Central nervous system involvement in multifocal demyelinating neuropathy with persistent conduction block.

abstract：:Cultured human skin fibroblasts from 9 patients with Duchenne muscular dystrophy (DMD) and 8 normal age- and sex-matched controls were examined for creatine kinase (CK) activity. Both the normal and the DMD fibroblasts were found to have significant levels of CK activity (approximately 10 x 10(-3) IU per milligram of ...

journal_title：Muscle & nerve

authors： Davis MH,Cappel R,Vester JW,Samaha FJ,Gruenstein E

更新日期：1982-01-01 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：:Eleven patients with previous polio were studied. The concentration of energy-related metabolites and energy charge was measured from the vastus lateralis muscle, as was isometric muscle strength of knee extension. Cross-sectional area of the quadriceps femoris muscle was calculated from magnetic resonance imaging. Re...

journal_title：Muscle & nerve

authors： Nordgren B,Falck B,Stålberg E,Ronquist G,Waldenström A,Ahlström H,Hemmingson A

更新日期：1997-11-01 00:00:00

abstract：INTRODUCTION:Conduction block (CB) has been included in the Rajabally criteria for axonal Guillain-Barré syndrome (GBS). Because the nerve roots may be affected early in GBS, detection of proximal CB by the triple stimulation technique (TST) can be useful. METHODS:We describe TST findings in 2 patients who presented w...

journal_title：Muscle & nerve

authors： Taieb G,Grapperon AM,Duclos Y,Franques J,Labauge P,Renard D,Yuki N,Attarian S

更新日期：2015-12-01 00:00:00

abstract：INTRODUCTION:We assessed antibodies against low-density lipoprotein receptor-related protein 4 (LRP4-Ab) in a Chinese population with myasthenia gravis (MG). METHODS:Serum samples from 116 patients and 80 controls were collected. Acetylcholine receptor antibodies(AChR-Ab) and muscle-specific receptor tyrosine kinase a...

journal_title：Muscle & nerve

authors： Li Y,Zhang Y,Cai G,He D,Dai Q,Xu Z,Chu L

更新日期：2017-11-01 00:00:00

abstract：INTRODUCTION:Muscle growth and regeneration are processes closely associated with proliferation, differentiation, and apoptosis of muscle cells. Death-associated protein 1 (DAP1) has been identified as a negative regulator of autophagy. Little is known about the function of DAP1 in the regulation of myogenesis and sate...

journal_title：Muscle & nerve

authors： Shin J,McFarland DC,Strasburg GM,Velleman SG

更新日期：2013-11-01 00:00:00

abstract：INTRODUCTION:In this study we noninvasively evaluated skeletal muscle mitochondrial capacity, muscle-specific endurance, and energy/fatigue feelings in persons with Friedreich ataxia (FRDA) and able-bodied controls (AB). METHODS:Forearm mitochondrial capacity was measured in FRDA (n = 16) and AB (n = 10) study partici...

journal_title：Muscle & nerve

authors： Bossie HM,Willingham TB,Schoick RAV,O'Connor PJ,McCully KK

更新日期：2017-10-01 00:00:00

abstract：:Schwartz-Jampel syndrome (SJS) is an autosomal-recessive condition characterized by muscle stiffness and chondrodysplasia. It is due to loss-of-function hypomorphic mutations in the HSPG2 gene that encodes for perlecan, a proteoglycan secreted into the basement membrane. The origin of muscle stiffness in SJS is debate...

journal_title：Muscle & nerve

authors： Echaniz-Laguna A,Rene F,Marcel C,Bangratz M,Fontaine B,Loeffler JP,Nicole S

更新日期：2009-07-01 00:00:00

abstract：:Influenza vaccination has been associated with adverse events including Guillain-Barré syndrome. Because the safety of influenza vaccination in patients with myasthenia gravis (MG) has not been established, some clinicians discourage vaccination for these patients. We explored whether the administration of influenza v...

