding the underlying disease mechanisms, particularly those involving sensory neurons, and also by improvement of gene vector design, therapeutic gene selection, and methods of delivery. Progress in the field has renewed our optimism for gene therapy as a treatment modality that can be used by neurologists, ophthalmologists and neurosurgeons. In this Review, we describe the promising gene therapy strategies that have the potential to treat patients with neurological diseases and discuss prospects for future development of gene therapy. PMID:23609618

  11. Brain Molecular Aging, Promotion of Neurological Disease and Modulation by Sirtuin5 Longevity Gene Polymorphism

    PubMed Central

    Glorioso, Christin; Oh, Sunghee; Douillard, Gaelle Guilloux; Sibille, Etienne

    2010-01-01

    Mechanisms determining characteristic age of onset for neurological diseases are largely unknown. Normal brain aging associates with robust and progressive transcriptome changes (“molecular aging”), but the intersection with disease pathways is mostly uncharacterized. Here, using cross-cohort microarray analysis of four human brain areas, we show that neurological disease pathways largely overlap with molecular aging and that subjects carrying a newly-characterized low-expressing polymorphism in a putative longevity gene (Sirtuin5; SIRT5prom2) have older brain molecular ages. Specifically, molecular aging was remarkably conserved across cohorts and brain areas, and included numerous developmental and transcription-regulator genes. Neurological disease-associated genes were highly overrepresented within age-related genes and changed almost unanimously in pro-disease directions, together suggesting an underlying genetic “program” of aging that progressively promotes disease. To begin testing this putative pathway, we developed and used an age-biosignature to assess five candidate longevity gene polymorphisms association with molecular aging rates. Most robustly, aging was accelerated in cingulate, but not amygdala, of subjects carrying a SIRT5 promoter polymorphism (+9yrs, p=0.004), in concordance with cingulate-specific decreased SIRT5 expression. This effect was driven by a set of core transcripts (+24 yrs, p=0.0004), many of which were mitochondrial, including Parkinson’s disease genes, PINK1 and DJ1/PARK7, hence suggesting that SIRT5prom2 may represent a risk factor for mitochondrial dysfunction-related diseases, including Parkinson s, through accelerated molecular aging of disease-related genes. Based on these results we speculate that a “common mechanism” may underlie age of onset across several neurological diseases. Confirming this pathway and its regulation by common genetic variants would provide new strategies for predicting, delaying, and

  12. Chapter 15: Jean-Martin Charcot and the anatomo-clinical method of neurology.

    PubMed

    Goetz, Christopher G

    2010-01-01

    Jean-Martin Charcot (1825-1893) was the premier clinical neurologist of the 19th century. Charcot's research was anchored in the anatomo-clinical method, a two-part methodology that linked clinical signs with anatomical lesions. The first step of this method involved the careful documentation of clinical signs with longitudinal observation. At the time of death, the second step involved autopsy examination of the brain and spinal cord. With combined clinical and anatomical data, Charcot was able to suggest concrete clinical-anatomical correlations. This method helped to define the tracts and nuclei responsible for normal and abnormal neurological signs and was pivotal to a new classification of neurological diseases based on anatomy. The best-developed example of this method was Charcot's work with motor system degenerative disorders, specifically amyotrophic lateral sclerosis. These studies led to the international designation of amyotrophic lateral sclerosis as Charcot's disease. Other examples of the fruits of the anatomo-clinical method included several stroke syndromes and the linkage of specific signs to specific lesions in multiple sclerosis. The discipline fostered cortical localization theory, which moved neurologists away from the concept of the brain as a homogenous organ in preference to the concept that brain regions controlled specific motor, sensory and language functions. Charcot's attempts to apply his anatomo-clinical method to the knotty neurological diagnosis of hysteria led him to experiments and conclusions that drew criticism and even scorn from colleagues. These events tarnished Charcot's reputation at the close of his career. In the context of Charcot's extensive discoveries and lasting contributions, the anatomo-clinical method remains the anchor of modern neurological diagnosis and is Charcot's most important contribution to clinical neurology.

  13. Neurologic Emergencies in the Elderly.

    PubMed

    Nentwich, Lauren M; Grimmnitz, Benjamin

    2016-08-01

    Neurologic diseases are a major cause of death and disability in elderly patients. Due to the physiologic changes and increased comorbidities that occur as people age, neurologic diseases are more common in geriatric patients and a major cause of death and disability in this population. This article discusses the elderly patient presenting to the emergency department with acute ischemic stroke, transient ischemic attack, intracerebral hemorrhage, subarachnoid hemorrhage, chronic subdural hematoma, traumatic brain injury, seizures, and central nervous system infections. This article reviews the subtle presentations, difficult workups, and complicated treatment decisions as they pertain to our older patients." PMID:27475016

  14. Neurological and ocular fascioliasis in humans.

    PubMed

    Mas-Coma, Santiago; Agramunt, Verónica H; Valero, María Adela

    2014-01-01

    Fascioliasis is a food-borne parasitic disease caused by the trematode species Fasciola hepatica, distributed worldwide, and Fasciola gigantica, restricted to given regions of Africa and Asia. This disease in humans shows an increasing importance, which relies on its recent widespread emergence related to climate and global changes and also on its pathogenicity in the invasive, biliary, and advanced chronic phases in the human endemic areas, mainly of developing countries. In spite of the large neurological affection capacity of Fasciola, this important pathogenic aspect of the disease has been pronouncedly overlooked in the past decades and has not even appear within the numerous reviews on the parasitic diseases of the central nervous system. The aim of this wide retrospective review is an in-depth analysis of the characteristics of neurological and ocular fascioliasis caused by these two fasciolid species. The terms of neurofascioliasis and ophthalmofascioliasis are restricted to cases in which the direct affection of the central nervous system or the eye by a migrant ectopic fasciolid fluke is demonstrated by an aetiological diagnosis of recovered flukes after surgery or spontaneous moving-out of the fluke through the orbit. Cases in which the ectopic fluke is not recovered and the symptoms cannot be explained by an indirect affection at distance may also be included in these terms. Neurofascioliasis and ophthalmofascioliasis cases are reviewed and discussed. With regard to fascioliasis infection giving an indirect rise to neurological affection, the distribution and frequency of cases are analysed according to geography, sex, and age. Minor symptoms and major manifestations are discussed. Three main types of cases are distinguished depending on the characteristics of their manifestations: genuine neurological, meningeal, and psychiatric or neuropsychic. The impressive symptoms and signs appearing in each type of these cases are included. Brain examination

  15. FT overexpression induces precocious flowering and normal reproductive development in Eucalyptus.

    PubMed

    Klocko, Amy L; Ma, Cathleen; Robertson, Sarah; Esfandiari, Elahe; Nilsson, Ove; Strauss, Steven H

    2016-02-01

    Eucalyptus trees are among the most important species for industrial forestry worldwide. However, as with most forest trees, flowering does not begin for one to several years after planting which can limit the rate of conventional and molecular breeding. To speed flowering, we transformed a Eucalyptus grandis × urophylla hybrid (SP7) with a variety of constructs that enable overexpression of FLOWERING LOCUS T (FT). We found that FT expression led to very early flowering, with events showing floral buds within 1-5 months of transplanting to the glasshouse. The most rapid flowering was observed when the cauliflower mosaic virus 35S promoter was used to drive the Arabidopsis thaliana FT gene (AtFT). Early flowering was also observed with AtFT overexpression from a 409S ubiquitin promoter and under heat induction conditions with Populus trichocarpa FT1 (PtFT1) under control of a heat-shock promoter. Early flowering trees grew robustly, but exhibited a highly branched phenotype compared to the strong apical dominance of nonflowering transgenic and control trees. AtFT-induced flowers were morphologically normal and produced viable pollen grains and viable self- and cross-pollinated seeds. Many self-seedlings inherited AtFT and flowered early. FT overexpression-induced flowering in Eucalyptus may be a valuable means for accelerating breeding and genetic studies as the transgene can be easily segregated away in progeny, restoring normal growth and form. PMID:26132805

  16. Neurological Soft Signs in Indian Children with Specific Developmental Disorders of Scholastic Skills

    ERIC Educational Resources Information Center

    Sadhu, Raja; Mehta, Manju; Kalra, Veena; Sagar, Rajesh; Mongia, Monica

    2008-01-01

    Aim: To compare the occurrence of neurological soft signs (NSS) in children with specific developmental disorders of scholastic skills (SDDSS) and normal children. Methods: 36 cases of SDDSS were compared with 30 control children regarding sociodemographic and clinical variables and neurological soft signs. Results: Children with SDDSS had…

  17. [Gangliosides in neurological pharmacotherapy].

    PubMed

    Małecki, A; Małecka, E; Trzeciak, H I

    1992-01-01

    Gangliosides take part in synaptic transmission, neuronal metabolism and development of nervous tissue. They cooperate with nerve growth factor (NGF) and have positive influence on regeneration of the nervous system impairments. There exist many behavioural and biochemical evidences of gangliosides participation in the regeneration of experimentally injured animal nervous system. The therapeutic effectivity of gangliosides in clinical practice is encouraging. Commercial preparates of gangliosides (Cronassial, Sygen) have been successfully used in the therapy of chronic neuropathies, strokes and subarachnoidal haemorrhages. Among the adverse reactions to these drugs are: local irritation, anxiety and possible detrimental effect in immunological system. Ganglioside preparations need further clinical examinations. PMID:1291908

  18. A comparative study of monocot and dicot root development in normal /earth/ and hypogravity /space/ environments

    NASA Technical Reports Server (NTRS)

    Slocum, R. D.; Galston, A. W.

    1982-01-01

    The anatomy and fine structure of roots from oat and mung bean seedlings grown under hypogravity conditions aboard NASA's Space Shuttle were examined and compared to those of roots from ground control plants grown under similar conditions. Oat roots from both sets of plants exhibited normal tissue organization and ultrastructural features, with the exception of cortex cell mitochondria, which characteristically showed a 'swollen' morphology. Flight-grown mung bean roots differed significantly from the controls in that root cap cells were somewhat disorganized and degraded in appearance, especially at the cap periphery. At the EM level, these cells exhibited a loss organelle integrity and a condensed cytoplasm. The potential significance of this finding for the putative gravity-sensing cap cells were noted.

  19. Personality traits and personality disorders in older women: an explorative study between normal development and psychopathology.

    PubMed

    Henriques-Calado, Joana; Duarte-Silva, Maria Eugénia; Keong, Ana Marta; Sacoto, Carlota; Junqueira, Diana

    2014-01-01

    The relationships between Axis II personality disorders (DSM-IV) and the Five-Factor Model (FFM) were explored in older women. The sample consists of 90 participants (M = 72.29 years, SD = 7.10) who were administered the NEO-Five-Factor Inventory and the Personality Diagnostic Questionnaire. The highest prevalence of A and C clusters and obsessive-compulsive personality disorder was observed. Also, elevated neuroticism and decreased agreeableness and openness appear as valuable traits in the description of psychopathology. The study of maladaptive personality functioning within an aging population can be described with the same traits that underlie normal personality functioning, extending the range of psychopathology to a dimensional approach.

  20. Sparring and Neurological Function in Professional Boxers

    PubMed Central

    Stiller, John W.; Yu, Steven S.; Brenner, Lisa A.; Langenberg, Patricia; Scrofani, Phillip; Pannella, Patrick; Hsu, Edbert B.; Roberts, Darryl W.; Monsell, Ray M. T.; Binks, Sidney W.; Guzman, Alvaro; Postolache, Teodor T.

    2014-01-01

    Despite increased interest regarding the potentially long-term negative impact of chronic traumatic brain injury, limited research has been conducted regarding such injuries and neurological outcomes in real world settings. To increase understanding regarding the relationship between sparring (e.g., training under the tutelage of an experienced boxing coach for the purpose of improving skills and/or fitness) and neurological functioning, professional boxers (n = 237) who competed in Maryland between 2003 and 2008 completed measures regarding sparring exposure (Cumulative Sparring Index, CSI) and performance on tests of cognition (Symbol Digit Modalities Test, SDMT) and balance (Sharpened Romberg Test, SRT). Measures were completed prior to boxing matches. Higher scores on the CSI (increased sparring exposure) were associated with poorer performance on both tests of cognition (SDMT) and balance (SRT). A threshold effect was noted regarding performance on the SDMT, with those reporting CSI values greater than about 150 experiencing a decline in cognition. A history of frequent and/or intense sparring may pose a significant risk for developing boxing associated neurological sequelae. Implementing administration of clinically meaningful tests before bouts, such as the CSI, SDMT, and/or the SRT, as well as documentation of results into the boxer’s physicals or medical profiles may be an important step for improving boxing safety. PMID:25101253

  1. [Jean-Martin Charcot in German neurology].

    PubMed

    Lehmann, H C; Hartung, H-P; Kieseier, B C

    2004-02-01

    Jean-Martin Charcot (1825-1893), well known as the founder of modern neurology, was the most celebrated neurologist in the nineteenth century. His international success stemmed not only from mastery descriptions of various neurological disorders but also from his many contacts with scientists all over the world. The aim of this article is to review Charcot's ambivalent relationship to German neuropsychiatry of the time and to examine the German reception of his personality and work. Wilhelm Erb, Ludwig Hirt, Ernst von Leyden, Max Nonne, Adolph Strümpell, and other German physicians cultivated -to varying degrees - professional contacts with Charcot and, based on the fascination of his personality and significance of his work, were long and intensively influenced by the Salpêtrière school. The extent of their admiration became apparent in 1882 by the award of an honorary doctorate to Charcot by the University of Würzburg. Along with increasingly severe criticism of Charcot's research on hysteria and hypnosis, most German neuropsychiatrists became estranged, without neglecting his importance to the development of neurology in Germany.

  2. Neurological Complications of Ebola Virus Infection.

    PubMed

    Billioux, Bridgette Jeanne; Smith, Bryan; Nath, Avindra

    2016-07-01

    Ebola virus disease is one of the deadliest pathogens known to man, with a mortality rate between 25-90% depending on the species and outbreak of Ebola. Typically, it presents with fever, headache, voluminous vomiting and diarrhea, and can progress to a hemorrhagic illness; neurologic symptoms, including meningoencephalitis, seizures, and coma, can also occur. Recently, an outbreak occurred in West Africa, affecting > 28,000 people, and killing > 11,000. Owing to the magnitude of this outbreak, and the large number (>17,000) of Ebola survivors, the medical and scientific communities are learning much more about the acute manifestations and sequelae of Ebola. A number of neurologic complications can occur after Ebola, such as seizures, memory loss, headaches, cranial nerve abnormalities, and tremor. Ebola may also persist in some immunologically privileged sites, including the central nervous system, and can rarely lead to relapse in disease. Owing to these findings, it is important that survivors are evaluated and monitored for neurologic symptoms. Much is unknown about this disease, and treatment remains largely supportive; however, with ongoing clinical and basic science, the mechanisms of how Ebola affects the central nervous system and how it persists after acute disease will hopefully become more clear, and better treatments and clinical practices for Ebola patients will be developed. PMID:27412684

  3. Edgar Allan Poe and neurology.

    PubMed

    Teive, Hélio Afonso Ghizoni; Paola, Luciano de; Munhoz, Renato Puppi

    2014-06-01

    Edgar Allan Poe was one of the most celebrated writers of all time. He published several masterpieces, some of which include references to neurological diseases. Poe suffered from recurrent depression, suggesting a bipolar disorder, as well as alcohol and drug abuse, which in fact led to his death from complications related to alcoholism. Various hypotheses were put forward, including Wernicke's encephalopathy.

  4. Neurological Complications of Lyme Disease

    MedlinePlus

    ... may begin with flu-like symptoms such as fever, chills, swollen lymph nodes, headaches, fatigue, muscle aches, and joint pain. Neurological complications most often occur in the second stage ... such as fever, stiff neck, and severe headache. Other problems, which ...

  5. HEW and the neurologically handicapped

    NASA Technical Reports Server (NTRS)

    Huber, W. V.

    1974-01-01

    Some of the neurological disorders and therapeutic devices are considered with which the Department of Health, Education, and Welfare (HEW) is most concerned. The organization of the Department, because it is a rather complex one with many different agencies involved, is also described.

  6. Neurology Case Studies: Cerebrovascular Disease.

    PubMed

    Farooq, Muhammad U; Gorelick, Philip B

    2016-08-01

    This article discusses interesting vascular neurology cases including the management of intracranial stenosis, migraine headache and stroke risk, retinal artery occlusions associated with impaired hearing, intracranial occlusive disease, a heritable cause of stroke and vascular cognitive impairment, and an interesting clinico-neuroradiologic disorder associated with eclampsia. PMID:27445238

  7. Fly model causes neurological rethink

    PubMed Central

    Sadanandappa, Madhumala K

    2013-01-01

    A Drosophila model for a neurological disorder called type 2B Charcot-Marie-Tooth disease reveals that it has its origins in a partial loss of function, rather than a gain of function, which points to the need for a new therapeutic approach. PMID:24336781

  8. Neurologic issues and obstetric anesthesia.

    PubMed

    Chang, Laura Y; Carabuena, Jean Marie; Camann, William

    2011-09-01

    The rising popularity of regional anesthesia in the last several decades has greatly changed the experience of labor. Although the use of regional anesthesia has aided in decreasing maternal morbidity and mortality, a new dimension of neurologic issues, particularly headache and peripheral neuropathy, is apparent. Obstetric anesthesiologists frequently encounter patients with preexisting neurologic disease. Although very few of these disorders contraindicate the use of neuraxial technique, there are limited published data on specific neurologic and neuromuscular disorders in pregnancy. Neurologists are often consulted by anesthesiologists and obstetricians to evaluate pregnant patients for the feasibility of labor analgesia and when postpartum neurologic complications arise. Early consultation with an obstetric anesthesiologist, discussion with a neurologist, and communication with the obstetrician allows for the education and discussion of the risks and benefits of both the mode of delivery and anesthetic options. This multidisciplinary approach is crucial in forming reasonable expectations for the patient. The aim of this discussion is to provide an obstetric anesthesiologist's perspective on regional anesthesia and its implications in obstetrics, and to enhance communication between our specialties. PMID:22113509

  9. An unusual neurological consequence of massive wasp stings

    PubMed Central

    Volders, José; Smits, Marcel; Folkersma, Gerbrich; Tjan, David H.

    2012-01-01

    Although rare, central and peripheral neurological manifestations after single or multiple wasp stings have been reported. The authors describe a 45-year-old man who developed periods of sudden loss of consciousness with a Glasgow Coma Scale of 6 after being stung by fifty wasps. These periods were seen directly after being stung and were continuing months later. Different mechanisms and pathophysiological findings of neurological sequela reported after wasp stings are being reviewed. PMID:22744238

  10. The neurology of poverty.

    PubMed

    Alvarez, G

    1982-01-01

    An intellectual deficit is known to exist in populations where extreme poverty is rife and is thus seen extensively in the lower socio-economic strata of underdeveloped nations. Poverty is a complex entity whose sociological and economic indicators often bear little relevance to the biological agents which can affect the central nervous system. An attempt is made to express poverty in terms of identifiable defects, physiological in nature. Thus adverse socio-economic factors are converted into specific biological entities which, though necessary for adequate development of the brain, are restricted where there is poverty. A number of causative deficiencies, including nutritional, visual, auditory, tactile, vestibular, affective, and other stimuli are postulated. These interact and potentiate one another. Each is capable of an independent action on the brain and examples are given of some sensory deprivations as well as malnutrition and their possible mechanism of action. If the various deficiencies can independently harm the brain, then a number of separate specific functions should be affected; examples are offered. The nature of this intellectual deficit is probably a non-fulfillment of genetic potential of certain specific functions of the brain, which may exhibit limited variations between one community and another, depending on cultural differences. The deleterious effect of this intellectual impairment is seen most clearly in figures of school desertion, for example in Latin America. Analogous data for adults is scarce.

