Benign course of glycogen storage disease type IIb in two brothers: nature or nurture?

abstract：:Laser Doppler flowmetry (LDF) was conducted on familial amyloidotic polyneuropathy (FAP) patients and asymptomatic carriers of FAP. Vasoconstrictive responses in the 11 FAP patients tested, induced by deep inspiration, were markedly depressed compared with those of the healthy controls. The responses decreased with th...

journal_title：Muscle & nerve

authors： Ando Y,Araki S,Shimoda O,Kano T

更新日期：1992-04-01 00:00:00

abstract：:A common misconception attributes sparing of the flexor carpi ulnaris (FCU) in ulnar neuropathy at the elbow (UNE) to its innervating branch arising "at or above the elbow." We examined the relationship of FCU branches to the medial epicondyle (ME) and humeroulnar aponeurotic arcade (HUA) in 30 cadaver elbows. In only...

journal_title：Muscle & nerve

authors： Campbell WW,Pridgeon RM,Riaz G,Astruc J,Leahy M,Crostic EG

更新日期：1989-12-01 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：:Patients with chronic inflammatory demyelinating polyneuropathy (CIDP) received immune globulin intravenous, 10% caprylate/chromatography purified (IGIV-C, Gamunex; n=59) or placebo (n=58) every 3 weeks for up to 24 weeks (first period) in a randomized, double-blind, parallel-group, response-conditional, crossover stu...

journal_title：Muscle & nerve

authors： Bril V,Katzberg H,Donofrio P,Banach M,Dalakas MC,Deng C,Hanna K,Hartung HP,Hughes RA,Latov N,Merkies IS,van Doorn PA,ICE Study Group.

更新日期：2009-04-01 00:00:00

abstract：:CNS conduction and autonomic nervous function were investigated in 15 patients with HMSN I. Central motor conduction time (CMCT) was estimated with magnetic brain stimulation and electrical nerve root stimulation. Somatosensory evoked potential (SEP) and visual evoked potential (VEP) were used for assessment of centra...

journal_title：Muscle & nerve

authors： Solders G,Andersson T,Persson A

更新日期：1991-11-01 00:00:00

abstract：INTRODUCTION:Skin-derived precursor cells (SKPs) are neural crest progenitor cells that can attain a Schwann cell-like phenotype through in vitro techniques (SKP-SCs). We hypothesized that SKP-SCs could produce mature myelin and, in doing so, facilitate the recovery of a focal demyelination injury. METHODS:We unilater...

journal_title：Muscle & nerve

authors： Grochmal J,Dhaliwal S,Stys PK,van Minnen J,Midha R

更新日期：2014-08-01 00:00:00

abstract：:Muscle weakness in myasthenia gravis is due to autoantibody-induced loss of functional acetylcholine receptors (AChR). About 15% of myasthenia gravis patients, however, do not have detectable anti-AChR antibodies. To investigate the effect of their plasma immunoglobulins on neuromuscular transmission, mice were inject...

journal_title：Muscle & nerve

authors： Burges J,Vincent A,Molenaar PC,Newsom-Davis J,Peers C,Wray D

更新日期：1994-12-01 00:00:00

abstract：INTRODUCTION:The aim of this study was to assess whether peripheral neuropathy is a feature of glycogen storage disease type IIIa (GSD IIIa) in adult patients. METHODS:Medical records of a cohort of adult GSD IIIa patients who underwent electromyography (EMG) and nerve conduction studies (NCS) were reviewed, and the r...

journal_title：Muscle & nerve

authors： Herlin B,Laforět P,Labrune P,Fournier E,Stojkovic T

更新日期：2016-02-01 00:00:00

abstract：:Polylysines, polyornithine, and protamine increased the release of creatine kinase (CK) and lactate dehydrogenase (LDH) from frog muscles. With 5 micrograms/ml (50 nM) of a polylysine of molecular weight 100,000, enzyme release reached peak values (ten-fold above control) within 30 minutes; subsequent removal of the p...