journal_title：Muscle & nerve

authors： Zinman L,Thoma J,Kwong JC,Kopp A,Stukel TA,Juurlink DN

更新日期：2009-12-01 00:00:00

abstract：INTRODUCTION:Sepsis often causes diaphragm contractile dysfunction. Dihydropyridine receptors (DHPRα1s and DHPRα1c) and ryanodine receptors (RyR1, RyR2, and RyR3) are essential for excitation-contraction coupling in striated muscles. However, their expression in diaphragm during sepsis have not been explored. METHODS:...

journal_title：Muscle & nerve

authors： Jiao GY,Hao LY,Gao CE,Chen L,Sun XF,Yang HL,Li Y,Dai YN

更新日期：2013-11-01 00:00:00

abstract：INTRODUCTION:Chediak-Higashi syndrome (CHS) is a very rare autosomal recessive disorder (gene CHS1/LYST) characterized by partial albinism, recurrent infections, and easy bruising. Survivors develop a constellation of slowly progressive neurological manifestations. METHODS:We describe clinical, laboratory, electrophys...

journal_title：Muscle & nerve

authors： Faber IV,Prota JRM,Martinez ARM,Nucci A,Lopes-Cendes I,Júnior MCF

更新日期：2017-05-01 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：: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：:Guillain-Barré syndrome (GBS) generally presents sporadically. Epidemics of GBS are unusual. We reviewed the medical records of 26 patients hospitalized for GBS during the 2007 GBS epidemic in northeast China. The objective was to determine whether there were clinical and electrophysiological characteristics. All pati...

journal_title：Muscle & nerve

authors： Ye Y,Zhu D,Wang K,Wu J,Feng J,Ma D,Xing Y,Jiang X

更新日期：2010-09-01 00:00:00

abstract：:Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder characterized by autoantibodies against presynaptic voltage-gated calcium channels that impair neuromuscular transmission. Malignancies, especially small cell lung cancer (SCLC), have been associated with LEMS and account for approximately 60% of cases...

journal_title：Muscle & nerve

authors： Wang C,Chen S,Feng B,Guan Y

更新日期：2014-03-01 00:00:00

abstract：INTRODUCTION:In this study we examined relationships among mechanomyographic (MMG), electromyographic (EMG), and peak twitch torque (PTT) responses as well as test-retest reliability when recorded during recruitment curves in the soleus muscle. METHODS:PTT, EMG (M-wave, H-reflex), and MMG responses were recorded durin...

journal_title：Muscle & nerve

authors： Miramonti AA,Jenkins NDM,Oza PD,Weir JP,Cramer JT

更新日期：2017-07-01 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：:To investigate whether transcranial magnetic stimulation (TMS) has an effect on isometric muscle force elicited by maximal voluntary contractions (MVC) and the ability to activate a muscle voluntarily (VA, as a percentage of full muscle activation), a twitch-interpolation technique was applied on the quadriceps femori...

journal_title：Muscle & nerve

authors： Urbach D,Awiszus F

更新日期：2000-07-01 00:00:00

abstract：:This longitudinal study of 194 patients with sporadic ALS demonstrated that it is possible for physicians to predict the approximate survival time for an individual ALS patient given: (1) the age of the patient, (2) the duration of his weakness and (3) an estimate of his clinical disability (ALS Score). This informati...

journal_title：Muscle & nerve

authors： Jablecki CK,Berry C,Leach J

更新日期：1989-10-01 00:00:00

abstract：:In this pilot study we use T2-weighted magnetic resonance imaging (MRI) to identify possible peripheral nerve inflammation in 4 patients with diffuse chronic pain. In all 4 patients, there was an increase in median and ulnar nerve T2 signal intensity at the wrist (P < 0.05 vs. controls). Positive clinical signs of per...

journal_title：Muscle & nerve

authors： Dilley A,Greening J,Walker-Bone K,Good C

更新日期：2011-12-01 00:00:00

abstract：:Genetic testing is rapidly becoming an increasingly significant part of the diagnostic armamentarium of neuromuscular clinicians. Although technically easy to order, the results of such testing, whether positive or negative, have potentially enormous consequences for the individual tested and for family members. As a ...

journal_title：Muscle & nerve

authors： Su X,Kang PB,Russell JA,Simmons Z

更新日期：2016-12-01 00:00:00

abstract：INTRODUCTION:Piriformis muscle syndrome (PS) is a disorder encompassing a constellation of symptoms, including buttock and hip pain. In this study we aimed to assess the value of ultrasound (US) in the diagnosis of PS. METHODS:Thirty-three clinically diagnosed PS patients and 26 healthy volunteers underwent a clinical...