  11. The neurology of poverty.

    PubMed

    Alvarez, G

    1982-01-01

    An intellectual deficit is known to exist in populations where extreme poverty is rife and is thus seen extensively in the lower socio-economic strata of underdeveloped nations. Poverty is a complex entity whose sociological and economic indicators often bear little relevance to the biological agents which can affect the central nervous system. An attempt is made to express poverty in terms of identifiable defects, physiological in nature. Thus adverse socio-economic factors are converted into specific biological entities which, though necessary for adequate development of the brain, are restricted where there is poverty. A number of causative deficiencies, including nutritional, visual, auditory, tactile, vestibular, affective, and other stimuli are postulated. These interact and potentiate one another. Each is capable of an independent action on the brain and examples are given of some sensory deprivations as well as malnutrition and their possible mechanism of action. If the various deficiencies can independently harm the brain, then a number of separate specific functions should be affected; examples are offered. The nature of this intellectual deficit is probably a non-fulfillment of genetic potential of certain specific functions of the brain, which may exhibit limited variations between one community and another, depending on cultural differences. The deleterious effect of this intellectual impairment is seen most clearly in figures of school desertion, for example in Latin America. Analogous data for adults is scarce. PMID:7112171

  12. Neurological emergencies: acute stroke

    PubMed Central

    Davenport, R.; Dennis, M.

    2000-01-01

    Stroke causes a vast amount of death and disability throughout the world, yet for many healthcare professionals it remains an area of therapeutic nihilism, and thus uninteresting. This negative perception is shared by the general public, who often have a poor understanding of the early symptoms and significance of a stroke. Yet within the past few years there have been many important developments in the approach to caring for stroke patients, for both the acute management and secondary prevention. After the completion of numerous clinical trials, there is now robust evidence to either support or discredit various interventions. Even more exciting is the prospect of yet more data becoming available in the near future, testing a whole array of treatments, as clinical interest in stroke expands exponentially. In this review an evidence based approach to the management of acute stroke within the first few days is presented, including ischaemic and haemorrhagic events, but not subarachnoid haemorrhage. It is explained why stroke is regarded as a medical emergency, and the importance of a rational, methodic approach to the initial assessment, which is the key to accurate diagnosis and subsequent management, is emphasised. The potential early problems associated with stroke are identified and specific interventions for different stroke types are discussed. The review ends with a brief discussion of the implications that the evolving treatments have for the organisation of modern stroke services.

 PMID:10675208

  13. Interpersonal Relatedness and Self-Definition in Normal and Disrupted Personality Development: Retrospect and Prospect

    ERIC Educational Resources Information Center

    Luyten, Patrick; Blatt, Sidney J.

    2013-01-01

    Two-polarities models of personality propose that personality development evolves through a dialectic synergistic interaction between two fundamental developmental psychological processes across the life span--the development of interpersonal relatedness on the one hand and of self-definition on the other. This article offers a broad review of…

  14. Understanding normal and abnormal development of the Wolffian/epididymal duct by using transgenic mice

    PubMed Central

    Murashima, Aki; Xu, Bingfang; Hinton, Barry T

    2015-01-01

    The development of the Wolffian/epididymal duct is crucial for proper function and, therefore, male fertility. The development of the epididymis is complex; the initial stages form as a transient embryonic kidney; then the mesonephros is formed, which in turn undergoes extensive morphogenesis under the influence of androgens and growth factors. Thus, understanding of its full development requires a wide and multidisciplinary view. This review focuses on mouse models that display abnormalities of the Wolffian duct and mesonephric development, the importance of these mouse models toward understanding male reproductive tract development, and how these models contribute to our understanding of clinical abnormalities in humans such as congenital anomalies of the kidney and urinary tract (CAKUT). PMID:26112482

  15. Understanding normal and abnormal development of the Wolffian/epididymal duct by using transgenic mice.

    PubMed

    Murashima, Aki; Xu, Bingfang; Hinton, Barry T

    2015-01-01

    The development of the Wolffian/epididymal duct is crucial for proper function and, therefore, male fertility. The development of the epididymis is complex; the initial stages form as a transient embryonic kidney; then the mesonephros is formed, which in turn undergoes extensive morphogenesis under the influence of androgens and growth factors. Thus, understanding of its full development requires a wide and multidisciplinary view. This review focuses on mouse models that display abnormalities of the Wolffian duct and mesonephric development, the importance of these mouse models toward understanding male reproductive tract development, and how these models contribute to our understanding of clinical abnormalities in humans such as congenital anomalies of the kidney and urinary tract (CAKUT). PMID:26112482

  16. A putative APSES transcription factor is necessary for normal growth and development of Aspergillus nidulans.

    PubMed

    Lee, Ji-Yeon; Kim, Lee-Han; Kim, Ha-Eun; Park, Jae-Sin; Han, Kap-Hoon; Han, Dong-Min

    2013-12-01

    The nsdD gene encoding a GATA type transcription factor positively controls sexual development in Aspergillus nidulans. According to microarray data, 20 genes that were upregulated by deleting nsdD during various life cycle stages were randomly selected and deleted for functional analysis. None of the mutants showed apparent changes in growth or development compared with those of the wild-type except the AN3154 gene that encodes a putative APSES transcription factor and is an ortholog of Saccharomyces cerevisiae swi4. Deleting AN3154 resulted in retarded growth and development, and the gene was named rgdA (retared growth and development). The rgdA deletion mutant developed a reduced number of conidia even under favorable conditions for asexual development. The retarded growth and development was partially suppressed by the veA1 mutation. The conidial heads of the mutant aborted, showing reduced and irregular shaped phialides. Fruiting body development was delayed compared with that in the wild-type. The mutant did not respond to various nutritional or environmental factors that affected the development patterns. The rgdA gene was expressed at low levels throughout the life cycle and was not significantly affected by several regulators of sexual and asexual development such as nsdD, veA, stuA, or brlA. However, the rgdA gene affected brlA and abaA expression, which function as key regulators of asexual sporulation, suggesting that rgdA functions upstream of those genes.

  17. Implicit attitudes towards children with autism versus normally developing children as predictors of professional burnout and psychopathology.

    PubMed

    Kelly, Amanda; Barnes-Holmes, Dermot

    2013-01-01

    Tutors trained in applied behaviour analysis (n = 16) and mainstream school teachers (n = 16) were exposed to an Implicit Relational Assessment Procedure (IRAP) designed to assess implicit attitudes towards individuals with autism versus normally developing individuals. Participants also completed a range of explicit measures, including measures of professional burnout and psychopathology. All participants produced more negative biases towards children with autism compared to children who were normally developing. Increased negativity towards autism on the IRAP predicted similar attitudes on some of the explicit measures and also correlated with increased levels of self-reported psychopathology and professional burnout for the tutors working with children with autism. Results suggest that implicit measures of attitudes may provide a marker for professional burnout.

  18. Development of normalized spectra manipulating spectrophotometric methods for simultaneous determination of Dimenhydrinate and Cinnarizine binary mixture.

    PubMed

    Lamie, Nesrine T; Yehia, Ali M

    2015-01-01

    Simultaneous determination of Dimenhydrinate (DIM) and Cinnarizine (CIN) binary mixture with simple procedures were applied. Three ratio manipulating spectrophotometric methods were proposed. Normalized spectrum was utilized as a divisor for simultaneous determination of both drugs with minimum manipulation steps. The proposed methods were simultaneous constant center (SCC), simultaneous derivative ratio spectrophotometry (S(1)DD) and ratio H-point standard addition method (RHPSAM). Peak amplitudes at isoabsorptive point in ratio spectra were measured for determination of total concentrations of DIM and CIN. For subsequent determination of DIM concentration, difference between peak amplitudes at 250 nm and 267 nm were used in SCC. While the peak amplitude at 275 nm of the first derivative ratio spectra were used in S(1)DD; then subtraction of DIM concentration from the total one provided the CIN concentration. The last RHPSAM was a dual wavelength method in which two calibrations were plotted at 220 nm and 230 nm. The coordinates of intersection point between the two calibration lines were corresponding to DIM and CIN concentrations. The proposed methods were successfully applied for combined dosage form analysis, Moreover statistical comparison between the proposed and reported spectrophotometric methods was applied. PMID:26037499

  19. The role of the basal stem cell of the human breast in normal development and cancer

    PubMed Central

    Russo, Jose; Russo, Irma H.

    2011-01-01

    MCF-10F a spontaneously immortalized ERα negative human breast epithelial cell line derived from breast tissues containing Lobules type 1, is able to form normal ductal structures in a tridimensional collagen matrix system. MCF-10F cells that are Estrogen-transformed [trMCF cells] progressively express phenotypes of in vitro cell transformation, including colony formation in agar methocel, and loss of the ductulogenic capacity. Further selection of these trMCF cells for invasiveness in a Matrigel invasion system identified cells [bcMCF] that formed tumors in severe combined immunodeficient [SCID] mice. The cell lines derived from those tumors [caMCF] were poorly differentiated ERα, PR and ERBB2 negative adenocarcinomas. These characteristics are similar to the human basal cell-like carcinomas. This in vitro in vivo model demonstrates the importance of the basal cell type as a stem cell that reconstitute the branching pattern of the breast and that is also target of a carcinogenic insult leading to transformation and cancer. PMID:21901623

  20. [COMPARISON OF PSYCHOMOTOR DEVELOPMENT IN PRESCHOOL CHILEAN NORMAL WEIGHT VERSUS OVERWEIGHT/OBESITY].

    PubMed

    Méndez Ruíz, Mariela; Estay Carvajal, Jessica; Calzadilla Nuñez, Aracelis; Durán Agüero, Samuel; Díaz-Narváez, Víctor Patricio

    2015-07-01

    Objetivo: comparar cómo se distribuye el Desarrollo Psicomotor (DSM) en tres estados nutricionales: normal, sobrepeso y obeso. Material y métodos: se aplicó el Test de DSM “TEPSI” a 58 niños (muestra intencionada) de un total de 150, de 4 a 4,5 años, los cuales se dividieron en 3 grupos: normopeso (n = 28); sobrepeso (n = 18) y obeso (n = 12). El peso y la talla se midieron utilizando una balanza SECA y un cartabón de pared (metodología de Frankfurt). La evaluación nutricional se realizó por IMC/edad según la propuesta del Center for Disease Control de Estados Unidos (CDC). Las comparaciones se realizaron mediante un análisis de varianza, prueba Tukey y prueba de Kruskal- Wallis. Se emplearon gráficos de cajas. El nivel de significación empleado fue de ≤ 0,05 en todos los casos. Resultados: los preescolares con obesidad y sobrepeso estudiados mostraron un perfil motriz clasificado como inferior y muy inferior, respectivamente, cuando eran comparados con el grupo de preescolares normopeso. Conclusiones: el sobrepeso/obesidad es un factor que influye en el desarrollo de la psicomotricidad de forma negativa en preescolares de 4 años de edad.

  1. Vision development in the monocular individual: implications for the mechanisms of normal binocular vision development and the treatment of infantile esotropia.

    PubMed Central

    Day, S

    1995-01-01

    PURPOSE: The purpose of this research is to study the vision development in monocular individuals so as to better understand normal binocular vision development and to refine the treatment of infants with infantile esotropia. METHODS: Thirty-six subjects with one clinically normal eye and one eye with no vision (no light perception or history of enucleation) are studied. In addition to measurement of standard parameters of development such as visual acuity, measurement of motion processing is made by both optokinetic and electrophysiologic techniques. A comparison is made of vision development among three populations: the monocular population, the normal population, and patients with a history of infantile esotropia. Such comparison is made to study the relative effects of interruption of binocularity and binocular competition. The monocular population represents individuals who have interruption of binocularity, whereas the infantile esotropia population has both interruption of binocularity and binocular competition. RESULTS: The OKN data suggest that the monucular population is more similar to the normal population than the esotropia population. The electrophysiologic data shows a statistically significant difference in the three populations. Motion processing is more fully developed in the monocular population than in the infantile esotropia population when compared to the normal population. CONCLUSIONS: 1. The development of motion processing appears to be particularly vulnerable to abnormal experience during the first year of life. 2. Monocular subjects have a less abnormal motion processing system when compared to patients with infantile esotropia even when monocularity is congenital. 3. The results indirectly support the premise that prealignment alternate occlusion is of benefit to the patient with infantile esotropia prior to realignment. 4. Development of the motion processing system does not necessarily parallel the development of other binocular

  2. Signaling Proteins and Transcription Factors in Normal and Malignant Early B Cell Development

    PubMed Central

    Pérez-Vera, Patricia; Reyes-León, Adriana; Fuentes-Pananá, Ezequiel M.

    2011-01-01

    B cell development starts in bone marrow with the commitment of hematopoietic progenitors to the B cell lineage. In murine models, the IL-7 and preBCR receptors, and the signaling pathways and transcription factors that they regulate, control commitment and maintenance along the B cell pathway. E2A, EBF1, PAX5, and Ikaros are among the most important transcription factors controlling early development and thereby conditioning mice homeostatic B cell lymphopoiesis. Importantly, their gain or loss of function often results in malignant development in humans, supporting conserved roles for these transcription factors. B cell acute lymphoblastic leukemia is the most common cause of pediatric cancer, and it is characterized by unpaired early B cell development resulting from genetic lesions in these critical signaling pathways and transcription factors. Fine mapping of these genetic abnormalities is allowing more specific treatments, more accurately predicting risk profiles for this disease, and improving survival rates. PMID:22046564

  3. Signaling proteins and transcription factors in normal and malignant early B cell development.

    PubMed

    Pérez-Vera, Patricia; Reyes-León, Adriana; Fuentes-Pananá, Ezequiel M

    2011-01-01

    B cell development starts in bone marrow with the commitment of hematopoietic progenitors to the B cell lineage. In murine models, the IL-7 and preBCR receptors, and the signaling pathways and transcription factors that they regulate, control commitment and maintenance along the B cell pathway. E2A, EBF1, PAX5, and Ikaros are among the most important transcription factors controlling early development and thereby conditioning mice homeostatic B cell lymphopoiesis. Importantly, their gain or loss of function often results in malignant development in humans, supporting conserved roles for these transcription factors. B cell acute lymphoblastic leukemia is the most common cause of pediatric cancer, and it is characterized by unpaired early B cell development resulting from genetic lesions in these critical signaling pathways and transcription factors. Fine mapping of these genetic abnormalities is allowing more specific treatments, more accurately predicting risk profiles for this disease, and improving survival rates.

  4. Wnt5a Is Necessary for Normal Kidney Development in Zebrafish and Mice

    PubMed Central

    Huang, Liwei; Xiao, An; Choi, Soo Young; Kan, Quane; Zhou, Weibin; Chacon-Heszele, Maria F.; Ryu, Yun Kyoung; McKenna, Sarah; Zuo, Xiaofeng; Kuruvilla, Rejji; Lipschutz, Joshua H.

    2015-01-01

    Background Wnt5a is important for the development of various organs and postnatal cellular function. Little is known, however, about the role of Wnt5a in kidney development, although WNT5A mutations were identified in patients with Robinow syndrome, a genetic disease which includes developmental defects in kidneys. Our goal in this study was to determine the role of Wnt5a in kidney development. Methods Whole-mount in situ hybridization was used to establish the expression pattern of Wnt5a during kidney development. Zebrafish with wnt5a knockdown and Wnt5a global knockout mice were used to identify kidney phenotypes. Results In zebrafish, wnt5a knockdown resulted in glomerular cyst formation and dilated renal tubules. In mice, Wnt5a global knockout resulted in pleiotropic, but severe, kidney phenotypes, including agenesis, fused kidney, hydronephrosis and duplex kidney/ureter. Conclusions Our data demonstrated the important role of Wnt5a in kidney development. Disrupted Wnt5a resulted in kidney cysts in zebrafish and pleiotropic abnormal kidney development in mice. PMID:25412793

  5. Validation of Tuba1a as Appropriate Internal Control for Normalization of Gene Expression Analysis during Mouse Lung Development

    PubMed Central

    Mehta, Aditi; Dobersch, Stephanie; Dammann, Reinhard H.; Bellusci, Saverio; Ilinskaya, Olga N.; Braun, Thomas; Barreto, Guillermo

    2015-01-01

    The expression ratio between the analysed gene and an internal control gene is the most widely used normalization method for quantitative RT-PCR (qRT-PCR) expression analysis. The ideal reference gene for a specific experiment is the one whose expression is not affected by the different experimental conditions tested. In this study, we validate the applicability of five commonly used reference genes during different stages of mouse lung development. The stability of expression of five different reference genes (Tuba1a, Actb Gapdh, Rn18S and Hist4h4) was calculated within five experimental groups using the statistical algorithm of geNorm software. Overall, Tuba1a showed the least variability in expression among the different stages of lung development, while Hist4h4 and Rn18S showed the maximum variability in their expression. Expression analysis of two lung specific markers, surfactant protein C (SftpC) and Clara cell-specific 10 kDA protein (Scgb1a1), normalized to each of the five reference genes tested here, confirmed our results and showed that incorrect reference gene choice can lead to artefacts. Moreover, a combination of two internal controls for normalization of expression analysis during lung development will increase the accuracy and reliability of results. PMID:25723738

  6. Dysprosody nonassociated with neurological diseases--a case report.

    PubMed

    Pinto, José Antonio; Corso, Renato José; Guilherme, Ana Cláudia Rocha; Pinho, Sílvia Rebelo; Nóbrega, Monica de Oliveira

    2004-03-01

    Dysprosody also known as pseudo-foreign dialect, is the rarest neurological speech disorder. It is characterized by alterations in intensity, in the timing of utterance segments, and in rhythm, cadency, and intonation of words. The terms refers to changes as to duration, fundamental frequency, and intensity of tonic and atonic syllables of the sentences spoken, which deprive an individual's particular speech of its characteristics. The cause of this disease is usually associated with neurological pathologies such as brain vascular accidents, cranioencephalic traumatisms, and brain tumors. The authors report a case of dysprosody attended to at the Núcleo de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço de São Paulo (NOSP). It is about a female patient with bilateral III degree Reinke's edema and normal neurological examinations that started presenting characteristics of the German dialect following a larynx microsurgery.

  7. Postnatal ovarian follicle development in hypogonadal (hpg) and normal mice and associated changes in the hypothalamic-pituitary ovarian axis.

    PubMed

    Halpin, D M; Jones, A; Fink, G; Charlton, H M

    1986-05-01

    Significant uterine growth occurred in normal and hypogonadal (hpg) mice between Days 7 and 21 but thereafter no further growth was observed in hpg mice. The ovaries of hpg mice were significantly smaller than those of normals at all ages, but there was no significant difference between the number of non-growing follicles in the ovaries of mutants and their normal littermates at any age studied, and normal and hpg mice showed a marked reduction in the number of non-growing follicles during the first month of life. The size and composition of the growing follicle population in hpg mice, however, differed markedly from those in normal animals and by 21 days of age the number of growing follicles in mutants was significantly reduced. There was no significant difference in the number of Type 3b follicles before 60 days of age, but the number of all other follicle types was significantly less in hpg mice at all ages studied. Follicles in which the antrum is fully developed (Type 7 and 8) were never seen in the ovaries of mutants and corpora lutea were never observed. Interstitial tissue development was also very poor in hpg ovaries. The hypothalamic GnRH content in normal mice remained low until Day 20, before rising sharply to adult levels (approximately 800 pg) between Days 20 and 30. The pituitary FSH content increased over the first 10 days of life to reach a peak of about 5000 ng, before declining to the adult value of about 2000 ng by Day 30, whilst the plasma FSH concentration was high in the first 10 days, but fell to adult levels over the next 20 days. Pituitary LH content increased significantly between Days 5 and 10 to reach the adult level of about 600 ng. Hypothalamic GnRH was undetectable at all ages in hypogonadal mice, but the pituitary content of FSH and LH had risen to the attenuated mutant adult value by Day 15, and unlike normals, plasma FSH concentrations were not elevated during the neonatal period. These results suggest that minimal gonadotrophic

  8. Multivariate normality

    NASA Technical Reports Server (NTRS)

    Crutcher, H. L.; Falls, L. W.

