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https://www.readbyqxmd.com/read/28716516/hypertonia
#1
Sarah Helen Evans, Mark William Cameron, Justin Michael Burton
Hypertonia is the abnormal increase in muscle tone as a result of upper motor neuron lesions. There are three following clinical types: spasticity, dystonia, and rigidity. Management of hypertonia is individualized and should be directed by the patient and/or family׳s goals of care as well as the underlying cause of the hypertonia. Treatment options include stretching, strengthening, positioning, oral medications, botulinum toxin injections, phenol injections, as well as surgical procedures. Without effective management, hypertonia can result in muscle imbalance, abnormal movement patterns, pain, joint contracture, joint deformity, and ultimately negatively impact a patient׳s function...
July 14, 2017: Current Problems in Pediatric and Adolescent Health Care
https://www.readbyqxmd.com/read/28713101/-extracorporeal-shock-waves-esw-as-an-alternative-treatment-method-for-improving-the-limb-muscles-spasticity-after-cerebral-stroke-a-systematic-review-of-the-literature
#2
Robert Dymarek, Kuba Ptaszkowski, Lucyna Słupska, Małgorzata Paprocka-Borowicz, Jakub Taradaj, Tomasz Halski, Joanna Rosińczuk
Extracorporeal shock wave (ESW) is a physical factor, of which the clinical use is observed in a wide range of disorders, particularly musculoskeletal dysfunctions. Recently, one can observe that the list of indications for ESW treatment is continuously growing and adapting the increasingly different systemic diseases in terms of etiology and pathomechanism. Nevertheless, it should be remembered that the potential biological mechanisms of ESW stimulation conditioning advantageous and desirable therapeutic effects are not clearly explained...
2017: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28705229/brugada-syndrome-in-a-patient-with-amyotrophic-lateral-sclerosis-a-case-report
#3
Anusha Battineni, Rohit Gummi, Naresh Mullaguri, Raghav Govindarajan
BACKGROUND: Amyotrophic lateral sclerosis is a fatal neuromuscular disorder characterized by progressive death of the upper and lower motor neurons in the central nervous system. Patients with this disease die mostly as a result of respiratory failure; however, owing to prolonged survival through assisted ventilation, cardiovascular causes are increasingly responsible for mortality. We report what is to the best of our knowledge the first case of type 2 Brugada syndrome causing ventricular tachyarrhythmia and cardiac arrest in a patient with upper limb onset amyotrophic lateral sclerosis...
July 14, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28705085/international-survey-of-als-experts-about-critical-questions-for-assessing-patients-with-als
#4
Mamede De Carvalho, Adam Ryczkowski, Peter Andersen, Marta Gromicho, Julian Grosskreutz, Magdalena Kuźma-Kozakiewicz, Susanne Petri, Maria Piotrkiewicz, Gabriel Miltenberger Miltenyi
OBJECTIVE: To define an applicable dataset for ALS patient registries we weighted specific clinical items as scored by worldwide ALS experts. METHODS: Sixty participants were invited based on relevant clinical work, publications and personal acquaintance. They rated 160 clinical items consensually agreed by the members of our project, incorporating specialists from five European Centres. Scoring scheme was defined as: 1 - essential; 2 - important; 3 - not very important...
July 13, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28687974/syne1-related-cerebellar-ataxia-presents-with-variable-phenotypes-in-a-consanguineous-family-from-turkey
#5
E Yucesan, Sibel A Ugur Iseri, B Bilgic, Z Gormez, B Bakir Gungor, A Sarac, O Ozdemir, M Sagiroglu, H Gurvit, H Hanagasi, U Ozbek
SYNE1 related autosomal recessive cerebellar ataxia type 1 (ARCA1) is a late-onset cerebellar ataxia with slow progression originally demonstrated in French-Canadian populations of Quebec, Canada. Nevertheless, recent studies on SYNE1 ataxia have conveyed the condition from a geographically limited pure cerebellar recessive ataxia to a complex multisystem phenotype that is relatively common on the global scale. To determine the underlying genetic cause of the ataxia phenotype in a consanguineous family from Turkey presenting with very slow progressive cerebellar symptoms including dysarthria, dysmetria, and gait ataxia, we performed SNP-based linkage analysis in the family along with whole exome sequencing (WES) in two affected siblings...
