Muscle metabolic alterations assessed by 31-phosphorus magnetic resonance spectroscopy in mild Becker muscular dystrophy.

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：:The objective of this study was to examine the contribution of lower motor neuron (LMN) and upper motor neuron (UMN) dysfunction to weakness and impaired motor control in 27 patients with amyotrophic lateral sclerosis (ALS). Isometric strength was measured by dorsiflexor maximum voluntary contraction force (MVC). LMN ...

journal_title：Muscle & nerve

authors： Kent-Braun JA,Walker CH,Weiner MW,Miller RG

更新日期：1998-06-01 00:00:00

abstract：:The F-wave was used to estimate motor nerve conduction velocity (MNCV) along the proximal segment of the fastest axons of the deep peroneal nerve (anterior-horn cells to knee) in 13 patients with amyotrophic lateral sclerosis (ALS) and in 21 age- and sex-matched control subjects. Measurements were based on the shortes...

journal_title：Muscle & nerve

authors： Argyropoulos CJ,Panayiotopoulos CP,Scarpalezos S

更新日期：1978-11-01 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：:Exercise intolerance with myalgia, muscle stiffness, and recurrent rhabdomyolysis due to mutations in the DMD gene can mimic metabolic myopathies leading to delayed or inaccurate diagnoses. In this retrospective chart review, we report 3 unrelated boys with exertional myalgia, muscle stiffness, myoglobinuria, and norm...

journal_title：Muscle & nerve

authors： Veerapandiyan A,Shashi V,Jiang YH,Gallentine WB,Schoch K,Smith EC

更新日期：2010-12-01 00:00:00

abstract：:The aim of this study was to estimate normal jitter in voluntarily activated extensor digitorum communis (EDC) and orbicularis oculi (OOc) muscles using a disposable concentric needle electrode (CNE). The EDC of 67 normal subjects (22 males and 45 females, mean age 35.5 +/- 10.2 years) and the OOc of 50 normal subject...

journal_title：Muscle & nerve

authors： Kouyoumdjian JA,Stålberg EV

更新日期：2008-06-01 00:00:00

abstract：INTRODUCTION:Premotor potentials (PMPs) precede compound muscle action potentials evoked from the second lumbrical muscle after median nerve stimulation. Although PMP has been identified as a median sensory nerve action potential, few reports have documented the significance of PMP parameters for diagnosing carpal tunn...

journal_title：Muscle & nerve

authors： Kodama M,Sasao Y,Tochikura M,Kasahara T,Koyama Y,Aono K,Fujii C,Hanayama K,Takahashi O,Kobayashi Y,Masakado Y

更新日期：2012-12-01 00:00:00

abstract：INTRODUCTION:The aim of this study was to examine the coping strategies of 49 patients with amyotrophic lateral sclerosis (ALS) and the relationships of these strategies to their perceived health-related quality of life (HRQoL). METHODS:Forty-nine subjects were assessed for collection of demographic and medical data. ...

journal_title：Muscle & nerve

authors： Montel S,Albertini L,Spitz E

更新日期：2012-01-01 00:00:00

abstract：INTRODUCTION:We evaluated diagnostic value of sensory tests during recovery from iatrogenic sensory neuropathy using intraoperatively verified nerve injury with subjective symptoms as gold standard. METHODS:Inferior alveolar nerves were monitored neurophysiologically throughout mandibular osteotomy in 19 patients. Sen...

journal_title：Muscle & nerve

authors： Teerijoki-Oksa T,Forssell H,Jääskeläinen SK

更新日期：2019-03-01 00:00:00

abstract：INTRODUCTION:Venous access for therapeutic plasma exchange (TPE) in myasthenia gravis (MG) can be achieved by central venous catheters (CVC) or peripheral veins (PV), and the preferred method varies among providers. We evaluated our institutional experience with TPE venous access method and complications. METHODS:We r...

journal_title：Muscle & nerve

authors： Guptill JT,Oakley D,Kuchibhatla M,Guidon AC,Hobson-Webb LD,Massey JM,Sanders DB,Juel VC

