Muscular dystrophy in a litter of golden retriever dogs.

abstract：INTRODUCTION:Hereditary transthyretin (hATTR) amyloidosis is a progressive, degenerative disease, with peripheral neuropathy, cardiomyopathy, and other clinical manifestations. In this study we examine the impact of hATTR amyloidosis on quality of life (QOL). METHODS:Neuropathy-specific QOL, measured with the Norfolk ...

journal_title：Muscle & nerve

authors： Yarlas A,Gertz MA,Dasgupta NR,Obici L,Pollock M,Ackermann EJ,Lovley A,Kessler AS,Patel PA,White MK,Guthrie SD

更新日期：2019-08-01 00:00:00

abstract：INTRODUCTION:Anecdotal reports from families and care providers suggest a wide variation in services received by individuals with Duchenne/Becker muscular dystrophy (DBMD). METHODS:We documented the type and frequency of health services received by individuals with DBMD using the Muscular Dystrophy Surveillance Tracki...

journal_title：Muscle & nerve

authors： Pandya SK,Campbell KA,Andrews JG,Meaney FJ,Ciafaloni E

更新日期：2016-02-01 00:00:00

abstract：:It has been suggested that vibration causes small changes in muscle length, but to the best of our knowledge, these have yet to be demonstrated during whole-body vibration (WBV). This was an observational study to determine whether acute WBV would result in muscle lengthening. We hypothesized that acute WBV would incr...

journal_title：Muscle & nerve

authors： Cochrane DJ,Loram ID,Stannard SR,Rittweger J

更新日期：2009-09-01 00:00:00

abstract：INTRODUCTION:Twenty-six patients with clinical symptoms of adult onset carnitine palmitoyltransferase II (CPTII) deficiency were examined. All patients had skeletal muscle CPTII enzyme activity levels indicative of heterozygosity for CPT2 mutations, however sequence analysis identified no pathogenic mutations within th...

journal_title：Muscle & nerve

authors： Isackson PJ,Sutton KA,Hostetler KY,Vladutiu GD

更新日期：2013-02-01 00:00:00

abstract：:We compared the diaphragmatic electromyographic (EMG) recordings from 32 patients with known neuromuscular disease and respiratory symptoms (23 neuropathies, 9 myopathies) to recordings from 23 normal subjects. Turns analysis of 219-ms sections, or epochs, of the EMG demonstrated a significant overlap between diagnost...

journal_title：Muscle & nerve

authors： McKeown MJ,Bolton CF

更新日期：1998-07-01 00:00:00

abstract：:Toxoplasma gondii encysts in skeletal muscle. Although only rarely found at muscle biopsy, this parasite has previously been regarded as a possible cause of polymyositis. We report a case of biopsy-proven toxoplasmic myositis in a non-HIV-infected patient that led to recognition of idiopathic CD4 lymphocytopenia (ICL)...

journal_title：Muscle & nerve

authors： Plonquet A,Bassez G,Authier FJ,Dray JM,Farcet JP,Gherardi RK

更新日期：2003-06-01 00:00:00

abstract：INTRODUCTION:The ability to distinguish between normal thymus, thymic hyperplasia, and thymoma should aid clinical management and decision making in patients with myasthenia gravis (MG). We sought to determine the accuracy of routine imaging in predicting thymic pathology. METHODS:We retrospectively analyzed records o...

journal_title：Muscle & nerve

authors： Klimiec E,Quirke M,Leite MI,Hilton-Jones D

更新日期：2018-02-09 00:00:00

abstract：:A prospective study was done on 222 consecutive new patients referred to our pediatric muscle clinic to assess the diagnostic value of ultrasound imaging. Ultrasound scans were interpreted without knowledge of clinical presentation or results of other tests. Muscular dystrophy produced a brightly speckled pattern of i...

journal_title：Muscle & nerve

authors： Heckmatt JZ,Pier N,Dubowitz V

更新日期：1988-01-01 00:00:00

abstract：INTRODUCTION:Duchenne muscular dystrophy (DMD) subjects ≥5 years with nonsense mutations were followed for 48 weeks in a multicenter, randomized, double-blind, placebo-controlled trial of ataluren. Placebo arm data (N = 57) provided insight into the natural history of the 6-minute walk test (6MWT) and other endpoints. ...

journal_title：Muscle & nerve

authors： McDonald CM,Henricson EK,Abresch RT,Florence JM,Eagle M,Gappmaier E,Glanzman AM,PTC124-GD-007-DMD Study Group.,Spiegel R,Barth J,Elfring G,Reha A,Peltz S

更新日期：2013-09-01 00:00:00

abstract：:There were 31 Duchenne patients placed on overnight mouth intermittent positive pressure ventilation for severe respiratory insufficiency at the average age of 19.9 years. Most patients had vital capacities less than 200 cc at their last evaluations. Of these, 23 patients are alive at the average age of 27 years and l...

