Clinical Follow-Up for Duchenne Muscular Dystrophy Newborn Screening: A Proposal.

abstract：:Although the effect of low temperature on the peripheral nervous system has been systematically studied, the effect of high temperature has not. We investigated the effect of elevating limb temperature from 32 degrees C to 42 degrees C by performing sequential motor studies, antidromic sensory studies, and 3-Hz repeti...

journal_title：Muscle & nerve

authors： Rutkove SB,Kothari MJ,Shefner JM

更新日期：1997-04-01 00:00:00

abstract：:The objective of this study was to determine the self-reported prevalence of needlestick injuries among practicing electromyographers. In January 2008, an anonymous electronic survey was sent to all active members of the American Association for Neuromuscular and Electrodiagnostic Medicine (AANEM) who provided e-mail ...

journal_title：Muscle & nerve

authors： Mateen FJ,Grant IA,Sorenson EJ

更新日期：2008-12-01 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：:Ultrasound has been used for visualizing peripheral nerve pathology. Our goal was to use ultrasound to quantitate the sizes of upper extremity nerves along their length in control subjects and patients with neuropathy. We measured median and ulnar nerve cross-sectional areas (NCSA) in the arms of 190 subjects, includi...

journal_title：Muscle & nerve

authors： Zaidman CM,Al-Lozi M,Pestronk A

更新日期：2009-12-01 00:00:00

abstract：:This study was designed to compare electromyogram (EMG) and acoustic myogram (AMG) recordings of biceps brachii muscles in patients with spastic cerebral palsy (CP). The maximal voluntary contraction (MVC) in the CP group was approximately one half of that of the normal group even after being normalized by the muscle ...

journal_title：Muscle & nerve

authors： Akataki K,Mita K,Itoh K,Suzuki N,Watakabe M

更新日期：1996-10-01 00:00:00

abstract：:Prolonged exposure to hand-transmitted vibration can cause debilitating neural and vascular dysfunction in humans. It is unclear whether the pathophysiology involves simultaneous or sequential injury of arteries and nerves. The mechanism of vibration injury was investigated in a rat tail model, containing arteries and...

journal_title：Muscle & nerve

authors： Curry BD,Bain JL,Yan JG,Zhang LL,Yamaguchi M,Matloub HS,Riley DA

更新日期：2002-04-01 00:00:00

abstract：:Cytoplasmic body myopathy (CBM) is characterized by proteinaceous inclusion bodies in muscle tissue. A 43-year-old woman presented with rapidly progressive weakness and dysphagia. Electromyography (EMG) elsewhere demonstrated lower-limb chronic partial denervation. Muscle biopsy showed fiber size variation without dia...

journal_title：Muscle & nerve

authors： Krishnan AV,Pamphlett R,Burke D,Wills EJ,Kiernan MC

更新日期：2004-11-01 00:00:00

abstract：INTRODUCTION:In this study, we determined normal levels of dysferlin expression in CD14+ monocytes by flow cytometry (FC) as a screening tool for dysferlinopathies. METHODS:Monocytes from 183 healthy individuals and 29 patients were immunolabeled, run on an FACScalibur flow cytometer, and analyzed by FlowJo software. ...

journal_title：Muscle & nerve

authors： Sánchez-Chapul L,Ángel-Muñoz MD,Ruano-Calderón L,Luna-Angulo A,Coral-Vázquez R,Hernández-Hernández Ó,Magaña JJ,León-Hernández SR,Escobar-Cedillo RE,Vargas S

更新日期：2016-12-01 00:00:00

abstract：:Calpain 3 (C3) is the only muscle-specific member of the calcium-dependent protease family. Although neither its physiological function nor its in vivo substrates are known, C3 must be an important protein for normal muscle function as mutations in the C3 gene result in limb-girdle muscular dystrophy type 2A. Previous...

journal_title：Muscle & nerve

authors： Guyon JR,Kudryashova E,Potts A,Dalkilic I,Brosius MA,Thompson TG,Beckmann JS,Kunkel LM,Spencer MJ

更新日期：2003-10-01 00:00:00

abstract：INTRODUCTION:Peripheral nerves are often exposed to mechanical stress leading to compression neuropathies. The pathophysiology underlying nerve dysfunction by chronic compression is largely unknown. METHODS:We analyzed molecular organization and fine structures at and near nodes of Ranvier in a compression neuropathy ...

