Proteasome inhibitors for malignancy-related Lambert-Eaton myasthenic syndrome.

abstract：:This investigation analyzes the temporal characteristics of maximal depolarization times for three waveforms: end-plate spikes, fibrillation potentials, and positive sharp waves (PSWs) to provide support for the electrode initiation hypothesis of PSW induction. The maximal depolarization times for PSWs are documented ...

journal_title：Muscle & nerve

authors： Dumitru D,Santa Maria DL

更新日期：2007-09-01 00:00:00

abstract：:The coronavirus disease 2019 (COVID-19) pandemic has resulted in the reorganization of health-care settings affecting clinical care delivery to patients with Duchenne and Becker muscular dystrophy (DBMD) as well as other inherited muscular dystrophies. The magnitude of the impact of this public health emergency on the...

journal_title：Muscle & nerve

authors： Veerapandiyan A,Wagner KR,Apkon S,McDonald CM,Mathews KD,Parsons JA,Wong BL,Eichinger K,Shieh PB,Butterfield RJ,Rao VK,Smith EC,Proud CM,Connolly AM,Ciafaloni E

更新日期：2020-07-01 00:00:00

abstract：INTRODUCTION:Mitochondria are semi-autonomous organelles that are able to change their shape, size, location, and number inside the living cell. Mitochondrial division is an extremely important process, because cell survival depends on there being an adequate number of mitochondria in each cell. The dynamics of these o...

journal_title：Muscle & nerve

authors： Fidziańska A,Glinka Z

更新日期：2012-07-01 00:00:00

abstract：:The clinical and neurophysiological features of multifocal motor neuropathy (MMN) indicate selective involvement of motor axons, but pathological abnormalities in sensory axons suggest a more widespread disease process. The present study was undertaken to determine whether the focal abnormalities are associated with w...

journal_title：Muscle & nerve

authors： Cappelen-Smith C,Kuwabara S,Lin CS,Burke D

更新日期：2002-12-01 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：:Amyotrophic lateral sclerosis (ALS) is a debilitating neurodegenerative disorder with complex biology and significant clinical heterogeneity. Many preclinical and early phase ALS clinical trials have yielded promising results that could not be replicated in larger phase 3 confirmatory trials. One reason for the lack o...

journal_title：Muscle & nerve

authors： Goyal NA,Berry JD,Windebank A,Staff NP,Maragakis NJ,van den Berg LH,Genge A,Miller R,Baloh RH,Kern R,Gothelf Y,Lebovits C,Cudkowicz M

更新日期：2020-08-01 00:00:00

abstract：:In normal skeletal muscle, prolonged stimulation results in some cellular adenosine triphosphate (ATP) being converted to adenosine monophosphate (AMP) and then deaminated to inosine monophosphate (IMP). Here, we investigate whether the build-up of IMP contributes to muscle fatigue and also determine what happens if A...

journal_title：Muscle & nerve

authors： Blazev R,Lamb GD

更新日期：1999-12-01 00:00:00

abstract：:The compound muscle action potential (M(MAX)) during a maximal voluntary contraction (MVC) may be measured to determine if the motor nerve has been supramaximally stimulated during the interpolated twitch technique (ITT). Ten males performed isometric knee extension MVCs. M(MAX) for the vastus medialis was recorded du...

journal_title：Muscle & nerve

authors： Berger MJ,Watson BV,Doherty TJ

更新日期：2010-10-01 00:00:00

abstract：:Muscle of donor origin was formed after implantation of H-2Kb-tsA58 muscle precursor cells (mpc) into irradiated mdx nu/nu mouse muscles. A series of injections of the myotoxin, notexin, which destroys mature muscle fibers but spares muscle precursor cells and other tissues, was made into the mpc-injected muscles, lea...

journal_title：Muscle & nerve

authors： Gross JG,Morgan JE

更新日期：1999-02-01 00:00:00

abstract：:Although serum creatine kinase (CK) levels are frequently modestly elevated in patients with hypothyroid myopathy, elevations in serum CK to the levels usually seen in inflammatory myopathies or dystrophies are rare. We report a patient with progressive proximal weakness and a serum CK level of over 29,000 IU/L, in wh...

