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Neuromuscular weakness

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https://www.readbyqxmd.com/read/28338488/respiratory-complications-management-and-treatments-for-neuromuscular-disease-in-children
#1
MyMy C Buu
PURPOSE OF REVIEW: To summarize current literature describing the respiratory complications of neuromuscular disease (NMD) and the effect of respiratory interventions and to explore new gene therapies for patients with NMD. RECENT FINDINGS: Measurements of respiratory function focus on vital capacity and maximal inspiratory and expiratory pressure and show decline over time. Management of respiratory complications includes lung volume recruitment, mechanical insufflation-exsufflation, chest physiotherapy and assisted ventilation...
March 23, 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28334989/impaired-fetal-muscle-development-and-jak-stat-activation-mark-disease-onset-and-progression-in-a-mouse-model-for-merosin-deficient-congenital-muscular-dystrophy
#2
Andreia M Nunes, Ryan D Wuebbles, Apurva Sarathy, Tatiana M Fontelonga, Marianne Deries, Dean J Burkin, Sólveig Thorsteinsdóttir
Merosin-deficient congenital muscular dystrophy type 1A (MDC1A) is a dramatic neuromuscular disease in which crippling muscle weakness is evident from birth. Here we use the dyW mouse model for human MDC1A to trace the onset of the disease during development in utero. We find that myotomal and primary myogenesis proceed normally in homozygous dyW-/-embryos. Fetal dyW-/-muscles display the same number of myofibers as wildtype muscles, but by E18.5 dyW-/-muscles are significantly smaller and muscle size is not recovered post-natally...
March 7, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28326313/lung-volume-recruitment-acutely-increases-respiratory-system-compliance-in-individuals-with-severe-respiratory-muscle-weakness
#3
Yannick Molgat-Seon, Liam M Hannan, Paolo B Dominelli, Carli M Peters, Renee J Fougere, Douglas A McKim, A William Sheel, Jeremy D Road
The aim of the present study was to determine whether lung volume recruitment (LVR) acutely increases respiratory system compliance (Crs) in individuals with severe respiratory muscle weakness (RMW). Individuals with RMW resulting from neuromuscular disease or quadriplegia (n=12) and healthy controls (n=12) underwent pulmonary function testing and the measurement of Crs at baseline, immediately after, 1 h after and 2 h after a single standardised session of LVR. The LVR session involved 10 consecutive supramaximal lung inflations with a manual resuscitation bag to the highest tolerable mouth pressure or a maximum of 50 cmH2O...
January 2017: ERJ Open Research
https://www.readbyqxmd.com/read/28325641/spectrum-of-nondystrophic-skeletal-muscle-channelopathies-in-children
#4
Fouad Al-Ghamdi, Basil T Darras, Partha S Ghosh
BACKGROUND: The nondystrophic skeletal muscle channelopathies are a group of disorders caused by mutations of various voltage-gated ion channel genes, including nondystrophic myotonia and periodic paralysis. METHODS: We identified patients with a diagnosis of muscle channelopathy from our neuromuscular database in a tertiary care pediatric center from 2005 to 2015. We then performed a retrospective review of their medical records for demographic characteristics, clinical features, investigations, treatment, and follow-up...
February 16, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28318818/twitch-mouth-pressure-for-detecting-respiratory-muscle-weakness-in-suspicion-of-neuromuscular-disorder
#5
Dante Brasil Santos, Gilbert Desmarais, Line Falaize, Adam Ogna, Sandrine Cognet, Bruno Louis, David Orlikowski, Hélène Prigent, Frédéric Lofaso
Twitch mouth pressure using magnetic stimulation of the phrenic nerves and an automated inspiratory trigger is a noninvasive, non-volitional assessment of diaphragmatic strength. Our aims were to validate this method in patients with suspected neuromuscular disease, to determine the best inspiratory-trigger pressure threshold, and to evaluate whether twitch mouth pressure decreased the overdiagnosis of muscle weakness frequently observed with noninvasive volitional tests. Maximal inspiratory pressure, sniff nasal pressure, and twitch mouth pressure were measured in 112 patients with restrictive disease and suspected neuromuscular disorder...
