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https://www.readbyqxmd.com/read/28098631/after-the-fall-the-tapestry-of-disturbance-and-recovery
#1
Nalini M Nadkarni
On July 3, 2015, Nalini Nadkarni, a world-renowned ecologist who had been studying the biologic processes of ecosystem disturbance and recovery, sustained a catastrophic 50-foot free-fall from the top of the rainforest canopy to the forest floor at her remote field research site. She lost consciousness in shock and sustained life-threatening injuries. Her accompanying students hiked out, radio-called 911, and the Harborview Medical Center (Seattle) Medivac team arrived 4 hours later to rescue her. Her prognosis was extremely grim; her family gathered in anticipation of her death as she underwent four operations during her 10 days in the ICU...
February 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28078312/biallelic-scn10a-mutations-in-neuromuscular-disease-and-epileptic-encephalopathy
#2
Marios Kambouris, Julien Thevenon, Ariane Soldatos, Allison Cox, Joshi Stephen, Tawfeg Ben-Omran, Yasser Al-Sarraj, Hala Boulos, William Bone, James C Mullikin, Alice Masurel-Paulet, Judith St-Onge, Yannis Dufford, Corrine Chantegret, Christel Thauvin-Robinet, Jamil Al-Alami, Laurence Faivre, Jean Baptiste Riviere, William A Gahl, Alexander G Bassuk, May Christine V Malicdan, Hatem El-Shanti
OBJECTIVES: Two consanguineous families, one of Sudanese ethnicity presenting progressive neuromuscular disease, severe cognitive impairment, muscle weakness, upper motor neuron lesion, anhydrosis, facial dysmorphism, and recurrent seizures and the other of Egyptian ethnicity presenting with neonatal hypotonia, bradycardia, and recurrent seizures, were evaluated for the causative gene mutation. METHODS AND RESULTS: Homozygosity mapping and whole exome sequencing (WES) identified damaging homozygous variants in SCN10A, namely c...
January 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/28077706/connexin-mediated-signaling-in-nonsensory-cells-is-crucial-for-the-development-of-sensory-inner-hair-cells-in-the-mouse-cochlea
#3
Stuart L Johnson, Federico Ceriani, Oliver Houston, Roman Polishchuk, Elena Polishchuk, Giulia Crispino, Veronica Zorzi, Fabio Mammano, Walter Marcotti
: Mutations in the genes encoding for gap junction proteins connexin 26 (Cx26) and connexin 30 (Cx30) have been linked to syndromic and nonsyndromic hearing loss in mice and humans. The release of ATP from connexin hemichannels in cochlear nonsensory cells has been proposed to be the main trigger for action potential activity in immature sensory inner hair cells (IHCs), which is crucial for the refinement of the developing auditory circuitry. Using connexin knock-out mice, we show that IHCs fire spontaneous action potentials even in the absence of ATP-dependent intercellular Ca(2+) signaling in the nonsensory cells...
January 11, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28074704/the-toxicology-of-zinc-chloride-smoke-producing-bombs-and-screens
#4
Ayman El Idrissi, Lisanne van Berkel, Nadia E Bonekamp, Diana J Z Dalemans, Marcel A G van der Heyden
CONTEXT: Zinc chloride (ZnCl2)-based smoke bombs and screens are in use since the Second World War (1939-1945). Many case descriptions on ZnCl2 smoke inhalation incidents appeared since 1945. OBJECTIVE: We provide a comprehensive overview of the clinical symptoms and underlying pathophysiology due to exposure to fumes from ZnCl2 smoke producing bombs. In addition, we give a historical overview of treatment regimens and their outcomes. METHODOLOGY: We performed a literature search on Medline, Scopus and Google Scholar databases using combinations of the following search terms "smoke bomb", "smoke screen", "ZnCl2", "intoxication", "poisoning", "case report", "HE smoke", "hexachloroethane smoke", "smoke inhalation" and "white smoke"...