journal_title：Muscle & nerve

authors： Scott KR,Simmons Z,Boyer PJ

更新日期：2002-07-01 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：:Nerve conduction studies (NCS) may be deferred because of a perceived risk of cardiac arrhythmia in the presence of same-limb peripheral intravenous lines. Patients with implanted pacemakers or defibrillators provide a model in whom this risk can be assessed. Twenty patients, seven with pacemakers and 13 with defibril...

journal_title：Muscle & nerve

authors： Mellion ML,Buxton AE,Iyer V,Almahameed S,Lorvidhaya P,Gilchrist JM

更新日期：2010-08-01 00:00:00

abstract：:The findings in two cases of congenital myasthenia investigated by intercostal muscle biopsy are presented. The first case, a 16-year-old boy, showed reduced miniature endplate potential amplitude and normal 125I-alpha-bungarotoxin binding to postsynaptic acetylcholine receptors. Muscle biopsy and endplate ultrastruct...

journal_title：Muscle & nerve

authors： Lecky BR,Morgan-Hughes JA,Murray NM,Landon DN,Wray D,Prior C

更新日期：1986-03-01 00:00:00

abstract：:Electron microscopic studies of long-term denervated rat muscles have identified very small, immature myofibers that are believed to arise from detached satellite cells that have fused to form new fibers within the interstitial space. At present, it is unknown whether and to what extent equivalent fibers exist in dene...

journal_title：Muscle & nerve

authors： Doppler K,Mittelbronn M,Bornemann A

更新日期：2008-01-01 00:00:00

abstract：:Ischemia-reperfusion injury is implicated in the failure of free flap and replant surgeries and is associated with the pathogenesis of a wide variety of clinical diseases including stroke, myocardial infarction, spinal injury, and compartment syndromes. We used a skeletal muscle flap model to test if the induction of ...

journal_title：Muscle & nerve

authors： Lille S,Su CY,Schoeller T,Suchy H,Lyons S,Russell RC,Neumeister M,Lai CC

更新日期：1999-03-01 00:00:00

abstract：:Nerve guides seeded with Schwann cells (SCs) promote axonal regeneration in peripheral nerve lesions. We examined the applicability of bioluminescent imaging (BLI) for monitoring the fate of SCs in nerve guides after implantation. Rat SCs were transfected with the firefly luciferase (Fluc) gene and subsequently seeded...

journal_title：Muscle & nerve

authors： Ma MS,Van Dam G,Meek M,Boddeke E,Copray S

更新日期：2009-11-01 00:00:00

abstract：:Myophosphorylase deficiency (McArdle's disease) may present with episodic renal failure following exertion. We present a case of adult-onset myophosphorylase deficiency in which recurrent bouts of renal failure could not be associated with any exertional events until a tonic seizure was witnessed after the fourth epis...

journal_title：Muscle & nerve

authors： Walker AR,Tschetter K,Matsuo F,Flanigan KM

更新日期：2003-11-01 00:00:00

abstract：INTRODUCTION:The contribution of cranial and thoracic region electromyography (EMG) to diagnostic criteria for amyotrophic lateral sclerosis (ALS) has not been evaluated. METHODS:Clinical and EMG data from each craniospinal region were retrospectively assessed in 470 patients; 214 had ALS. Changes to diagnostic classi...

journal_title：Muscle & nerve

authors： Jenkins TM,Alix JJ,Kandler RH,Shaw PJ,McDermott CJ

更新日期：2016-09-01 00:00:00

abstract：INTRODUCTION:Pretreatment with pyridostigmine bromide (PB) of human intercostal muscle fibers exposed to the irreversible acetylcholinesterase (AChE) inhibitor soman was investigated. METHODS:Muscles were pretreated with 3 × 10(-6) M PB or saline for 20 minutes, then exposed to 10(-7) M soman for 10 minutes. RESULTS:...

journal_title：Muscle & nerve

authors： Maselli RA,Henderson JD,Ng J,Follette D,Graves G,Wilson BW

更新日期：2011-04-01 00:00:00

abstract：:The aim of this work was to determine the role of peripheral facial muscle reinnervation in the central reorganization of the blink reflex (BR) after hypoglossal-facial anastomosis (HFA). An electrophysiological study was performed on seven patients who underwent HFA after facial nerve transection during surgery for a...

