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https://www.readbyqxmd.com/read/29026661/tenth-case-of-bilateral-hemifacial-spasm-treated-by-microvascular-decompression-review-of-the-pathophysiology
#1
REVIEW
Warley Carvalho da Silva Martins, Lucas Alverne Freitas de Albuquerque, Gervásio Teles Cardoso de Carvalho, Jules Carlos Dourado, Marcos Dellaretti, Atos Alves de Sousa
BACKGROUND: Bilateral hemifacial spasm (BHFS) is a rare neurological syndrome whose diagnosis depends on excluding other facial dyskinesias. We present a case of BHFS along with a literature review. METHODS: A 64-year-old white, hypertense male reported involuntary left hemiface contractions in 2001 (aged 50). In 2007, right hemifacial symptoms appeared, without spasm remission during sleep. Botulinum toxin type A application produced partial temporary improvement...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/29019321/beyond-excitation-inhibition-imbalance-in-multidimensional-models-of-neural-circuit-changes-in-brain-disorders
#2
Cian O'Donnell, J Tiago Gonçalves, Carlos Portera-Cailliau, Terrence J Sejnowski
A leading theory holds that neurodevelopmental brain disorders arise from imbalances in excitatory and inhibitory (E/I) brain circuitry. However, it is unclear whether this one-dimensional model is rich enough to capture the multiple neural circuit alterations underlying brain disorders. Here we combined computational simulations with analysis of in vivo 2-photon Ca(2+) imaging data from somatosensory cortex of Fmr1 knock-out (KO) mice, a model of Fragile-X Syndrome, to test the E/I imbalance theory. We found that: 1) The E/I imbalance model cannot account for joint alterations in the observed neural firing rates and correlations; 2) Neural circuit function is vastly more sensitive to changes in some cellular components over others; 3) The direction of circuit alterations in Fmr1 KO mice changes across development...
October 11, 2017: ELife
https://www.readbyqxmd.com/read/28938422/prepubertal-development-of-gonadotropin-releasing-hormone-gnrh-neuron-activity-is-altered-by-sex-age-and-prenatal-androgen-exposure
#3
Eden A Dulka, Suzanne M Moenter
Gonadotropin-releasing hormone (GnRH) neurons regulate reproduction though pulsatile hormone release. Disruption of GnRH release as measured via LH pulses occurs in polycystic ovary syndrome (PCOS), and in young hyperandrogenemic girls. In adult prenatally androgenized (PNA) mice, which exhibit many aspects of PCOS, increased LH is associated with increased GnRH neuron action potential firing. How GnRH neuron activity develops over the prepubertal period and whether or not this is altered by sex or prenatal androgen treatment are unknown...
September 15, 2017: Endocrinology
https://www.readbyqxmd.com/read/28931075/excitability-is-increased-in-hippocampal-ca1-pyramidal-cells-of-fmr1-knockout-mice
#4
M Angeles Luque, Pablo Beltran-Matas, M Carmen Marin, Blas Torres, Luis Herrero
Fragile X syndrome (FXS) is caused by a failure of neuronal cells to express the gene encoding the fragile mental retardation protein (FMRP). Clinical features of the syndrome include intellectual disability, learning impairment, hyperactivity, seizures and anxiety. Fmr1 knockout (KO) mice do not express FMRP and, as a result, reproduce some FXS behavioral abnormalities. While intrinsic and synaptic properties of excitatory cells in various part of the brain have been studied in Fmr1 KO mice, a thorough analysis of action potential characteristics and input-output function of CA1 pyramidal cells in this model is lacking...
2017: PloS One
https://www.readbyqxmd.com/read/28917552/crispr-correction-of-the-prkag2-gene-mutation-in-a-patient-s-induced-pluripotent-stem-cell-derived-cardiomyocytes-eliminates-electrophysiological-and-structural-abnormalities
#5
Ronen Ben Jehuda, Binyamin Eisen, Yuval Shemer, Lucy N Mekies, Agnes Szantai, Irina Reiter, Huanhuan Cui, Kaomei Guan, Shiraz Haron-Khun, Dov Freimark, Silke R Sperling, Mihaela Gherghiceanu, Michael Arad, Ofer Binah
BACKGROUND: Mutations in the PRKAG2 gene encoding the γ-subunit of adenosine monophosphate kinase (AMPK) cause hypertrophic cardiomyopathy (HCM) and familial Wolff-Parkinson-White (WPW) syndrome. Patients carrying the R302Q mutation in PRKAG2 present with sinus bradycardia, escape rhythms, ventricular preexcitation, supraventricular tachycardia, and atrioventricular block. This mutation affects AMPK activity and increases glycogen storage in cardiomyocytes. The link between glycogen storage, WPW syndrome, HCM, and arrhythmias remains unknown...