    1976-01-01

    Sets of experimentally determined or routinely observed data provide information about the past, present and, hopefully, future sets of similarly produced data. An infinite set of statistical models exists which may be used to describe the data sets. The normal distribution is one model. If it serves at all, it serves well. If a data set, or a transformation of the set, representative of a larger population can be described by the normal distribution, then valid statistical inferences can be drawn. There are several tests which may be applied to a data set to determine whether the univariate normal model adequately describes the set. The chi-square test based on Pearson's work in the late nineteenth and early twentieth centuries is often used. Like all tests, it has some weaknesses which are discussed in elementary texts. Extension of the chi-square test to the multivariate normal model is provided. Tables and graphs permit easier application of the test in the higher dimensions. Several examples, using recorded data, illustrate the procedures. Tests of maximum absolute differences, mean sum of squares of residuals, runs and changes of sign are included in these tests. Dimensions one through five with selected sample sizes 11 to 101 are used to illustrate the statistical tests developed.

  9. Adverse event reporting and developments in radiation biology after normal tissue injury: International Atomic Energy Agency consultation

    SciTech Connect

    Chen Yuhchyau . E-mail: Yuhchyau_chen@urmc.rochester.edu; Trotti, Andy; Coleman, C. Norman; Machtay, Mitchell; Mirimanoff, Rene O.; Hay, John; O'Brien, Peter C.; El-Gueddari, Brahim; Salvajoli, Joao V.; Jeremic, Branislav

    2006-04-01

    Purpose: Recent research has enhanced our understanding of radiation injury at the molecular-cellular and tissue levels; significant strides have occurred in standardization of adverse event reporting in clinical trials. In response, the International Atomic Energy Agency, through its Division of Human Health and its section for Applied Radiation Biology and Radiotherapy, organized a consultation meeting in Atlanta (October 2, 2004) to discuss developments in radiobiology, normal tissue reactions, and adverse event reporting. Methods and Materials: Representatives from cooperative groups of African Radiation Oncology Group, Curriculo Radioterapeutica Ibero Latino Americana, European Organization for Research and Treatment of Cancer, National Cancer Institute of Canada Clinical Trials Group, Radiation Therapy Oncology Group, and Trans-Tasman Radiation Oncology Group held the meeting discussion. Results: Representatives of major radiotherapy groups/organizations and prominent leaders in radiotherapy discussed current understanding of normal tissue radiobiologic effects, the design and implementation of future clinical and translational projects for normal tissue injury, and the standardization of adverse-event reporting worldwide. Conclusions: The consensus was to adopt NCI comprehensive adverse event reporting terminology and grading system (CTCAE v3.0) as the new standard for all cooperative group trials. Future plans included the implementation of coordinated research projects focusing on normal tissue biomarkers and data collection methods.

  10. Human neuro-specimen banking 1961-1992. The National Neurological Research Specimen Bank (a donor program of pre- and post-mortem tissues and cerebrospinal fluid/blood; and a collection of cryopreserved human neurological specimens for neuroscientists).

    PubMed

    Tourtellotte, W W; Rosario, I P; Conrad, A; Syndulko, K

    1993-01-01

    The National Neurological Research Specimen Bank (The Bank) was established in 1961 to provide a vital service to neuroscientists. Our purpose is to support medical research which seeks the etiopathogenesis for devastating neurological disorders with no known cause, treatment or precise animal model. It serves as a bridge between the neurology clinician who diagnoses and cares for patients with incurable disease and the basic scientist who has need for specimens to find their etiopathogenesis. In the long run this service should advance neurologic diagnoses and serve as the basis for designing treatment. The Bank has grown to be the largest, most diverse neurological specimen bank of its kind in the world. It is a type of "tissue bank" where donor members "will" their central nervous system tissues to science. Then we collect, photograph, seal in air tight bags, quick freeze, cryogenically store and distribute on request the donated tissue to research scientists around the world. All tissue diagnoses are confirmed by clinical records and neuropathologic examination; further histology is conducted on request. In addition to brain and spinal cord tissues, the Bank has samples of other tissues. There are also samples of pre- and post-mortem CSF and sera from normal individuals and patients with various neurological disorders, especially serial specimens on multiple sclerosis patients and HIV-1 seropositive and at risk individuals. This paper outlines the global operations of our human brain bank, based on protocols developed and used by the authors. These operations include donor solicitation, tissue acquisition and documentation, tissue processing and storage, specimen dissemination to users, outcome assessment of banking, quality control, cost of our operation, table of organization and the future. PMID:8360665

  11. Neurological abnormalities and neurocognitive functions in healthy elder people: A structural equation modeling analysis

    PubMed Central

    2011-01-01

    Background/Aims Neurological abnormalities have been reported in normal aging population. However, most of them were limited to extrapyramidal signs and soft signs such as motor coordination and sensory integration have received much less attention. Very little is known about the relationship between neurological soft signs and neurocognitive function in healthy elder people. The current study aimed to examine the underlying relationships between neurological soft signs and neurocognition in a group of healthy elderly. Methods One hundred and eighty healthy elderly participated in the current study. Neurological soft signs were evaluated with the subscales of Cambridge Neurological Inventory. A set of neurocognitive tests was also administered to all the participants. Structural equation modeling was adopted to examine the underlying relationship between neurological soft signs and neurocognition. Results No significant differences were found between the male and female elder people in neurocognitive function performances and neurological soft signs. The model fitted well in the elderly and indicated the moderate associations between neurological soft signs and neurocognition, specifically verbal memory, visual memory and working memory. Conclusions The neurological soft signs are more or less statistically equivalent to capture the similar information done by conventional neurocognitive function tests in the elderly. The implication of these findings may serve as a potential neurological marker for the early detection of pathological aging diseases or related mental status such as mild cognitive impairment and Alzheimer's disease. PMID:21827719

  12. [Neuropediatrics: epidemiological features and etiologies at the Dakar neurology service].

    PubMed

    Ndiaye, M; Sene-Diouf, F; Diop, A G; Ndao, A K; Ndiaye, M M; Ndiaye, I P

    1999-01-01

    Child neurology is a relatively young speciality of neurosciences which is at the frontier of Neurology and Paediatrics. Its development has been impulsed by the diagnosis techniques such as Neurobiology, Genetics, Neuroimaging and pedo-psychology. We conducted a retrospective survey among the in-patients from January 1980 to December 1997 in the service of Neurology of the University Hospital. Have been included children ranged from 0 to 15 years old without any racial, sexual or origin distinctive. In Neurology Department, children of 0 to 15 years old represent 10.06% of the in-patients received from 1980 to 1997. The mortality rate was 9.23%. The diseases are dominated by epilepsy and infantile encephalopathies with 31.02%, infectious diseases with 19.36% represented by tuberculosis, other bacterial, viral and parasitical etiologies, tumors with 10.36%, vascular pathology and degenerative disorders. PMID:11957278

  13. [Neuropediatrics: epidemiological features and etiologies at the Dakar neurology service].

    PubMed

    Ndiaye, M; Sene-Diouf, F; Diop, A G; Ndao, A K; Ndiaye, M M; Ndiaye, I P

    1999-01-01

    Child neurology is a relatively young speciality of neurosciences which is at the frontier of Neurology and Paediatrics. Its development has been impulsed by the diagnosis techniques such as Neurobiology, Genetics, Neuroimaging and pedo-psychology. We conducted a retrospective survey among the in-patients from January 1980 to December 1997 in the service of Neurology of the University Hospital. Have been included children ranged from 0 to 15 years old without any racial, sexual or origin distinctive. In Neurology Department, children of 0 to 15 years old represent 10.06% of the in-patients received from 1980 to 1997. The mortality rate was 9.23%. The diseases are dominated by epilepsy and infantile encephalopathies with 31.02%, infectious diseases with 19.36% represented by tuberculosis, other bacterial, viral and parasitical etiologies, tumors with 10.36%, vascular pathology and degenerative disorders.

  14. Unusual neurologic manifestations occurring during dengue fever infection.

    PubMed

    Patey, O; Ollivaud, L; Breuil, J; Lafaix, C

    1993-06-01

    This is a report on dengue fever in two young patients in France that were infected in New Caledonia and Thailand. Both presented with unusual neurologic manifestations. The first patient developed a focal subarachnoid hemorrhage that was associated with transient thrombocytopenia. No neurologic vascular malformation was detected; a mild dengue hemorrhagic fever after a previous dengue infection was suspected. The second patient showed peripheral facial palsy one week after apyrexia without any other etiology except the dengue infection. This case was probably a postinfectious syndrome associated with dengue virus. Both patients recovered spontaneously. The circumstances of the neurologic manifestations in these patients may be attributed to the dengue fever virus. However, although neurologic complications reported for dengue fever are unusual, it is reasonable to consider these manifestations as being due to immunopathologic consequences.

  15. Interpersonal relatedness and self-definition in normal and disrupted personality development: retrospect and prospect.

    PubMed

    Luyten, Patrick; Blatt, Sidney J

    2013-04-01

    Two-polarities models of personality propose that personality development evolves through a dialectic synergistic interaction between two fundamental developmental psychological processes across the life span-the development of interpersonal relatedness on the one hand and of self-definition on the other. This article offers a broad review of extant research concerning these models, discusses their implications for psychology and psychiatry, and addresses future research perspectives deriving from these models. We first consider the implications of findings in this area for clinical research and practice. This is followed by a discussion of emerging research findings concerning the role of developmental, cross-cultural, evolutionary, and neurobiological factors influencing the development of these two fundamental personality dimensions. Taken together, this body of research suggests that theoretical formulations that focus on interpersonal relatedness and self-definition as central coordinates in personality development and psychopathology provide a comprehensive conceptual paradigm for future research in psychology and psychiatry exploring the interactions among neurobiological, psychological, and sociocultural factors in adaptive and disrupted personality development across the life span.

  16. p120 Catenin is required for normal tubulogenesis but not epithelial integrity in developing mouse pancreas.

    PubMed

    Hendley, Audrey M; Provost, Elayne; Bailey, Jennifer M; Wang, Yue J; Cleveland, Megan H; Blake, Danielle; Bittman, Ross W; Roeser, Jeffrey C; Maitra, Anirban; Reynolds, Albert B; Leach, Steven D

    2015-03-01

    The intracellular protein p120 catenin aids in maintenance of cell-cell adhesion by regulating E-cadherin stability in epithelial cells. In an effort to understand the biology of p120 catenin in pancreas development, we ablated p120 catenin in mouse pancreatic progenitor cells, which resulted in deletion of p120 catenin in all epithelial lineages of the developing mouse pancreas: islet, acinar, centroacinar, and ductal. Loss of p120 catenin resulted in formation of dilated epithelial tubules, expansion of ductal epithelia, loss of acinar cells, and the induction of pancreatic inflammation. Aberrant branching morphogenesis and tubulogenesis were also observed. Throughout development, the phenotype became more severe, ultimately resulting in an abnormal pancreas comprised primarily of duct-like epithelium expressing early progenitor markers. In pancreatic tissue lacking p120 catenin, overall epithelial architecture remained intact; however, actin cytoskeleton organization was disrupted, an observation associated with increased cytoplasmic PKCζ. Although we observed reduced expression of adherens junction proteins E-cadherin, β-catenin, and α-catenin, p120 catenin family members p0071, ARVCF, and δ-catenin remained present at cell membranes in homozygous p120(f/f) pancreases, potentially providing stability for maintenance of epithelial integrity during development. Adult mice homozygous for deletion of p120 catenin displayed dilated main pancreatic ducts, chronic pancreatitis, acinar to ductal metaplasia (ADM), and mucinous metaplasia that resembles PanIN1a. Taken together, our data demonstrate an essential role for p120 catenin in pancreas development.

  17. Endothelial Jarid2/Jumonji is required for normal cardiac development and proper Notch1 expression.

    PubMed

    Mysliwiec, Matthew R; Bresnick, Emery H; Lee, Youngsook

    2011-05-13

    Jarid2/Jumonji critically regulates developmental processes including cardiovascular development. Jarid2 knock-out mice exhibit cardiac defects including hypertrabeculation with noncompaction of the ventricular wall. However, molecular mechanisms underlying Jarid2-mediated cardiac development remain unknown. To determine the cardiac lineage-specific roles of Jarid2, we generated myocardial, epicardial, cardiac neural crest, or endothelial conditional Jarid2 knock-out mice using Cre-loxP technology. Only mice with an endothelial deletion of Jarid2 recapitulate phenotypic defects observed in whole body mutants including hypertrabeculation and noncompaction of the ventricle. To identify potential targets of Jarid2, combinatorial approaches using microarray and candidate gene analyses were employed on Jarid2 knock-out embryonic hearts. Whole body or endothelial deletion of Jarid2 leads to increased endocardial Notch1 expression in the developing ventricle, resulting in increased Notch1-dependent signaling to the adjacent myocardium. Using quantitative chromatin immunoprecipitation analysis, Jarid2 was found to occupy a specific region on the endogenous Notch1 locus. We propose that failure to properly regulate Notch signaling in Jarid2 mutants likely leads to the defects in the developing ventricular chamber. The identification of Jarid2 as a potential regulator of Notch1 signaling has broad implications for many cellular processes including development, stem cell maintenance, and tumor formation.

  18. Ictal kissing behavior: neurological and psychodynamic overview.

    PubMed

    Taşkıran, Emine; Özmen, Mine; Kılıc, Özge; Sentürk, Aslı; Özkara, Cigdem

    2013-11-01

    This study is based on the psychodynamic and neurological analysis of three Turkish patients who displayed ictal kissing automatism during their seizures. To unveil the probable underlying causes of their kissing behaviors, all patients underwent psychiatric interviews after being evaluated by ictal video-EEG recordings. The group consisted of two females (ages 35 and 29) and one male (age 26). In addition to prominent oral automatisms, each patient also displayed behaviors of kissing or blowing kisses to individuals at close proximity. Seizures were related to the right temporal lobe in two patients and the left temporal lobe in one patient. Magnetic resonance imaging showed mesial temporal sclerosis in two of the patients (one left, one right) and was normal in one. According to the DSM-IV-TR criteria, each of the three patients also suffered from major depression, while the psychodynamic interviews revealed traumatic childhood histories and intense unfulfilled affective needs. PMID:24029004

  19. The diffusion tensor imaging (DTI) component of the NIH MRI study of normal brain development (PedsDTI).

    PubMed

    Walker, Lindsay; Chang, Lin-Ching; Nayak, Amritha; Irfanoglu, M Okan; Botteron, Kelly N; McCracken, James; McKinstry, Robert C; Rivkin, Michael J; Wang, Dah-Jyuu; Rumsey, Judith; Pierpaoli, Carlo

    2016-01-01

    The NIH MRI Study of normal brain development sought to characterize typical brain development in a population of infants, toddlers, children and adolescents/young adults, covering the socio-economic and ethnic diversity of the population of the United States. The study began in 1999 with data collection commencing in 2001 and concluding in 2007. The study was designed with the final goal of providing a controlled-access database; open to qualified researchers and clinicians, which could serve as a powerful tool for elucidating typical brain development and identifying deviations associated with brain-based disorders and diseases, and as a resource for developing computational methods and image processing tools. This paper focuses on the DTI component of the NIH MRI study of normal brain development. In this work, we describe the DTI data acquisition protocols, data processing steps, quality assessment procedures, and data included in the database, along with database access requirements. For more details, visit http://www.pediatricmri.nih.gov. This longitudinal DTI dataset includes raw and processed diffusion data from 498 low resolution (3 mm) DTI datasets from 274 unique subjects, and 193 high resolution (2.5 mm) DTI datasets from 152 unique subjects. Subjects range in age from 10 days (from date of birth) through 22 years. Additionally, a set of age-specific DTI templates are included. This forms one component of the larger NIH MRI study of normal brain development which also includes T1-, T2-, proton density-weighted, and proton magnetic resonance spectroscopy (MRS) imaging data, and demographic, clinical and behavioral data.

  20. Neuromarketing and consumer neuroscience: contributions to neurology

    PubMed Central

    2013-01-01

    Background ‘Neuromarketing’ is a term that has often been used in the media in recent years. These public discussions have generally centered around potential ethical aspects and the public fear of negative consequences for society in general, and consumers in particular. However, positive contributions to the scientific discourse from developing a biological model that tries to explain context-situated human behavior such as consumption have often been neglected. We argue for a differentiated terminology, naming commercial applications of neuroscientific methods ‘neuromarketing’ and scientific ones ‘consumer neuroscience’. While marketing scholars have eagerly integrated neuroscientific evidence into their theoretical framework, neurology has only recently started to draw its attention to the results of consumer neuroscience. Discussion In this paper we address key research topics of consumer neuroscience that we think are of interest for neurologists; namely the reward system, trust and ethical issues. We argue that there are overlapping research topics in neurology and consumer neuroscience where both sides can profit from collaboration. Further, neurologists joining the public discussion of ethical issues surrounding neuromarketing and consumer neuroscience could contribute standards and experience gained in clinical research. Summary We identify the following areas where consumer neuroscience could contribute to the field of neurology: First, studies using game paradigms could help to gain further insights into the underlying pathophysiology of pathological gambling in Parkinson’s disease, frontotemporal dementia, epilepsy, and Huntington’s disease. Second, we identify compulsive buying as a common interest in neurology and consumer neuroscience. Paradigms commonly used in consumer neuroscience could be applied to patients suffering from Parkinson’s disease and frontotemporal dementia to advance knowledge of this important behavioral symptom

  1. "Is My Child Developing Normally?": A Critical Review of Web-Based Resources for Parents

    ERIC Educational Resources Information Center

    Williams, Nia; Mughal, Sabena; Blair, Mitch

    2008-01-01

    Early detection of developmental problems improves outcomes for parents and children. Parents want to be involved in assessment and need high-quality, accurate, and reliable data on child development to help monitor progress and inform decisions on referral. The aim of this paper is to review which websites are readily accessible to parents on…

  2. A nucleostemin-like GTPase required for normal apical and floral meristem development in Arabidopsis

    PubMed Central

    Wang, Xiaomin; Gingrich, Daniel K.; Deng, Yunfei; Hong, Zonglie

    2012-01-01

    Mammalian nucleostemin (NS) is preferentially expressed in stem cells and acts to promote cell cycle progression. In plants, stem cell activities have to be terminated during flower development, and this process requires the activation of AGAMOUS (AG) gene expression. Here, a nucleostemin-like 1 gene, NSN1, is shown to be required for flower development in Arabidopsis. The NSN1 mRNA was found in the inflorescence meristem and floral primordia, and its protein was localized to the nucleoli. Both heterozygous and homozygous plants developed defective flowers on inflorescences that were eventually terminated by the formation of carpelloid flowers. Overexpression of NSN1 resulted in loss of apical dominance and formation of defective flowers. Expression of the AG gene was found to be up-regulated in nsn1. The carpelloid flower defect of nsn1 was suppressed by the ag mutation in the nsn1 ag double mutant, whereas double mutants of nsn1 apetala2 (ap2) displayed enhanced defective floral phenotypes. These results suggest that in the delicately balanced regulatory network, NSN1 acts to repress AG and plays an additive role with AP2 in floral organ specification. As a midsize nucleolar GTPase, NSN1 represents a new class of regulatory proteins required for flower development in Arabidopsis. PMID:22357616

  3. Developing High Quality Decision-Making Discussions about Biological Conservation in a Normal Classroom Setting

    ERIC Educational Resources Information Center

    Grace, Marcus

    2009-01-01

    The conservation of biodiversity is an important socio-scientific issue that is often regarded as a precondition to sustainable development. The foundation for citizens' understanding of conservation issues can be laid down in formal school education. This research focuses on decision-making discussions about biological conservation issues among…

  4. The Interface between Neighborhood Density and Optional Infinitives: Normal Development and Specific Language Impairment

    ERIC Educational Resources Information Center

    Hoover, Jill R.; Storkel, Holly L.; Rice, Mabel L.