更新日期：2013-02-01 00:00:00

abstract：:The Moberg Pick-Up Test is a standardized test for assessing hand dexterity. Although reduction of sensation in the hand occurs with aging, the effect of age on a subject's performance of the Moberg Pick-Up Test has not been examined. The primary goal of this study was to examine the impact of aging and, secondarily, ...

journal_title：Muscle & nerve

authors： Amirjani N,Ashworth NL,Gordon T,Edwards DC,Chan KM

更新日期：2007-06-01 00:00:00

abstract：:Acetylcholinesterase (AChE) and butyrylcholinesterase (BuChE) were studied in the diaphragm of early postnatal rats, using radiometric determination of enzyme activities, velocity sedimentation for separation of molecular forms, and electron microscopic cytochemistry to localize enzymes. AChE activity did not change s...

journal_title：Muscle & nerve

authors： Brzin M,Sketelj J,Tennyson VM,Kiauta T,Budininkas-Schoenebeck M

更新日期：1981-11-01 00:00:00

abstract：:The dropped head syndrome occurs in a variety of neuromuscular disorders. We present a woman with chronic inflammatory demyelinating polyneuropathy who developed this syndrome, likely reflecting severe demyelination of nerves to cervical paraspinal muscles. ...

journal_title：Muscle & nerve

authors： Hoffman D,Gutmann L

更新日期：1994-07-01 00:00:00

abstract：:A patient with neuromyotonia, peripheral neuropathy and myasthenia gravis (MG) is described. Neurophysiological studies, at rest, showed continuous muscle discharges of motor unit action potentials (MUAPs) in duplets and triplets. Motor (MNCV) and sensory (SNCV) nerve conduction studies revealed mild axonal and demyel...

journal_title：Muscle & nerve

authors： Martinelli P,Patuelli A,Minardi C,Cau A,Riviera AM,Dal Pozzo F

更新日期：1996-04-01 00:00:00

abstract：:We have assessed the reliability of hand-held myometry in 33 patients with spinal muscular atrophy (SMA), testing elbow flexion, handgrip, three-point pinch, knee flexion, knee extension, and foot dorsiflexion, and determining intraclass correlation coefficients (ICC). Interrater reliability was high for upper limbs, ...

journal_title：Muscle & nerve

authors： Merlini L,Mazzone ES,Solari A,Morandi L

更新日期：2002-07-01 00:00:00

abstract：INTRODUCTION:Duchenne muscular dystrophy (DMD) is characterized by absence of the subsarcolemmal protein dystrophin, present in skeletal muscles and cardiomyocytes. We hypothesized that progressive respiratory and left ventricular (LV) insufficiencies in DMD could be parallel and interrelated phenomena. METHODS:We con...

journal_title：Muscle & nerve

authors： Khokhar A,Nair A,Midya V,Kumar A,Sinharoy A,Ahmad TA,Abu-Hasan M,Mondal P

更新日期：2019-09-01 00:00:00

abstract：:Mitochondrial trifunctional protein (TFP) deficiency is a rare disorder of the fatty acid beta-oxidation cycle with heterogeneous phenotypes and occurs secondary to either alpha- or beta-subunit mutations. We characterized the neuromyopathic phenotype of TFP deficiency through adolescence or adulthood in 11 patients, ...

journal_title：Muscle & nerve

authors： Spiekerkoetter U,Bennett MJ,Ben-Zeev B,Strauss AW,Tein I

更新日期：2004-01-01 00:00:00

abstract：:We describe two cases of neuralgic amyotrophy with electrophysiological evidence of conduction block across the lower trunk of the brachial plexus. Low-output impedance stimulation of the cervical spinal roots in combination with collision was used to accurately demonstrate the conduction block. Complete electrophysio...

journal_title：Muscle & nerve

authors： Watson BV,Nicolle MW,Brown JD

更新日期：2001-04-01 00:00:00

abstract：INTRODUCTION:This study was undertaken to describe a method for quantifying vibration when using a conventional tuning fork (CTF) in comparison to a Rydel-Seiffer tuning fork (RSTF) and to provide reference values. METHODS:Vibration thresholds at index finger and big toe were obtained in 281 participants. Spearman's c...