July 7, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28677231/development-of-deep-and-upper-neuronal-layers-in-the-domestic-cat-sheep-and-pig-neocortex
#6
M Glatzle, M Hoops, J Kauffold, J Seeger, S A Fietz
The neocortex plays a key role in cognition, volitional motor control and sensory perception and has undergone tremendous expansion during evolution. The mature neocortex consists of radially aligned neurons that are arranged in six layers. Layers II-VI are often split into two groups: deep and upper layers, both building up the so-called cortical plate during embryonic and foetal development. So far cortical neurogenesis, including the generation of deep and upper layers, has mostly been studied in laboratory rodents and primates...
July 4, 2017: Anatomia, Histologia, Embryologia
https://www.readbyqxmd.com/read/28669297/establishing-reconstructive-neurosurgery-as-a-subspecialty
#7
Justin M Brown, Mark A Mahan, Ross Mandeville, Bob S Carter
Neurosurgery is experiencing the emergence of a new subspecialty focused on function restoration. New, evolving, and reappraised surgical procedures have provided an opportunity to restore function to many patients with previously undertreated disorders. Candidates for reconstruction were previously limited to those with peripheral nerve and brachial plexus injuries, but this has been expanded to include stroke, spinal cord injury, and a host of other paralyzing disorders affecting both upper and lower motor neurons...
July 2017: Neurosurgical Focus
https://www.readbyqxmd.com/read/28651542/evidence-for-an-early-innate-immune-response-in-the-motor-cortex-of-als
#8
Javier H Jara, Barış Genç, Macdonell J Stanford, Peter Pytel, Raymond P Roos, Sandra Weintraub, M Marsel Mesulam, Eileen H Bigio, Richard J Miller, P Hande Özdinler
BACKGROUND: Recent evidence indicates the importance of innate immunity and neuroinflammation with microgliosis in amyotrophic lateral sclerosis (ALS) pathology. The MCP1 (monocyte chemoattractant protein-1) and CCR2 (CC chemokine receptor 2) signaling system has been strongly associated with the innate immune responses observed in ALS patients, but the motor cortex has not been studied in detail. METHODS: After revealing the presence of MCP1 and CCR2 in the motor cortex of ALS patients, to elucidate, visualize, and define the timing, location and the extent of immune response in relation to upper motor neuron vulnerability and progressive degeneration in ALS, we developed MCP1-CCR2-hSOD1(G93A) mice, an ALS reporter line, in which cells expressing MCP1 and CCR2 are genetically labeled by monomeric red fluorescent protein-1 and enhanced green fluorescent protein, respectively...
June 26, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/28645622/in-vivo-effects-of-knocking-down-metabotropic-glutamate-receptor-5-in-the-sod1-g93a-mouse-model-of-amyotrophic-lateral-sclerosis
#9
Tiziana Bonifacino, Luca Cattaneo, Elena Gallia, Aldamaria Puliti, Marcello Melone, Francesca Provenzano, Simone Bossi, Ilaria Musante, Cesare Usai, Fiorenzo Conti, Giambattista Bonanno, Marco Milanese
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder due to loss of upper and lower motor neurons (MNs). The mechanisms of neuronal death are largely unknown, thus prejudicing the successful pharmacological treatment. One major cause for MN degeneration in ALS is represented by glutamate(Glu)-mediated excitotoxicity. We have previously reported that activation of Group I metabotropic Glu receptors (mGluR1 and mGluR5) at glutamatergic spinal cord nerve terminals produces abnormal Glu release in the widely studied SOD1(G93A) mouse model of ALS...