    2012-01-01

    The effect of neighborhood density on optional infinitives was evaluated for typically developing (TD) children and children with Specific Language Impairment (SLI). Forty children, twenty in each group, completed two production tasks that assessed third person singular production. Half of the sentences in each task presented a dense verb, and…

  5. A new neurological focus in neonatal intensive care.

    PubMed

    Bonifacio, Sonia L; Glass, Hannah C; Peloquin, Susan; Ferriero, Donna M

    2011-09-01

    Advances in the care of high-risk newborn babies have contributed to reduced mortality rates for premature and term births, but the surviving neonates often have increased neurological morbidity. Therapies aimed at reducing the neurological sequelae of birth asphyxia at term have brought hypothermia treatment into the realm of standard care. However, this therapy does not provide complete protection from neurological complications and a need to develop adjunctive therapies for improved neurological outcomes remains. In addition, the care of neurologically impaired neonates, regardless of their gestational age, clearly requires a focused approach to avoid further injury to the brain and to optimize the neurodevelopmental status of the newborn baby at discharge from hospital. This focused approach includes, but is not limited to, monitoring of the patient's brain with amplitude-integrated and continuous video EEG, prevention of infection, developmentally appropriate care, and family support. Provision of dedicated neurocritical care to newborn babies requires a collaborative effort between neonatologists and neurologists, training in neonatal neurology for nurses and future generations of care providers, and the recognition that common neonatal medical problems and intensive care have an effect on the developing brain. PMID:21808297

  6. Different Neurologic Aspects of Nutritional B12 Deficiency in Infancy.

    PubMed

    Yilmaz, Sanem; Serdaroglu, Gul; Tekgul, Hasan; Gokben, Sarenur

    2016-04-01

    The objective of this study is to evaluate neurologic problems caused by nutritional vitamin B12 deficiency in infancy. Twenty-four cases between 2 and 18 months of age with neurologic symptoms and/or signs and diagnosed as nutritional vitamin B12 deficiency were analyzed. The most common symptoms were developmental retardation, afebrile seizures, and involuntary movements. The mean vitamin B12 levels were lower in patients with both neurologic and extraneurologic involvement when compared to those with only neurologic symptoms. All of the cases were treated with vitamin B12. In patients with severe deficiencies, involuntary movements were observed during vitamin B12 treatment using cyanocobalamin form. At the 1-year follow-up, all but 3 patients were considered neurodevelopmentally normal. The 3 patients that did not fully recover, on admission, had the lowest vitamin B12 levels. It is of great importance to prevent, diagnose, and treat vitamin B12 deficiency promptly to prevent the long-term neurologic problems.

  7. [Neurology].

    PubMed

    Sokolov, Arseny A; Rossetti, Andrea O; Michel, Patrik; Benninger, David; Nater, Bernard; Wider, Christian; Hirt, Lorenz; Kuntzer, Thierry; Démonet, Jean-François; Du Pasquier, Renaud A; Vingerhoets, François

    2016-01-13

    In 2015, cerebral stimulation becomes increasingly established in the treatment of pharmacoresistant epilepsy. Efficacy of endovascular treatment has been demonstrated for acute ischemic stroke. Deep brain stimulation at low frequency improves dysphagia and freezing of gait in Parkinson patients. Bimagrumab seems to increase muscular volume and force in patients with inclusion body myositis. In cluster-type headache, a transcutaneous vagal nerve stimulator is efficient in stopping acute attacks and also reducing their frequency. Initial steps have been undertaken towards modulating memory by stimulation of the proximal fornix. Teriflunomide is the first oral immunomodulatory drug for which efficacy has been shown in preventing conversion from clinical isolated syndrome to multiple sclerosis. PMID:26946707

  8. Cyclin D and cdk4 Are Required for Normal Development beyond the Blastula Stage in Sea Urchin Embryos

    PubMed Central

    Moore, Jennifer C.; Sumerel, Jan L.; Schnackenberg, Bradley J.; Nichols, Jason A.; Wikramanayake, Athula; Wessel, Gary M.; Marzluff, William F.

    2002-01-01

    cdk4 mRNA and protein are constitutively expressed in sea urchin eggs and throughout embryonic development. In contrast, cyclin D mRNA is barely detectable in eggs and early embryos, when the cell cycles consist of alternating S and M phases. Cyclin D mRNA increases dramatically in embryos at the early blastula stage and remains at a constant level throughout embryogenesis. An increase in cdk4 kinase activity occurs concomitantly with the increase in cyclin D mRNA. Ectopic expression of cyclin D mRNA in eggs arrests development before the 16-cell stage and causes eventual embryonic death, suggesting that activation of cyclin D/cdk4 in cleavage cell cycles is lethal to the embryo. In contrast, blocking cyclin D or cdk4 expression with morpholino antisense oligonucleotides results in normal development of early gastrula-stage embryos but abnormal, asymmetric larvae. These results suggest that in sea urchins, cyclin D and cdk4 are required for normal development and perhaps the patterning of the developing embryo, but may not be directly involved in regulating entry into the cell cycle. PMID:12052892

  9. The Interface between Neighborhood Density & Optional Infinitives: Normal Development and Specific Language Impairment

    PubMed Central

    Hoover, Jill R.; Storkel, Holly L.; Rice, Mabel L.

    2011-01-01

    The effect of neighborhood density on optional infinitives was evaluated for typically developing (TD) children and children with Specific Language Impairment (SLI). Forty children, 20 in each group, completed two production tasks that assessed third person singular production. Half of the sentences in each task presented a dense verb, and half presented a sparse verb. Children’s third person singular accuracy was compared across dense and sparse verbs. Results showed that the TD group was significantly less likely to use optional infinitives with dense, rather than sparse verbs. In contrast, the distribution of optional infinitives for the SLI group was independent of verb neighborhood density. Follow-up analyses showed that the lack of neighborhood density effect for the SLI group could not be attributed to heterogeneous neighborhood density effects or floor effects. Results were interpreted within the Optional Infinitive/Extended Optional Infinitive accounts for typical language development and SLI for English speaking children. PMID:22123500

  10. Cyclin dependent kinase 5 is required for the normal development of oligodendrocytes and myelin formation.

    PubMed

    Yang, Yan; Wang, Haibo; Zhang, Jie; Luo, Fucheng; Herrup, Karl; Bibb, James A; Lu, Richard; Miller, Robert H

    2013-06-15

    The development of oligodendrocytes, the myelinating cells of the vertebrate CNS, is regulated by a cohort of growth factors and transcription factors. Less is known about the signaling pathways that integrate extracellular signals with intracellular transcriptional regulators to control oligodendrocyte development. Cyclin dependent kinase 5 (Cdk5) and its co-activators play critical roles in the regulation of neuronal differentiation, cortical lamination, neuronal cell migration and axon outgrowth. Here we demonstrate a previously unrecognized function of Cdk5 in regulating oligodendrocyte maturation and myelination. During late embryonic development Cdk5 null animals displayed a reduction in the number of MBP+ cells in the spinal cord, but no difference in the number of OPCs. To determine whether the reduction of oligodendrocytes reflected a cell-intrinsic loss of Cdk5, it was selectively deleted from Olig1+ oligodendrocyte lineage cells. In Olig1(Cre/+); Cdk5(fl/fl) conditional mutants, reduced levels of expression of MBP and PLP mRNA were observed throughout the CNS and ultrastructural analyses demonstrated a significant reduction in the proportion of myelinated axons in the optic nerve and spinal cord. Pharmacological inhibition or RNAi knockdown of Cdk5 in vitro resulted in the reduction in oligodendrocyte maturation, but had no effect on OPC cell proliferation. Conversely, over-expression of Cdk5 promoted oligodendrocyte maturation and enhanced process outgrowth. Consistent with this data, Cdk5(-/-) oligodendrocytes developed significantly fewer primary processes and branches than control cells. Together, these findings suggest that Cdk5 function as a signaling integrator to regulate oligodendrocyte maturation and myelination.

  11. Social networks and neurological illness.

    PubMed

    Dhand, Amar; Luke, Douglas A; Lang, Catherine E; Lee, Jin-Moo

    2016-10-01

    Every patient is embedded in a social network of interpersonal connections that influence health outcomes. Neurologists routinely need to engage with a patient's family and friends due to the nature of the illness and its social sequelae. Social isolation is a potent determinant of poor health and neurobiological changes, and its effects can be comparable to those of traditional risk factors. It would seem reasonable, therefore, to map and follow the personal networks of neurology patients. This approach reveals influential people, their habits, and linkage patterns that could facilitate or limit health behaviours. Personal network information can be particularly valuable to enhance risk factor management, medication adherence, and functional recovery. Here, we propose an agenda for research and clinical practice that includes mapping the networks of patients with diverse neurological disorders, evaluating the impact of the networks on patient outcomes, and testing network interventions. PMID:27615420

  12. Botulinum Toxin in Pediatric Neurology

    PubMed Central

    Abdallah, Enas Abdallah Ali

    2015-01-01

    Botulinum neurotoxins are natural molecules produced by anaerobic spore-forming bacteria called Clostradium boltulinum. The toxin has a peculiar mechanism of action by preventing the release of acetylcholine from the presynaptic membrane. Consequently, it has been used in the treatment of various neurological conditions related to muscle hyperactivity and/or spasticity. Also, it has an impact on the autonomic nervous system by acting on smooth muscle, leading to its use in the management of pain syndromes. The use of botulinum toxin in children separate from adults has received very little attention in the literature. This review presents the current data on the use of botulinum neurotoxin to treat various neurological disorders in children. PMID:27335961

  13. Clinical neurology and executive dysfunction.

    PubMed

    Filley, C M

    2000-01-01

    Executive function is a uniquely human ability that permits an individual to plan, carry out, and monitor a sequence of actions that is intended to accomplish a goal. This crucial neurobehavioral capacity depends on the integrity of the frontal lobes, most importantly the dorsolateral prefrontal cortices and their connections. Executive dysfunction is associated with a wide range of neurologic disorders that affect these regions. In this paper, executive dysfunction is considered from the perspective of behavioral neurology, and the lesion method is employed to illustrate this impairment in a diverse group of disorders. Frontal system damage leading to disturbed executive function is common and clinically significant. Recognition of this syndrome is critical for ensuring the correct diagnosis, accurate prognosis, and appropriate treatment of affected patients. Executive dysfunction also represents an intriguing aspect of brain-behavior relationships and offers important insights into one of the highest cerebral functions. PMID:10879543

  14. Reaction time of patients with Parkinson's disease, with reference to asymmetry of neurological signs.

    PubMed Central

    Yokochi, F; Nakamura, R; Narabayashi, H

    1985-01-01

    Electromyographic reaction times of the left and the right finger extensor muscles in extension movement of the wrist were examined in 42 patients with Parkinson's disease, and 20 normal subjects. Compared to the normal subjects and the patients with neurological signs confined to the right side, the patients with neurological signs on the left side or on both sides showed slowing of reaction times regardless of the side of responding hand. The patients with asymmetry of bilateral neurological signs showed slower RTs on the more affected side. PMID:4031916

  15. Neurologic injuries in baseball players.

    PubMed

    Treihaft, M M

    2000-01-01

    In baseball pitchers, injuries to the throwing arm are common due to the extreme stresses placed on the elbow and shoulder joints. These result in peripheral nerve syndromes including ulnar neuropathy at the elbow and suprascapular neuropathy at the shoulder. Recurrent trauma to the axillary artery causing aneurysm and thrombus formation may lead to distal ischemia and stroke. Careful evaluation is required to identify musculoskeletal, neurologic, and vascular causes of upper extremity symptoms in the throwing athlete.

  16. Early school outcomes for extremely preterm infants with transient neurological abnormalities

    PubMed Central

    Harmon, Heidi; Taylor, H Gerry; Minich, Nori; Wilson-Costello, Deanne; Hack, Maureen

    2015-01-01

    AIM To determine if transient neurological abnormalities (TNA) at 9 months corrected age predict cognitive, behavioral, and motor outcomes at 6 years of age in extremely preterm infants. METHOD A cohort of 124 extremely preterm infants (mean gestational age 25.5wk; 55 males, 69 females), admitted to our unit between 2001 and 2003, were classified based on the Amiel-Tison Neurological Assessment at 9 months and 20 months corrected age as having TNA (n=17), normal neurological assessment (n=89), or neurologically abnormal assessment (n=18). The children were assessed at a mean age of 5 years 11 months (SD 4mo) on cognition, academic achievement, motor ability, and behavior. RESULTS Compared with children with a normal neurological assessment, children with TNA had higher postnatal exposure to steroids (35% vs 9%) and lower adjusted mean scores on spatial relations (84 [standard error {SE} 5] vs 98 [SE 2]), visual matching (79 [SE 5] vs 91 [SE 2]), letter–word identification (97 [SE 4] vs 108 [SE 1]), and spelling (76 [SE 4] vs 96 [SE 2]) (all p<0.05). INTERPRETATION Despite a normalized neurological assessment, extremely preterm children with a history TNA are at higher risk for lower cognitive and academic skills than those with normal neurological findings during their first year of school. PMID:26014665

  17. T Cells Development Is Different between Thymus from Normal and Intrauterine Growth Restricted Pig Fetus at Different Gestational Stage.

    PubMed

    Lin, Yan; Wang, Junjun; Wang, Xiaoqiu; Wu, Weizong; Lai, Changhua

    2013-03-01

    This experiment was conducted to evaluate the development of T cells in intrauterine growth retarded (IUGR) piglets at different gestational stages, and tentatively explore the relationship between T cells development and the Notch signaling pathway. A total of 18 crossbred (Landrace×Large white) primiparous sows were mated at similar weights and estruses and euthanized at d 60, 90 and 110 of gestation with six replicates for each time point. One IUGR and one normal fetus were picked from each litter. The T-cell subsets, mRNA expression of Delta-like1, Delta-like4, Jagged1, and Notch2 genes in the thymus were investigated. Compared to normal piglets, CD3(+)CD4(-)CD8(+) cells in IUGR fetuses at d 90 was 0.13% lower (p<0.05). At d 110 of gestation CD8(+) T cells in IUGR fetuses was 0.19% lower (p<0.05). The percentage of CD8(+) T cells was 3.14% lower (p<0.05) of the total T cells in IUGR pigs at d 60. The abundance of Notch2 and Delta-like4 mRNA at d 110 was 20.93% higher and 0.77% (p<0.05) lower, and Delta-like1 mRNA at d 90 was 0.19% (p<0.05) higher compared to normal pigs. These results suggested that normal fetuses had a greater proportion of T-cell subsets at earlier gestation periods, and the Notch signaling pathway was likely partially responsible for these differences to some degree.

  18. Genomic medicine and neurological disease

    PubMed Central

    Boone, Philip M.; Wiszniewski, Wojciech; Lupski, James R.

    2011-01-01

    “Genomic medicine” refers to the diagnosis, optimized management, and treatment of disease—as well as screening, counseling, and disease gene identification—in the context of information provided by an individual patient’s personal genome. Genomic medicine, to some extent synonymous with “personalized medicine,” has been made possible by recent advances in genome technologies. Genomic medicine represents a new approach to health care and disease management that attempts to optimize the care of a patient based upon information gleaned from his or her personal genome sequence. In this review, we describe recent progress in genomic medicine as it relates to neurological disease. Many neurological disorders either segregate as Mendelian phenotypes or occur sporadically in association with a new mutation in a single gene. Heritability also contributes to other neurological conditions that appear to exhibit more complex genetics. In addition to discussing current knowledge in this field, we offer suggestions for maximizing the utility of genomic information in clinical practice as the field of genomic medicine unfolds. PMID:21594611

  19. Neurological prognostication after cardiac arrest

    PubMed Central

    Sandroni, Claudio; Geocadin, Romergryko G.

    2016-01-01

    Purpose of review Prediction of neurological prognosis in patients who are comatose after successful resuscitation from cardiac arrest remains difficult. Previous guidelines recommended ocular reflexes, somatosensory evoked potentials and serum biomarkers for predicting poor outcome within 72h from cardiac arrest. However, these guidelines were based on patients not treated with targeted temperature management and did not appropriately address important biases in literature. Recent findings Recent evidence reviews detected important limitations in prognostication studies, such as low precision and, most importantly, lack of blinding, which may have caused a self-fulfilling prophecy and overestimated the specificity of index tests. Maintenance of targeted temperature using sedatives and muscle relaxants may interfere with clinical examination, making assessment of neurological status before 72 h or more after cardiac arrest unreliable. Summary No index predicts poor neurological outcome after cardiac arrest with absolute certainty. Prognostic evaluation should start not earlier than 72 h after ROSC and only after major confounders have been excluded so that reliable clinical examination can be made. Multimodality appears to be the most reasonable approach for prognostication after cardiac arrest. PMID:25922894

  20. Normal development following chromatin transfer correlates with donor cell initial epigenetic state.

    PubMed

    McLean, Cameron A; Wang, Zhongde; Babu, Kavitha; Edwards, Angie; Kasinathan, Poothappillai; Robl, James; Sheppard, Allan M

    2010-04-01

    If the full potential of chromatin transfer (CT) technology is to be realized for both animal production and biomedical applications it is imperative that the efficiency of the reprogramming process be improved, and the potential for deleterious development be eliminated. Generation of the first cloned animals from adult somatic cells demonstrated that development is substantially an epigenetic process (Wilmut I, Schnieke AE, McWhir J, Kind AJ, Campbell KH, 1997. Viable offspring derived from fetal and adult mammalian cells. Nature. 385(6619): 810-813.). In this study, we provide preliminary evidence that the epigenetic state of the donor cell, may be valuable in assessing potential cloning success. We have measured key indicators of cellular epigenetic state in both serially derived cell populations of the same genetic origin, but differing in epigenomic status, and in a distinct cohort of donor cell populations with diverse genetic origins and epigenomic status. Specifically, the relative abundance of particular histone modifications in donor populations prior to manipulation has been correlated with the measurable variance in reprogramming efficiencies observed following CT, as defined by the number of resulting live births and healthy progeny, and the concomitant incidence of deleterious growth measures (notably the appearance of large offspring syndrome (LOS)). Thus, we suggest that the likely outcome and relative success of cloning may be predictable based on the expression of discriminating histone marks present in the donor cell population before CT. This approach may provide the basis of a prognostic signature for the future evaluation and risk assessment of putative donor cells prior to CT, and thus increase future cloning success and alleviate the incidence of abnormal development.

