The cooperative international neuromuscular research group Duchenne natural history study--a longitudinal investigation in the era of glucocorticoid therapy: design of protocol and the methods used.

abstract：INTRODUCTION:Thymectomy is required for the treatment of thymoma-associated myasthenia gravis (MG). However, MG may develop only after thymectomy, a condition known as post-thymectomy MG. This study aimed to investigate the risk factors for post-thymectomy MG in patients with thymoma. METHODS:We retrospectively identi...

journal_title：Muscle & nerve

authors： Kim A,Choi SJ,Kang CH,Lee S,Son H,Kim JA,Shin JY,Kim SM,Hong YH,Sung JJ

更新日期：2021-01-12 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：:Modern neuromuscular electrodiagnosis (EDX) and neuromuscular ultrasound (NMUS) require a universal language for effective communication in clinical practice and research and, in particular, for teaching young colleagues. Therefore, the AANEM and the IFCN have decided to publish a joint glossary as they feel the need ...

journal_title：Muscle & nerve

authors： Dengler R,de Carvalho M,Shahrizaila N,Nodera H,Vucic S,Grimm A,Padua L,Schreiber S,Kneiser MK,Hobson-Webb LD,Boon AJ,Smith BE,Litchy WJ,Li Y,Lenihan M,Thompson VB,Stalberg E,Sanders DB,Kincaid JC

更新日期：2020-07-01 00:00:00

abstract：:Clinicians who use electromyographic (EMG) signals to help determine the presence or absence of abnormality in a muscle often, with varying degrees of success, evaluate sets of motor unit potentials (MUPs) qualitatively and/or quantitatively to characterize the muscle in a clinically meaningful way. The resulting musc...

journal_title：Muscle & nerve

authors： Pino LJ,Stashuk DW,Boe SG,Doherty TJ

更新日期：2010-01-01 00:00:00

abstract：INTRODUCTION:Full-length tyrosine kinase B (TrkB.FL) and truncated TrkB (TrkB.t1) receptors are colocalized with acetylcholine receptors (AChRs) at the neuromuscular junction. We have recently shown that reduced TrkB expression leads to age-related alterations in AChR structure, neurotransmission failure, and muscle we...

journal_title：Muscle & nerve

authors： Personius KE,Parker SD

更新日期：2013-04-01 00:00:00

abstract：:Cerebral palsy (CP) is the most prevalent neurologic disease in children and a leading cause of severe physical disability. Research and clinical experience indicate that children with CP have abnormal neuromuscular junctions (NMJs), and we present evidence that nonapposition of neuromuscular junction components is as...

journal_title：Muscle & nerve

authors： Theroux MC,Oberman KG,Lahaye J,Boyce BA,Duhadaway D,Miller F,Akins RE

更新日期：2005-11-01 00:00:00

abstract：:Chronic inflammatory demyelinating polyneuropathy (CIDP) presents in rare instances with focal or multifocal upper limb involvement. We reviewed the clinical and electromyographic (EMG) characteristics of 10 such patients (UL-CIDP) and compared them with patients with typical generalized CIDP (G-CIDP) and multifocal m...

journal_title：Muscle & nerve

authors： Gorson KC,Ropper AH,Weinberg DH

更新日期：1999-06-01 00:00:00

abstract：:We studied the effect of a gluten-free diet in patients with idiopathic sensorimotor axonal neuropathy and circulating antigliadin antibodies. Consecutive patients underwent baseline neurophysiological assessment and were offered a gluten-free diet. Those who went on the diet formed the intention-to-treat group and th...

journal_title：Muscle & nerve

authors： Hadjivassiliou M,Kandler RH,Chattopadhyay AK,Davies-Jones AG,Jarratt JA,Sanders DS,Sharrack B,Grünewald RA

更新日期：2006-12-01 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：: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：BACKGROUND:In adult males, cross-sectional area (CSA) for type II muscle fibers is generally larger than for type I fibers. In this cross-sectional study the aim was to compare sex-related CSAs of various muscle fiber types during childhood-to-adulthood transition. METHODS:Percutaneous biopsy samples were obtained fro...