February 2, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28316843/anesthetic-considerations-in-a-patient-with-myotonic-dystrophy-for-hip-labral-repair
#6
Ramon Go, David Wang, Danielle Ludwin
Myotonic Dystrophy (DM) affects multiple organ systems. Disorders such as hyperthyroidism, progressive musculoskeletal weakness, cardiac dysrhythmias, hypoventilation, and cognitive-behavioral disorders may be present in these patients. Thorough preoperative assessment and anesthetic planning are required to minimize the risk of anesthetic complications. Patients with DM can exhibit exquisite sensitivity to sedatives, neuromuscular blocking agents, and volatile anesthetics, resulting in potential postoperative complications...
2017: Case Reports in Anesthesiology
https://www.readbyqxmd.com/read/28284391/neuromuscular-manifestations-in-mitochondrial-diseases-in-children
#7
Andrés Nascimento, Carlos Ortez, Cristina Jou, Mar O'Callaghan, Federico Ramos, Àngels Garcia-Cazorla
Mitochondrial diseases exhibit significant clinical and genetic heterogeneity. Mitochondria are highly dynamic organelles that are the major contributor of adenosine triphosphate, through oxidative phosphorylation. These disorders may be developed at any age, with isolated or multiple system involvement, and in any pattern of inheritance. Defects in the mitochondrial respiratory chain impair energy production and almost invariably involve skeletal muscle and peripheral nerves, causing exercise intolerance, cramps, recurrent myoglobinuria, or fixed weakness, which often affects extraocular muscles and results in droopy eyelids (ptosis), progressive external ophthalmoplegia, peripheral ataxia, and peripheral polyneuropathy...
November 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28283324/early-mobilization-why-what-for-and-how
#8
A R Miranda Rocha, B P Martinez, V Z Maldaner da Silva, L A Forgiarini Junior
Early mobilization strategies in the intensive care unit may result in the prevention and reduction of polyneuromyopathy in the critical patient, improved quality of life, shortened ICU and hospital stay, and lesser mortality during hospitalization. However, it is well known that factors such as the protocol used, the population included in the studies, the timing of the strategy, the severity of the patients and different barriers directly influence the outcomes. This study examines the main protocols described in the literature and their associated results...
March 7, 2017: Medicina Intensiva
https://www.readbyqxmd.com/read/28273554/head-drop-after-botox-electrodiagnostic-evaluation-of-iatrogenic-botulinum-toxicity
#9
Eliza Szuch, James B Caress, Bandhu Paudyal, Allison Brashear, Michael S Cartwright, Roy E Strowd
BACKGROUND: Botulinum is a potent neurotoxin with increasing indications for neurologic disorders. While clinical benefit manifests primarily due to local actions at the neuromuscular junction, regional and systemic effects do occur. Rarely, systemic symptoms including weakness, dysarthria, dysphagia and other side effects occur as a result of iatrogenic botulinum neurotoxicity. CASE: A 72 year-old female with right leg dystonia developed head drop, bulbar and systemic weakness following right lower extremity botulinum toxin injection...
March 5, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28269792/ryr1-related-myopathies-clinical-histopathologic-and-genetic-heterogeneity-among-17-patients-from-a-portuguese-tertiary-centre
#10
Raquel Samões, Jorge Oliveira, Ricardo Taipa, Teresa Coelho, Márcio Cardoso, Ana Gonçalves, Rosário Santos, Manuel Melo Pires, Manuela Santos
BACKGROUND: Pathogenic variants in ryanodine receptor type 1 (RYR1) gene are an important cause of congenital myopathy. The clinical, histopathologic and genetic spectrum is wide. OBJECTIVE: Review a group of the patients diagnosed with ryanodinopathy in a tertiary centre from North Portugal, as an attempt to define some phenotypical patterns that may help guiding future diagnosis. METHODS: Patients were identified from the database of the reference centre for Neuromuscular Disorders in North Portugal...