  1. Ubiquitin Specific Protease 21 Is Dispensable for Normal Development, Hematopoiesis and Lymphocyte Differentiation

    PubMed Central

    Pannu, Jaspreet; Belle, Jad I.; Förster, Michael; Duerr, Claudia U.; Shen, Shiyang; Kane, Leanne; Harcourt, Katherine; Fritz, Jörg H.; Clare, Simon; Nijnik, Anastasia

    2015-01-01

    USP21 is a ubiquitin specific protease that catalyzes protein deubiquitination, however the identification of its physiological substrates remains challenging. USP21 is known to deubiquitinate transcription factor GATA3 and death-domain kinase RIPK1 in vitro, however the in vivo settings where this regulation plays a biologically significant role remain unknown. In order to determine whether USP21 is an essential and non-redundant regulator of GATA3 or RIPK1 activity in vivo, we characterized Usp21-deficient mice, focusing on mouse viability and development, hematopoietic stem cell function, and lymphocyte differentiation. The Usp21-knockout mice were found to be viable and fertile, with no significant dysmorphology, in contrast to the GATA3 and RIPK1 knockout lines that exhibit embryonic or perinatal lethality. Loss of USP21 also had no effect on hematopoietic stem cell function, lymphocyte development, or the responses of antigen presenting cells to TLR and TNFR stimulation. GATA3 levels in hematopoietic stem cells or T lymphocytes remained unchanged. We observed that aged Usp21-knockout mice exhibited spontaneous T cell activation, however this was not linked to altered GATA3 levels in the affected cells. The contrast in the phenotype of the Usp21-knockout line with the previously characterized GATA3 and RIPK1 knockout mice strongly indicates that USP21 is redundant for the regulation of GATA3 and RIPK1 activity during mouse development, in hematopoietic stem cells, and in lymphocyte differentiation. The Usp21-deficient mouse line characterized in this study may serve as a useful tool for the future characterization of USP21 physiological functions. PMID:25680095

  2. Morphogenesis of the inner ear at different stages of normal human development.

    PubMed

    Toyoda, Saki; Shiraki, Naoto; Yamada, Shigehito; Uwabe, Chigako; Imai, Hirohiko; Matsuda, Tetsuya; Yoneyama, Akio; Takeda, Tohoru; Takakuwa, Tetsuya

    2015-12-01

    This study examined the external morphology and morphometry of the human embryonic inner ear membranous labyrinth and documented its three-dimensional position in the developing embryo using phase-contrast X-ray computed tomography and magnetic resonance imaging. A total of 27 samples between Carnegie stage (CS) 17 and the postembryonic phase during trimester 1 (approximately 6-10 weeks after fertilization) were included. The otic vesicle elongated along the dorso-ventral axis and differentiated into the end lymphatic appendage and cochlear duct (CD) at CS 17. The spiral course of the CD began at CS18, with anterior and posterior semicircular ducts (SDs) forming prominent circles with a common crus. The spiral course of the CD comprised more than two turns at the postembryonic phase, at which time the height of the CD was evident. A linear increase was observed in the length of anterior, posterior, and lateral SDs, in that order, and the length of the CD increased exponentially over the course of development. Bending in the medial direction was observed between the cochlear and vestibular parts from the latero-caudal view, with the angle decreasing during development. The position of the inner ear was stable throughout the period of observation on the lateral to ventral side of the rhombencephalon, caudal to the pontine flexure, and adjacent to the auditory ganglia. The plane of the lateral semicircular canal was approximately 8.0°-14.6° with respect to the cranial caudal (z-)axis, indicating that the orientation of the inner ear changes during growth to adulthood.

  3. Genome-wide association studies in neurology

    PubMed Central

    Tan, Meng-Shan; Jiang, Teng

    2014-01-01

    Genome-wide association studies (GWAS) are a powerful tool for understanding the genetic underpinnings of human disease. In this article, we briefly review the role and findings of GWAS in common neurological diseases, including Stroke, Alzheimer’s disease, Parkinson’s disease, epilepsy, multiple sclerosis, migraine, amyotrophic lateral sclerosis, frontotemporal lobar degeneration, restless legs syndrome, intracranial aneurysm, human prion diseases and moyamoya disease. We then discuss the present and future implications of these findings with regards to disease prediction, uncovering basic biology, and the development of potential therapeutic agents. PMID:25568877

  4. Neurologic Parasitic Infections in Immigrants and Travelers

    PubMed Central

    Thakur, Kiran; Zunt, Joseph

    2012-01-01

    Infectious diseases are increasingly common in modern clinical practice and the contemporary neurologist must be aware of the clinical manifestations, potential complications, and management of common travel-related infections. The authors provide an approach to patients who present with neurologic symptoms, with a history of travel to or residence in tropical and developing countries. Although many other infections are important in this demographic, they focus on three parasitic infections that neurologists may encounter: cerebral malaria, neuroschistosomiasis, and neurocysticercosis. The epidemiology, pathophysiology, clinical manifestations, diagnostic evaluation, and treatment are discussed for each infection. PMID:21964842

  5. [Specific features of neurological complications developing in patients with type 2 diabetes mellitus and metabolic syndrome: possibility for correction and prevention].

    PubMed

    Shishkova, V N

    2015-01-01

    The prevalence of type 2 diabetes mellitus (DM) and preceding metabolic disturbances has reached epidemic proportions. Oxidative stress plays a significant role in the development of micro- and macrovascular complications in patients with DM. The accumulation of free radicals is responsible for the development of systemic and vascular inflammation, endothelial dysfunction, and hypercoagulable and ischemic states. Since vascular and nervous system damages do not level off even under adequate glycemic control, there is a need for complex pathogenetic treatment strategies. Antioxidant therapy using mexidol is one of the compulsory components of combination therapy for complications of DM.

  6. [The mirror stage ("Stade du Miroir" by J. Lacan) as a crucial stage of development for finding one's identity - normal, neurotic, psychotic development].

    PubMed

    Gruber, J

    1979-01-01

    The socalled "Mirror Stage" ("Stade du Miroir" by J. Lacan) seems to be a normal stage of development between the sixth and the eighteenth month of age. In the centre of this study you find terms of developmental psychology and especially of psychoanalysis like: 'mother-child relationship', 'first relationship to objects', 'passage from primary to secondary narcism', 'ego-ideal' and 'ideal of the ego' and others. The Mirror Stage seems to be especially important for the formation of the identity: wether the child becomes 'normal', 'neurotic' or 'psychotic'. Here we also find terms, invented by Lacan like the 'Real', the 'Imaginary' and the 'Symbolic'.

  7. Animal Models to Study Placental Development and Function throughout Normal and Dysfunctional Human Pregnancy.

    PubMed

    Grigsby, Peta L

    2016-01-01

    Abnormalities of placental development and function are known to underlie many pathologies of pregnancy, including spontaneous preterm birth, fetal growth restriction, and preeclampsia. A growing body of evidence also underscores the importance of placental dysfunction in the lifelong health of both mother and offspring. However, our knowledge regarding placental structure and function throughout pregnancy remains limited. Understanding the temporal growth and functionality of the human placenta throughout the entirety of gestation is important if we are to gain a better understanding of placental dysfunction. The utilization of new technologies and imaging techniques that could enable safe monitoring of placental growth and function in vivo has become a major focus area for the National Institutes of Child Health and Human Development, as evident by the establishment of the "Human Placenta Project." Many of the objectives of the Human Placenta Project will necessitate preclinical studies and testing in appropriately designed animal models that can be readily translated to the clinical setting. This review will describe the advantages and limitations of relevant animals such as the guinea pig, sheep, and nonhuman primate models that have been used to study the role of the placenta in fetal growth disorders, preeclampsia, or other maternal diseases during pregnancy.

  8. Animal Models to Study Placental Development and Function throughout Normal and Dysfunctional Human Pregnancy

    PubMed Central

    Grigsby, Peta L.

    2016-01-01

    Abnormalities of placental development and function are known to underlie many pathologies of pregnancy, including spontaneous preterm birth, fetal growth restriction and preeclampsia. A growing body of evidence also underscores the importance of placental dysfunction in the lifelong health of both mother and offspring. However, our knowledge regarding placental structure and function throughout pregnancy remains limited. Understanding the temporal growth and functionality of the human placenta throughout the entirety of gestation is important if we are to gain a better understanding of placental dysfunction. The utilization of new technologies and imaging techniques that could enable safe monitoring of placental growth and function in vivo has become a major focus area for the National Institutes of Child Health & Human Development, as evident by the establishment of the “Human Placenta Project”. Many of the objectives of the Human Placenta Project will necessitate pre-clinical studies and testing in appropriately designed animal models that can be readily translated to the clinical setting. This review will describe the advantages and limitations of relevant animals such as the guinea pig, sheep and non-human primate models that have been used to study the role of the placenta in fetal growth disorders, preeclampsia or other maternal diseases during pregnancy. PMID:26752715

  9. Cellular Distribution of NDRG1 Protein in the Rat Kidney and Brain During Normal Postnatal Development

    PubMed Central

    Wakisaka, Yoshinobu; Furuta, Akiko; Masuda, Katsuaki; Morikawa, Wataru; Kuwano, Michihiko; Iwaki, Toru

    2003-01-01

    N-myc downregulated gene 1 (NDRG1) is a 43-kD protein whose mRNA is induced by DNA damage, hypoxia, or prolonged elevation of intracellular calcium. Although NDRG1 is also upregulated during cell differentiation, there are few studies on NDRG1 expression during postnatal development. Here we investigated the expression and cellular distribution of NDRG1 protein in rat kidney and brain during postnatal development. Immunohistochemical analysis revealed that the cellular localization of NDRG1 protein in the kidney changed from the proximal convoluted tubules to the collecting ducts between postnatal days 10 and 20. In the brain, a change in cellular expression was also found from the hippocampal pyramidal neurons to the astrocytes in the gray matter during the same postnatal period. These alterations in the cellular distribution of NDRG1 were associated with shifts in the molecular assembly on Western blots. Under non-reduced conditions, the main NDRG1 band was found only around 215 kD in both kidney and brain during the early postnatal stage. After postnatal day 10, the immunoreactive bands shifted to 43 kD in the kidney and 129 kD in the brain. These changes in the cellular distribution and state of assembly may correlate with the functional maturation of both organs. PMID:14566023

  10. Ischemia may be the primary cause of the neurologic deficits in classic migraine

    SciTech Connect

    Skyhoj Olsen, T.; Friberg, L.; Lassen, N.A.

    1987-02-01

    This study investigates whether the cerebral blood flow reduction occurring in attacks of classic migraine is sufficient to cause neurologic deficits. Regional cerebral blood flow measured with the xenon 133 intracarotid injection technique was analyzed in 11 patients in whom a low-flow area developed during attacks of classic migraine. When measured with this technique, regional cerebral blood flow in focal low-flow areas will be overestimated because of the effect of scattered radiation (Compton scatter) on the recordings. In this study, this effect was particularly taken into account when evaluating the degree of blood flow reduction. During attacks of classic migraine, cerebral blood flow reductions averaging 52% were observed focally in the 11 patients. Cerebral blood flow levels known to be insufficient for normal cortical function (less than 16 to 23 mL/100 g/min) were measured in seven patients during the attacks. This was probably also the case in the remaining four patients, but the effect of scattered radiation made a reliable evaluation of blood flow impossible. It is concluded that the blood flow reduction that occurs during attacks of classic migraine is sufficient to cause ischemia and neurologic deficits. Hence, this study suggests a vascular origin of the prodromal neurologic deficits that may accompany attacks of classic migraine.

  11. Mammary Gland Specific Knockdown of the Physiological Surge in Cx26 during Lactation Retains Normal Mammary Gland Development and Function

    PubMed Central

    Stewart, Michael K. G.; Plante, Isabelle; Bechberger, John F.; Naus, Christian C.; Laird, Dale W.

    2014-01-01

    Connexin26 (Cx26) is the major Cx protein expressed in the human mammary gland and is up-regulated during pregnancy while remaining elevated throughout lactation. It is currently unknown if patients with loss-of-function Cx26 mutations that result in hearing loss and skin diseases have a greater susceptibility to impaired breast development. To investigate if Cx26 plays a critical role in mammary gland development and differentiation, a novel Cx26 conditional knockout mouse model was generated by crossing Cx26fl/fl mice with mice expressing Cre under the β-Lactoglobulin promoter. Conditional knockdown of Cx26 from the mammary gland resulted in a dramatic reduction in detectable gap junction plaques confirmed by a significant ∼65-70% reduction in Cx26 mRNA and protein throughout parturition and lactation. Interestingly, this reduction was accompanied by a decrease in mammary gland Cx30 gap junction plaques at parturition, while no change was observed for Cx32 or Cx43. Whole mount, histological and immunofluorescent assessment of breast tissue revealed comparatively normal lobuloalveolar development following pregnancy in the conditionally knockdown mice compared to control mice. In addition, glands from genetically-modified mice were capable of producing milk proteins that were evident in the lumen of alveoli and ducts at similar levels as controls, suggesting normal gland function. Together, our results suggest that low levels of Cx26 expression throughout pregnancy and lactation, and not the physiological surge in Cx26, is sufficient for normal gland development and function. PMID:24988191

  12. Huntingtin Is Required for Normal Excitatory Synapse Development in Cortical and Striatal Circuits

    PubMed Central

    McKinstry, Spencer U.; Karadeniz, Yonca B.; Worthington, Atesh K.; Hayrapetyan, Volodya Y.; Ozlu, M. Ilcim; Serafin-Molina, Karol; Risher, W. Christopher; Ustunkaya, Tuna; Dragatsis, Ioannis; Zeitlin, Scott; Yin, Henry H.

    2014-01-01

    Huntington's disease (HD) is a neurodegenerative disease caused by the expansion of a poly-glutamine (poly-Q) stretch in the huntingtin (Htt) protein. Gain-of-function effects of mutant Htt have been extensively investigated as the major driver of neurodegeneration in HD. However, loss-of-function effects of poly-Q mutations recently emerged as potential drivers of disease pathophysiology. Early synaptic problems in the excitatory cortical and striatal connections have been reported in HD, but the role of Htt protein in synaptic connectivity was unknown. Therefore, we investigated the role of Htt in synaptic connectivity in vivo by conditionally silencing Htt in the developing mouse cortex. When cortical Htt function was silenced, cortical and striatal excitatory synapses formed and matured at an accelerated pace through postnatal day 21 (P21). This exuberant synaptic connectivity was lost over time in the cortex, resulting in the deterioration of synapses by 5 weeks. Synaptic decline in the cortex was accompanied with layer- and region-specific reactive gliosis without cell loss. To determine whether the disease-causing poly-Q mutation in Htt affects synapse development, we next investigated the synaptic connectivity in a full-length knock-in mouse model of HD, the zQ175 mouse. Similar to the cortical conditional knock-outs, we found excessive excitatory synapse formation and maturation in the cortices of P21 zQ175, which was lost by 5 weeks. Together, our findings reveal that cortical Htt is required for the correct establishment of cortical and striatal excitatory circuits, and this function of Htt is lost when the mutant Htt is present. PMID:25009276

  13. Loss of all 3 Extended Synaptotagmins does not affect normal mouse development, viability or fertility.

    PubMed

    Tremblay, Michel G; Moss, Tom

    2016-09-01

    The extended synaptotagmins, E-Syt1, 2 and 3, are multiple C2 domain membrane proteins that are tethered to the endoplasmic reticulum and interact in a calcium dependent manner with plasma membrane phospholipids to form endoplasmic reticulum - plasma membrane junctions. These junctions have been implicated in the exchange of phospholipids between the 2 organelles. The E-Syts have further been implicated in receptor signaling and endocytosis and can interact directly with fibroblast growth factor and other cell surface receptors. Despite these multiple functions, the search for a requirement in vivo has been elusive. Most recently, we found that the genes for E-Syt2 and 3 could be inactivated without effect on mouse development, viability, fertility or morphology. We have now created insertion and deletion mutations in the last of the mouse E-Syt genes. We show that E-Syt1 is specifically expressed throughout the embryonic skeleton during the early stages of chrondrogenesis in a pattern quite distinct from that of E-Syt2 or 3. Despite this, E-Syt1 is also not required for mouse development and propagation. We further show that even the combined inactivation of all 3 E-Syt genes has no effect on mouse viability or fertility in the laboratory. However, this inactivation induces an enhancement in the expression of the genes encoding Orp5/8, Orai1, STIM1 and TMEM110, endoplasmic reticulum - plasma membrane junction proteins that potentially could compensate for E-Syt loss. Given the multiple functions suggested for the E-Syts and their evolutionary conservation, our unexpected findings suggest that they may only provide a survival advantage under specific conditions that have as yet to be identified.

  14. Loss of all 3 Extended Synaptotagmins does not affect normal mouse development, viability or fertility

    PubMed Central

    Tremblay, Michel G.; Moss, Tom

    2016-01-01

    ABSTRACT The extended synaptotagmins, E-Syt1, 2 and 3, are multiple C2 domain membrane proteins that are tethered to the endoplasmic reticulum and interact in a calcium dependent manner with plasma membrane phospholipids to form endoplasmic reticulum - plasma membrane junctions. These junctions have been implicated in the exchange of phospholipids between the 2 organelles. The E-Syts have further been implicated in receptor signaling and endocytosis and can interact directly with fibroblast growth factor and other cell surface receptors. Despite these multiple functions, the search for a requirement in vivo has been elusive. Most recently, we found that the genes for E-Syt2 and 3 could be inactivated without effect on mouse development, viability, fertility or morphology. We have now created insertion and deletion mutations in the last of the mouse E-Syt genes. We show that E-Syt1 is specifically expressed throughout the embryonic skeleton during the early stages of chrondrogenesis in a pattern quite distinct from that of E-Syt2 or 3. Despite this, E-Syt1 is also not required for mouse development and propagation. We further show that even the combined inactivation of all 3 E-Syt genes has no effect on mouse viability or fertility in the laboratory. However, this inactivation induces an enhancement in the expression of the genes encoding Orp5/8, Orai1, STIM1 and TMEM110, endoplasmic reticulum - plasma membrane junction proteins that potentially could compensate for E-Syt loss. Given the multiple functions suggested for the E-Syts and their evolutionary conservation, our unexpected findings suggest that they may only provide a survival advantage under specific conditions that have as yet to be identified. PMID:27399837

  15. Brain MRI Findings in Neurological Complications of Cancer Treatment.

    PubMed

    Cabaj, Astra; Bekiesińska-Figatowska, Monika; Duczkowska, Agnieszka; Duczkowski, Marek

    2016-01-01

    The amount of people living with cancer is increasing; they live longer and have thus a higher risk of developing neurological complications. Magnetic resonance as a diagnostic procedure of choice in detecting the reasons of neurological/psychiatric symptoms in oncological patients is nowadays relatively easily accessible. Early diagnosis established by radiologists familiar with neurological entities that may follow cancer treatment allow clinicians to provide proper treatment, even if the diagnosis seems unbelievable. The review of MR images of acute and chronic neurological complications of cancer treatment from the authors' own archive is the focus of this report. Neurological complications of cancer can be metastatic and non-metastatic; the first cannot be considered as a treatment complication, the latter can be chemoor radiotherapy-induced, acute, chronic and delayed. In our material we dealt with complications with dramatic course (stroke, PRES, acute leukoencephalopathy, Wernicke's encephalopathy) and with cases with milder and/or longer course (neuro-infections, chronic leukoencephalopathy, telangiectasias and/or cavernous hemangiomas, second tumors: glioma and meningioma after irradiation). The central nervous system is very susceptible to complications of systemic cancer and its treatment. Even though the first thought of clinicians and radiologists after a patient's first neurological/psychiatric symptoms appears concerns the metastatic spread of the disease, they need to have an understanding that there are a number of other causes of such symptoms. The knowledge of entities which can be expected and diagnostic experience prevent clinicians from making wrong diagnosis. PMID:27629856

  16. Backdoor pathway for dihydrotestosterone biosynthesis: implications for normal and abnormal human sex development.

    PubMed

    Fukami, Maki; Homma, Keiko; Hasegawa, Tomonobu; Ogata, Tsutomu

    2013-04-01

    We review the current knowledge about the "backdoor" pathway for the biosynthesis of dihydrotestosterone (DHT). While DHT is produced from cholesterol through the conventional "frontdoor" pathway via testosterone, recent studies have provided compelling evidence for the presence of an alternative "backdoor" pathway to DHT without testosterone intermediacy. This backdoor pathway is known to exist in the tammar wallaby pouch young testis and the immature mouse testis, and has been suggested to be present in the human as well. Indeed, molecular analysis has identified pathologic mutations of genes involved in the backdoor pathway in genetic male patients with undermasculinized external genitalia, and urine steroid profile analysis has argued for the relevance of the activated backdoor pathway to abnormal virilization in genetic females with cytochrome P450 oxidoreductase deficiency and 21-hydroxylase deficiency. It is likely that the backdoor pathway is primarily operating in the fetal testis in a physiological condition to produce a sufficient amount of DHT for male sex development, and that the backdoor pathway is driven with a possible interaction between fetal and permanent adrenals in pathologic conditions with increased 17-hydroxyprogesterone levels. These findings provide novel insights into androgen biosynthesis in both physiological and pathological conditions. PMID:23073980