journal_title：Muscle & nerve

authors： Esbjörnsson ME,Dahlström MS,Gierup JW,Jansson EC

更新日期：2020-12-21 00:00:00

abstract：:A 66-year-old professional golf instructor developed pain, numbness, and weakness in an ulnar distribution, but the lesion could not be localized to either elbow or wrist by routine nerve conduction techniques. Stimulation along serial 1-cm increments in the distal forearm disclosed a point of focal conduction block a...

journal_title：Muscle & nerve

authors： Campbell WW

更新日期：1989-05-01 00:00:00

abstract：:There is a paucity of validated tests to quantify hand function impairment due to carpal tunnel syndrome (CTS). The aim of this study was to test the validity and reliability of the Purdue Pegboard Test (PPT) in CTS patients. We compared 190 CTS patients with 122 healthy, age-matched controls. CTS severity was determi...

journal_title：Muscle & nerve

authors： Amirjani N,Ashworth NL,Olson JL,Morhart M,Chan KM

更新日期：2011-02-01 00:00:00

abstract：:Reinnervation of a free muscle graft by nerves from an adjacent intact muscle is called muscular neurotization. This paper investigates the mechanisms and stimuli responsible for muscular neurotization in the rat. Sternohyoid or sternomastoid muscles were transplanted as free muscle grafts to the ventral surface of an...

journal_title：Muscle & nerve

authors： Must R

更新日期：1987-07-01 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：INTRODUCTION:In this study we aimed to clarify whether muscle ultrasound (US) of the forearm can be used to differentiate between patients with sporadic inclusion body myositis (s-IBM) and those with s-IBM-mimicking diseases. METHODS:We compared the echo intensity (EI) of the flexor digitorum profundus (FDP) muscle an...

journal_title：Muscle & nerve

authors： Noto Y,Shiga K,Tsuji Y,Kondo M,Tokuda T,Mizuno T,Nakagawa M

更新日期：2014-05-01 00:00:00

abstract：INTRODUCTION:In this study we determined the reliability and validity of electrical impedance myography (EIM) in facioscapulohumeral muscular dystrophy (FSHD). METHODS:We performed a prospective study of EIM on 16 bilateral limb and trunk muscles in 35 genetically defined and clinically affected FSHD patients (reliabi...

journal_title：Muscle & nerve

authors： Statland JM,Heatwole C,Eichinger K,Dilek N,Martens WB,Tawil R

更新日期：2016-10-01 00:00:00

abstract：:Recent years have seen steady progress in the identification of genetic muscle diseases as well as efforts to develop treatment for these diseases. Consequently, sensitive and objective new methods are required to identify and monitor muscle pathology. Magnetic resonance imaging offers multiple potential biomarkers of...

journal_title：Muscle & nerve

authors： Leung DG

更新日期：2019-10-01 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：:Based on the known trophic effects of growth hormone (GH) on nerve and muscle 75 patients with ALS were treated for up to 18 months with synthetic human growth hormone (Protropin) or a placebo. The course of ALS was assessed serially using a quantitative (TQNE) neuromuscular and manual exam (MRC) and laboratory chemis...

journal_title：Muscle & nerve

authors： Smith RA,Melmed S,Sherman B,Frane J,Munsat TL,Festoff BW

更新日期：1993-06-01 00:00:00

abstract：:Our experience and review of the literature reveal that Sjogren's syndrome (SS) is an important, poorly recognized cause of peripheral neuropathy. Several forms of peripheral nerve dysfunction occur in SS including trigeminal sensory neuropathy, mononeuropathy multiplex, distal sensory neuropathy, distal sensorimotor ...

