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Behavioral neurology

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https://www.readbyqxmd.com/read/28918504/autism-spectrum-disorders-and-disease-modeling-using-stem-cells
#1
REVIEW
Anita Brito, Fabiele Baldino Russo, Alysson Renato Muotri, Patricia Cristina Baleeiro Beltrão-Braga
Autism spectrum disorders (ASD) represent a variety of disorders characterized as complex lifelong neurodevelopment disabilities, which may affect the ability of communication and socialization, including typical comportments like repetitive and stereotyped behavior. Other comorbidities are usually present, such as echolalia, hypotonia, intellectual disability and difficulties in processing figured speech. Furthermore, some ASD individuals may present certain abilities, such as eidetic memory, outstanding musical or painting talents and special mathematical skills, among others...
September 16, 2017: Cell and Tissue Research
https://www.readbyqxmd.com/read/28916806/rewiring-of-neuronal-networks-during-synaptic-silencing
#2
Jana Katharina Wrosch, Vicky von Einem, Katharina Breininger, Marc Dahlmanns, Andreas Maier, Johannes Kornhuber, Teja Wolfgang Groemer
Analyzing the connectivity of neuronal networks, based on functional brain imaging data, has yielded new insight into brain circuitry, bringing functional and effective networks into the focus of interest for understanding complex neurological and psychiatric disorders. However, the analysis of network changes, based on the activity of individual neurons, is hindered by the lack of suitable meaningful and reproducible methodologies. Here, we used calcium imaging, statistical spike time analysis and a powerful classification model to reconstruct effective networks of primary rat hippocampal neurons in vitro...
September 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28916543/cryopyrin-associated-periodic-syndromes-in-italian-patients-evaluation-of-the-rate-of-somatic-nlrp3-mosaicism-and-phenotypic-characterization
#3
Denise Lasigliè, Anna Mensa-Vilaro, Denise Ferrera, Roberta Caorsi, Federica Penco, Giuseppe Santamaria, Marco Di Duca, Giulia Amico, Kenji Nakagawa, Francesca Antonini, Alberto Tommasini, Rita Consolini, Antonella Insalaco, Marco Cattalini, Laura Obici, Romina Gallizzi, Francesca Santarelli, Genny Del Zotto, Mariasavina Severino, Anna Rubartelli, Roberto Ravazzolo, Alberto Martini, Isabella Ceccherini, Ryuta Nishikomori, Marco Gattorno, Juan I Arostegui, Silvia Borghini
OBJECTIVE: To evaluate the rate of somatic NLRP3 mosaicism in an Italian cohort of mutation-negative patients with cryopyrin-associated periodic syndrome (CAPS). METHODS: The study enrolled 14 patients with a clinical phenotype consistent with CAPS in whom Sanger sequencing of the NLRP3 gene yielded negative results. Patients' DNA were subjected to amplicon-based NLRP3 deep sequencing. RESULTS: Low-level somatic NLRP3 mosaicism has been detected in 4 patients, 3 affected with chronic infantile neurological cutaneous and articular syndrome and 1 with Muckle-Wells syndrome...
September 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28911972/sex-specific-effects-of-developmental-lead-exposure-on-the-immune-neuroendocrine-network
#4
REVIEW
Jane Kasten-Jolly, David A Lawrence
The environmental toxicant lead (Pb) has long been known to induce neurological deficits. The 1st century Greek physician Pedanius Dioscorides noted that "lead makes the mind give way". Current studies are suggesting the effects of Pb on behaviors may involve the immune system and conversely some immunomodulatory changes may be due to Pb effects in the central nervous system. Although Pb-induced disorders do not appear to discriminate among females and males, this report discusses the differences observed in human and animal studies regarding differential gender effects on gene expression after Pb exposure...
September 11, 2017: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/28910378/neuroinflammation-myelin-and-behavior-temporal-patterns-following-mild-traumatic-brain-injury-in-mice
#5
Toufik Taib, Claire Leconte, Juliette Van Steenwinckel, Angelo H Cho, Bruno Palmier, Egle Torsello, Rene Lai Kuen, Somfieme Onyeomah, Karine Ecomard, Chiara Benedetto, Bérard Coqueran, Anne-Catherine Novak, Edwige Deou, Michel Plotkine, Pierre Gressens, Catherine Marchand-Leroux, Valérie C Besson
Traumatic brain injury (TBI) results in white matter injury (WMI) that is associated with neurological deficits. Neuroinflammation originating from microglial activation may participate in WMI and associated disorders. To date, there is little information on the time courses of these events after mild TBI. Therefore we investigated (i) neuroinflammation, (ii) WMI and (iii) behavioral disorders between 6 hours and 3 months after mild TBI. For that purpose, we used experimental mild TBI in mice induced by a controlled cortical impact...