  17. Qilin Is Essential for Cilia Assembly and Normal Kidney Development in Zebrafish

    PubMed Central

    Li, Jade; Sun, Zhaoxia

    2011-01-01

    Defects in the cilium, a once thought vestigial organelle, have recently been implicated in many human diseases, including a number of cystic kidney diseases such as polycystic kidney disease (PKD), Bardet Bieldl Syndrome, and Meckel-Gruber Syndrome. In a forward genetic screen, qilin was identified as a novel gene important in the pathogenesis of kidney cysts in zebrafish. In this paper we characterized qilinhi3959A mutant's phenotypes in detail, investigated cilia formation in this mutant and performed structural and functional analysis of the Qilin protein. Results reveal Qilin's essential role in cilia assembly and maintenance in multiple organs, including the kidney, the lateral line organ, and the outer segment of the photoreceptor cell. In addition, rescue experiments suggest that defective pronephric cilia correlate with the formation of kidney cysts in qilinhi3959A mutants. Further, genetic analysis suggests that qilin interacts with multiple intraflagellar transport (IFT) complex B genes, which is supported by the striking phenotypic similarities between qilinhi3959A and IFT complex B mutants. Finally, through deletion analysis we provide evidence that the well-conserved N-terminus and the coiled-coil domain of Qilin are both essential and sufficient for its function. Taken all the observations together, we propose that Qilin acts in a similar role as IFT complex B proteins in cilia assembly, maintenance and kidney development in zebrafish. PMID:22102889

  18. C4.4A gene ablation is compatible with normal epidermal development and causes modest overt phenotypes

    PubMed Central

    Kriegbaum, Mette Camilla; Jacobsen, Benedikte; Füchtbauer, Annette; Hansen, Gert Helge; Christensen, Ib Jarle; Rundsten, Carsten Friis; Persson, Morten; Engelholm, Lars Henning; Madsen, Andreas Nygaard; Di Meo, Ivano; Lund, Ida Katrine; Holst, Birgitte; Kjaer, Andreas; Lærum, Ole Didrik; Füchtbauer, Ernst-Martin; Ploug, Michael

    2016-01-01

    C4.4A is a modular glycolipid-anchored Ly6/uPAR/α-neurotoxin multidomain protein that exhibits a prominent membrane-associated expression in stratified squamous epithelia. C4.4A is also expressed in various solid cancer lesions, where high expression levels often are correlated to poor prognosis. Circumstantial evidence suggests a role for C4.4A in cell adhesion, migration, and invasion, but a well-defined biological function is currently unknown. In the present study, we have generated and characterized the first C4.4A-deficient mouse line to gain insight into the functional significance of C4.4A in normal physiology and cancer progression. The unchallenged C4.4A-deficient mice were viable, fertile, born in a normal Mendelian distribution and, surprisingly, displayed normal development of squamous epithelia. The C4.4A-deficient mice were, nonetheless, significantly lighter than littermate controls predominantly due to differences in fat mass. Congenital C4.4A deficiency delayed migration of keratinocytes enclosing incisional skin wounds in male mice. In chemically induced bladder carcinomas, C4.4A deficiency attenuated the incidence of invasive lesions despite having no effect on total tumour burden. This new C4.4A-deficient mouse line provides a useful platform for future studies on functional aspects of C4.4A in tumour cell invasion in vivo. PMID:27169360

  19. Neural substrates of a schizotypal spectrum in typically-developing children: Further evidence of a normal-pathological continuum.

    PubMed

    Evans, David W; Michael, Andrew M; Ularević, Mirko; Lusk, Laina G; Buirkle, Julia M; Moore, Gregory J

    2016-12-15

    Schizophrenia represents the extreme end of a distribution of traits that extends well into the general population. Using a recently developed measure of psychotic-like traits in children, we examined the neural substrates of psychotic (and other psychiatric) symptoms using structural magnetic resonance imaging (MRI). Twenty-eight typically-developing children (14 males) between the ages of 6-17 years underwent a 3T MRI scan. Parents completed the Psychiatric and Schizotypal Inventory for Children. Results revealed that caudate, amygdala, hippocampal and middle temporal gyrus volumes were associated with quantitative dimensions of psychiatric traits. Furthermore, results suggest a differential a sexually-dimorphic pattern of brain-schizotypy associations. These findings highlight brain-behavior continuities between clinical conditions such as schizophrenia and normal trait variation in typical development. PMID:27555534

  20. The Philadelphia Neurodevelopmental Cohort: A publicly available resource for the study of normal and abnormal brain development in youth.

    PubMed

    Satterthwaite, Theodore D; Connolly, John J; Ruparel, Kosha; Calkins, Monica E; Jackson, Chad; Elliott, Mark A; Roalf, David R; Ryan Hopsona, Karthik Prabhakaran; Behr, Meckenzie; Qiu, Haijun; Mentch, Frank D; Chiavacci, Rosetta; Sleiman, Patrick M A; Gur, Ruben C; Hakonarson, Hakon; Gur, Raquel E

    2016-01-01

    The Philadelphia Neurodevelopmental Cohort (PNC) is a large-scale study of child development that combines neuroimaging, diverse clinical and cognitive phenotypes, and genomics. Data from this rich resource is now publicly available through the Database of Genotypes and Phenotypes (dbGaP). Here we focus on the data from the PNC that is available through dbGaP and describe how users can access this data, which is evolving to be a significant resource for the broader neuroscience community for studies of normal and abnormal neurodevelopment.

  1. The neurology of folic acid deficiency.

    PubMed

    Reynolds, E H

    2014-01-01

    The metabolism of folic acid and the metabolism of vitamin B12 are intimately linked such that deficiency of either vitamin leads to an identical megaloblastic anemia. The neurologic manifestations of folate deficiency overlap with those of vitamin B12 deficiency and include cognitive impairment, dementia, depression, and, less commonly, peripheral neuropathy and subacute combined degeneration of the spinal cord. In both deficiency states there is often dissociation between the neuropsychiatric and the hematologic complications. There is a similar overlap and dissociation between neurologic and hematologic manifestations of inborn errors of folate and vitamin B12 metabolism. Low folate and raised homocysteine levels are risk factors for dementia, including Alzheimer's disease, and depression. Even when folate deficiency is secondary to psychiatric illness due to apathy or poor diet it may eventually aggravate the underlying disorder in a vicious circle effect. Clinical responses to treatment with folates are usually slow over weeks and months, probably due to the efficient blood-brain barrier mechanism for the vitamin, perhaps in turn related to the experimentally demonstrated excitatory properties of folate derivatives. The inappropriate administration of folic acid in the presence of vitamin B12 deficiency may lead to both neurologic and, later, hematologic relapse. Impaired maternal folate intake and status increases the risk of neural tube defects. Periconceptual prophylactic administration of the vitamin reduces, but does not eliminate the risk of neural tube defects even in the absence of folate deficiency. Folates and vitamin B12 have fundamental roles in central nervous system function at all ages, especially in purine, thymidine, neucleotide, and DNA synthesis, genomic and nongenomic methylation and, therefore, in tissue growth, differentiation and repair. There is interest in the potential role of both vitamins in the prevention of disorders of central

  2. Society of Vascular and Interventional Neurology (SVIN) Stroke Interventional Laboratory Consensus (SILC) Criteria: A 7M Management Approach to Developing a Stroke Interventional Laboratory in the Era of Stroke Thrombectomy for Large Vessel Occlusions.

    PubMed

    Shams, Tanzila; Zaidat, Osama; Yavagal, Dileep; Xavier, Andrew; Jovin, Tudor; Janardhan, Vallabh

    2016-06-01

    Brain attack care is rapidly evolving with cutting-edge stroke interventions similar to the growth of heart attack care with cardiac interventions in the last two decades. As the field of stroke intervention is growing exponentially globally, there is clearly an unmet need to standardize stroke interventional laboratories for safe, effective, and timely stroke care. Towards this goal, the Society of Vascular and Interventional Neurology (SVIN) Writing Committee has developed the Stroke Interventional Laboratory Consensus (SILC) criteria using a 7M management approach for the development and standardization of each stroke interventional laboratory within stroke centers. The SILC criteria include: (1) manpower: personnel including roles of medical and administrative directors, attending physicians, fellows, physician extenders, and all the key stakeholders in the stroke chain of survival; (2) machines: resources needed in terms of physical facilities, and angiography equipment; (3) materials: medical device inventory, medications, and angiography supplies; (4) methods: standardized protocols for stroke workflow optimization; (5) metrics (volume): existing credentialing criteria for facilities and stroke interventionalists; (6) metrics (quality): benchmarks for quality assurance; (7) metrics (safety): radiation and procedural safety practices. PMID:27610118

  3. Society of Vascular and Interventional Neurology (SVIN) Stroke Interventional Laboratory Consensus (SILC) Criteria: A 7M Management Approach to Developing a Stroke Interventional Laboratory in the Era of Stroke Thrombectomy for Large Vessel Occlusions

    PubMed Central

    Shams, Tanzila; Zaidat, Osama; Yavagal, Dileep; Xavier, Andrew; Jovin, Tudor; Janardhan, Vallabh

    2016-01-01

    Brain attack care is rapidly evolving with cutting-edge stroke interventions similar to the growth of heart attack care with cardiac interventions in the last two decades. As the field of stroke intervention is growing exponentially globally, there is clearly an unmet need to standardize stroke interventional laboratories for safe, effective, and timely stroke care. Towards this goal, the Society of Vascular and Interventional Neurology (SVIN) Writing Committee has developed the Stroke Interventional Laboratory Consensus (SILC) criteria using a 7M management approach for the development and standardization of each stroke interventional laboratory within stroke centers. The SILC criteria include: (1) manpower: personnel including roles of medical and administrative directors, attending physicians, fellows, physician extenders, and all the key stakeholders in the stroke chain of survival; (2) machines: resources needed in terms of physical facilities, and angiography equipment; (3) materials: medical device inventory, medications, and angiography supplies; (4) methods: standardized protocols for stroke workflow optimization; (5) metrics (volume): existing credentialing criteria for facilities and stroke interventionalists; (6) metrics (quality): benchmarks for quality assurance; (7) metrics (safety): radiation and procedural safety practices. PMID:27610118

  4. Society of Vascular and Interventional Neurology (SVIN) Stroke Interventional Laboratory Consensus (SILC) Criteria: A 7M Management Approach to Developing a Stroke Interventional Laboratory in the Era of Stroke Thrombectomy for Large Vessel Occlusions

    PubMed Central

    Shams, Tanzila; Zaidat, Osama; Yavagal, Dileep; Xavier, Andrew; Jovin, Tudor; Janardhan, Vallabh

    2016-01-01

    Brain attack care is rapidly evolving with cutting-edge stroke interventions similar to the growth of heart attack care with cardiac interventions in the last two decades. As the field of stroke intervention is growing exponentially globally, there is clearly an unmet need to standardize stroke interventional laboratories for safe, effective, and timely stroke care. Towards this goal, the Society of Vascular and Interventional Neurology (SVIN) Writing Committee has developed the Stroke Interventional Laboratory Consensus (SILC) criteria using a 7M management approach for the development and standardization of each stroke interventional laboratory within stroke centers. The SILC criteria include: (1) manpower: personnel including roles of medical and administrative directors, attending physicians, fellows, physician extenders, and all the key stakeholders in the stroke chain of survival; (2) machines: resources needed in terms of physical facilities, and angiography equipment; (3) materials: medical device inventory, medications, and angiography supplies; (4) methods: standardized protocols for stroke workflow optimization; (5) metrics (volume): existing credentialing criteria for facilities and stroke interventionalists; (6) metrics (quality): benchmarks for quality assurance; (7) metrics (safety): radiation and procedural safety practices.

  5. Efficacy of Intravenous Immunoglobulin in Neurological Diseases.

    PubMed

    Lünemann, Jan D; Quast, Isaak; Dalakas, Marinos C

    2016-01-01

    Owing to its anti-inflammatory efficacy in various autoimmune disease conditions, intravenous immunoglobulin (IVIG)-pooled IgG obtained from the plasma of several thousands individuals-has been used for nearly three decades and is proving to be efficient in a growing number of neurological diseases. IVIG therapy has been firmly established for the treatment of Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy, either as first-line therapy or adjunctive treatment. IVIG is also recommended as rescue therapy in patients with worsening myasthenia gravis and is beneficial as a second-line therapy for dermatomyositis and stiff-person syndrome. Subcutaneous rather than intravenous administration of IgG is gaining momentum because of its effectiveness in patients with primary immunodeficiency and the ease with which it can be administered independently from hospital-based infusions. The demand for IVIG therapy is growing, resulting in rising costs and supply shortages. Strategies to replace IVIG with recombinant products have been developed based on proposed mechanisms that confer the anti-inflammatory activity of IVIG, but their efficacy has not been tested in clinical trials. This review covers new developments in the immunobiology and clinical applications of IVIG in neurological diseases.

  6. Distribution of poly(A)-containing RNA during normal pollen development and during induced pollen embryogenesis in Hyoscyamus niger

    PubMed Central

    1981-01-01

    The distribution of poly(A)-containing RNA [poly(A)+RNA] in pollen grains of Hyoscyamus niger during normal gametophytic development and embryogenic development induced by culture of anther segments was followed by in situ hybridization with [3H]-polyuridylic acid as a probe. No binding of the isotope occurred in pollen grains during the uninucleate phase of their development. Although [3H]polyuridylic acid binding sites were present in the generative and vegetative cells of maturing pollen grains, they almost completely disappeared from mature grains ready to germinate. During pollen germination, poly(A)+RNA formation was transient and was due to the activity of the generative nucleus, whereas the vegetative nucleus and the sperm cells failed to interact with the applied probe. In cultured anther segments, moderate amounts of poly(A)+RNA were detected in the uninucleate, nonvacuolate, embryogenically determined pollen grains. Poly(A)+RNA accumulation in these grains was sensitive to actinomycin D, suggesting that it represents newly transcribed mRNA. After the first haploid mitosis in the embryogenically determined pollen grains, only those grains in which the generative nucleus alone or along with the vegetative nucleus accumulated poly(A)+RNA in the surrounding cytoplasm were found to divide in the embryogenic pathway. Overall, the results suggest that, in contrast to normal gametophytic development, embryogenic development in the uninucleate pollen grains of cultured anther segments of H. niger is due to the transcriptional activation of an informational type of RNA. Subsequent divisions in the potentially embryogenic binucleate pollen grains appeared to be mediated by the continued synthesis of mRNA either in the generative nucleus or in both the generative and vegetative nuclei. PMID:6166618

  7. WDR82, a key epigenetics-related factor, plays a crucial role in normal early embryonic development in mice.

    PubMed

    Bi, Ye; Lv, Zhuo; Wang, Ying; Hai, Tang; Huo, Ran; Zhou, Zuomin; Zhou, Qi; Sha, Jiahao

    2011-04-01

    Epigenetic regulation is considered one of the most important mechanisms by which changes in gene expression occur without changes in the underlying DNA sequence. More and more studies have shown that this kind of regulation plays a very important role during the process of early embryonic development. Methylation of histones is a special process in epigenetic regulations that plays a dual role: some activate gene expression, while some inhibit it; trimethylation of histone 3 lysine 4 has been shown to be a marker of gene expression activation. Previous research has led us to focus on the role of WDR82, which has been shown to recognize a subunit in the methyltransferases complex that catalyzes H3K4me3 in early embryonic development. Although it has been shown that a defect in WDR82 causes dysfunction of SETD1A/SETD1B and results in loss of H3K4me3 in human cell lines, the exact role of WDR82 in the methyltransferases complex during early embryonic development is not clear. Our study has shown that a defect in WDR82 causes dysfunction of SETD1A/SETD1B and affects the normal H3K4me3 status in the transcription start region of POU5F1, which then causes the down-regulation of POU5F1 as well as its downstream factors STAT3/BIRC5, which are responsible for the extremely high apoptotic rates of blastocysts. Finally, the result of a blocked WDR82 consists of stunted embryonic development and death. Thus, WDR82 can be considered a key epigenetic regulation-related factor crucial in the normal growth and development of embryos. PMID:21123813

  8. Improved neurological outcome in children with chronic renal disease from infancy.

    PubMed

    Elzouki, A; Carroll, J; Butinar, D; Moosa, A

    1994-04-01

    Progressive encephalopathy, developmental delay, microcephaly, electroencephalogram (EEG) and computed tomographic (CT) scan abnormalities have been reported in 80% of children with chronic renal failure (CRF) in infancy. Malnutrition, aluminium intoxication and psychosocial deprivation are proposed as causes. In 15 children with CRF from infancy we evaluated the effect of no aluminium salts and early vigorous nutritional and psychosocial support, in addition to the standard therapy, on neurological development. Six patients underwent dialysis (2 at birth) and 3 received transplants. None of our patients were given aluminium therapy. The nutritional status of the patients in the first 2 years of life was assessed with the waterlow classification. At the end of the follow-up period (mean 50 months range 14-148 months), patients underwent neurodevelopmental assessment, head CT scan, EEG, nerve conduction velocity (NCV) and auditory brain stem evoked response (ABER). None of our patients developed progressive encephalopathy or recurrent seizures. All have a normal neurological examination apart from hypotonia. Microcephaly was present in 5 patients. There was a good correlation between malnutrition in the first 2 years of life and microcephaly. Developmental delay was present in 3 patients; all 3 were microcephalic. There was evidence of brain atrophy on CT scan in only 3 patients. EEG was abnormal in 6 patients, but only severe in 1 patient. Only 1 patient had diminished NCV; all patients had a normal ABER. We conclude that a policy of no oral aluminium therapy and early nutritional support leads to better neurological outcome in children with CRF from infancy. PMID:8018500

  9. Neurologic injuries from scuba diving.

    PubMed

    Hawes, Jodi; Massey, E Wayne

    2009-02-01

    Interest in scuba (self-contained underwater breathing apparatus) diving increased in the 1970s, and undersea diving continues to be a popular sport early in the 21st century, with approximately 3 million certified divers in the United States. The Divers Alert Network (DAN), an institution created in 1981 by the Commerce Department, National Oceanic and Atmospheric Administration, has collected diving injury data for US and Canadian divers since 1987 that can be studied to suggest the epidemiologic characteristics of diving. This article examines neurologic injuries resulting from scuba diving.

  10. Neurologic injuries from scuba diving.

    PubMed

    Hawes, Jodi; Massey, E Wayne

    2008-02-01

    Interest in scuba (self-contained underwater breathing apparatus) diving increased in the 1970s, and undersea diving continues to be a popular sport early in the 21st century, with approximately 3 million certified divers in the United States. The Divers Alert Network (DAN), an institution created in 1981 by the Commerce Department, National Oceanic and Atmospheric Administration, has collected diving injury data for US and Canadian divers since 1987 that can be studied to suggest the epidemiologic characteristics of diving. This article examines neurologic injuries resulting from scuba diving.

  11. Emerging and Reemerging Neurologic Infections

    PubMed Central

    Glaser, Carol A.