June 21, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28641533/role-and-therapeutic-potential-of-astrocytes-in-amyotrophic-lateral-sclerosis
#10
Mariana Pehar, Benjamin A Harlan, Kelby M Killoy, Marcelo R Vargas
Amyotrophic lateral sclerosis (ALS) is characterized by the progressive degeneration of motor neurons in the spinal cord, brain stem, and motor cortex. The molecular mechanism underlying the progressive degeneration of motor neuron remains uncertain but involves a non-cell autonomous process. In acute injury or degenerative diseases astrocytes adopt a reactive phenotype known as astrogliosis. Astrogliosis is a complex remodeling of astrocyte biology and most likely represents a continuum of potential phenotypes that affect neuronal function and survival in an injury-specific manner...
June 21, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28638596/environmental-insults-critical-triggers-for-amyotrophic-lateral-sclerosis
#11
REVIEW
Bing Yu, Roger Pamphlett
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterised by a rapid loss of lower and upper motor neurons. As a complex disease, the ageing process and complicated gene-environment interactions are involved in the majority of cases. MAIN BODY: Significant advances have been made in unravelling the genetic susceptibility to ALS with massively parallel sequencing technologies, while environmental insults remain a suspected but largely unexplored source of risk...
2017: Translational Neurodegeneration
https://www.readbyqxmd.com/read/28637976/coordinated-control-of-the-tongue-during-suckling-like-activity-and-respiration
#12
Tomio Inoue, Kiyomi Nakayama, Yoshiaki Ihara, Satoshi Tachikawa, Shiro Nakamura, Ayako Mochizuki, Koji Takahashi, Takehiko Iijima
The tongue can move freely and is important in oral motor functions. Tongue movement must be coordinated with movement of the hyoid, mandible, and pharyngeal wall, to which it is attached. Our previous study using isolated brainstem-spinal cord preparations showed that application of N-methyl-D-aspartate induces rhythmic activity in the hypoglossal nerve that is coincident with rhythmic activity in the ipsilateral trigeminal motor nerve. Partial or complete midline transection of the preparation only abolishes activity in the trigeminal motor nerve; therefore, the neuronal network contributing to coordinated activity of the jaw/tongue muscles is located on both sides of the preparation and sends motor commands to contralateral trigeminal motoneurons...
2017: Journal of Oral Science
https://www.readbyqxmd.com/read/28634003/spasticity-video-challenge-a-look-at-methods-for-addressing-difficult-cases
#13
Katharine E Alter, Mark Gormley, Atul T Patel
As seen in this CME online activity (available at http://courses.elseviercme.com/spasticity/662e), treatment of patients with spasticity due to upper motor neuron syndromes, including traumatic brain injury, stroke, and cerebral palsy, is multifaceted, involving chemodenervation, systemic medications, surgical therapy, rehabilitation efforts, and home care. Optimal care begins with the recognition that each patient's impairments are unique and must be assessed carefully to determine the impact of muscle overactivity, loss of dexterity, and weakness on passive and active function in the context of the patients' goals...
June 19, 2017: PM & R: the Journal of Injury, Function, and Rehabilitation
https://www.readbyqxmd.com/read/28631958/-anything-that-makes-life-s-journey-better-exploring-the-use-of-digital-technology-by-people-living-with-motor-neurone-disease
#14
Esther V Hobson, Saima Fazal, Pamela J Shaw, Christopher J McDermott
Our aim was to explore the attitudes of those living with motor neuron disease towards digital technology. Postal and online questionnaires surveyed 83 people with MND (pwMND) and 54 friends and family members (fMND). Five pwMND and five fMND underwent semi-structured interviews. 82% of pwMND and 87% of fMND use technology every day with iPads and laptops being the devices most commonly used. pwMND used technology to help them continue to participate in everyday activities such as socialising, entertainment and accessing the internet...
February 20, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28630754/nerve-injuries-of-the-upper-extremity-and-hand
#15
Lars B Dahlin, Mikael Wiberg
A nerve injury has a profound impact on the patient's daily life due to the impaired sensory and motor function, impaired dexterity, sensitivity to cold as well as eventual pain problems.To perform an appropriate treatment of nerve injuries, a correct diagnosis must be made, where the injury is properly classified, leading to an optimal surgical approach and technique, where timing of surgery is also important for the outcome.Knowledge about the nerve regeneration process, where delicate processes occur in neurons, non-neuronal cells (i...