2017: PloS One
https://www.readbyqxmd.com/read/28905935/age-related-differences-in-interferon-regulatory-factor-4-and-5-signaling-in-ischemic-brains-of-mice
#6
Shou-Cai Zhao, Chun Wang, Heng Xu, Wen-Qian Wu, Zhao-Hu Chu, Ling-Song Ma, Ying-Dong Zhang, Fudong Liu
Stroke is a disease that mainly affects the elderly. Since the age-related differences in stroke have not been well studied, modeling stroke in aged animals is clinically more relevant. The inflammatory responses to stroke are a fundamental pathological procedure, in which microglial activation plays an important role. Interferon regulatory factor-5 (IRF5) and IRF4 regulate M1 and M2 activation of macrophages, respectively, in peripheral inflammation; but it is unknown whether IRF5/IRF4 are also involved in cerebral inflammatory responses to stroke and whether age-related differences of the IRF5/IRF4 signaling exist in ischemic brain...
September 14, 2017: Acta Pharmacologica Sinica
https://www.readbyqxmd.com/read/28904513/autoimmune-thyroiditis-presenting-as-psychosis
#7
Soumitra DAS, Nimisha Doval, Vikas Moun
Hashimoto's thyroiditis is a rare condition associated mainly with neurological symptoms. It contains an abundant amount of auto-antibodies in the blood. Only a few cases of behavioral symptoms without significant neurological disturbances have been recorded in the literature. In this view, our case is unique as it was not associated with overt hypothyroid manifestations.
June 25, 2017: Shanghai Archives of Psychiatry
https://www.readbyqxmd.com/read/28900300/rogdi-defines-gabaergic-control-of-a-wake-promoting-dopaminergic-pathway-to-sustain-sleep-in-drosophila
#8
Minjong Kim, Donghoon Jang, Eunseok Yoo, Yangkyun Oh, Jun Young Sonn, Jongbin Lee, Yoonhee Ki, Hyo Jin Son, Onyou Hwang, Changwook Lee, Chunghun Lim, Joonho Choe
Kohlschutter-Tönz syndrome (KTS) is a rare genetic disorder with neurological dysfunctions including seizure and intellectual impairment. Mutations at the Rogdi locus have been linked to development of KTS, yet the underlying mechanisms remain elusive. Here we demonstrate that a Drosophila homolog of Rogdi acts as a novel sleep-promoting factor by supporting a specific subset of gamma-aminobutyric acid (GABA) transmission. Rogdi mutant flies displayed insomnia-like behaviors accompanied by sleep fragmentation and delay in sleep initiation...
September 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28899758/chronic-global-analysis-of-vascular-permeability-and-cerebral-blood-flow-after-bone-marrow-stromal-cell-treatment-of-traumatic-brain-injury-in-the-rat-a-long-term-mri-study
#9
Lian Li, Michael Chopp, Guangliang Ding, Qingjiang Li, Asim Mahmood, Quan Jiang
Vascular permeability and hemodynamic alteration in response to the transplantation of human bone marrow stromal cells (hMSCs) after traumatic brain injury (TBI) were longitudinally investigated in non directly injured and normal-appearing cerebral tissue using magnetic resonance imaging (MRI). Male Wistar rats (300-350g, n=30) subjected to controlled cortical impact TBI were intravenously injected with 1 ml of saline (at 6-hours or 1-week post-injury, n=5/group) or with hMSCs in suspension (∼3x10(6) hMSCs, at 6-hours or 1-week post-injury, n=10/group)...
September 9, 2017: Brain Research
https://www.readbyqxmd.com/read/28899744/neural-and-metabolic-basis-of-dynamic-resting-state-fmri
#10
REVIEW
Garth J Thompson
Resting state fMRI (rsfMRI) as a technique showed much initial promise for use in psychiatric and neurological diseases where diagnosis and treatment were difficult. To realize this promise, many groups have moved towards examining "dynamic rsfMRI," which relies on the assumption that rsfMRI measurements on short time scales remain relevant to the underlying neural and metabolic activity. Many dynamic rsfMRI studies have demonstrated differences between clinical or behavioral groups beyond what static rsfMRI measured, suggesting a neurometabolic basis...