    2014-01-01

    The list of emerging and reemerging pathogens that cause neurologic disease is expanding. Various factors, including population growth and a rise in international travel, have contributed to the spread of pathogens to previously nonendemic regions. Recent advances in diagnostic methods have led to the identification of novel pathogens responsible for infections of the central nervous system. Furthermore, new issues have arisen surrounding established infections, particularly in an increasingly immunocompromised population due to advances in the treatment of rheumatologic disease and in transplant medicine. PMID:25360203

  12. Evaluation of Traumatic Spine by Magnetic Resonance Imaging and Correlation with Neurological Recovery

    PubMed Central

    Magu, Sarita; Yadav, Rohtas Kanwar; Bala, Manju

    2015-01-01

    Study Design Prospective study. Purpose To compare magnetic resonance imaging (MRI) findings with clinical profile and neurological status of the patient and to correlate the MRI findings with neurological recovery of the patients and predict the outcome. Overview of Literature Previous studies have reported poor neurological recovery in patients with cord hemorrhage, as compared to cord edema in spine injury patients. High canal compromise, cord compression along with higher extent of cord injury also carries poor prognostic value. Methods Neurological status of patients was assessed at the time of admission and discharge in as accordance with the American Spine Injury Association (ASIA) impairment scale. Mean stay in hospital was 14.11±5.74 days. Neurological status at admission and neurological recovery at discharge was compared with various qualitative cord findings and quantitative parameters on MRI. In 27 patients, long-term follow-up was done at mean time of 285.9±43.94 days comparing same parameters. Results Cord edema and normal cord was associated with favorable neurological outcome. Cord contusion showed lesser neurological recovery, as compared to cord edema. Cord hemorrhage was associated with worst neurological status at admission and poor neurological recovery. Mean canal compromise (MCC), mean spinal cord compression (MSCC) and lesion length values were higher in patients presenting with ASIA A impairment scale injury and showed decreasing trends towards ASIA E impairment scale injury. Patients showing neurological recovery had lower mean MCC, MSCC, and lesion length, as compared to patients showing no neurological recovery (p<0.05). Conclusions Cord hemorrhage, higher MCC, MSCC, and lesion length values have poor prognostic value in spine injury patients. PMID:26435794

  13. Paraneoplastic Neurological Syndrome in Fallopian Tube Cancer.

    PubMed

    Maas, E; Skoberne, T; Werner, A; Braun, S; Jackisch, C

    2014-10-01

    We report on a rare case of paraneoplastic syndrome (PS) that was discovered on completion of diagnostic work-up to be an undifferentiated fallopian tube carcinoma. A 49-year-old Caucasian woman was admitted to neurology with vertigo, gait ataxia and dysarthria, transient ischaemic attack (TIA) and stroke were quickly excluded. Indicative for the further diagnosis of a paraneoplastic syndrome was the identification of onconeuronal antibodies the detection of which can be associated with certain tumour entities. The strongly positive anti-Yo antibody that is formed above all by breast and lung cancers as well as ovarian cancer led to a corresponding staging. The tumour markers CEA, CA 125 and CA 15-3 were in the normal ranges. Mammography and thorax CT were also unremarkable; on transvaginal sonography the internal genitals were inconspicuous except for a follicular cyst on the left. On abdominal CT the only conspicuous finding was a 1.5 cm ovarian cyst. After consensual agreement a bilateral laparoscopic adenexectomy was performed but with unremarkable abdominal findings. The histological examination confirmed a right-sided undifferentiated tubal carcinoma with the provisional classification FIGO IIA. After a stage-related staging operation, the final classification was found to be the FIGO-IIIC stage on account of positive retroperitoneal lymph nodes. Thus adjuvant chemotherapy with 6 cycles of carboplatin and paclitaxel was performed. By means of a timely, guideline-conform therapy for tubal carcinoma, the neurological symptoms and, above all, the dysarthria could be improved after 10 months.

  14. The glycocalyx is present as soon as blood flow is initiated and is required for normal vascular development.

    PubMed

    Henderson-Toth, Caitlin E; Jahnsen, Espen D; Jamarani, Roya; Al-Roubaie, Sarah; Jones, Elizabeth A V

    2012-09-15

    The glycocalyx, and the thicker endothelial surface layer (ESL), are necessary both for endothelial barrier function and for sensing mechanical forces in the adult. The goal of this study is to use a combination of imaging techniques to establish when the glycocalyx and endothelial surface layer form during embryonic development and to determine the biological significance of the glycocalyx layer during vascular development in quail embryos. Using transmission electron microscopy, we show that the glycocalyx layer is present as soon as blood flow starts (14 somites). The early endothelial glycocalyx (14 somites) lacks the distinct hair-like morphology that is present later in development (17 and 25 somites). The average thickness does not change significantly (14 somites, 182 nm ± 33 nm; 17 somites, 218 ± 30 nm; 25 somites, 212 ± 32 nm). The trapping of circulating fluorescent albumin was used to evaluate the development of the ESL. Trapped fluorescent albumin was first observed at 25 somites. In order to assess a functional role for the glycocalyx during development, we selectively degraded luminal glycosaminoglycans. Degradation of hyaluronan compromised endothelial barrier function and prevented vascular remodeling. Degradation of heparan sulfate down regulated the expression of shear-sensitive genes but does not inhibit vascular remodeling. Our findings show that the glycocalyx layer is present as soon as blood flow starts (14 somites). Selective degradations of major glycocalyx components were shown to inhibit normal vascular development, examined through morphology, vascular barrier function, and gene expression.

  15. A novel rat model to study the functions of macrophages during normal development and pathophysiology of the eye.

    PubMed

    Hose, Stacey; Zigler, J Samuel; Sinha, Debasish

    2005-01-31

    Several studies have shown that macrophages play an active role in the initiation and completion of the programmed cell death process during development. Macrophages are called professional phagocytes, as their primary role is phagocytosis. The process of phagocytosis is complex and to date only poorly defined. It has also been postulated that macrophages around the developing lens likely migrate into the neural retina and differentiate into microglia after completion of their role as debris removers. We have identified ED1 immunopositive macrophages and CD11b/18 (OX-42) immunopositive macrophage-like cells in the vitreous chamber and sub-retinal space of a rat spontaneous mutation that we have termed Nuc1. The mutation appears to affect the programmed cell death process and is highly eye specific in its effects. While ED1 and ED2-immunopositive macrophages have previously been found surrounding the developing lens and are thought to play a role in the programmed regression of the tunica vasculosa lentis (part of the vascular structure present on the posterior surface of the lens during development), OX-42-immunopositive cells have not previously been identified in the vitreous chamber under normal or pathological conditions. Macrophage subpopulations surrounding the lens may differentiate into OX-42+ cells in Nuc1 following the release of lens material into the vitreous after the posterior capsule ruptures. In Nuc1 homozygotes, the posterior lens capsule ruptures before birth, causing lens material to be extruded into the vitreous compartment and damaging the tunica vasculosa lentis. Alternatively, OX-42+ cells may be recruited due to an inflammatory response both in the vitreous compartment and sub-retinal space. Inflammation is known to have an enhanced influx of phagocytic cells. Our data suggests that subpopulations of macrophages perform distinct functions in inducing apoptosis and phagocytic activity during normal conditions and in disease.

  16. Cone Photoreceptors Develop Normally in the Absence of Functional Rod Photoreceptors in a Transgenic Swine Model of Retinitis Pigmentosa

    PubMed Central

    Fernandez de Castro, Juan P.; Scott, Patrick A.; Fransen, James W.; Demas, James; DeMarco, Paul J.; Kaplan, Henry J.; McCall, Maureen A.

    2014-01-01

    Purpose. Human and swine retinas have morphological and functional similarities. In the absence of primate models, the swine is an attractive model to study retinal function and disease, with its cone-rich visual streak, our ability to manipulate their genome, and the differences in susceptibility of rod and cone photoreceptors to disease. We characterized the normal development of cone function and its subsequent decline in a P23H rhodopsin transgenic (TgP23H) miniswine model of autosomal dominant RP. Methods. Semen from TgP23H miniswine 53-1 inseminated domestic swine and produced TgP23H and Wt hybrid littermates. Retinal function was evaluated using ERGs between postnatal days (P) 14 and 120. Retinal ganglion cell (RGC) responses were recorded to full-field stimuli at several intensities. Retinal morphology was assessed using light and electron microscopy. Results. Scotopic retinal function matures in Wt pigs up to P60, but never develops in TgP23H pigs. Wt and TgP23H photopic vision matures similarly up to P30 and diverges at P60 where TgP23H cone vision declines. There are fewer TgP23H RGCs with visually evoked responses at all ages and their response to light is compromised. Photoreceptor morphological changes mirror these functional changes. Conclusions. Lack of early scotopic function in TgP23H swine suggests it as a model of an aggressive form of RP. In this mammalian model of RP, normal cone function develops independent of rod function. Therefore, its retina represents a system in which therapies to rescue cones can be developed to prolong photopic visual function in RP patients. PMID:24618325

  17. Neurological assessment of preterm infants for predicting neuromotor status at 2 years: results from the LIFT cohort

    PubMed Central

    Leroux, Bénédicte Gaillard; N'Guyen The Tich, Sylvie; Branger, Bernard; Gascoin, Géraldine; Rouger, Valérie; Berlie, Isabelle; Montcho, Yannis; Ancel, Pierre-Yves; Rozé, Jean-Christophe; Flamant, Cyril

    2013-01-01

    Objective To develop a predictive risk stratification model for the identification of preterm infants at risk of 2-year suboptimal neuromotor status. Design Population-based observational study. Setting Regional preterm infant follow-up programme (Loire Infant Follow-up Team (LIFT) cohort) implemented in 2003. Participants 4030 preterm infants were enrolled in the LIFT cohort, and examined by neonatologists using a modified version of the Amiel-Tison neurological assessment tool. Main outcome criteria 2 year neuromotor status based on clinical examinations was conducted by trained paediatricians and parents’ responses to the Ages and Stages Questionnaire were reported. Results At 2 years of corrected age, 3321 preterm infants were examined, and suboptimal neuromotor status was found in 355 (10.7%). The study population was divided into training and validation sets. In the training set, 13 neonatal neurological items were associated with a 2-year suboptimal neuromotor status. Having at least one abnormal item was defined as an abnormal neurological status at term. In the validation set, these data predicted a 2-year suboptimal neuromotor status with a sensitivity of 0.55 (95% CI 0.47 to 0.62) and a specificity of 0.65 (95% CI 0.62 to 0.67). Two predictive risk stratification trees were built using the training set, which were based on the neurological assessment at term along with either gestational age or severe cranial lesions or birth weight. Using the validation set, the first tree identified a subgroup with a relatively low risk of suboptimal neuromotor status (3%), representing 32% of infants, and the second tree identified a subgroup with a risk of 5%, representing 42% of infants. Conclusion A normal neurological assessment at term allows the identification of a subgroup of preterm infants with a lower risk of non-optimal neuromotor development at 2 years. PMID:23435797

  18. Neurology and psychiatry in Babylon.

    PubMed

    Reynolds, Edward H; Wilson, James V Kinnier

    2014-09-01

    We here review Babylonian descriptions of neurological and psychiatric disorders, including epilepsy, stroke, psychoses, obsessive compulsive disorder, phobias, psychopathic behaviour, depression and anxiety. Most of these accounts date from the first Babylonian dynasty of the first half of the second millennium BC, within a millennium and a half of the origin of writing. The Babylonians were remarkably acute and objective observers of medical disorders and human behaviour. Their detailed descriptions are surprisingly similar to modern 19th and 20th century AD textbook accounts, with the exception of subjective thoughts and feelings which are more modern fields of enquiry. They had no knowledge of brain or psychological function. Some neuropsychiatric disorders, e.g. stroke or facial palsy, had a physical basis requiring the attention of a physician or asû, using a plant and mineral based pharmacology; some disorders such as epilepsy, psychoses, depression and anxiety were regarded as supernatural due to evil demons or spirits, or the anger of personal gods, and thus required the intervention of the priest or ašipu; other disorders such as obsessive compulsive disorder and psychopathic behaviour were regarded as a mystery. The Babylonians were the first to describe the clinical foundations of neurology and psychiatry. We discuss these accounts in relation to subsequent and more modern clinical descriptions.

  19. Neurological Complications of VZV Reactivation

    PubMed Central

    Nagel, Maria A.

    2014-01-01

    Purpose of the review Varicella zoster virus (VZV) reactivation results in zoster, which may be complicated by postherpetic neuralgia, myelitis, meningoencephalitis and VZV vasculopathy. This review highlights the clinical features, laboratory abnormalities, imaging changes and optimal treatment of each of those conditions. Because all of these neurological disorders produced by VZV reactivation can occur in the absence of rash, the virological tests proving that VZV caused disease are discussed. Recent findings After primary infection, VZV becomes latent in ganglionic neurons along the entire neuraxis. With a decline in VZV-specific cell-mediated immunity, VZV reactivates from ganglia and travels anterograde to the skin to cause zoster, which is often complicated by postherpetic neuralgia. VZV can also travel retrograde to produce meningoencephaltis, myelitis and stroke. When these complications occur without rash, VZV-induced disease can be diagnosed by detection of VZV DNA or anti-VZV antibody in CSF and treated with intravenous acyclovir. Summary Awareness of the expanding spectrum of neurological complications caused by VZV reactivation with and without rash will improve diagnosis and treatment. PMID:24792344

  20. Neurologic infections in diabetes mellitus.

    PubMed

    Jay, Cheryl A; Solbrig, Marylou V

    2014-01-01

    Even at a time when HIV/AIDS and immunosuppressive therapy have increased the number of individuals living with significant immunocompromise, diabetes mellitus (DM) remains a major comorbid disorder for several rare but potentially lethal infections, including rhino-orbital-cerebral mucormycosis and malignant external otitis. DM is also a commonly associated condition in patients with nontropical pyomyositis, pyogenic spinal infections, Listeria meningitis, and blastomycosis. As West Nile virus spread to and across North America over a decade ago, DM appeared in many series as a risk factor for death or neuroinvasive disease. More recently, in several large international population-based studies, DM was identified as a risk factor for herpes zoster. The relationships among infection, DM, and the nervous system are multidirectional. Viral infections have been implicated in the pathogenesis of type 1 and type 2 DM, while parasitic infections have been hypothesized to protect against autoimmune disorders, including type 1 DM. DM-related neurologic disease can predispose to systemic infection - polyneuropathy is the predominant risk factor for diabetic foot infection. Because prognosis for many neurologic infections depends on timely institution of antimicrobial and sometimes surgical therapy, neurologists caring for diabetic patients should be familiar with the clinical features of the neuroinfectious syndromes associated with DM. PMID:25410222

  1. Neurologic complications after liver transplantation

    PubMed Central

    Živković, Saša A

    2013-01-01

    Neurologic complications are relatively common after solid organ transplantation and affect 15%-30% of liver transplant recipients. Etiology is often related to immunosuppressant neurotoxicity and opportunistic infections. Most common complications include seizures and encephalopathy, and occurrence of central pontine myelinolysis is relatively specific for liver transplant recipients. Delayed allograft function may precipitate hepatic encephalopathy and neurotoxicity of calcineurin inhibitors typically manifests with tremor, headaches and encephalopathy. Reduction of neurotoxic immunosuppressants or conversion to an alternative medication usually result in clinical improvement. Standard preventive and diagnostic protocols have helped to reduce the prevalence of opportunistic central nervous system (CNS) infections, but viral and fungal CNS infections still affect 1% of liver transplant recipients, and the morbidity and mortality in the affected patients remain fairly high. Critical illness myopathy may also affect up to 7% of liver transplant recipients. Liver insufficiency is also associated with various neurologic disorders which may improve or resolve after successful liver transplantation. Accurate diagnosis and timely intervention are essential to improve outcomes, while advances in clinical management and extended post-transplant survival are increasingly shifting the focus to chronic post-transplant complications which are often encountered in a community hospital and an outpatient setting. PMID:24023979

  2. Neurologic infections in diabetes mellitus.

    PubMed

    Jay, Cheryl A; Solbrig, Marylou V

    2014-01-01

    Even at a time when HIV/AIDS and immunosuppressive therapy have increased the number of individuals living with significant immunocompromise, diabetes mellitus (DM) remains a major comorbid disorder for several rare but potentially lethal infections, including rhino-orbital-cerebral mucormycosis and malignant external otitis. DM is also a commonly associated condition in patients with nontropical pyomyositis, pyogenic spinal infections, Listeria meningitis, and blastomycosis. As West Nile virus spread to and across North America over a decade ago, DM appeared in many series as a risk factor for death or neuroinvasive disease. More recently, in several large international population-based studies, DM was identified as a risk factor for herpes zoster. The relationships among infection, DM, and the nervous system are multidirectional. Viral infections have been implicated in the pathogenesis of type 1 and type 2 DM, while parasitic infections have been hypothesized to protect against autoimmune disorders, including type 1 DM. DM-related neurologic disease can predispose to systemic infection - polyneuropathy is the predominant risk factor for diabetic foot infection. Because prognosis for many neurologic infections depends on timely institution of antimicrobial and sometimes surgical therapy, neurologists caring for diabetic patients should be familiar with the clinical features of the neuroinfectious syndromes associated with DM.

  3. Neurology and psychiatry in Babylon.

    PubMed

    Reynolds, Edward H; Wilson, James V Kinnier

    2014-09-01

    We here review Babylonian descriptions of neurological and psychiatric disorders, including epilepsy, stroke, psychoses, obsessive compulsive disorder, phobias, psychopathic behaviour, depression and anxiety. Most of these accounts date from the first Babylonian dynasty of the first half of the second millennium BC, within a millennium and a half of the origin of writing. The Babylonians were remarkably acute and objective observers of medical disorders and human behaviour. Their detailed descriptions are surprisingly similar to modern 19th and 20th century AD textbook accounts, with the exception of subjective thoughts and feelings which are more modern fields of enquiry. They had no knowledge of brain or psychological function. Some neuropsychiatric disorders, e.g. stroke or facial palsy, had a physical basis requiring the attention of a physician or asû, using a plant and mineral based pharmacology; some disorders such as epilepsy, psychoses, depression and anxiety were regarded as supernatural due to evil demons or spirits, or the anger of personal gods, and thus required the intervention of the priest or ašipu; other disorders such as obsessive compulsive disorder and psychopathic behaviour were regarded as a mystery. The Babylonians were the first to describe the clinical foundations of neurology and psychiatry. We discuss these accounts in relation to subsequent and more modern clinical descriptions. PMID:25037816

  4. Dysphagia associated with neurological disorders.

    PubMed

    Buchholz, D W

    1994-01-01

    Neurogenic dysphagia results from sensorimotor impairment of the oral and pharyngeal phases of swallowing due to a neurologic disorder. The symptoms of neurogenic dysphagia include drooling, difficulty initiating swallowing, nasal regurgitation, difficulty managing secretions, choke/cough episodes while feeding, and food sticking in the throat. If unrecognized and untreated, neurogenic dysphagia can lead to dehydration, malnutrition, and respiratory complications. The symptoms of neurogenic dysphagia may be relatively inapparent on account of both compensation for swallowing impairment and diminution of the laryngeal cough reflex due to a variety of factors. Patients with symptoms of oropharyngeal dysphagia should undergo videofluoroscopy of swallowing, which in the case of neurogenic dysphagia typically reveals impairment of oropharyngeal motor performance and/or laryngeal protection. The many causes of neurogenic dysphagia include stroke, head trauma, Parkinson's disease, motor neuron disease and myopathy. Evaluation of the cause of unexplained neurogenic dysphagia should include consultation by a neurologist, magnetic resonance imaging of the brain, blood tests (routine studies plus muscle enzymes, thyroid screening, vitamin B12 and anti-acetylcholine receptor antibodies), electromyography/nerve conduction studies, and, in certain cases, muscle biopsy or cerebrospinal fluid examination. Treatment of neurogenic dysphagia involves treatment of the underlying neurologic disorder (if possible), swallowing therapy (if oral feeding is reasonably safe to attempt) and gastrostomy (if oral feeding is unsafe or inadequate).