January 11, 2017: Clinical Toxicology
https://www.readbyqxmd.com/read/28069353/effects-of-chronic-exposure-to-low-dose-thip-on-brainstem-neuronal-excitability-in-mouse-models-of-rett-syndrome-evidence-from-symptomatic-females
#5
Weiwei Zhong, Christopher Mychal Johnson, Ningren Cui, Hao Xing, Yang Wu, Chun Jiang
Rett Syndrome (RTT) is a neurodevelopmental disorder caused by mutations of the MECP2 gene, affecting predominantly females. One of the characteristic features of the disease is defective brainstem autonomic function. In Mecp2(-/Y) mice, several groups of brainstem neurons are overly excitable, which causes destabilization of neuronal networks for the autonomic control. We have previously shown that the extrasynaptic GABAA receptor agonist THIP relieves many RTT-like symptoms in Mecp2(-/Y) mice. Although neuronal activity is inhibited by acute THIP exposure, how a chronic treatment affects neuronal excitability remains elusive...
January 6, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28067582/the-relationship-of-hip-muscle-performance-to-leg-ankle-and-foot-injuries-a-systematic-review
#6
Nili Steinberg, Gali Dar, Martin Dunlop, James Edmund Gaida
OBJECTIVES: Hip control affects movement and muscle firing patterns in the leg, ankle and foot, and may contribute to overuse injuries. Muscle performance can be measured as strength, endurance or muscle activation patterns. Our objective was to systematically review whether hip muscle performance is associated with leg, ankle and foot injuries. DATA SOURCES: A structured and comprehensive search of six medical literature databases was combined with forward and backward citation tracking (AMED, CINAHL, EMBASE, Medline, Scopus and SportDiscus)...
January 9, 2017: Physician and Sportsmedicine
https://www.readbyqxmd.com/read/27919115/febrile-infection-related-epilepsy-syndrome-clinical-review-and-hypotheses-of-epileptogenesis
#7
Andreas van Baalen, Annamaria Vezzani, Martin Häusler, Gerhard Kluger
Febrile infection-related epilepsy syndrome (FIRES, AERRPS, or DESC) is one of the most severe, mostly irreversible, and presumably immune-mediated epileptic encephalopathies affecting healthy children. Refractory status epilepticus or a cluster of seizures start a few days after the onset of an acute febrile illness; however, encephalitis cannot be proved. Sequelae of FIRES are drug-resistant epilepsy and neuropsychological impairments occurring without latency. Clinical knowledge is limited because FIRES is sporadic and extremely rare...
December 5, 2016: Neuropediatrics
https://www.readbyqxmd.com/read/27910058/gene-delivery-for-the-generation-of-bioartificial-pacemaker
#8
Patrick K W Chan, Ronald A Li
Electronic pacemakers have been used in patients with heart rhythm disorders for device-supported pacing. While effective, there are such shortcomings as limited battery life, permanent implantation of catheters, the lack of autonomic neurohumoral responses, and risks of lead dislodging. Here we describe protocols for establishing porcine models of sick sinus syndrome and complete heart block, and the generation of bioartificial pacemaker by delivering a strategically engineered form of hyperpolarization-activated cyclic nucleotide-gated pacemaker channel protein via somatic gene transfer to convert atrial or ventricular muscle cardiomyocytes into nodal-like cells that rhythmically fire action potentials...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/27864337/connexin-mediated-signalling-in-non-sensory-cells-is-crucial-for-the-development-of-sensory-inner-hair-cells-in-the-mouse-cochlea
#9
Stuart L Johnson, Federico Ceriani, Oliver Houston, Roman Polishchuk, Elena Polishchuk, Giulia Crispino, Veronica Zorzi, Fabio Mammano, Walter Marcotti
: Mutations in the genes encoding for gap junction proteins connexin 26 (Cx26) and connexin 30 (Cx30) have been linked to syndromic and non-syndromic hearing loss in mice and humans. The release of ATP from connexin hemichannels in cochlear non-sensory cells has been proposed to be the main trigger for action potential (AP) activity in immature sensory inner hair cells (IHCs), which is crucial for the refinement of the developing auditory circuitry. Using connexin knockout mice, we show that IHCs fire spontaneous APs even in the absence of ATP-dependent intercellular Ca(2+) signalling in the non-sensory cells...
November 18, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/27856530/diagnostic-conundrum-lessons-from-the-man-who-accidently-set-fire-to-his-home
#10
Sahan Benedict Mendis, Victoria Lukats
A 62-year-old man presented to psychiatric services with a 3-month history of a range of symptoms which included obsessional thoughts, self-neglect, lack of mental flexibility, reduced ability to plan, organise and follow instructions, reduced capacity to empathise and disinhibition. He also accidently set fire to his house. Overall these findings are compatible with a dysexecutive syndrome. This man has a significant history of polysubstance misuse and chronic hepatitis C infection. Neuroimaging revealed an acquired traumatic brain injury which could account for his dysexecutive syndrome...