journal_title：Muscle & nerve

authors： Bach JR,O'Brien J,Krotenberg R,Alba AS

更新日期：1987-02-01 00:00:00

abstract：:A 62-year-old woman with axonal Guillain-Barré syndrome developed weakness and urinary retention simultaneously. The retention failed to recover for 10 months even after she regained the ability to walk. The patient exhibited no postural hypotension. Videourodynamics showed that the retention was caused not by the bla...

journal_title：Muscle & nerve

authors： Sakakibara R,Uchiyama T,Tamura N,Kuwabara S,Asahina M,Hattori T

更新日期：2007-01-01 00:00:00

abstract：:The distribution of collagen types I, III, and V and fibronectin was investigated by means of immunofluorescent techniques in denervated and normal rat skeletal muscle. During a period of 28 days, a distinct atrophy developed in the denervated gastrocnemius muscle and was accompanied by an increase in types I and III ...

journal_title：Muscle & nerve

authors： Salonen V,Lehto M,Kalimo M,Penttinen R,Aro H

更新日期：1985-02-01 00:00:00

abstract：INTRODUCTION:Many prognostic factors have been studied in carpal tunnel decompression, but most studies consider only a subset of variables. METHODS:Three thousand three hundred thirty-two operations were used to develop prognostic models, and 885 operations were used for validation. Outcome recorded on a Likert scale...

journal_title：Muscle & nerve

authors： Bowman A,Rudolfer S,Weller P,Bland JDP

更新日期：2018-12-01 00:00:00

abstract：:Contractions of rat extensor digitorum longus (EDL, a fast muscle) and soleus (SOL, a slow muscle) muscles of different ages (1-4 weeks) were recorded in vitro with direct stimulation and at different temperatures (range 35-10 degrees C). Twitch tension in 4-week-old EDL muscle increased in cooling from 35 to 20 degre...

journal_title：Muscle & nerve

authors： Wylie SR,Ranatunga KW

更新日期：1987-11-01 00:00:00

abstract：INTRODUCTION:The minimal clinically important difference (MCID) is the smallest outcome change that has clinical significance. Its use has not been established in the study of myasthenia gravis (MG). METHODS:Patients from a published intravenous immunoglobulin (IVIg) vs. placebo study were studied. One anchor-based an...

journal_title：Muscle & nerve

authors： Katzberg HD,Barnett C,Merkies IS,Bril V

更新日期：2014-05-01 00:00:00

abstract：:The latency fluctuation of single motor unit potentials (MUPH) in the H-reflex is greater than the latency fluctuation of MUPs in the direct (MUPM) and recurrent (MUPF) responses. This has been attributed to the variability in the impulse generation at the site of nerve stimulation, and to the variation in the synapti...

journal_title：Muscle & nerve

authors： Soliven B,Maselli RA

更新日期：1992-06-01 00:00:00

abstract：INTRODUCTION:Mesenchymal stem cells (MSCs) can differentiate into various cell types. METHODS:In this study we investigated the potential of human tonsil-derived MSCs (T-MSCs) for neuromuscular regeneration in trembler-J (Tr-J) mice, a model for Charcot-Marie-Tooth disease type 1A (CMT1A). RESULTS:T-MSCs differentiat...

journal_title：Muscle & nerve

authors： Park S,Choi Y,Kwak G,Hong YB,Jung N,Kim J,Choi BO,Jung SC

更新日期：2018-03-01 00:00:00

abstract：:High-density multichannel electromyography (EMG) recordings add spatial information to the temporal information content of the surface EMG (sEMG) signal. This study explores the potential value of such multichannel information at a single motor unit level, in particular for the improvement of motor unit number estimat...

journal_title：Muscle & nerve

authors： Blok JH,Van Dijk JP,Zwarts MJ,Stegeman DF

更新日期：2005-09-01 00:00:00

abstract：INTRODUCTION:In this study we estimated the costs paid by U.S. health plans for treating myasthenia gravis (MG) in 2009 and determined the major cost drivers. METHODS:One hundred thirteen MG patients were matched by propensity scores with 339 non-MG patients from a comprehensive health-care insurance database. The mea...

journal_title：Muscle & nerve

authors： Guptill JT,Sharma BK,Marano A,Soucy A,Krueger A,Sanders DB

更新日期：2012-03-01 00:00:00

abstract：INTRODUCTION:Diaphragm excursion measured via ultrasound may be an important imaging outcome measure of respiratory function. We developed a new method for measuring diaphragm movement and compared it to the more traditional M-mode method. METHODS:Ultrasound images of the right and left hemidiaphragms were collected t...