journal_title：Muscle & nerve

authors： Alanazy MH,Alfurayh NA,Almweisheer SN,Aljafen BN,Muayqil T

更新日期：2018-01-01 00:00:00

abstract：BACKGROUND:To collect preliminary data on the effects of mexiletine on cortical and axonal hyperexcitability in sporadic amyotrophic lateral sclerosis (ALS) in a phase 2 double-blind randomized controlled trial. METHODS:Twenty ALS subjects were randomized to placebo and mexiletine 300 or 600 mg daily for 4 wk and asse...

journal_title：Muscle & nerve

authors： Weiss MD,Macklin EA,McIlduff CE,Vucic S,Wainger BJ,Kiernan MC,Goutman SA,Goyal NA,Rutkove SB,Ladha SS,Chen IA,Harms MB,Brannagan TH,Lacomis D,Zivkovic S,Ma M,Wang LH,Simmons Z,Rivner MH,Shefner JM,Cudkowicz ME,A

更新日期：2020-12-19 00:00:00

abstract：:Comparison was made between simultaneous recordings of the electromyogram (EMG) and acoustic myogram (AMG) signals and the torque produced by the lumbar erectores spinae muscles of three healthy subjects while they performed isometric contractions. Repeatability of each signal was studied. The AMG had a relationship t...

journal_title：Muscle & nerve

authors： Stokes IA,Moffroid MS,Rush S,Haugh LD

更新日期：1988-04-01 00:00:00

abstract：INTRODUCTION:The ability to distinguish between normal thymus, thymic hyperplasia, and thymoma should aid clinical management and decision making in patients with myasthenia gravis (MG). We sought to determine the accuracy of routine imaging in predicting thymic pathology. METHODS:We retrospectively analyzed records o...

journal_title：Muscle & nerve

authors： Klimiec E,Quirke M,Leite MI,Hilton-Jones D

更新日期：2018-02-09 00:00:00

abstract：INTRODUCTION:Pes cavus often signals the presence of Charcot-Marie-Tooth (CMT) in adult patients, although its prevalence in the general population makes it a finding of unclear significance. METHODS:We undertook a pilot double cohort study to investigate the feasibility of comparing preselected bedside and radiograph...

journal_title：Muscle & nerve

authors： Stino AM,Atway S,Anthony M,Kline D,Kissel JT

更新日期：2019-01-01 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：:The spectrum of neuromuscular disorders among intensive care unit (ICU) patients has shifted toward disorders acquired within the ICU and away from "traditional" neuromuscular disorders that lead to ICU admission. We sought to assess this spectrum by determining the causes and relative frequencies of neuromuscular dis...

journal_title：Muscle & nerve

authors： Lacomis D,Petrella JT,Giuliani MJ

更新日期：1998-05-01 00:00:00

abstract：:Muscle makes up the largest tissue volume of the body, yet its size makes muscle-specific therapy difficult. This becomes particularly relevant when approaches to gene therapy for inherited myopathies are evaluated. Thus, a mechanism to target constructs or pharmaceuticals to muscle following intravenous injection wou...

journal_title：Muscle & nerve

authors： Samoylova TI,Smith BF

更新日期：1999-04-01 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：: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：:Frog muscles bathed in solutions equilibrated with 0.5%-2.0% halothane develop large contractures when cooled to 2-4 degrees C. These halothane-cooling contractures (HCC) are dose dependent and reproducible; they are potentiated by lidocaine (5 mM), abolished by procaine (5 mM), and partially inhibited by procainamide...

journal_title：Muscle & nerve

authors： Suarez-Kurtz G,Sudo RT

更新日期：1986-01-01 00:00:00

abstract：INTRODUCTION:An open-label trial suggested that valproic acid (VPA) improved strength in adults with spinal muscular atrophy (SMA). We report a 12-month, double-blind, cross-over study of VPA in ambulatory SMA adults. METHODS:There were 33 subjects, aged 20–55 years, included in this investigation. After baseline asse...

journal_title：Muscle & nerve

authors： Kissel JT,Elsheikh B,King WM,Freimer M,Scott CB,Kolb SJ,Reyna SP,Crawford TO,Simard LR,Krosschell KJ,Acsadi G,Schroth MK,D'Anjou G,LaSalle B,Prior TW,Sorenson S,Maczulski JA,Swoboda KJ,Project Cure Spinal Muscular Atr

更新日期：2014-02-01 00:00:00