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https://www.readbyqxmd.com/read/28644548/repetitive-peripheral-magnetic-stimulation-for-activities-of-daily-living-and-functional-ability-in-people-after-stroke
#1
REVIEW
Ryo Momosaki, Naoki Yamada, Erika Ota, Masahiro Abo
BACKGROUND: Repetitive peripheral magnetic stimulation (rPMS) is a form of therapy that creates painless stimulation of deep muscle structures to improve motor function in people with physical impairment from brain or nerve disorders. Use of rPMS for people after stroke has been identified as a feasible approach to improve activities of daily living and functional ability. However, no systematic reviews have assessed the findings of available trials. The effect and safety of this intervention for people after stroke currently remain uncertain...
June 23, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28642891/safety-tolerability-of-the-anti-semaphorin-4d-antibody-vx15-2503-in-a-randomized-phase-1-trial
#2
Christopher LaGanke, Lawrence Samkoff, Keith Edwards, Lily Jung Henson, Pavle Repovic, Sharon Lynch, Lael Stone, David Mattson, Aaron Galluzzi, Terrence L Fisher, Christine Reilly, Laurie A Winter, John E Leonard, Maurice Zauderer
OBJECTIVE: To evaluate the safety, tolerability, pharmacokinetics (PK), and pharmacodynamics (PD) of VX15/2503 in a randomized, single-dose, dose-escalation, double-blind, placebo-controlled study enrolling adult patients with MS. METHODS: Single IV doses of VX15/2503 or placebo were administered. Ten patients each were randomized (4:1 randomization ratio) into 5 ascending dose cohorts of 1, 3, 6, 10, or 20 mg/kg. Safety, immunogenicity, PK/PD, MRI, ECG, and lymphocyte subset levels were evaluated...
July 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28642828/surgical-orthodontic-treatment-of-a-patient-affected-by-type-1-myotonic-dystrophy-steinert-syndrome
#3
Laura Cacucci, Beatrice Ricci, Maria Moretti, Giulio Gasparini, Sandro Pelo, Cristina Grippaudo
Myotonic dystrophy, or Steinert's disease, is the most common form of muscular dystrophy that occurs in adults. This multisystemic form involves the skeletal muscles but affects also the eye, the endocrine system, the central nervous system, and the cardiac system. The weakness of the facial muscles causes a characteristic facial appearance frequently associated with malocclusions. Young people with myotonic dystrophy, who also have severe malocclusions, have bad oral functions such as chewing, breathing, and phonation...
2017: Case Reports in Dentistry
https://www.readbyqxmd.com/read/28642160/a-novel-missense-variant-gln220arg-of-gnb4-encoding-guanine-nucleotide-binding-protein-subunit-beta-4-in-a-japanese-family-with-autosomal-dominant-motor-and-sensory-neuropathy
#4
Shiroh Miura, Takuya Morikawa, Ryuta Fujioka, Kazuhito Noda, Kengo Kosaka, Takayuki Taniwaki, Hiroki Shibata
Dominant intermediate Charcot-Marie-Tooth disease F (CMTDIF) is an autosomal dominant hereditary form of Charcot-Marie-Tooth disease (CMT) caused by variations in the guanine nucleotide-binding protein, subunit beta-4 gene (GNB4). We examined two Japanese familial cases with CMT. Case 1 was a 49-year-old male whose chief complaint was slowly progressive gait disturbance and limb dysesthesia that appeared at the age of 47. On neurological examination, he showed hyporeflexia or areflexia, distal limb muscle weakness, and distal sensory impairment with lower dominancy...