journal_title：Muscle & nerve

authors： Kaplan JG,Rosenberg R,Reinitz E,Buchbinder S,Schaumburg HH

更新日期：1990-07-01 00:00:00

abstract：INTRODUCTION:Meralgia paresthetica is a common clinical complaint for which some patients ultimately undergo surgical treatment. The lateral femoral cutaneous nerve (LFCN) has been difficult to reliably test electrophysiologically, likely due to anatomic variability and lack of responses in asymptomatic obese subjects....

journal_title：Muscle & nerve

authors： Boon AJ,Bailey PW,Smith J,Sorenson EJ,Harper CM,Hurdle MF

更新日期：2011-10-01 00:00:00

abstract：BACKGROUND:We aimed at evaluating the differential involvement of large myelinated Aβ-, small myelinated Aδ-, and unmyelinated C-fibers in patients with diabetic polyneuropathy and how they contribute to neuropathic pain. METHODS:We collected clinical and diagnostic test variables in 133 consecutive patients with diab...

journal_title：Muscle & nerve

authors： Galosi E,Di Pietro G,La Cesa S,Di Stefano G,Leone C,Fasolino A,Di Lionardo A,Leonetti F,Buzzetti R,Mollica C,Cruccu G,Truini A

更新日期：2021-01-01 00:00:00

abstract：:We describe a consanguineous family that had progressive myopathy with rimmed vacuole (RV) formation and amyloid deposition. Patient 1 is a 71-year-old woman with muscle atrophy in the lumbar girdle and lower extremities. Patient 2 is a 40-year-old man (the son of Patient 1) with fatty changes in the biceps femoris mu...

journal_title：Muscle & nerve

authors： Kojima Y,Sakai K,Ishida C,Asaka T,Hamaguchi T,Nozaki I,Fukushima K,Tsuchiya A,Kametani F,Yazaki M,Okino S,Yamada M

更新日期：2009-09-01 00:00:00

abstract：:A randomized double-blind therapeutic trial of isaxonine was completed over a 2-year period for 20 ambulant boys with Duchenne muscular dystrophy aged 5 1/2-10 years. The effect of the drug was monitored by measurement of walking times over 28 and 150 ft, motor ability score, MRC score based on 32 muscle groups, and m...

journal_title：Muscle & nerve

authors： Heckmatt JZ,Hyde SA,Gabain A,Dubowitz V

更新日期：1988-08-01 00:00:00

abstract：:Patients with prior poliomyelitis may experience muscle function deterioration decades after onset of disease. The present study is aimed at describing electromyographic and morphometric evidence of muscular compensation and of on-going muscular instability. Ten subjects 42-62 years of age with onset of polio 25-52 ye...

journal_title：Muscle & nerve

authors： Einarsson G,Grimby G,Stålberg E

更新日期：1990-02-01 00:00:00

abstract：INTRODUCTION:Magnetic resonance imaging (MRI) is an excellent imaging modality for soft tissues. Magnetization transfer (MT) imaging is an MRI technique that is sensitive to the bound protons of macromolecules and therefore can aid in the assessment of nerve damage. METHODS:Measurements of the MT ratio (MTR), which pr...

journal_title：Muscle & nerve

authors： Gambarota G,Krueger G,Theumann N,Mekle R

更新日期：2012-01-01 00:00:00

abstract：:Two brothers with the childhood variant of type II glycogenosis (GSD-IIb) treated with nutrition and exercise therapy (NET) from a young age showed an unusually benign course. Muscle biopsy from the older brother, which showed characteristic vacuolar glycogen accumulation at age 2, had reverted to normal by age 16. A ...

journal_title：Muscle & nerve

authors： Slonim AE,Bulone L,Minikes J,Hays AP,Shanske S,Tsujino S,DiMauro S

更新日期：2006-04-01 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：:Skeletal muscle involvement can occur at all stages of human immunodeficiency virus (HIV) infection, and may represent the first manifestation of the disease. Myopathies in HIV-infected patients are classified as follows: (1) HIV-associated myopathies and related conditions, including HIV polymyositis, inclusion-body ...

journal_title：Muscle & nerve

authors： Authier FJ,Chariot P,Gherardi RK

更新日期：2005-09-01 00:00:00