May 2017: EFORT open reviews
https://www.readbyqxmd.com/read/28627708/alterations-in-aqp4-expression-and-polarization-in-the-course-of-motor-neuron-degeneration-in-sod1g93a-mice
#16
Jiaying Dai, Weihao Lin, Minying Zheng, Qiang Liu, Baixuan He, Chuanming Luo, Xilin Lu, Zhong Pei, Huanxing Su, Xiaoli Yao
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by selective degeneration of upper and lower motor neurons. The disease progression is associated with the astrocytic environment. Aquaporin-4 (AQP4) water channels are the most abundant AQPs expressed in astrocytes, exerting important influences on central nervous system homeostasis. The present study aimed to characterize the alterations in AQP4 expression and loca-lization in superoxide dismutase 1 (SOD1) G93A transgenic mice...
August 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28616022/therapeutic-opportunities-and-challenges-of-induced-pluripotent-stem-cells-derived-motor-neurons-for-treatment-of-amyotrophic-lateral-sclerosis-and-motor-neuron-disease
#17
REVIEW
Manoj Kumar Jaiswal
Amyotrophic lateral sclerosis (ALS) and motor neuron diseases (MNDs) are progressive neurodegenerative diseases that affect nerve cells in the brain affecting upper and lower motor neurons (UMNs/LMNs), brain stem and spinal cord. The clinical phenotype is characterized by loss of motor neurons (MNs), muscular weakness and atrophy eventually leading to paralysis and death due to respiratory failure within 3-5 years after disease onset. No effective treatment or cure is currently available that halts or reverses ALS and MND except FDA approved drug riluzole that only modestly slows the progression of ALS in some patients...
May 2017: Neural Regeneration Research
https://www.readbyqxmd.com/read/28613097/dynamic-muscle-ultrasound-identifies-upper-motor-neuron-involvement-in-amyotrophic-lateral-sclerosis
#18
Yu-Ichi Noto, Neil Simon, Kazumoto Shibuya, José M Matamala, Thanuja Dharmadasa, Matthew C Kiernan
OBJECTIVE: The aim of the present study was to elucidate the pattern of change in bulbar muscles using ultrasound in patients diagnosed with amyotrophic lateral sclerosis (ALS). METHODS: Changes in the mylohyoid and geniohyoid muscle complex (mylohyoid-geniohyoid-muscle-complex) thickness were recorded while swallowing 5 ml of water using M-mode ultrasound in 30 ALS patients compared to 20 healthy controls. The ratio of mylohyoid-geniohyoid-muscle-complex thickness as determined by the maximum thickness of mylohyoid-geniohyoid-muscle-complex during swallowing divided by thickness at rest, was compared between ALS patients and controls, with the correlation between thickness ratio, echogenicity and clinical parameters assessed...
February 10, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28611587/downregulation-of-microrna-193b-3p-promotes-autophagy-and-cell-survival-by-targeting-tsc1-mtor-signaling-in-nsc-34-cells
#19
ChunYu Li, YongPing Chen, XuePing Chen, QianQian Wei, Bei Cao, HuiFang Shang
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the death of upper and lower motor neurons. MicroRNAs (miRNAs) are reported to be closely related to the development of ALS. However, the precise functions of miRNAs in the pathogenesis of ALS remain largely unknown. In previous studies, we determined that miRNA-193b-3p was significantly downregulated in patients with sporadic ALS (sALS). Here, we observed that miRNA-193b-3p was downregulated in the SOD1(G93A) mouse model of ALS and promoted cell death in NSC-34 cells...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28602631/clinical-electrodiagnostic-and-outcome-correlation-in-als-patients-in-thailand
#20
Kongkiat Kulkantrakorn, Dararat Suksasunee
INTRODUCTION: The diagnosis of amyotrophic lateral sclerosis (ALS) requires both clinical and electrodiagnostic (EDx) data. The correlation between the two may aid in outcome prognostication. METHODS: Retrospective review of patients with ALS in tow tertiary hospitals in Thailand. RESULTS: Data from 25 ALS patients out of 38 patients with motor neuron disease was reviewed. Male was predominant with mean age of onset of 60.1±10.7years old...
June 8, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
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