journal_title：Muscle & nerve

authors： Otani Y,Yermakov LM,Dupree JL,Susuki K

更新日期：2017-04-01 00:00:00

abstract：INTRODUCTION:RASopathies are a group of genetic conditions due to alterations of the Ras/MAPK pathway. Neurocutaneous findings are hallmark features of the RASopathies, but musculoskeletal abnormalities are also frequent. The objective was to evaluate handgrip strength in the RASopathies. METHODS:Individuals with RASo...

journal_title：Muscle & nerve

authors： Stevenson DA,Allen S,Tidyman WE,Carey JC,Viskochil DH,Stevens A,Hanson H,Sheng X,Thompson BA,Okumura MJ,Reinker K,Johnson B,Rauen KA

更新日期：2012-09-01 00:00:00

abstract：INTRODUCTION:Although formal spirometry is the gold standard for monitoring respiratory function in patients with myasthenia gravis (MG), such testing is often delayed or unavailable. There is a need for a simple bedside test that can accurately measure respiratory function. METHODS:We conducted a prospective, cross-s...

journal_title：Muscle & nerve

authors： Elsheikh B,Arnold WD,Gharibshahi S,Reynolds J,Freimer M,Kissel JT

更新日期：2016-01-01 00:00:00

abstract：INTRODUCTION:Psoriasis is a T-cell-mediated skin disorder with uncommon extracutaneous manifestations. Rare patients with psoriasis and myopathy have been reported. METHODS:We conducted a retrospective review of medical records of psoriasis patients seen at the Mayo Clinic during the period from January 1, 1996 to May...

journal_title：Muscle & nerve

authors： Liewluck T,Ernste FC,Tracy JA

更新日期：2013-11-01 00:00:00

abstract：:We studied the mechanism underlying acute organophosphate intoxication (OPI) through in-vivo and in-vitro electrophysiologic studies in rats injected with diisopropylfluorophosphate. Intoxicated rats showed weakness, repetitive compound muscle action potentials (CMAPs) in response to a single stimulus, and decremental...

journal_title：Muscle & nerve

authors： Maselli RA,Soliven BC

更新日期：1991-12-01 00:00:00

abstract：INTRODUCTION:Duration of training to reliably measure nerve cross-sectional area with ultrasound is unknown. METHODS:A retrospective review was performed of ultrasound data, acquired and recorded by 2 examiners-an expert and either a trainee with 2 months (novice) or a trainee with 12 months (experienced) of experienc...

journal_title：Muscle & nerve

authors： Garcia-Santibanez R,Dietz AR,Bucelli RC,Zaidman CM

更新日期：2018-02-01 00:00:00

abstract：BACKGROUND:The goals of this study were to determine whether serum concentrations of endocannabinoids (eCB) and related lipids predict disease status in patients with amyotrophic lateral sclerosis (ALS) relative to healthy controls, and whether concentrations correlate with disease duration and severity. METHODS:Serum...

journal_title：Muscle & nerve

authors： Carter GT,McLaughlin RJ,Cuttler C,Sauber GJ,Weeks DL,Hillard CJ,Weiss MD

更新日期：2021-01-01 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：:L1 radiculopathy is very rare and difficult to diagnose with needle electromyography. A patient presented with pain and hypesthesia on the anterolateral aspect of the left thigh. Nerve conduction studies and needle electromyography were normal, except for the quadratus lumborum and iliopsoas muscles, which showed abno...

journal_title：Muscle & nerve

authors： Yang SN,Kim DH

更新日期：2010-04-01 00:00:00

abstract：:To better define electrophysiological abnormalities in myasthenia gravis (MG) patients with muscle-specific tyrosine kinase (MuSK) antibodies (Ab), we compared electrophysiological features of 14 MuSK Ab-positive, 73 acetylcholine receptor antibody (AChR Ab)-positive, and 22 MuSK and AChR Ab-negative (seronegative) pa...

journal_title：Muscle & nerve

authors： Oh SJ,Hatanaka Y,Hemmi S,Young AM,Scheufele ML,Nations SP,Lu L,Claussen GC,Wolfe GI

更新日期：2006-04-01 00:00:00

abstract：:Hereditary neuropathy with liability to pressure palsy (HNPP) is typified as isolated nerve palsies caused by trivial compression or trauma. It rarely presents in two extremities and even more infrequently affects all four limbs simultaneously. We present a patient who concurrently experienced right shoulder, left han...

