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Upper motor neuron

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https://www.readbyqxmd.com/read/28794983/multimodal-structural-mri-in-the-diagnosis-of-motor-neuron-diseases
#1
Pilar M Ferraro, Federica Agosta, Nilo Riva, Massimiliano Copetti, Edoardo Gioele Spinelli, Yuri Falzone, Gianni Sorarù, Giancarlo Comi, Adriano Chiò, Massimo Filippi
This prospective study developed an MRI-based method for identification of individual motor neuron disease (MND) patients and test its accuracy at the individual patient level in an independent sample compared with mimic disorders. 123 patients with amyotrophic lateral sclerosis (ALS), 44 patients with predominantly upper motor neuron disease (PUMN), 20 patients with ALS-mimic disorders, and 78 healthy controls were studied. The diagnostic accuracy of precentral cortical thickness and diffusion tensor (DT) MRI metrics of corticospinal and motor callosal tracts were assessed in a training cohort and externally proved in a validation cohort using a random forest analysis...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28790120/zinc-containing-dental-fixative-causing-copper-deficiency-myelopathy
#2
Liam Stuart Carroll, Azmil H Abdul-Rahim, Rosanne Murray
A 62-year-old male, previously well, was referred to neurology clinic following 6 months history of worsening lower limbs instability, paraesthesia, pain and weakness rendering him housebound. Examination revealed upper motor neuron pattern of weakness of the lower limbs and loss of proprioception. Serum analysis revealed reduced caeruloplasmin and copper levels with raised zinc. Spinal imaging revealed subtle dorsal column intensity changes in C2-C7, confirmed with 3T MRI. A copper deficiency myeloneuropathy was diagnosed secondary to chronic use of a zinc-containing dental fixative paste...
August 8, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28780847/-characteristics-of-repetitive-nerve-stimulation-in-53-cases-of-amyotrophic-lateral-sclerosis
#3
W X Liu, Z H Chen, L Ling, Y T Ren, B Sun, F Yang, F Cui, X S Huang
Objective: To investigate the feature of repetitive nerve stimulation (RNS) in patients with amyotrophic lateral sclerosis (ALS) and correlation between RNS and clinical features and electromyography (EMG) findings of the corresponding muscle. Methods: Needle EMG and RNS were performed in 53 patients with ALS, who were recruited into Department of Neurology of Chinese PLA general hospital during April to December in 2016. Decrement of the compond muscle action potential (CMAP) in response to RNS of different nerves and stimulus frequencies was compared...
August 1, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28769755/broadband-prosthetic-interfaces-combining-nerve-transfers-and-implantable-multichannel-emg-technology-to-decode-spinal-motor-neuron-activity
#4
Konstantin D Bergmeister, Ivan Vujaklija, Silvia Muceli, Agnes Sturma, Laura A Hruby, Cosima Prahm, Otto Riedl, Stefan Salminger, Krisztina Manzano-Szalai, Martin Aman, Michael-Friedrich Russold, Christian Hofer, Jose Principe, Dario Farina, Oskar C Aszmann
Modern robotic hands/upper limbs may replace multiple degrees of freedom of extremity function. However, their intuitive use requires a high number of control signals, which current man-machine interfaces do not provide. Here, we discuss a broadband control interface that combines targeted muscle reinnervation, implantable multichannel electromyographic sensors, and advanced decoding to address the increasing capabilities of modern robotic limbs. With targeted muscle reinnervation, nerves that have lost their targets due to an amputation are surgically transferred to residual stump muscles to increase the number of intuitive prosthetic control signals...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28750498/emerging-clinical-issues-and-multivariate-analyses-in-pet-investigations
#5
Javier Arbizu, Alessandro Giuliani, Jaime G Perez-Larraya, Mario Riverol, Cathrine Jonsson, Berta García-García, Maribel Morales, Laura Imaz, Marco Pagani
PET using 18F-2-fluoro-2-deoxy-D-glucose (FDG-PET) has been gradually introduced in the diagnostic clinical criteria of the most prevalent neurodegenerative diseases. Moreover, an increasing amount of literature have shown that the information provided by FDG-PET enhances the sensitivity of standard imaging biomarkers in less frequent disorders in which an early differential diagnosis can be of paramount relevance for patient management and outcome. Therefore emerging uses of FDG-PET may be important in prion diseases, autoimmune encephalitis and amyotrophic lateral sclerosis...