journal_title：Muscle & nerve

authors： Goutman SA,Hamilton JD,Swihart B,Foerster B,Feldman EL,Rubin JM

更新日期：2017-01-01 00:00:00

abstract：:Laser Doppler flowmetry (LDF) was conducted on familial amyloidotic polyneuropathy (FAP) patients and asymptomatic carriers of FAP. Vasoconstrictive responses in the 11 FAP patients tested, induced by deep inspiration, were markedly depressed compared with those of the healthy controls. The responses decreased with th...

journal_title：Muscle & nerve

authors： Ando Y,Araki S,Shimoda O,Kano T

更新日期：1992-04-01 00:00:00

abstract：INTRODUCTION:The purpose of this study was to investigate in vivo median nerve longitudinal mobility in different segments of the carpal tunnel associated with active finger motion in carpal tunnel syndrome (CTS) patients in a comparison with healthy controls. METHODS:Eleven healthy volunteers and 11 CTS patients part...

journal_title：Muscle & nerve

authors： Yao Y,Grandy E,Evans PJ,Seitz WH Jr,Li ZM

更新日期：2020-10-01 00:00:00

abstract：:A case study of a 50-year-old man who awakened from aortobifemoral bypass graft surgery with painful burning and numbness of the left foot is reported. Angiography showed embolic occlusion of the left popliteal artery proximal to the knee. Nerve conduction studies performed 1 year after occlusion showed loss or severe...

journal_title：Muscle & nerve

authors： Levin KH

更新日期：1989-10-01 00:00:00

abstract：INTRODUCTION:The current practice of single-fiber electromyography (SFEMG) requires that 20 fiber pairs with normal jitter be collected to exclude myasthenia gravis (MG). We applied principles of futility analysis from clinical trials in an attempt to reduce that requirement. METHODS:We utilized conditional power futi...

journal_title：Muscle & nerve

authors： Narayanaswami P,Pantoja-Galicia N,Betensky RA,Rutkove SB

更新日期：2012-04-01 00:00:00

abstract：:Conduction velocity of A delta fibers of the human peripheral nerves was measured by using pain-related somatosensory evoked potentials following CO2 laser stimulation. It was found to be approximately 9 m/s in the forearm as well as in the lower leg. Because conventional conduction study using electric stimulation re...

journal_title：Muscle & nerve

authors： Kakigi R,Endo C,Neshige R,Kuroda Y,Shibasaki H

更新日期：1991-12-01 00:00:00

abstract：INTRODUCTION:The risk of cancer in patients diagnosed with myotonic dystrophy (DM) is reported for the homogeneous Utah population. METHODS:Clinical data accessed from the largest Utah healthcare providers have been record-linked to the Utah Population Database, a population-based resource also linked to the Utah Canc...

journal_title：Muscle & nerve

authors： Abbott D,Johnson NE,Cannon-Albright LA

更新日期：2016-10-01 00:00:00

abstract：:Ten adult myotonic dystrophy patients underwent measurements of lung function, maximal dynamic and static ventilatory efforts, and respiratory muscle electromyography (EMG). EMG studies were performed during spontaneous breathing or when subjects breathed through high inspiratory or expiratory resistive loads. Present...

journal_title：Muscle & nerve

authors： Jammes Y,Pouget J,Grimaud C,Serratrice G

更新日期：1985-09-01 00:00:00

abstract：:Immersion of rat hemidiaphragms in Ca2+-free Krebs solution (KS) containing Ca2+ chelator in vitro leads to separation of basal lamina from the plasma membrane, as well as transient contracture and rapid loss of twitch response [calcium paradox (CP) phase 1]. Subsequent immersion in regular KS results in necrosis of m...

journal_title：Muscle & nerve

authors： Soza M,Karpati G,Carpenter S

更新日期：1986-03-01 00:00:00

abstract：:Techniques to estimate motor unit number (MUNE) measure the number of functioning motor units in a muscle. As amyotrophic lateral sclerosis (ALS) is characterized by progressive motor unit loss, this disease offers an ideal setting for the use of MUNE. Statistical MUNE was employed in a recent multicenter trial of cre...

journal_title：Muscle & nerve

authors： Shefner JM,Cudkowicz ME,Zhang H,Schoenfeld D,Jillapalli D,Northeast ALS Consortium.

更新日期：2007-02-01 00:00:00

abstract：:The fastest median and ulnar velocities derived by recording motor and mixed nerve action potentials, F waves, H-reflexes, and somatosensory evoked potentials (SEPs) were compared. H-reflex recording was facilitated by employing selective group Ia excitation during voluntary muscular contraction. Mixed nerve, SEP, and...

journal_title：Muscle & nerve

authors： Eisen A,Hoirch M,White J,Calne D

更新日期：1984-10-01 00:00:00