November 17, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27855662/microarray-expression-profile-analysis-of-mrnas-and-long-non-coding-rnas-in-pulmonary-tuberculosis-with-different-traditional-chinese-medicine-syndromes
#11
Ting-Ting Jiang, Li-Liang Wei, Li-Ying Shi, Zhong-Liang Chen, Chong Wang, Chang-Ming Liu, Zhong-Jie Li, Ji-Cheng Li
BACKGROUND: Combination chemotherapy with Western anti-tuberculosis (TB) drugs is the mainstay of TB treatment. Chinese herbal medicines with either heat clearing and detoxifying effects or nourishing Yin and reducing fire effects have been used to treat TB based on the Traditional Chinese Medicine (TCM) syndromes of TB patients. This study analyzed the expression profiles of long non-coding RNAs (lncRNAs) and mRNAs in TB patients with different TCM syndromes. METHODS: TB patients were classified as pulmonary Yin deficiency (PYD) syndrome, hyperactivity of fire due to Yin deficiency (HFYD) syndrome, and deficiency of Qi and Yin (DQY) syndrome...
November 17, 2016: BMC Complementary and Alternative Medicine
https://www.readbyqxmd.com/read/27805592/in-vitro-recording-of-mesenteric-afferent-nerve-activity-in-mouse-jejunal-and-colonic-segments
#12
Sara Nullens, Annemie Deiteren, Wen Jiang, Christopher Keating, Hannah Ceuleers, Sven Francque, David Grundy, Joris G De Man, Benedicte Y De Winter
Afferent nerves not only convey information concerning normal physiology, but also signal disturbed homeostasis and pathophysiological processes of the different organ systems from the periphery towards the central nervous system. As such, the increased activity or 'sensitization' of mesenteric afferent nerves has been allocated an important role in the pathophysiology of visceral hypersensitivity and abdominal pain syndromes. Mesenteric afferent nerve activity can be measured in vitro in an isolated intestinal segment that is mounted in a purpose-built organ bath and from which the splanchnic nerve is isolated, allowing researchers to directly assess nerve activity adjacent to the gastrointestinal segment...
October 25, 2016: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/27770579/febrile-infection-related-epilepsy-syndrome-treated-with-anakinra
#13
Daniel L Kenney-Jung, Annamaria Vezzani, Robert J Kahoud, Reghann G LaFrance-Corey, Mai-Lan Ho, Theresa Wampler Muskardin, Elaine C Wirrell, Charles L Howe, Eric T Payne
Febrile infection-related epilepsy syndrome (FIRES) is a devastating epileptic encephalopathy with limited treatment options and an unclear etiology. Anakinra is a recombinant version of the human interleukin-1 receptor antagonist used to treat autoinflammatory disorders. This is the first report of anakinra for treatment of a child with super-refractory status epilepticus secondary to FIRES. Anakinra was well tolerated and effective. Cerebral spinal fluid analysis revealed elevated levels of proinflammatory cytokines before treatment that normalized on anakinra, suggesting a potential pathogenic role for neuroinflammation in FIRES...
December 2016: Annals of Neurology
https://www.readbyqxmd.com/read/27767173/euchromatin-histone-methyltransferase-1-regulates-cortical-neuronal-network-development
#14
Marijn Bart Martens, Monica Frega, Jessica Classen, Lisa Epping, Elske Bijvank, Marco Benevento, Hans van Bokhoven, Paul Tiesinga, Dirk Schubert, Nael Nadif Kasri
Heterozygous mutations or deletions in the human Euchromatin histone methyltransferase 1 (EHMT1) gene cause Kleefstra syndrome, a neurodevelopmental disorder that is characterized by autistic-like features and severe intellectual disability (ID). Neurodevelopmental disorders including ID and autism may be related to deficits in activity-dependent wiring of brain circuits during development. Although Kleefstra syndrome has been associated with dendritic and synaptic defects in mice and Drosophila, little is known about the role of EHMT1 in the development of cortical neuronal networks...