September 9, 2017: NeuroImage
https://www.readbyqxmd.com/read/28898175/an-unusual-presentation-of-creutzfeldt-jakob-disease-and-an-example-of-how-hickam-s-dictum-and-ockham-s-razor-can-both-be-right
#11
Eli S Neiman, Amtul Farheen, Nancy Gadallah, Thomas Steineke, Peter Parsells, Zev A Kizelnik, Michael Rosenberg
Patients can have more than one neurological problem, and sorting out acute from chronic disease can be challenging. The authors report a middle-aged patient who presented with ataxia, right hemiparesis, and abnormal nystagmus. Magnetic resonance imaging (MRI) showed a Chiari and an arachnoid cyst with brainstem compression that appeared to explain his abnormal examination. Shortly after admission, he was noted to have intermittent abnormal behavior and confusion. History from family revealed significant acute and chronic psychiatric problems that appeared to explain his abnormal mental status; this delayed the diagnosis of intermittent complex partial seizures...
2017: Neurodiagnostic Journal
https://www.readbyqxmd.com/read/28898133/differential-impact-of-treadmill-training-on-stroke-induced-neurological-disorders
#12
Rasoul Rezaei, Maryam Nourshahi, Fariba Khodagholi, Abbas Haghparast, Sanaz Nasoohi, Mohammadreza Bigdeli, Ghorbangol Ashabi
OBJECTIVE: Physical exercise contributes to improving stability against nerve injury caused by ischaemic stroke. Here we aimed to preliminarily investigate the effects of continuous endurance training (CET) and high-intensity interval training (HIT) on stroke-associated anxiety, locomotion, neurological assessments and P70S6 Kinase (P70S6K) activation as well. To do this, rats were trained according to HIT and CET protocols for 2 months prior to being subject to middle cerebral artery occlusion surgery...
September 12, 2017: Brain Injury: [BI]
https://www.readbyqxmd.com/read/28894950/autism-spectrum-disorder-an-early-and-frequent-feature-in-cerebrotendinous-xanthomatosis
#13
Bianca M L Stelten, Olivier Bonnot, Hidde H Huidekoper, Francjan J van Spronsen, Peter M van Hasselt, Leo A J Kluijtmans, Ron A Wevers, Aad Verrips
BACKGROUND: Cerebrotendinous xanthomatosis (CTX) is an autosomal recessively inherited inborn error of metabolism (IEM) due to mutations in the CYP27A1 gene. The clinical picture ranges from being nearly asymptomatic in early childhood, up to severe disability at adult age. Infantile-onset diarrhea and juvenile-onset cataract are the earliest symptoms in childhood. In the current study, we evaluated the presence of autism spectrum disorder (ASD) in a large cohort of CTX patients. METHODS: We performed a retrospective patient file study in 77 genetically confirmed Dutch CTX patients to determine the frequency of ASD...
September 11, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28894819/computational-modelling-of-cough-function-and-airway-penetrant-behavior-in-patients-with-disorders-of-laryngeal-function
#14
Bari Hoffman Ruddy, Don Nadun Kuruppumullage, Giselle Carnaby, Michael Crary, Jeffery Lehman, Olusegun J Ilegbusi
OBJECTIVE/HYPOTHESIS: Patients with laryngeal disorders often exhibit changes to cough function contributing to aspiration episodes. Two primary cough variables (peak cough flow: PCF and compression phase duration: CPD) were examined within a biomechanical model to determine their impact on characteristics that impact airway compromise. STUDY DESIGN: Computational study. METHODS: A Computational Fluid Dynamics (CFD) technique was used to simulate fluid flow within an upper airway model reconstructed from patient CT images...
February 2017: Laryngoscope Investigative Otolaryngology
https://www.readbyqxmd.com/read/28891432/neonatal-treatment-with-ovarian-hormones-and-suckling-among-distinct-litter-sizes-differential-effects-on-recognition-memory-and-spreading-depression-at-adulthood
#15
Noranege Epifânio Accioly, Rubem Carlos Araújo Guedes
OBJECTIVES: Ovarian hormones (OH) and early malnutrition may affect the developing brain, with repercussions on behavioral and excitability-dependent processes. However, the possible synergistic effects of both factors have not been analyzed. In this study, we investigated the effect of treatment in early life with OH and suckling among distinct litter sizes on recognition memory, anxiety behavior, and the excitability-dependent phenomenon known as cortical spreading depression (CSD)...