September 14, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28898171/febrile-infection-related-epilepsy-syndrome-fires-a-literature-review-and-case-study
#6
Kristy Fox, Mary Ellen Wells, Michael Tennison, Bradley Vaughn
Febrile infection-related epilepsy syndrome (FIRES) is a catastrophic epileptic syndrome that strikes previously healthy children aged 3-15 years and has an unknown pathogenesis and few treatments. These children experience a nonspecific febrile illness that is followed by prolonged refractory status epilepticus. Although the etiology is unknown, FIRES has a biphasic presentation, with the acute phase beginning as seizure activity lasting 1-12 weeks, then followed by the chronic phase, which is characterized by refractory seizures that cluster every 2-4 weeks, and may continue to be multifocal and independent...
2017: Neurodiagnostic Journal
https://www.readbyqxmd.com/read/28893864/the-beach-protein-lrba-is-required-for-hair-bundle-maintenance-in-cochlear-hair-cells-and-for-hearing
#7
Christian Vogl, Tanvi Butola, Natja Haag, Torben J Hausrat, Michael G Leitner, Michel Moutschen, Philippe P Lefèbvre, Carsten Speckmann, Lillian Garrett, Lore Becker, Helmut Fuchs, Martin Hrabe de Angelis, Sandor Nietzsche, Michael M Kessels, Dominik Oliver, Matthias Kneussel, Manfred W Kilimann, Nicola Strenzke
Lipopolysaccharide-responsive beige-like anchor protein (LRBA) belongs to the enigmatic class of BEACH domain-containing proteins, which have been attributed various cellular functions, typically involving intracellular protein and membrane transport processes. Here, we show that LRBA deficiency in mice leads to progressive sensorineural hearing loss. In LRBA knockout mice, inner and outer hair cell stereociliary bundles initially develop normally, but then partially degenerate during the second postnatal week...
September 11, 2017: EMBO Reports
https://www.readbyqxmd.com/read/28869495/what-can-synergetics-contribute-to-embodied-aesthetics
#8
Hermann Haken
I deal with perception and action (e.g., movements) using results from synergetics, a comprehensive mathematical theory of the self-organized formation (emergence) of spatial, temporal, or functional structures in complex systems. I illustrate basic concepts such as order parameters (OPs), enslavement, complexity reduction, circular causality first by examples of well-known collective, spontaneous modes of human behavior such as rhythmic clapping of hands, and so forth, and then by face recognition. The role played by OPs depends on context...
September 2, 2017: Behavioral Sciences
https://www.readbyqxmd.com/read/28855130/pten-deletion-increases-hippocampal-granule-cell-excitability-in-male-and-female-mice
#9
Victor R Santos, Raymund Y K Pun, Salwa R Arafa, Candi L LaSarge, Shane Rowley, Shadi Khademi, Tom Bouley, Katherine D Holland, Norberto Garcia-Cairasco, Steve C Danzer
Deletion of the mTOR pathway inhibitor PTEN from postnatally-generated hippocampal dentate granule cells causes epilepsy. Here, we conducted field potential, whole cell recording and single cell morphology studies to begin to elucidate the mechanisms by which granule cell-specific PTEN-loss produces disease. Cells from both male and female mice were recorded to identify sex-specific effects. PTEN knockout granule cells showed altered intrinsic excitability, evident as a tendency to fire in bursts. PTEN knockout granule cells also exhibited increased frequency of spontaneous excitatory synaptic currents (sEPSCs) and decreased frequency of inhibitory currents (sIPSCs), further indicative of a shift towards hyperexcitability...