June 19, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28641925/safety-pharmacokinetics-and-sialic-acid-production-after-oral-administration-of-n-acetylmannosamine-mannac-to-subjects-with-gne-myopathy
#5
Xin Xu, Amy Q Wang, Lea L Latham, Frank Celeste, Carla Ciccone, May Christine Malicdan, Barry Goldspiel, Pramod Terse, James Cradock, Nora Yang, Selwyn Yorke, John C McKew, William A Gahl, Marjan Huizing, Nuria Carrillo
GNE myopathy is a rare, autosomal recessive, inborn error of sialic acid metabolism, caused by mutations in GNE, the gene encoding UDP-N-acetyl-glucosamine-2-epimerase/N-acetylmannosamine kinase. The disease manifests as an adult-onset myopathy characterized by progressive skeletal muscle weakness and atrophy. There is no medical therapy available for this debilitating disease. Hyposialylation of muscle glycoproteins likely contributes to the pathophysiology of this disease. N-acetyl-D-mannosamine (ManNAc), an uncharged monosaccharide and the first committed precursor in the sialic acid biosynthetic pathway, is a therapeutic candidate that prevents muscle weakness in the mouse model of GNE myopathy...
April 26, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28641283/-healing-of-amyotrophic-lateral-sclerosis-a-case-report
#6
Inge Mangelsdorf, Harald Walach, Joachim Mutter
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a devastating disease leading to death within 3-5 years in most cases. New approaches to treating this disease are needed. Here, we report a successful therapy. CASE REPORT: In a 49-year-old male patient suffering from muscle weakness and fasciculations, progressive muscular atrophy, a variant of ALS, was diagnosed after extensive examinations ruling out other diseases. Due to supposed mercury exposure from residual amalgam, the patient's teeth were restored...
June 12, 2017: Complementary Medicine Research
https://www.readbyqxmd.com/read/28640120/working-hand-syndrome-a-new-definition-of-non-classified-polyneuropathy-condition
#7
Gökhan Özdemir
The aim of this paper was to define an unexplained non-classified polyneuropathy condition as a new neurological disease. This new diagnosis of occupation related polyneuropathy has been named as "WORKING HAND SYNDROME (WHS)."This study collected and compared clinic and electrophysiological analyze data from healthy controls, WHS patients, carpal tunnel syndrome (CTS) patients and polyneuropathy patients. The WHS patients presented to the clinic with pain, numbness, tingling, and burning sensations in their hands that increased significantly during rest and nighttime...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28637129/patient-reported-outcomes-in-gne-myopathy-incorporating-a-valid-assessment-of-physical-function-in-a-rare-disease
#8
Christina Slota, Margaret Bevans, Li Yang, Joseph Shrader, Galen Joe, Nuria Carrillo
BACKGROUND: The aim of this analysis was to evaluate the psychometric properties of three patient reported outcome (PRO) measures characterizing physical function in GNE myopathy: the Human Activity Profile, the Inclusion Body Myositis Functional Rating Scale, and the Activities-specific Balance Confidence scale. METHODS: This analysis used data from 35 GNE myopathy subjects participating in a natural history study. For construct validity, correlational and known-group analyses were between the PROs and physical assessments...
February 7, 2017: Disability and Rehabilitation
https://www.readbyqxmd.com/read/28637125/bilateral-movements-increase-sustained-extensor-force-in-the-paretic-arm
#9
Nyeonju Kang, James H Cauraugh
PURPOSE: Muscle weakness in the extensors poststroke is a common motor impairment. Unfortunately, research is unclear on whether bilateral movements increase extensor force production in the paretic arm. This study investigated sustained force production while stroke individuals maximally extended their wrist and fingers on their paretic arm. Specifically, we determined isometric force production in three conditions: (a) unilateral paretic arm, (b) unilateral nonparetic arm, and (c) bilateral (both arms executing the same movement simultaneously)...
February 6, 2017: Disability and Rehabilitation
https://www.readbyqxmd.com/read/28636643/neuromuscular-electrical-stimulation-prevents-skeletal-muscle-dysfunction-in-adjuvant-induced-arthritis-rat
#10
Koichi Himori, Daisuke Tatebayashi, Keita Kanzaki, Masanobu Wada, Håkan Westerblad, Johanna T Lanner, Takashi Yamada
Skeletal muscle weakness is a prominent feature in patients with rheumatoid arthritis (RA). In this study, we investigated whether neuromuscular electrical stimulation (NMES) training protects against skeletal muscle dysfunction in rats with adjuvant-induced arthritis (AIA). AIA was produced by intraarticular injection of complete Freund's adjuvant into the knees of Wistar rats. For NMES training, dorsiflexor muscles were stimulated via a surface electrode (0.5 ms pulse, 50 Hz, 2 s on/4 s off). NMES training was performed every other day for three weeks and consisted of three sets produced at three min intervals...