September 10, 2017: Nutritional Neuroscience
https://www.readbyqxmd.com/read/28891034/rheb1-insufficiency-in-aged-male-mice-is-associated-with-stress-induced-seizures
#16
Qi Tian, Pavel Gromov, Joachim H Clement, Yingming Wang, Marc Riemann, Falk Weih, Xiao-Xin Sun, Mu-Shui Dai, Lev M Fedorov
The mechanistic target of rapamycin (mTOR), a protein kinase, is a central regulator of mammalian metabolism and physiology. Protein mTOR complex 1 (mTORC1) functions as a major sensor for the nutrient, energy, and redox state of a cell and is activated by ras homolog enriched in brain (RHEB1), a GTP-binding protein. Increased activation of mTORC1 pathway has been associated with developmental abnormalities, certain form of epilepsy (tuberous sclerosis), and cancer. Clinically, those mTOR-related disorders are treated with the mTOR inhibitor rapamycin and its rapalogs...
September 10, 2017: GeroScience
https://www.readbyqxmd.com/read/28890690/a-cohesive-framework-for-motor-stereotypy-in-typical-and-atypical-development-the-role-of-sensorimotor-integration
#17
Robin L Shafer, Karl M Newell, Mark H Lewis, James W Bodfish
Stereotyped motor behavior manifests as rhythmic, repetitive movements. It is common in several neurologic and psychiatric disorders where it is considered maladaptive. However, it also occurs early in typical development where it serves an adaptive function in the development of complex, controlled motor behavior. Currently, no framework accounts for both adaptive and maladaptive forms of motor stereotypy. We propose a conceptual model that implicates sensorimotor mechanisms in the phenomenology of adaptive and maladaptive stereotypy...
2017: Frontiers in Integrative Neuroscience
https://www.readbyqxmd.com/read/28890029/natural-course-and-predictors-of-severe-disability-and-death-in-thai-patients-with-dementia
#18
Pornpatr A Dharmasaroja, Praween Lolekha, Kongkiat Kulkantrakorn, Thammanard Charernboon, Arvemas Watcharakorn, Pritsana Piyabhan
More than half of patients with dementia lived in countries with low and middle incomes. However, there have been few studies on the natural course of disease in these countries. The purpose of this study was to study the natural course and the predictive factors of advanced stage and death in Thai patients with dementia. Patients with dementia who were treated in neurologic and psychiatric clinic from September 2004 to February 2016, were included. Data about natural course of diseases, behavioral and psychological symptoms in dementia (BPSD) and complications were studied...
September 7, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28889962/neurobehavioral-outcomes-in-autoimmune-encephalitis
#19
Anusha K Yeshokumar, Eliza Gordon-Lipkin, Ana Arenivas, Jesse Cohen, Arun Venkatesan, Deanna Saylor, John C Probasco
This study evaluates long-term neurobehavioral function in patients with clinically diagnosed autoimmune encephalitis (AE) of various etiologies through retrospective chart review and a cross-sectional structured telephone interview. Of 77 patients meeting clinical diagnostic criteria for AE over a ten year period, 39/77 (51%) patients had known AE-associated antibodies, and 38/77 (49%) had no detected antibody. 9/77 (12%) died, and 26/77 (34%) had "poor" neurologic disability score (mRS 3-5) at the last documented follow-up...
August 31, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28889260/developmental-neurotoxicity-of-lead
#20
Samuel Caito, Michael Aschner
Lead exposure is a major concern for the developing nervous system. Environmental exposures to lead, predominantly from contaminated water or lead paint chips, account for the majority of exposures to children. In utero and early life exposures to lead have been associated with lower IQ, antisocial and delinquent behaviors, and attention-deficit hyperactivity disorder. In this review, we will discuss sources of developmental lead exposure and mechanisms of lead neurotoxicity. We will highlight both human epidemiological studies showing associations between lead exposure and behavioral abnormalities as well as experimental data from animal studies...
2017: Advances in Neurobiology
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