October 21, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27753838/sy-06-1-obesity-diabetes-and-hypertension-are-adipokines-responsible
#15
Anthony Heagerty
There is now a considerable body of evidence to suggest that the fat cells that surround blood vessels (perivascular adipose tissue, PVAT) can influence profoundly arterial tone by releasing vasodilator adipokines which can act locally in a paracrine fashion. In healthy lean individuals the primary vasodilator released appears to be adiponectin and there is a complex interation between autonomic nerve firing in PVAT and the release of nitric oxide from adipocytes and an increased bioavailability of adiponectin...
September 2016: Journal of Hypertension
https://www.readbyqxmd.com/read/27704337/modeling-the-differentiation-of-a-and-c-type-baroreceptor-firing-patterns
#16
Jacob Sturdy, Johnny T Ottesen, Mette S Olufsen
The baroreceptor neurons serve as the primary transducers of blood pressure for the autonomic nervous system and are thus critical in enabling the body to respond effectively to changes in blood pressure. These neurons can be separated into two types (A and C) based on the myelination of their axons and their distinct firing patterns elicited in response to specific pressure stimuli. This study has developed a comprehensive model of the afferent baroreceptor discharge built on physiological knowledge of arterial wall mechanics, firing rate responses to controlled pressure stimuli, and ion channel dynamics within the baroreceptor neurons...
October 5, 2016: Journal of Computational Neuroscience
https://www.readbyqxmd.com/read/27703430/early-impairment-of-synaptic-and-intrinsic-excitability-in-mice-expressing-als-dementia-linked-mutant-ubqln2
#17
Daniel Radzicki, Erdong Liu, Han-Xiang Deng, Teepu Siddique, Marco Martina
Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) are believed to represent the different outcomes of a common pathogenic mechanism. However, while researchers have intensely studied the involvement of motor neurons in the ALS/FTD syndrome, very little is known about the function of hippocampal neurons, although this area is critical for memory and other cognitive functions. We investigated the electrophysiological properties of CA1 pyramidal cells in slices from 1 month-old UBQLN2(P497H) mice, a recently generated model of ALS/FTD that shows heavy depositions of ubiquilin2-positive aggregates in this brain region...
2016: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/27688999/mechanisms-underlying-prorenin-actions-on-hypothalamic-neurons-implicated-in-cardiometabolic-control
#18
Soledad Pitra, Yumei Feng, Javier E Stern
BACKGROUND: Hypertension and obesity are highly interrelated diseases, being critical components of the metabolic syndrome. Despite the growing prevalence of this syndrome in the world population, efficient therapies are still missing. Thus, identification of novel targets and therapies are warranted. An enhanced activity of the hypothalamic renin-angiotensin system (RAS), including the recently discovered prorenin (PR) and its receptor (PRR), has been implicated as a common mechanism underlying aberrant sympatho-humoral activation that contributes to both metabolic and cardiovascular dysregulation in the metabolic syndrome...
October 2016: Molecular Metabolism
https://www.readbyqxmd.com/read/27670841/hyperexcitability-of-mesencephalic-trigeminal-neurons-and-reorganization-of-ion-channel-expression-in-a-rett-syndrome-model
#19
Max F Oginsky, Ningren Cui, Weiwei Zhong, Christopher M Johnson, Chun Jiang
People with Rett syndrome (RTT) have defects in motor function also seen in Mecp2-null mice. Motor function depends on not only central motor commands but also sensory feedback that is vulnerable to changes in excitability of propriosensory neurons. Here we report evidence for hyperexcitability of mesencephalic trigeminal (Me5) neurons in Mecp2-null mice and a novel cellular mechanism for lowering its impact. In in vitro brain slices, the Me5 neurons in both Mecp2(-/Y) male and symptomatic Mecp2(+/-) female mice were overly excitable showing increased firing activity in comparison to their wild-type (WT) male and asymptomatic counterparts...
May 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/27642933/sy-06-1-obesity-diabetes-and-hypertension-are-adipokines-responsible
#20
Anthony Heagerty
There is now a considerable body of evidence to suggest that the fat cells that surround blood vessels (perivascular adipose tissue, PVAT) can influence profoundly arterial tone by releasing vasodilator adipokines which can act locally in a paracrine fashion. In healthy lean individuals the primary vasodilator released appears to be adiponectin and there is a complex interation between autonomic nerve firing in PVAT and the release of nitric oxide from adipocytes and an increased bioavailability of adiponectin...
September 2016: Journal of Hypertension
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