  5. Screening EEG in Aircrew Selection: Clinical Aerospace Neurology Perspective

    NASA Technical Reports Server (NTRS)

    Clark, Jonathan B.; Riley, Terrence

    2001-01-01

    As clinical aerospace neurologists we do not favor using screening EEG in pilot selection on unselected and otherwise asymptomatic individuals. The role of EEG in aviation screening should be as an adjunct to diagnosis, and the decision to disqualify a pilot should never be based solely on the EEG. Although a policy of using a screening EEG in an unselected population might detect an individual with a potentially increased relative risk, it would needlessly exclude many applicants who would probably never have a seizure. A diagnostic test performed on an asymptomatic individual without clinical indications, in a population with a low prevalence of disease (seizure) may be of limited or possibly detrimental value. We feel that rather than do EEGs on all candidates, a better approach would be to perform an EEG for a specific indication, such as family history of seizure, single convulsion (seizure) , history of unexplained loss of consciousness or head injury. Routine screening EEGs in unselected aviation applications are not done without clinical indication in the U.S. Air Force, Navy, or NASA. The USAF discontinued routine screening EEGs for selection in 1978, the U.S. Navy discontinued it in 1981 , and NASA discontinued it in 1995. EEG as an aeromedical screening tool in the US Navy dates back to 1939. The US Navy routinely used EEGs to screen all aeromedical personnel from 1961 to 1981. The incidence of epileptiform activity on EEG in asymptomatic flight candidates ranges from 0.11 to 2.5%. In 3 studies of asymptomatic flight candidates with epileptiform activity on EEG followed for 2 to 15 years, 1 of 31 (3.2%), 1 of 30 (3.3%), and 0 of 14 (0%) developed a seizure, for a cumulative risk of an individual with an epileptiform EEG developing a seizure of 2.67% (2 in 75). Of 28,658 student naval aviation personnel screened 31 had spikes and/or slow waves on EEG, and only 1 later developed a seizure. Of the 28,627 who had a normal EEG, 4 later developed seizures, or

  6. Elevated mitochondrial superoxide disrupts normal T-cell development to impair adaptive immune responses to an influenza challenge

    PubMed Central

    Case, Adam J.; McGill, Jodi L.; Tygrett, Lorraine T.; Shirasawa, Takuji; Spitz, Douglas R.; Waldschmidt, Thomas J.; Legge, Kevin L.; Domann, Frederick E.

    2010-01-01

    Reactive oxygen species (ROS) are critical in a broad spectrum of cellular processes including signaling, tumor progression, and innate immunity. The essential nature of ROS signaling in the immune systems of Drosophila and zebrafish has been demonstrated; however, the role of ROS, if any, in mammalian adaptive immune system development and function remains unknown. The current work provides the first clear demonstration that thymus specific elevation of mitochondrial superoxide (O2·−) disrupts normal T-cell development to impair function of the mammalian adaptive immune system. To assess the effect of elevated mitochondrial superoxide in the developing thymus, we used a T-cell specific knockout of manganese superoxide dismutase (i.e. SOD2) and have thus established a murine model to examine the role of mitochondrial superoxide in T-cell development. Conditional loss of SOD2 led to increased superoxide, apoptosis, and developmental defects in the T-cell population resulting in immunodeficiency and susceptibility to influenza A virus (IAV), H1N1. This phenotype was rescued with mitochondrially targeted superoxide scavenging drugs. These new findings demonstrate that loss of regulated levels of mitochondrial superoxide lead to aberrant T-cell development and function, and further suggest that manipulations of mitochondrial superoxide levels may significantly alter clinical outcomes resulting from viral infection. PMID:21130157

  7. Proteomic profiles of the embryonic chorioamnion and uterine caruncles in buffaloes (Bubalus bubalis) with normal and retarded embryonic development.

    PubMed

    Balestrieri, Maria Luisa; Gasparrini, Bianca; Neglia, Gianluca; Vecchio, Domenico; Strazzullo, Maria; Giovane, Alfonso; Servillo, Luigi; Zicarelli, Luigi; D'Occhio, Michael J; Campanile, Giuseppe

    2013-05-01

    The aim of this study was to compare the proteome profiles of the chorioamnion and corresponding caruncle for buffalo embryos that had either normal or retarded development on Day 25 after artificial insemination (AI). In experiment 1, embryos that were to subsequently undergo late embryonic mortality had a smaller width on Day 25 after AI than embryos associated with pregnancy on Day 45 after AI. In experiment 2, 25 Italian Mediterranean buffaloes underwent transrectal ultrasonography on Day 25 after AI, and pregnant animals were categorized as one of two groups based on embryonic width: normal embryos (embryonic width > 2.7 mm) and retarded embryos (embryonic width < 2.7 mm). Three buffaloes of each group were slaughtered on Day 27 after AI to collect chorioamnion and caruncle tissues for subsequent proteomic analyses. Two-dimensional difference gel electrophoresis (2D-DIGE) and matrix-assisted laser desorption/ionization-time-of-flight/time-of-flight mass spectrometer analysis were used to ascertain the proteomic profiles. To confirm 2D-DIGE-results, three selected proteins were analyzed by Western blot. The proteomic profiles of the chorioamnion of retarded embryos and the corresponding caruncles showed differences in the expression of several proteins compared to normal embryos. In particular, a down-regulation was observed for proteins involved in protein folding (HSP 90-alpha, calreticulin), calcium binding (annexin A1, annexin A2), and coagulation (fibrinogen alpha-chain) (P < 0.05), whereas proteins involved in protease inhibition (alpha-1-antiproteinase, serpin H1, serpin A3-8), DNA and RNA binding (heterogeneous nuclear ribonucleoproteins A2/B1 and K), chromosome segregation (serine/threonine-protein phosphatase 2A), cytoskeletal organization (ezrin), cell redox homeostasis (amine oxidase-A), and hemoglobin binding (haptoglobin) were up-regulated (P < 0.05). PMID:23575152

  8. [Can music therapy for patients with neurological disorders?].

    PubMed

    Myskja, Audun

    2004-12-16

    Recent developments in brain research and in the field of music therapy have led to the development of music-based methods specifically aimed at relieving symptoms of Parkinson's disease and other neurologic disorders. Rhythmic auditory stimulation uses external rhythmic auditory cues from song, music or metronome to aid patients improving their walking functioning and has been shown to be effective both within sessions and as a result of training over time. Melodic intonation therapy and related vocal techniques can improve expressive dysphasia and aid rehabilitation of neurologic disorders, particularly Parkinson's disease, stroke and developmental disorders. PMID:15608775

  9. Paraneoplastic neurological syndromes--diagnosis and management.

    PubMed

    Blaes, Franz

    2012-01-01

    Paraneoplastic neurological syndromes (PNS) are rare nervous system dysfunctions in cancer patients, which are not due to a local effect of the tumour or its metastases. PNS in adults are mainly associated with lung cancer, especially small cell lung cancer, lymphoma and gynaecological tumours. In some cases an overlapping of different clinical syndromes can be observed. Since autoantibodies directed against tumour and nervous system tissue can be observed, an autoimmune aetiology has been suspected in PNS patients. Currently, one group of patients exhibit surface-binding receptor or ion channel autoantibodies which are thought to be pathogenic and many of these patients respond well to immunotherapies. Another group of PNS is associated with highly specific autoantibodies directed against intracellular onconeuronal antigens. The latter group seem to be T-cell-mediated and do not respond well to immunotherapies. The childhood PNS, especially the neuroblastoma-associated opsoclonus-myoclonus syndrome also respond to immunosuppressive therapies, plasmapheresis and intravenous immunoglobulins. The current review summarizes recent developments in physiopathology, diagnosis and treatment of paraneoplastic neurological syndromes.

  10. Neurological caricatures since the 15th century.

    PubMed

    Lorusso, Lorenzo

    2008-01-01

    During the Renaissance, different artists began to draw medical illustrations from various viewpoints. Leonardo da Vinci was among those who sought to portray the emotional as well as the physical qualities of man. Other European artists described caricatural aspects of medical activities. In Northern Europe, Albrecht Durer, Hieronymus Bosch, and Pieter Brueghel were also famous for drawing caricatures. Later English artists, notably William Hogarth, Thomas Rowlandson, James Gillray, and the Cruikshanks, satirized life in general and the medical profession in particular. In Spain, Francisco Goya's works became increasingly macabre and satirical following his own mysterious illness and, in France, Honore Daumier used satire and humor to expose medical quackery. Also physicians such as Charles Bell and Jean-Martin Charcot were talented caricaturists. Their own personal artistic styles reflected their approach and gave a different "image" of neurology. Caricatures were popular portraits of developments in science and medicine and were frequently used whenever scientific language was too difficult to disseminate, in particular in the field of neurology.

  11. Williams syndrome: pediatric, neurologic, and cognitive development.

    PubMed

    Carrasco, Ximena; Castillo, Silvia; Aravena, Teresa; Rothhammer, Paula; Aboitiz, Francisco

    2005-03-01

    This study examines the developmental history of 32 Williams syndrome patients, positive to the fluorescence in situ hybridization (FISH) test. The information is intended to provide help for early diagnosis and appropriate stimulation of these patients. In the sample reported here, only about half of the patients referred with presumptive diagnosis were in fact FISH+, indicating that facial dysmorphism may not be the most reliable sign for diagnosis. Initial pediatric signs are developmental delay and nocturnal irritability. In consultation, facial dysmorphies and heart murmur are detected. There is also low birth weight, failure to thrive, unsuccessful breastfeeding, and gastroesophageal reflux. All these symptoms are strongly suggestive of Williams syndrome. Subsequent steps consist of cardiologic studies. Our results indicate that the triad of symptoms consisting of infantile hypercalcemia, dysmorphic facies, and supravalvular aortic stenosis, which until recently was considered fundamental for Williams syndrome diagnosis, is not usually present and does not lead to an early diagnosis. Cognitively, these children are characterized by hypersociability, hyperacusia, deficient visuoconstructive abilities, attentional deficit and hyperactivity, and in some cases, spontaneous musical interests. There are no special verbal skills. The results of this study indicate that the concept of Williams syndrome patients as language- and musically-gifted is not fully accurate. PMID:15730896

  12. Calmodulin binding to glutamate decarboxylase is required for regulation of glutamate and GABA metabolism and normal development in plants.

    PubMed Central

    Baum, G; Lev-Yadun, S; Fridmann, Y; Arazi, T; Katsnelson, H; Zik, M; Fromm, H

    1996-01-01

    Glutamate decarboxylase (GAD) catalyzes the decarboxylation of glutamate to CO2 and gamma-aminobutyrate (GABA). GAD is ubiquitous in prokaryotes and eukaryotes, but only plant GAD has been shown to bind calmodulin (CaM). Here, we assess the role of the GAD CaM-binding domain in vivo. Transgenic tobacco plants expressing a mutant petunia GAD lacking the CaM-binding domain (GADdeltaC plants) exhibit severe morphological abnormalities, such as short stems, in which cortex parenchyma cells fail to elongate, associated with extremely high GABA and low glutamate levels. The morphology of transgenic plants expressing the full-length GAD (GAD plants) is indistinguishable from that of wild-type (WT) plants. In WT and GAD plant extracts, GAD activity is inhibited by EGTA and by the CaM antagonist trifluoperazine, and is associated with a CaM-containing protein complex of approximately 500 kDa. In contrast, GADdeltaC plants lack normal GAD complexes, and GAD activity in their extracts is not affected by EGTA and trifluoperazine. We conclude that CaM binding to GAD is essential for the regulation of GABA and glutamate metabolism, and that regulation of GAD activity is necessary for normal plant development. This study is the first to demonstrate an in vivo function for CaM binding to a target protein in plants. Images PMID:8670800

  13. Calmodulin binding to glutamate decarboxylase is required for regulation of glutamate and GABA metabolism and normal development in plants.

    PubMed

    Baum, G; Lev-Yadun, S; Fridmann, Y; Arazi, T; Katsnelson, H; Zik, M; Fromm, H

    1996-06-17

    Glutamate decarboxylase (GAD) catalyzes the decarboxylation of glutamate to CO2 and gamma-aminobutyrate (GABA). GAD is ubiquitous in prokaryotes and eukaryotes, but only plant GAD has been shown to bind calmodulin (CaM). Here, we assess the role of the GAD CaM-binding domain in vivo. Transgenic tobacco plants expressing a mutant petunia GAD lacking the CaM-binding domain (GADdeltaC plants) exhibit severe morphological abnormalities, such as short stems, in which cortex parenchyma cells fail to elongate, associated with extremely high GABA and low glutamate levels. The morphology of transgenic plants expressing the full-length GAD (GAD plants) is indistinguishable from that of wild-type (WT) plants. In WT and GAD plant extracts, GAD activity is inhibited by EGTA and by the CaM antagonist trifluoperazine, and is associated with a CaM-containing protein complex of approximately 500 kDa. In contrast, GADdeltaC plants lack normal GAD complexes, and GAD activity in their extracts is not affected by EGTA and trifluoperazine. We conclude that CaM binding to GAD is essential for the regulation of GABA and glutamate metabolism, and that regulation of GAD activity is necessary for normal plant development. This study is the first to demonstrate an in vivo function for CaM binding to a target protein in plants.

  14. Brain-derived neurotrophic factor is required for normal development of the central respiratory rhythm in mice

    PubMed Central

    Balkowiec, Agnieszka; Katz, David M

    1998-01-01

    Molecular mechanisms underlying maturation of the central respiratory rhythm are largely unknown. Previously, we found that brain-derived neurotrophic factor (BDNF) is required for expression of normal breathing behaviour in newborn mice, raising the possibility that maturation of central respiratory output is dependent on BDNF. Respiratory activity was recorded in vitro from cervical ventral roots (C1 or C4) using the isolated brainstem–spinal cord preparation from postnatal day (P) 0.5–2.0 and P4.5 wild-type mice and mice lacking functional bdnf alleles. Loss of one or both bdnf alleles resulted in an approximately 50 % depression of central respiratory frequency compared with wild-type controls. In addition, respiratory cycle length variability was 214 % higher in bdnf null (bdnf−/−) animals compared with controls at P4.5. In contrast, respiratory burst duration was unaffected by bdnf gene mutation. These derangements of central respiratory rhythm paralleled the ventilatory depression and irregular breathing characteristic of bdnf mutants in vivo, indicating that central deficits can largely account for the abnormalities in resting ventilation produced by genetic loss of BDNF. BDNF is thus the first growth factor identified that is required for normal development of the central respiratory rhythm, including the stabilization of central respiratory output that occurs after birth. PMID:9706001

  15. [Traumatic cervical disc prolapse with severe neurological impact].

    PubMed

    Knudsen, Roland; Gundtoft, Per

    2014-12-15

    A 51-year-old male drove into a ditch on his scooter. Immediately after the trauma the patient complained of neck pain and decreased ability to feel and move his extremities. An initial trauma computed tomography (CT) of the columna showed normal conditions. Because the patient had neurological deficiencies, magnetic resonance imaging of the columna was performed 12 days later, and a disc prolapse at the C3/C4 level with spinal cord compression was visible. Despite decompression the patient did not recover. Traumatic cervical disc prolapse is a rare and positionally dangerous condition, which can be present despite a CT showing normal conditions.

  16. Pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis. A report of 50 cases.

    PubMed

    Gómez-Aranda, F; Cañadillas, F; Martí-Massó, J F; Díez-Tejedor, E; Serrano, P J; Leira, R; Gracia, M; Pascual, J

    1997-07-01

    This is the first large series, comprising 50 patients who suffered a total of 164 episodes, of pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis (PMP syndrome). Onset of PMP was between the ages of 14 and 39 years and was most frequent in males (68%). Eight males (24%) and five females (31%) had a personal history of migraine. One-quarter had had a viral-like illness up to 3 weeks prior to the onset of the syndrome. The clinical picture consisted of one to 12 episodes of changing variable neurological deficits accompanied by moderate-to-severe headache and occasionally fever. The headaches were described as predominantly throbbing and bilateral with variable duration (mean, 19 h). The mean duration of the transient neurological deficits was 5 h. Sensory symptoms were most common (78% of episodes), followed by aphasic (66%) and motor (56%) symptoms. Visual symptoms appeared in only 12% of episodes. The most frequent combinations were motor aphasia plus sensory and motor right hemibody symptoms (19% of episodes), motor aphasia plus right sensory symptoms (10%) and isolated right (9%) or left (9%) sensory symptoms. All patients were asymptomatic between episodes and following the symptomatic period (maximum duration 49 days). Lymphocytic pleocytosis ranged from 10 to 760 lymphocytic cells/mm3 CSF (mean, 199). In CSF, protein was increased in 96% of patients, IgG was normal in 80% of cases and oligoclonal bands were not found. Adensoine deaminase values were slightly above normal in two out of 16 patients tested. Extensive microbiological determinations, including viral HIV and borrelia serologies, were negative. Brain CT and MRI were always within normal limits, while EEG frequently showed focal slowing. Conventional cranial angiography was performed on 12 patients. In only one were there abnormalities suggestive of localized vascular inflammation, coincident with the focal neurological symptoms. Two patients developed PMP symptoms

  17. Perceptual Development of Nasal Consonants in Children with Normal Hearing and in Children Who Use Cochlear Implants

    ERIC Educational Resources Information Center

    Guillot, Kathryn M.; Ohde, Ralph N.; Hedrick, Mark

    2013-01-01

    Purpose: This study was conducted to determine whether the perceptions of nasal consonants in children with normal hearing and children with cochlear implants were predicted by the discontinuity hypothesis. Methods: Four groups participated: 8 adults, 8 children with normal hearing (ages 5-7 years), 8 children with normal hearing (ages 3.5-4…

  18. How normal is grammatical development in the right hemisphere following hemispherectomy? The root infinitive stage and beyond.

    PubMed

    Curtiss, Susan; de Bode, Stella

    2003-08-01

    We examined the morphosyntax of eight left hemispherectomized children at two different stages and compared it to MLU-matched normals. We found that the language of the hemispherectomies paralleled that of their MLU matches with respect to the specific morphosyntactic characteristics of each stage. Our findings provide strong evidence for the presence of functional categories in all early grammars and demonstrate that grammatical development, regardless of its neural substrate, is highly constrained by UG and follows a narrowly determined course. We discuss our findings within a neurobiological framework in which etiology defines the integrity of the remaining hemisphere, which in turn, determines its potential for linguistic reorganization and/or acquisition. PMID:12921764

  19. Initiation and development of normal faults within the German alpine foreland basin: The inconspicuous role of basement structures

    NASA Astrophysics Data System (ADS)

    Hartmann, Hartwig; Tanner, David C.; Schumacher, Sandra

    2016-06-01

    In a large seismic cube within the German Alpine Molasse Basin, we recognize large normal faults with lateral alternating dips that displace the Molasse sediments. They are disconnected but strike parallel to fault lineaments of the underlying carbonate platform. This raises the question how such faults could independently develop. Structural analysis suggests that the faults grew both upward and downward from the middle of the Molasse package, i.e., they newly initiated within the Molasse sediments and were not caused by reactivation of the faults in the carbonate platform and/or crystalline basement. Numerical modeling of the basin proves that temporarily and spatially confined extensional stresses existed within the Molasse sediments but not in the carbonate platform and basement during lithospheric bending. The workflow shown here gives a new and as yet undocumented insight in the tectonic and structural processes within a foreland basin that was affected by buckling and bending in front of the orogen.

  20. (/sup 14/C)sucrose uptake and labeling of starch in developing grains of normal segl barley

    SciTech Connect

    Felker, F.C.; Peterson, D.M.; Nelson, O.E.

    1984-01-01

    Previous work showed that the segl mutant of barley (Hordeum vulgare o Betzes) did not differ from normal Betzes in plant growth, photosynthesis, or fertility, but it produced only shrunken seeds regardless of pollen source. To determine whether defects in sucrose uptake or starch synthesis resulted in the shrunken condition, developing grains of Betzes and segl were cultured in (/sup 14/C)sucrose solutions after slicing transversely to expose the endosperm cavity and free space. In both young grains (before genotypes differed in dry weight) and older grains (17 days after anthesis, when segl grains were smaller than Betzes), sucrose uptake and starch synthesis were similar in both genotypes on a dry weight basis. To determine if sucrose was hydrolyzed during uptake, spikes of Betzes and segl were allowed to take up (fructose-U-/sup 14/C)sucrose 14 days after anthesis and the radioactivity of endosperm sugars was examined during 3 hours of incubation. Whereas less total radioactivity entered the endosperm and the endosperm cavity (free space) of segl, in both genotypes over 96% of the label of endosperm sugars was in sucrose, and there was no apparent initial or progressive randomization of label among hexose moieties of sucrose as compared to the free space sampled after 1 hour of incubation. The authors conclude that segl endosperms are capable of normal sucrose uptake and starch synthesis and that hydrolysis of sucrose is not required for uptake in either genotype. Evidence suggests abnormal development of grain tissue of maternal origin during growth of segl grains.