journal_title：Muscle & nerve

authors： Zhang W,Luo F,Sun H,Ding H

更新日期：2019-04-01 00:00:00

abstract：:The frequency of muscle involvement in TSP/HAM is not known, nor is the precise role that HTLV-1 and the diverse cytokines play in the genesis of HTLV-1-associated diseases. In order to better define the frequency and characteristics of the skeletal muscle involvement in TSP/HAM, we studied 11 affected patients. EMG w...

journal_title：Muscle & nerve

authors： Gabbai AA,Wiley CA,Oliveira AS,Smith R,Schmidt B,Nóbrega JA,Bordin JO,Román GC

更新日期：1994-08-01 00:00:00

abstract：:Some have suggested that nonfamilial motor neuron disease (MND) may be autoimmune, and the neurological disorder may benefit from immunotherapy. There have been reports of over 30 cases of lymphoproliferative disease (lymphoma, multiple myeloma, Waldenström's macroglobulinemia) with MND, and these patients might he of...

journal_title：Muscle & nerve

authors： Louis ED,Hanley AE,Brannagan TH,Sherman W,Murphy P,Lange DJ,Trojaborg W,Younger DS,Lovelace RE,Latov N,Rowland LP

更新日期：1996-10-01 00:00:00

abstract：:Evaluation of the precise molecular dynamics of endplate maintenance and reorganization has been limited by the lack of available in vitro preparations. We describe an organ culture preparation of mouse diaphragm muscle which permits long-term maintenance of muscle viability. Spontaneous fibrillations, increased level...

journal_title：Muscle & nerve

authors： Wetzel DM,Salpeter MM

更新日期：1991-10-01 00:00:00

abstract：:Large muscle genes are often sequenced using complementary DNA (cDNA) made from muscle messenger RNA (mRNA) to reduce the cost and workload associated with sequencing from genomic DNA. Two potential barriers are the availability of a frozen muscle biopsy, and difficulties in detecting nonsense mutations due to nonsens...

journal_title：Muscle & nerve

authors： Waddell LB,Monnier N,Cooper ST,North KN,Clarke NF

更新日期：2011-08-01 00:00:00

abstract：:To determine which sensory nerve conduction studies (S-NCS) are helpful in detecting supraclavicular axon loss brachial plexopathies, we selected 53 cases (of 417 reviewed) in whom complicating factors were absent and which, by needle electrode examination findings, involved only a single "truncal" element (upper, mid...

journal_title：Muscle & nerve

authors： Ferrante MA,Wilbourn AJ

更新日期：1995-08-01 00:00:00

abstract：:Factors that affect the shape of the so-called "normal cloud" of the turns and amplitude measurements of the electromyographic interference pattern are investigated. As the force of voluntary contraction increases from low to moderate levels, the number of turns in the signal increase faster than does the mean amplitu...

journal_title：Muscle & nerve

authors： Nandedkar SD,Sanders DB,Stalberg EV

更新日期：1991-01-01 00:00:00

abstract：:Muscle biopsy and computerized tomography indicated that unilateral enlargement of the gastrocnemius muscle in a 12-year-old girl was the result of an infiltrating lipoma. This rare condition may cause diagnostic confusion before EMG and muscle biopsy studies are performed, but it can be readily distinguished from oth...

journal_title：Muscle & nerve

authors： Fetell MR,Duffy PE,Rowland LP

更新日期：1978-01-01 00:00:00

abstract：INTRODUCTION:We describe the nerve ultrasound findings in patients with type II diabetes mellitus who have neuropathic symptoms and signs. METHODS:Fifty-five healthy controls and 44 diabetic patients underwent clinical, sonographic, and electrophysiological evaluation. Patients were studied at a mean of 14.3 years aft...

journal_title：Muscle & nerve

authors： Pitarokoili K,Kerasnoudis A,Behrendt V,Labedi A,Ayzenberg I,Gold R,Yoon MS

更新日期：2016-06-01 00:00:00