Satisfaction with ankle foot orthoses in individuals with Charcot-Marie-Tooth disease.

abstract：INTRODUCTION:Herein we present an exploratory study of orofacial function in children with congenital myotonic dystrophy (CDM) vs. healthy controls. METHODS:We evaluated 41 children with CDM and 29 healthy controls for speech and swallow function and for lingual and labial strength. RESULTS:The Iowa Oral Performance ...

journal_title：Muscle & nerve

authors： Berggren KN,Hung M,Dixon MM,Bounsanga J,Crockett B,Foye MD,Gu Y,Campbell C,Butterfield RJ,Johnson NE

更新日期：2018-09-01 00:00:00

abstract：:We studied the effects of a 12-week progressive resistance strength training program in weakened muscles of 5 patients with sporadic inclusion body myositis (IBM). Strength was evaluated with Medical Research Council (MRC) scale ratings and quantitative isometric and dynamic tests. Changes in serum creatine kinase (CK...

journal_title：Muscle & nerve

authors： Spector SA,Lemmer JT,Koffman BM,Fleisher TA,Feuerstein IM,Hurley BF,Dalakas MC

更新日期：1997-10-01 00:00:00

abstract：:We propose a comprehensive model of spastic hypertonia based on clinical neurophysiology and validated using experimental data obtained from the pendulum test of the leg in 8 healthy volunteers and 15 spastic patients. This nonlinear computational model includes mechanical parameters and a stretch reflex representatio...

journal_title：Muscle & nerve

authors： Le Cavorzin P,Poudens SA,Chagneau F,Carrault G,Allain H,Rochcongar P

更新日期：2001-12-01 00:00:00

abstract：:Three cases of chronic distal sensorimotor neuropathy are described in patients with neurofibromatosis. One had type 2 or central neurofibromatosis with a chromosome 22 deletion; the precise form of the disease was not established in the other two. A striking clinical feature was a diffuse nodular enlargement of the p...

journal_title：Muscle & nerve

authors： Thomas PK,King RH,Chiang TR,Scaravilli F,Sharma AK,Downie AW

更新日期：1990-02-01 00:00:00

abstract：:We applied the singular value decomposition (SVD) method to study single motor unit firing patterns. Two projects were carried out: (1) a computer simulation study to confirm the meanings of two SVD parameters, the eigenvalue corresponding to the positive-slope eigenvector (PEV) and that corresponding to the negative-...

journal_title：Muscle & nerve

authors： Sun TY,Chen JJ,Lin TS

更新日期：2000-07-01 00:00:00

abstract：:Platelet-derived microparticle (PDMP) levels were measured using an enzyme-linked immunosorbent assay (ELISA) to elucidate the role of platelet activation in patients with polymyositis or dermatomyositis (PM/DM). PDMP levels in active PM/DM patients (median 13.3 U/ml, interquartile range 9.9-20.7 U/ml, n = 16) and tho...

journal_title：Muscle & nerve

authors： Shirafuji T,Hamaguchi H,Higuchi M,Kanda F

更新日期：2009-05-01 00:00:00

abstract：:New developments in the rapid diagnosis and treatment of boys with Duchenne muscular dystrophy (DMD) have led to growing enthusiasm for instituting DMD newborn screening (NBS) in the United States. Our group has been interested in developing clinical guidance to be implemented consistently in specialty care clinics ch...

journal_title：Muscle & nerve

authors： Kwon JM,Abdel-Hamid HZ,Al-Zaidy SA,Mendell JR,Kennedy A,Kinnett K,Cwik VA,Street N,Bolen J,Day JW,Connolly AM

更新日期：2016-08-01 00:00:00

abstract：INTRODUCTION:Glucocorticosteroids (GC) are effective in slowing weakness in boys with Duchenne muscular dystrophy (DMD). METHODS:This is a multisite, 1-year, open-label trial of twice-weekly prednisolone (5 mg/kg/dose) in infants/young boys (0.4-2.4 years) with DMD. We compared changes in Bayley III Scales of Infant D...

journal_title：Muscle & nerve

authors： Connolly AM,Zaidman CM,Golumbek PT,Cradock MM,Flanigan KM,Kuntz NL,Finkel RS,McDonald CM,Iannaccone ST,Anand P,Siener CA,Florence JM,Lowes LP,Alfano LN,Johnson LB,Nicorici A,Nelson LL,Mendell JR,MDA DMD Clinical Resea