2017: PloS One
https://www.readbyqxmd.com/read/28635954/phenotypic-extremes-of-bicd2-opathies-from-lethal-congenital-muscular-atrophy-with-arthrogryposis-to-asymptomatic-with-subclinical-features
#11
Markus Storbeck, Beate Horsberg Eriksen, Andreas Unger, Irmgard Hölker, Ingvild Aukrust, Lilian A Martínez-Carrera, Wolfgang A Linke, Andreas Ferbert, Raoul Heller, Matthias Vorgerd, Gunnar Houge, Brunhilde Wirth
Heterozygous variants in BICD cargo adapter 2 (BICD2) cause autosomal dominant spinal muscular atrophy, lower extremity-predominant 2 (SMALED2). The disease is usually characterized by a benign or slowly progressive, congenital or early onset muscle weakness and atrophy that mainly affects the lower extremities, although some affected individuals show involvement of the arms and the shoulder girdle. Here we report unusual extremes of BICD2-related diseases: A severe form of congenital muscular atrophy with arthrogryposis multiplex, respiratory insufficiency and lethality within four months...
June 21, 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28635523/long-term-paired-associative-stimulation-enhances-motor-output-of-the-tetraplegic-hand
#12
Aleksandra Tolmacheva, Sarianna Savolainen, Erika Kirveskari, Pantelis Lioumis, Linda Kuusela, Nina Maria Brandstack, Aarne Ylinen, Jyrki Mäkelä, Anastasia Shulga
A large proportion of spinal cord injuries are incomplete. Even in clinically complete injuries, silent nonfunctional connections can be present. Therapeutic approaches that can strengthen transmission in weak neural connections to improve motor performance are needed. Our aim was to determine whether long-term delivery of paired associative stimulation (PAS, a combination of transcranial magnetic stimulation (TMS) with peripheral nerve stimulation (PNS)) can enhance motor output in the hands of patients with chronic traumatic tetraplegia, and to compare this technique with long-term PNS...
June 21, 2017: Journal of Neurotrauma
https://www.readbyqxmd.com/read/28635482/diagnosis-and-management-of-myasthenia-gravis
#13
Christopher Barber
Myasthenia gravis is a rare long-term neurological condition that is characterised by fluctuating skeletal muscle weakness and fatigue, as well as respiratory difficulties. It is both an acquired autoimmune disease and a chronic neuromuscular disorder. Because of its rarity, myasthenia gravis is relatively unknown and may be unfamiliar to many nurses. While there are various types of myasthenia, this article focuses on myasthenia gravis, exploring its symptoms, diagnosis and treatment, and examining the nurse's role in managing the condition...
June 21, 2017: Nursing Standard
https://www.readbyqxmd.com/read/28634552/inherited-paediatric-motor-neuron-disorders-beyond-spinal-muscular-atrophy
#14
REVIEW
Hooi Ling Teoh, Kate Carey, Hugo Sampaio, David Mowat, Tony Roscioli, Michelle Farrar
Paediatric motor neuron diseases encompass a group of neurodegenerative diseases characterised by the onset of muscle weakness and atrophy before the age of 18 years, attributable to motor neuron loss across various neuronal networks in the brain and spinal cord. While the genetic underpinnings are diverse, advances in next generation sequencing have transformed diagnostic paradigms. This has reinforced the clinical phenotyping and molecular genetic expertise required to navigate the complexities of such diagnoses...
2017: Neural Plasticity
https://www.readbyqxmd.com/read/28634003/spasticity-video-challenge-a-look-at-methods-for-addressing-difficult-cases
#15
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/28633725/well-differentiated-neuroendocrine-tumor-a-low-b-grade-tumor-s-aggressive-course-and-dismal-outcome-a-case-report
#16
Dinesh Atwal, Krishna Prasad Joshi, Susanne Jeffus, James Ntambi, Fade Mahmoud
INTRODUCTION: Incidence of well-differentiated neuroendocrine tumors (NETs) of the colon and rectum is increasing and is now approximately 1 per 100,000 in the US. NETs are either well-differentiated (indolent) or poorly differentiated (aggressive). The majority of these tumors are found incidentally during screening colonoscopies and rarely are associated with symptoms of hormonal syndrome, even during the advanced stage. Metastatic well-differentiated NETs of the colon and rectum are incurable, hard to treat, and associated with a poor prognosis and survival rates similar to colorectal adenocarcinoma survival...