2017: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/28269496/the-correlation-between-transcranial-magnetic-stimulation-parameters-and-neuromuscular-properties-in-children-with-cerebral-palsy
#11
H Marzbani, Sh Parvin, S Amiri, M Lotfian, M R Kharazi, Sh Azizi, M M Mirbagheri
We studied the correlation between corticospinal signaling and neuromuscular properties in children with Cerebral Palsy (CP). Corticospinal signaling was evaluated using Transcranial Magnetic Stimulation (TMS). Neuromuscular properties were quantified using Hoffmann reflex (H-reflex), sonoelastography, clinical measurements, and isokinetic measures. In particular, we determined the relationship between the TMS parameters of the ankle joint and the neuromuscular features of ankle extensors and flexors as well as popular clinical measures of gait speed, endurance, balance and mobility...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28268771/knee-orthosis-with-variable-stiffness-and-damping-that-simulates-hemiparetic-gait
#12
Christina-Anne Lahiff, Tyagi Ramakrishnan, Seok Hun Kim, Kyle Reed
Individuals with unilateral stroke have neuromuscular weakness or paralysis on one side of the body caused by some muscles disengaging and others overexciting. Hyperextension of the knee joint and complete lack of plantar flexion of the ankle joint are common symptoms of stroke. This paper focuses on the creation and implementation of a small, lightweight, and adjustable orthotic device to be positioned around the knee of an able-bodied person to simulate hemiparetic gait. Force and range of motion data from able-bodied subjects fitted with the orthosis, inducing hemiparetic gait, was collected using the Computer Assisted Rehabilitation ENvironment (CAREN) system...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28268468/detection-of-myasthenia-gravis-using-electrooculography-signals
#13
T Liang, M I Boulos, B J Murray, S Krishnan, H Katzberg, K Umapathy
Myasthenia gravis (MG) is an autoimmune neuromuscular disorder resulting from skeletal muscle weakness and fatigue. An early common symptom is fatigable weakness of the extrinsic ocular muscles; if symptoms remain confined to the ocular muscles after a few years, this is classified as ocular myasthenia gravis (OMG). Diagnosis of MG when there are mild, isolated ocular symptoms can be difficult, and currently available diagnostic techniques are insensitive, non-specific or technically cumbersome. In addition, there are no accurate biomarkers to follow severity of ocular dysfunction in MG over time...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28262570/peripheral-muscle-abnormalities-in-cystic-fibrosis-etiology-clinical-implications-and-response-to-therapeutic-interventions
#14
Mathieu Gruet, Thierry Troosters, Samuel Verges
Peripheral muscle dysfunction is an important systemic consequence of cystic fibrosis (CF) with major clinical implications, such as exercise intolerance and reduced quality of life. Evidence is now accumulating that lack of physical activity is unlikely to be the sole explanation for peripheral muscle dysfunction of patients with CF. Particularly, the demonstration of CFTR expression in both murine and human skeletal muscle suggests the potential implication of intrinsic CF-related factors. By combining data from both human and animal models, this review describes CF peripheral muscle abnormalities and critically reviews the advances in understanding the impact of the underlying mechanisms...
March 2, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28256693/-communication-and-language-problems-in-children-with-nemaline-myopathy
#15
J F Cervera-Merida, I Villa-Garcia, A Ygual-Fernandez
INTRODUCTION: Nemaline myopathy is a rare disease with an incidence of 1 in every 50,000 live births. It is the most prevalent of the congenital myopathies, a heterogeneous set of neuromuscular disorders present at birth or manifesting at a very early age, which affect the skeletal muscles and give rise to weakness, hypotonia and psychomotor retardation, although cognitive development remains normal. AIM: To review the studies conducted to date on the communication difficulties and dysphagia of children with nemaline myopathy and their possible management based on speech therapy...
February 24, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28255658/symmetry-tensiomyographic-neuromuscular-response-after-chronic-anterior-cruciate-ligament-acl-reconstruction
#16
Noriaki Maeda, Yukio Urabe, Syogo Tsutsumi, Hironori Fujishita, Shuhei Numano, Takuya Takeuchi, Kazuhiko Hirata, Yukio Mikami, Hiroaki Kimura
PURPOSE: Quadriceps muscle weakness is common following anterior cruciate ligament (ACL) reconstruction. Tensiomyography is a recent method to assess muscle strength, and one that also enables evaluation of individual muscles. The purpose of this study was to evaluate motor unit recruitment and investigate the effects on mechanical and contractile characteristics of the quadriceps and hamstring muscles after chronic ACL reconstruction. METHODS: This study recruited 20 participants: three males and seven females at 24 months after ACL reconstruction, and three males and seven females with no history of knee injury (control group)...