更新日期：2019-06-01 00:00:00

abstract：:Physicians have an ethical and legal duty to obtain patients' informed consent before ordering testing and treatment. Informed consent encompasses informed refusal, the correlative right of patients to refuse tests and therapies they do not want. A patient's informed consent requires adequate information, capacity to ...

journal_title：Muscle & nerve

authors： Bernat JL

更新日期：2001-05-01 00:00:00

abstract：:We studied histopathological changes in the biceps brachii muscle in relation to age and the degree of muscle weakness in 64 patients (aged 11-59 years) with myotonic dystrophy. The proportion of type 1 fibers was unaltered in the adolescent patients compared with control values, but increased with age. The average di...

journal_title：Muscle & nerve

authors： Tohgi H,Kawamorita A,Utsugisawa K,Yamagata M,Sano M

更新日期：1994-09-01 00:00:00

abstract：:The cerebrospinal fluid (CSF) of patients with demyelinating neurological disease, such as Guillain-Barré syndrome or multiple sclerosis, contains factors that inhibit the excitatory Na+ current. Such antiexcitatory factors are occasionally also detectable in CSF from patients with other neurological diseases but were...

journal_title：Muscle & nerve

authors： Brinkmeier H,Seewald MJ,Wollinsky KH,Rüdel R

更新日期：1996-01-01 00:00:00

abstract：:Compound muscle action potentials (CMAPs) evoked by magnetic brain stimuli are larger if the subject provides a steady background voluntary contraction of the target muscle. This facilitation could be due either to cortical or spinal mechanisms, or both. Both magnetic and electrical stimuli given immediately after the...

journal_title：Muscle & nerve

authors： Mills KR,Kimiskidis V

更新日期：1996-08-01 00:00:00

abstract：:We investigated the possible role of extracellular matrix in specifying the expression of superfast myosin during cat jaw muscle regeneration. Equal proportions of muscle tissue from jaw and limb were minced together after killing cellular elements from one source. We allowed the mince to regenerate in the bed of a fa...

journal_title：Muscle & nerve

authors： Hoh JF,Hughes S

更新日期：1991-05-01 00:00:00

abstract：:Seven 2-day-old golden retriever pups were given focal intramuscular injections of a first generation adenovirus-dystrophin minigene construct and adenovirus-beta-galactosidase construct as a 2:1 mixture into the left anterior tibial muscle. The spread of transgene expression within the anterior tibial muscle was comp...

journal_title：Muscle & nerve

authors： O'Hara AJ,Howell JM,Taplin RH,Fletcher S,Lloyd F,Kakulas B,Lochmüller H,Karpati G

更新日期：2001-04-01 00:00:00

abstract：:In muscle of whole animals, pituitary growth hormone, the thyroid hormones, and insulin are major growth-promoting hormones, and the glucocorticoids have significant catabolic actions. At the cellular level the primary anabolic hormones for cultured myoblasts are the somatomedins (insulin-like growth factors) and fibr...

journal_title：Muscle & nerve

authors： Florini JR

更新日期：1987-09-01 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：INTRODUCTION:In preparation for clinical trials we examine the validity, reliability, and patient understanding of the Myotonic Dystrophy Health Index (MDHI). METHODS:Initially we partnered with 278 myotonic dystrophy type-1 (DM1) patients and identified the most relevant questions for the MDHI. Next, we used factor a...

journal_title：Muscle & nerve

authors： Heatwole C,Bode R,Johnson N,Dekdebrun J,Dilek N,Heatwole M,Hilbert JE,Luebbe E,Martens W,Mcdermott MP,Rothrock N,Thornton C,Vickrey BG,Victorson D,Moxley R 3rd

更新日期：2014-06-01 00:00:00

abstract：:Sucrose gap and intra-axonal recording techniques were used to identify the types of ion channels and inward rectification that are present in regenerated axons of adult (greater than 8 weeks) rat sciatic nerve after crush injury. In sucrose gap recordings, 4-aminopyridine (4-AP) led to slight broadening of the compou...

journal_title：Muscle & nerve

authors： Gordon TR,Kocsis JD,Waxman SG

更新日期：1991-07-01 00:00:00

abstract：:At least 50 disease-causing mutations in the skeletal muscle voltage-gated chloride channel gene (CLCN1), almost all of which originate from Caucasian families, have been identified. We investigated a Japanese family with Thomsen's myotonia congenita that included 16 affected individuals (8 men and 8 women) through fi...