journal_title：Muscle & nerve

authors： Crum BA,Sorenson EJ,Abad GA,Dyck PJ

更新日期：2000-06-01 00:00:00

abstract：:The consequences of general hypoxemia (PaO2 = 51 mmHg) on two muscle groups (adductor pollicis and diaphragm) sustaining 80% maximal isometric voluntary contraction were studied in healthy individuals. For adductor pollicis, contractions were also executed after 10-s or 3-min rest ischemia. Compared to control, i.e., ...

journal_title：Muscle & nerve

authors： Badier M,Guillot C,Lagier-Tessonnier F,Jammes Y

更新日期：1994-05-01 00:00:00

abstract：:Earlier studies have shown that prior denervation of muscle prevents myotonia induced by 2,4-dichlorophenoxy acetic acid (2,4-D) both in vivo and in vitro. This work studied the effect of reinnervation on 2,4-D myotonia. Twenty Sprague-Dawley rats were injected with 2,4-D at specific intervals following unilateral sci...

journal_title：Muscle & nerve

authors： Al-Sulaiman A,Al-Rajeh S,Iyer V

更新日期：1986-05-01 00:00:00

abstract：:The loss of contractile protein in Duchenne muscular dystrophy could result from low rates of synthesis, abnormally high rates of protein degradation, or a combination of both. We measured overall protein degradation rates in cultured human muscle cells obtained at biopsy from patients with Duchenne dystrophy or vario...

journal_title：Muscle & nerve

authors： Neville HE,Harrold S

更新日期：1985-03-01 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：:Cortical and spinal somatosensory evoked potentials (SEP) were recorded in healthy individuals following stimulation of digits I, III, and V with an intensity below discomfort level. Peak latency of the earliest cortical negativity (N1) was found to be the most consistent and easily measured parameter, whereas a spina...

journal_title：Muscle & nerve

authors： Kwast-Rabben O,Libelius R,Heikkilä H

更新日期：2002-10-01 00:00:00

abstract：:Motoneurons can be activated both reflexly and antidromically following electrical stimulation of peripheral nerves. These H reflexes and F waves are clinically useful responses which interface at the level of the peripheral nerves and the spinal cord. Because these responses are commonly employed in the electrodiagno...

journal_title：Muscle & nerve

authors： Fisher MA

更新日期：1992-11-01 00:00:00

abstract：:Ultrasound (US) technique was applied to measure the thickness, cross-sectional area (CSA), and internal structure of the quadriceps muscle in 70- to 80-year-old endurance and power athletes and untrained men. Isometric knee extension strength was higher in the power athletes than in the other groups. The mean values ...

journal_title：Muscle & nerve

authors： Sipilä S,Suominen H

更新日期：1991-06-01 00:00:00

abstract：:Focal myositis is a rare inflammatory pseudotumor of skeletal muscle which usually has a benign course. We report a 56-year-old woman with a painful mass in the left arm with a radial nerve palsy. Magnetic resonance imaging (MRI) of the left arm showed a mass in the triceps muscle that was suggestive of a soft-tissue ...

journal_title：Muscle & nerve

authors： Alzagatiti BI,Bertorini TE,Horner LH,Maccarino VS,O'Brien T

更新日期：1999-07-01 00:00:00

abstract：INTRODUCTION:When treating ocular myasthenia gravis (MG), the risk/benefit profile of corticosteroids is unclear, and acetylcholinesterase inhibitors are not very effective. We examined the efficacy of topical naphazoline in the treatment of myasthenic blepharoptosis. METHODS:Sixty MG patients with blepharoptosis (32 ...

journal_title：Muscle & nerve

authors： Nagane Y,Utsugisawa K,Suzuki S,Masuda M,Shimizu Y,Utsumi H,Uchiyama S,Suzuki N

更新日期：2011-07-01 00:00:00

abstract：:Impaired torque production is a major physical impairment following stroke, and has been studied extensively in isometric conditions. However, functional use of a limb requires torque production during movement, and the effects of velocity on maximal torque production may be abnormally enhanced in the paretic limb. Th...

journal_title：Muscle & nerve

authors： Lum PS,Patten C,Kothari D,Yap R

更新日期：2004-12-01 00:00:00