2017: Permanente Journal
https://www.readbyqxmd.com/read/28632467/effects-of-a-single-session-of-transcranial-direct-current-stimulation-on-upper-limb-movements-in-children-with-cerebral-palsy-a-randomized-sham-controlled-study
#17
Renata Calhes Franco Moura, Cibele Santos, Luanda Collange Grecco, Giorgio Albertini, Veronica Cimolin, Manuela Galli, Claudia Oliveira
INTRODUCTION: Motor impairment in children with spastic hemiparetic cerebral palsy (CP) is generally more prominent in the affected upper limb, leading to limitations in hand function stemming from deficiencies in motor coordination and selective motor control as well as muscle weakness, slower execution of movements and deficient integration of sensory-motor information. OBJECTIVE: Determine the effect of a single session of anodal transcranial direct current stimulation (tDCS) combined with functional training on the spatiotemporal variables of upper arm movements in children with spastic hemiparesis...
February 25, 2017: Developmental Neurorehabilitation
https://www.readbyqxmd.com/read/28630772/severe-rhabdomyolysis-due-to-presumed-drug-interactions-between-atorvastatin-with-amlodipine-and-ticagrelor
#18
Iouri Banakh, Kavi Haji, Ross Kung, Sachin Gupta, Ravindranath Tiruvoipati
Atorvastatin and ticagrelor combination is a widely accepted therapy for secondary prevention of ischaemic heart disease. However, rhabdomyolysis is a well-known rare side effect of statins which should be considered when treatments are combined with cytochrome P450 3A4 enzyme inhibitors. We report a case of atorvastatin and ticagrelor associated severe rhabdomyolysis that progressed to multiorgan failure requiring renal replacement therapy, inotropes, intubation, and mechanical ventilation. Despite withdrawal of the precipitating cause and the supportive measures including renal replacement therapy, creatinine kinase increased due to ongoing rhabdomyolysis rapidly progressing to upper and lower limbs weakness...
2017: Case Reports in Critical Care
https://www.readbyqxmd.com/read/28630226/thyrotoxic-periodic-paralysis-as-an-initial-presentation-of-graves-disease-in-a-saudi-patient
#19
S F Alqahtani, M M Aleithan
Thyrotoxic periodic paralysis (TPP) is a well-known complication of hyperthyroidism, characterised by recurrent flaccid paralysis with hypokalaemia. To date, only five cases of this rare disorder have been reported in Saudi Arabia. Here, we report an additional case involving a 25-year-old Saudi man who presented with lower limb paralysis and severe hypokalaemia. Clinically, he showed symptoms and signs suggestive of Graves' disease, which was confirmed by laboratory investigations. Carbimazole, a beta-blocker and potassium replacement were administered, resulting in dramatic improvement of the TTP...
June 19, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28629899/high-adherence-to-a-mediterranean-diet-and-lower-risk-of-frailty-among-french-older-adults-community-dwellers-results-from-the-three-city-bordeaux-study
#20
Berna Rahi, Soufiane Ajana, Maturin Tabue-Teguo, Jean-François Dartigues, Karine Peres, Catherine Feart
BACKGROUND & AIMS: Mediterranean diet (MeDi) is considered as a key component for healthy aging, including prevention of age-related disability, while its association with frailty, independent of disability has never been assessed. Our objective was to investigate the relation between MeDi adherence and frailty incidence among persons aged ≥75 years participating at the prospective population-based French Three-City Study. METHODS: The study sample consisted of 560 initially non-frail participants of the Three-City-Bordeaux center, seen at the 2009-2010 follow-up, and re-examined two years later...
May 31, 2017: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
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