journal_title：Muscle & nerve

authors： Bernat I,Vitte E,Lamas G,Soudant J,Willer JC,Tankéré F

更新日期：2006-03-01 00:00:00

abstract：:An appreciation of the comparative functions of the corticospinal tract is of direct relevance to the understanding of how results from animal models can advance knowledge of the human motor system and its disorders. Two critical functions of the corticospinal tract are discussed: first, the role of descending project...

journal_title：Muscle & nerve

authors： Lemon RN,Griffiths J

更新日期：2005-09-01 00:00:00

abstract：:We have previously demonstrated that patients with mitochondrial myopathies can benefit from short-term aerobic exercise training. In this study, we compared the responses to short-term aerobic training of patients with mitochondrial myopathies, patients with nonmetabolic myopathies, and sedentary normal subjects. Tra...

journal_title：Muscle & nerve

authors： Taivassalo T,De Stefano N,Chen J,Karpati G,Arnold DL,Argov Z

更新日期：1999-09-01 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：INTRODUCTION:Transitioning from adolescence to adulthood can be problematic for individuals with rare disabilities such as muscular dystrophy (MD). METHODS:We identified a cohort of 220 individuals with MD and 440 matched comparison individuals and measured emergency room (ER) and inpatient (IP) encounters for the yea...

journal_title：Muscle & nerve

authors： Mann JR,Royer JA,Mcdermott S,Hardin JW,Ozturk O,Street N

更新日期：2015-11-01 00:00:00

abstract：:A large Swedish family with members affected by progressive external ophthalmoplegia with hypogonadism were followed-up and reviewed. Hypogonadism included delayed sexual maturation, primary amenorrhea, early menopause, and testicular atrophy. Cataracts, cerebellar ataxia, neuropathy, hypoacusia, pes cavus, tremor, pa...

journal_title：Muscle & nerve

authors： Melberg A,Arnell H,Dahl N,Stålberg E,Raininko R,Oldfors A,Bakall B,Lundberg PO,Holme E

更新日期：1996-12-01 00:00:00

abstract：:Many patients with the limb-girdle variant of congenital myasthenic syndrome (CMS) possess mutations in the human Dok-7 gene (DOK7). We identified six unrelated CMS patients with DOK7 mutations. Two patients, one mildly and the other moderately affected, were homozygous for the previously described 1263insC mutation. ...

journal_title：Muscle & nerve

authors： Anderson JA,Ng JJ,Bowe C,McDonald C,Richman DP,Wollmann RL,Maselli RA

更新日期：2008-04-01 00:00:00

abstract：INTRODUCTION:Muscle fatigue could have a greater impact on position sense when antagonists of the movement are fatigued. Hence, this study aimed to compare the effects of antagonist and agonist exercise-induced muscle fatigue on knee joint position sense. METHODS:This within-subjects repeated-measures study included 4...

journal_title：Muscle & nerve

authors： Ribeiro F,Venâncio J,Quintas P,Oliveira J

更新日期：2011-08-01 00:00:00

abstract：:We measured the peak voltage induced in a sensing loop by a Magstim 200 magnetic stimulator. Coil output varied little for repeated stimulation at the same intensity over a wide range of coil output. In contrast, the first stimulus immediately after a change in intensity was of larger amplitude and showed greater vari...

journal_title：Muscle & nerve

authors： Reutens DC,MacDonell RA,Berkovic SF

更新日期：1993-12-01 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：:The physiological and phenotypic properties of motor units in the cat soleus muscle were studied after 4 months of inactivity induced by spinal cord isolation (SI). The soleus of some SI cats were stimulated for 30 min/day during an isometric (SI-I), shortening (SI-S), or lengthening (SI-L) phase of a simulated step c...

journal_title：Muscle & nerve

authors： Zhong H,Roy RR,Hodgson JA,Talmadge RJ,Grossman EJ,Edgerton VR

更新日期：2002-08-01 00:00:00