March 2, 2017: Knee Surgery, Sports Traumatology, Arthroscopy: Official Journal of the ESSKA
https://www.readbyqxmd.com/read/28253561/the-neurology-of-acutely-failing-respiratory-mechanics
#17
Eelco F M Wijdicks
Forces involved in breathing-which effectively pull in air-are the diaphragmatic, intercostal, spine, and neck muscles. Equally important are the bulbar musculature maintaining the architecture of a patent airway conduit and abdominal wall and internal intercostal muscles providing cough. Acute injury along a neural trajectory from brainstem to muscle will impair the coordinated interaction between these muscle groups. Acutely failing respiratory mechanics can be caused by central and peripheral lesions. In central lesions, the key lesion is in the nucleus ambiguus innervating the dilator muscles of the soft palate, pharynx, and larynx, but abnormal respiratory mechanics rarely coincide with abnormalities of the respiratory pattern generator...
March 2, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28249029/the-sensitivity-and-specificity-of-the-neurological-examination-in-polyneuropathy-patients-with-clinical-and-electrophysiological-correlations
#18
Alon Abraham, Majed Alabdali, Abdulla Alsulaiman, Hana Albulaihe, Ari Breiner, Hans D Katzberg, Danah Aljaafari, Leif E Lovblom, Vera Bril
INTRODUCTION: Polyneuropathy is one of the most prevalent neurologic disorders. Although several studies explored the role of the neurological examination in polyneuropathy, they were mostly restricted to specific subgroups of patients and have not correlated examination findings with symptoms and electrophysiological results. OBJECTIVES: To explore the sensitivity and specificity of different neurological examination components in patients with diverse etiologies for polyneuropathy, find the most sensitive combination of examination components for polyneuropathy detection, and correlate examination findings with symptoms and electrophysiological results...
2017: PloS One
https://www.readbyqxmd.com/read/28246134/precision-orthotics-optimising-ankle-foot-orthoses-to-improve-gait-in-patients-with-neuromuscular-diseases-protocol-of-the-proof-afo-study-a-prospective-intervention-study
#19
Niels F J Waterval, Frans Nollet, Jaap Harlaar, Merel-Anne Brehm
INTRODUCTION: In patients with neuromuscular disorders and subsequent calf muscle weakness, metabolic walking energy cost (EC) is nearly always increased, which may restrict walking activity in daily life. To reduce walking EC, a spring-like ankle-foot-orthosis (AFO) can be prescribed. However, the reduction in EC that can be obtained from these AFOs is stiffness dependent, and it is unknown which AFO stiffness would optimally support calf muscle weakness. The PROOF-AFO study aims to determine the effectiveness of stiffness-optimised AFOs on reducing walking EC, and improving gait biomechanics and walking speed in patients with calf muscle weakness, compared to standard, non-optimised AFOs...
February 28, 2017: BMJ Open
https://www.readbyqxmd.com/read/28245733/targeted-fascicular-biopsy-of-the-brachial-plexus-rationale-and-operative-technique
#20
Pierre Laumonerie, Stepan Capek, Kimberly K Amrami, P James B Dyck, Robert J Spinner
OBJECTIVE Nerve biopsy is useful in the management of neuromuscular disorders and is commonly performed in distal, noncritical cutaneous nerves. In general, these procedures are diagnostic in only 20%-50%. In selected cases in which preoperative evaluation points toward a more localized process, targeted biopsy would likely improve diagnostic yield. The authors report their experience with targeted fascicular biopsy of the brachial plexus and provide a description of the operative technique. METHODS All cases of targeted biopsy of the brachial plexus biopsy performed between 2003 and 2015 were reviewed...
March 2017: Neurosurgical Focus
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