journal_title：Muscle & nerve

authors： Sasaki R,Ito N,Shimamura M,Murakami T,Kuzuhara S,Uchino M,Uyama E

更新日期：2001-03-01 00:00:00

abstract：:The possibility has been raised that in familial periodic paralysis, the relatively large depolarization of the muscle fiber membrane seen during periods of paralysis might be attributable to a reduction in activity of the electrogenic Na(+)-K+ pump. The theoretical analysis presented here shows that although such hyp...

journal_title：Muscle & nerve

authors： Martin AR,Levinson SR

更新日期：1985-06-01 00:00:00

abstract：:Involuntary muscle contractions are common after spinal cord injury (SCI). Increased sensitivity to Ia muscle afferent input may contribute to the development of these spasms. Since tendon vibration results in a period of postactivation depression of the Ia synapse, we sought to determine whether Achilles tendon vibra...

journal_title：Muscle & nerve

authors： Butler JE,Godfrey S,Thomas CK

更新日期：2006-05-01 00:00:00

abstract：:We investigated factors affecting maximal voluntary torque and the assessment of the level of voluntary drive in the elbow flexor muscles. First, the effective compliance of the system was tested by using single, paired, and trains of four stimuli to measure voluntary activation. At high voluntary torques the response...

journal_title：Muscle & nerve

authors： Allen GM,McKenzie DK,Gandevia SC

更新日期：1998-03-01 00:00:00

abstract：:Data are scarce relating to the plasticity with strength training of dynamic muscle performance in older humans. Hence, we investigated alterations in the torque-velocity relation with strength training in old age, and their origin. Knee extension and leg-press exercises were performed three times per week for 14 week...

journal_title：Muscle & nerve

authors： Reeves ND,Maganaris CN,Narici MV

更新日期：2005-03-01 00:00:00

abstract：:Ion channels are complex proteins that span the lipid bilayer of the cell membrane, where they orchestrate the electrical signals necessary for normal function of the central nervous system, peripheral nerve, and both skeletal and cardiac muscle. The role of ion channel defects in the pathogenesis of numerous disorder...

journal_title：Muscle & nerve

authors： Kleopa KA,Barchi RL

更新日期：2002-09-01 00:00:00

abstract：INTRODUCTION:We sought to determine which muscles to choose for better assessment of the craniobulbar region in establishing the diagnosis of amyotrophic lateral sclerosis (ALS). METHODS:We studied the frontalis muscle in 83 controls and compared it with the tongue, sternocleidomastoid (SCM), and trapezius muscles in ...

journal_title：Muscle & nerve

authors： Pan H,Jian F,Lin J,Chen N,Zhang Z,Wang Y,Cui L,Kimura J

更新日期：2016-12-01 00:00:00

abstract：INTRODUCTION:Improving axonal outgrowth and remyelination is crucial for peripheral nerve regeneration. Miconazole appears to enhance remyelination in the central nervous system. In this study we assess the effect of miconazole on axonal regeneration using a sciatic nerve crush injury model in rats. METHODS:Fifty Spra...

journal_title：Muscle & nerve

authors： Lin T,Qiu S,Yan L,Zhu S,Zheng C,Zhu Q,Liu X

更新日期：2018-05-01 00:00:00

abstract：:The purpose of this long-term, open parallel-group, double-consent study of alternate-day, low-dose prednisone in 2-4-year-old patients with Duchenne muscular dystrophy (DMD) was to determine whether prednisone produces a beneficial effect when given earlier than usual. Muscle function was evaluated by timed tests, an...

journal_title：Muscle & nerve

authors： Merlini L,Cicognani A,Malaspina E,Gennari M,Gnudi S,Talim B,Franzoni E

更新日期：2003-02-01 00:00:00

abstract：INTRODUCTION:Myelinating Schwann cells compartmentalize their outermost layer to form actin-rich channels known as Cajal bands. Herein we investigate changes in Schwann cell architecture and cytoplasmic morphology in a novel mouse model of carpal tunnel syndrome. METHODS:Chronic nerve compression (CNC) injury was crea...

journal_title：Muscle & nerve

authors： Gupta R,Nassiri N,Hazel A,Bathen M,Mozaffar T

更新日期：2012-02-01 00:00:00

abstract：:The intramembrane particle (IMP) profile of control and dystrophic (Bio 14.6) hamster cardiac muscle plasma membrane was assessed in freeze-fracture replicas to determine whether this animal model of muscular dystrophy exhibits the same membrane characteristics found in skeletal muscle from other more thoroughly studi...

journal_title：Muscle & nerve

authors： Graham KA,Shivers RR,Atkinson BG

更新日期：1984-09-01 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