September 20, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28734788/neuropathic-pain-in-experimental-autoimmune-neuritis-is-associated-with-altered-electrophysiological-properties-of-nociceptive-drg-neurons
#10
Omneya Taha, Thoralf Opitz, Marcus Mueller, Julika Pitsch, Albert Becker, Bernd Oliver Evert, Heinz Beck, Monika Jeub
Guillain-Barré syndrome (GBS) is an acute, immune-mediated polyradiculoneuropathy characterized by rapidly progressive paresis and sensory disturbances. Moderate to severe and often intractable neuropathic pain is a common symptom of GBS, but its underlying mechanisms are unknown. Pathology of GBS is classically attributed to demyelination of large, myelinated peripheral fibers. However, there is increasing evidence that neuropathic pain in GBS is associated with impaired function of small, unmyelinated, nociceptive fibers...
November 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28730852/cannabinoid-hyperemesis-syndrome-and-topical-capsaicin-treating-smoke-with-fire-the-authors-reply
#11
Zachary Hafez, Michael E Mullins, Eike Blohm, Mark J O'Connor, Laurel Dezieck, Evan S Schwarz, Albert Conicella
No abstract text is available yet for this article.
July 21, 2017: Clinical Toxicology
https://www.readbyqxmd.com/read/28681715/motor-cortex-trns-improves-pain-affective-and-cognitive-impairment-in-patients-with-fibromyalgia-preliminary-results-of-a-randomised-sham-controlled-trial
#12
RANDOMIZED CONTROLLED TRIAL
Massimiliano Curatolo, Giuseppe La Bianca, Giuseppe Cosentino, Roberta Baschi, Giuseppe Salemi, Rossella Talotta, Marcello Romano, Giovanni Triolo, Marina De Tommaso, Brigida Fierro, Filippo Brighina
OBJECTIVES: Fibromyalgia (FM) is a clinical syndrome characterised by widespread musculoskeletal pain, chronic fatigue, cognitive deficits, and sleep and mood disorders. The effectiveness of most pharmacological treatments is limited, and there is a need for new, effective and well-tolerated therapies. It has recently been shown that transcranial direct-current stimulation (tDCS) of the motor cortex reduces pain, and that tDCS of the dorso-lateral prefrontal cortex (DLPFC) improves anxiety, depression and cognitive impairment in FM patients...
May 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28681654/cannabinoid-hyperemesis-syndrome-and-topical-capsaicin-treating-smoke-with-fire
#13
Josh J Wang, Eric Villeneuve, Jacques-Alexandre Amiel, Sophie Gosselin
No abstract text is available yet for this article.
July 6, 2017: Clinical Toxicology
https://www.readbyqxmd.com/read/28674175/enhanced-excitatory-connectivity-and-disturbed-sound-processing-in-the-auditory-brainstem-of-fragile-x-mice
#14
Elisabet Garcia-Pino, Nikodemus Gessele, Ursula Koch
Hypersensitivity to sounds is one of the prevalent symptoms in individuals with Fragile X syndrome (FXS). It manifests behaviorally early during development and is often used as a landmark for treatment efficacy. However, the physiological mechanisms and circuit-level alterations underlying this aberrant behavior remain poorly understood. Using the mouse model of FXS (Fmr1 KO), we demonstrate that functional maturation of auditory brainstem synapses is impaired in FXS. Fmr1 KO mice showed a greatly enhanced excitatory synaptic input strength in neurons of the lateral superior olive (LSO), a prominent auditory brainstem nucleus, which integrates ipsilateral excitation and contralateral inhibition to compute interaural level differences...
August 2, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28651475/high-frequency-ventilation-modalities-as-salvage-therapy-for-smoke-inhalation-associated-acute-lung-injury-a-systematic-review
#15
Andrew C Miller, Paula A Ferrada, Sameer S Kadri, Krupa Nataraj-Bhandari, Amir Vahedian-Azimi, Sadeq A Quraishi
BACKGROUND: Smoke inhalation-associated acute lung injury (SI-ALI) is a major cause of morbidity and mortality in victims of fire tragedies. To date, there are no evidence-based guidelines on ventilation strategies in acute respiratory distress syndrome (ARDS) after smoke inhalation. We reviewed the existing literature for clinical studies of salvage mechanical ventilation (MV) strategies in patients with SI-ALI, focusing on mortality and pneumonia as outcomes. METHODS: A systematic search was designed in accordance with preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines...