July 27, 2017: Quarterly Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28748847/clinical-features-of-isolated-bulbar-palsy-of-amyotrophic-lateral-sclerosis-in-chinese-population
#6
Hua-Gang Zhang, Lu Chen, Lu Tang, Nan Zhang, Dong-Sheng Fan
BACKGROUND: Progressive bulbar palsy (PBP) is a classic phenotype of bulbar onset amyotrophic lateral sclerosis (ALS) with more rapid progression and worse prognosis. However, as an often under-understood variant of ALS, isolated bulbar palsy (IBP) appears to progress more slowly and has a relatively benign prognosis. This study aimed to investigate the natural course and clinical features of IBP in Chinese population and to compare them with those of PBP. METHODS: The clinical data of patients with bulbar onset ALS were collected from January 2009 to December 2013...
August 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28747261/enterovirus-d68-infection-in-a-cluster-of-children-with-acute-flaccid-myelitis-buenos-aires-argentina-2016
#7
Victor Ruggieri, Maria Ivana Paz, María Gabriela Peretti, Carlos Rugilo, Rosa Bologna, Cecilia Freire, Silvana Vergel, Andrea Savransky
OBJECTIVE: To report a outbreak of 11 cases of acute asymmetric flaccid myelopathy due to spinal motor neuron injury. MATERIAL AND METHODS: Eleven children, six male, with a mean age of 3 years presented with acute flaccid myelitis. We analyzed clinical features, etiology, neuroradiological images, treatment, and outcome. RESULTS: Nine children had bilateral and asymmetric flaccid myelitis of the upper limbs, 1 had upper limb monoplegia, and 1 presented with hemiparesis...
July 19, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28737516/pre-therapy-neural-state-of-bilateral-motor-and-premotor-cortices-predicts-therapy-gain-after-subcortical-stroke-a-pilot-study
#8
Carmen M Cirstea, Phil Lee, Sorin C Craciunas, In-Young Choi, Joseph E Burris, Randolph J Nudo
OBJECTIVE: The aim of the study was to examine whether neural state of spared motor and premotor cortices captured before a therapy predicts therapy-related motor gains in chronic subcortical stroke. DESIGN: Ten survivors, presenting chronic moderate upper limb impairment, underwent proton magnetic resonance spectroscopy, magnetic resonance imaging, clinical, and kinematics assessments before a 4-wk impairment-oriented training. Clinical/kinematics assessments were repeated after therapy, and motor gain was defined as positive values of clinical upper limb/elbow motion changes and negative values of trunk motion changes...
July 22, 2017: American Journal of Physical Medicine & Rehabilitation
https://www.readbyqxmd.com/read/28736326/tone-inhibiting-insoles-enhance-the-reciprocal-inhibition-of-ankle-plantarflexors-of-post-stroke-hemiparetic-subjects-an-electromyographic-study
#9
Nobushige Takahashi, Hidetoshi Takahashi, Osamu Takahashi, Ryosuke Ushijima, Rie Umebayashi, Junji Nishikawa, Yasutomo Okajima
BACKGROUND: Spasticity is a common sequela of upper motor neuron pathology, such as cerebrovascular diseases and cerebral palsy. Intervention for spasticity of the ankle plantarflexors in physical therapy may include tone-inhibiting casting and/or orthoses for the ankle and foot. However, the physiological mechanism of tone reduction by such orthoses remains unclarified. OBJECTIVE: To investigate the electrophysiologic effects of tone-inhibiting insoles in stroke subjects with hemiparesis by measuring changes in reciprocal Ia inhibition (RI) in the ankle plantarflexor...
July 20, 2017: PM & R: the Journal of Injury, Function, and Rehabilitation
https://www.readbyqxmd.com/read/28726023/benefits-and-risks-of-non-approved-injection-regimens-for-botulinum-toxins-in-spasticity
#10
Andrea Santamato, Francesco Panza
Spasticity with muscle paresis and loss of dexterity is a common feature of upper motor neuron syndrome due to injuries or the pyramidal tract in several neurological conditions. Botulinum toxin type A has been considered the gold standard treatment for spasticity and movement disorders, with efficacy, reversibility, and low prevalence of complications. During the last 30 years, thousands of studies of its use have been performed, but few guidelines are available. Therefore, there is great variability in both the doses and intervals of administration and the approaches taken by clinicians with considerable experience in spasticity and movement disorder treatment...
July 19, 2017: Drugs
https://www.readbyqxmd.com/read/28716516/hypertonia
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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...
September 1, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28641533/role-and-therapeutic-potential-of-astrocytes-in-amyotrophic-lateral-sclerosis
#20
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
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