January 1, 2017: Journal of Intensive Care Medicine
https://www.readbyqxmd.com/read/28606559/adiponectin-at-physiological-level-glucose-independently-enhances-inhibitory-postsynaptic-current-onto-npy-neurons-in-the-hypothalamic-arcuate-nucleus
#16
Shigetomo Suyama, Wang Lei, Naoto Kubota, Takashi Kadowaki, Toshihiko Yada
Adiponectin regulates glucose and lipid metabolism, acting against atherosclerosis and metabolic syndrome. Accumulating evidences suggest that adiponectin acts on the brain including the arcuate nucleus of hypothalamus (ARC). The ARC contains orexigenic neuropeptide Y (NPY)/agouti related peptide (AgRP) neurons and anorexigenic proopiomelanocortin (POMC) neurons, the first order neurons for feeding regulation. We recently reported that intracerebroventricular injection of adiponectin at low glucose level suppressed food intake, while at elevated glucose level it promoted food intake, exhibiting glucose-dependent reciprocal effects...
March 22, 2017: Neuropeptides
https://www.readbyqxmd.com/read/28589518/forty-years-of-sodium-channels-structure-function-pharmacology-and-epilepsy
#17
William A Catterall
Voltage-gated sodium channels initiate action potentials in brain neurons. In the 1970s, much was known about the function of sodium channels from measurements of ionic currents using the voltage clamp method, but there was no information about the sodium channel molecules themselves. As a postdoctoral fellow and staff scientist at the National Institutes of Health, I developed neurotoxins as molecular probes of sodium channels in cultured neuroblastoma cells. During those years, Bruce Ransom and I crossed paths as members of the laboratories of Marshall Nirenberg and Philip Nelson and shared insights about sodium channels in neuroblastoma cells from my work and electrical excitability and synaptic transmission in cultured spinal cord neurons from Bruce's pioneering electrophysiological studies...
June 7, 2017: Neurochemical Research
https://www.readbyqxmd.com/read/28560313/urothelial-tight-junction-barrier-dysfunction-sensitizes-bladder-afferents
#18
Nicolas Montalbetti, Anna C Rued, Stefanie N Taiclet, Lori A Birder, F Aura Kullmann, Marcelo D Carattino
Interstitial cystitis/bladder pain syndrome (IC/BPS) is a chronic voiding disorder that presents with pain in the urinary bladder and surrounding pelvic region. A growing body of evidence suggests that an increase in the permeability of the urothelium, the epithelial barrier that lines the interior of the bladder, contributes to the symptoms of IC/BPS. To examine the consequence of increased urothelial permeability on pelvic pain and afferent excitability, we overexpressed in the urothelium claudin 2 (Cldn2), a tight junction (TJ)-associated protein whose message is significantly upregulated in biopsies of IC/BPS patients...
May 2017: ENeuro
https://www.readbyqxmd.com/read/28556479/tonic-and-phasic-changes-in-anteromedial-globus-pallidus-activity-in-tourette-syndrome
#19
Michal Israelashvili, Anouk Y J M Smeets, Maya Bronfeld, Dagmar H Zeef, Albert F G Leentjens, Vivianne van Kranen-Mastenbroek, Marcus L F Janssen, Yasin Temel, Linda Ackermans, Izhar Bar-Gad
BACKGROUND: Tourette syndrome is a hyperkinetic neurodevelopmental disorder characterized by tics. OBJECTIVE: Assess the neuronal changes in the associative/limbic GP associated with Tourette syndrome. METHODS: Neurophysiological recordings were performed from the anterior (associative/limbic) GPe and GPi of 8 awake patients during DBS electrode implantation surgeries. RESULTS: The baseline firing rate of the neurons was low in a state-dependent manner in both segments of the GP...
July 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28555777/early-ictal-and-interictal-patterns-in-fires-the-sparks-before-the-blaze
#20
Raquel Farias-Moeller, Luca Bartolini, Katelyn Staso, John M Schreiber, Jessica L Carpenter
OBJECTIVE: Febrile infection-related epilepsy syndrome (FIRES) is a catastrophic epileptic encephalopathy described as explosive onset of super refractory status epilepticus (SRSE) in previously healthy children. We describe electroencephalography (EEG) abnormalities in the hyperacute phase of FIRES, with the aim of contributing to the diagnostic characterization of a syndrome otherwise lacking specific biomarkers. METHODS: This is a retrospective single-center, case series of seven children with FIRES...
August 2017: Epilepsia
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