journal_title：Muscle & nerve

authors： Suarez-Kurtz G

更新日期：1985-02-01 00:00:00

abstract：:Spatial distribution of motor endplates affects the shape of the electrical activity recorded from muscle. In order to provide information for realistic models of action potential propagation within muscles, we assembled three-dimensional maps of the motor endplates of the rat medial gastrocnemius (MGM) and lateral ga...

journal_title：Muscle & nerve

authors： Prodanov D,Thil MA,Marani E,Delbeke J,Holsheimer J

更新日期：2005-09-01 00:00:00

abstract：BACKGROUND:Diagnosing cervical radiculopathy (CR) can be difficult because of symptomatic overlap with peripheral neuropathies. In this retrospective observational study, we aimed to determine whether short-tau inversion recovery (STIR) magnetic resonance imaging (MRI) sequences are useful for detecting signs of denerv...

journal_title：Muscle & nerve

authors： Yoshida T,Suwazono S,Sueyoshi T,Izumi Y,Nodera H

更新日期：2020-12-13 00:00:00

abstract：:Although the blood level of creatine kinase (CK) is the most commonly used marker of muscle injury, there is marked interindividual variability in this measure. Part of this variability may be attributed to variability in the rate of CK clearance from the circulation. In this study, we assessed the possibility that CK...

journal_title：Muscle & nerve

authors： Warren GL,O'farrell L,Rogers KR,Billings KM,Sayers SP,Clarkson PM

更新日期：2006-09-01 00:00:00

abstract：:We observed a marked prolongation of the transcranially evoked silent period during continuous intrathecal administration of high doses of the gamma-aminobutyric acid (GABA)B receptor agonist baclofen in a patient with generalized dystonia. Size of motor evoked potentials and central conduction time remained unchanged...

journal_title：Muscle & nerve

authors： Siebner HR,Dressnandt J,Auer C,Conrad B

更新日期：1998-09-01 00:00:00

abstract：:Motor unit action potentials (MUAPs) evoked by repetitive, low-intensity transcranial magnetic stimulation can be modeled as a Poisson process. A mathematical consequence of such a model is that the ratio of the variance to the mean of the amplitudes of motor evoked potentials (MEPs) should provide an estimate of the ...

journal_title：Muscle & nerve

authors： Kaelin-Lang A,Conforto AB,Z'Graggen W,Hess CW

更新日期：2010-11-01 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：:Focal entrapment of the ulnar nerve occurs most frequently in the region of the elbow, at the ulnar groove or beneath the humeroulnar aponeurosis. Surgical treatment commonly involves transposition of the nerve anterior to the medial epicondyle, in the antecubital fossa. Symptoms may recur after surgery, and, to asses...

journal_title：Muscle & nerve

authors： Matei CI,Logigian EL,Shefner JM

更新日期：2004-10-01 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：:We measured distal sympathetic and vagal function in 73 consecutive patients with diabetic neuropathy seen at the Mayo Autonomic Reflex Laboratory. Postganglionic sympathetic failure measured proximally within the foot occurred as commonly as vagal failure (58% and 55%, respectively) and occurred much more frequently ...

journal_title：Muscle & nerve

authors： Low PA,Zimmerman BR,Dyck PJ

更新日期：1986-09-01 00:00:00

abstract：:Mutations in CLCN1, the gene encoding the ClC-1 chloride channel in skeletal muscle, lead to myotonia congenita. The effects on the intramembranous channel forming domains have been investigated more than that at the intracellular C-terminus. We have performed a mutation screen involving the whole CLCN1 gene of patien...

journal_title：Muscle & nerve

authors： Chen L,Schaerer M,Lu ZH,Lang D,Joncourt F,Weis J,Fritschi J,Kappeler L,Gallati S,Sigel E,Burgunder JM

更新日期：2004-05-01 00:00:00

abstract：:Epstein-Barr virus infection can affect both the central and peripheral nervous system. In some patients this occurs concurrently. Two patients are presented with encephalopathy and acute quadriparesis with diminished reflexes. Positive serology for Epstein-Barr virus was found in both patients. Both patients had a ce...

journal_title：Muscle & nerve

authors： Morgenlander JC

更新日期：1996-08-01 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：:Left spinal accessory nerve palsy occurred in a young man when he quickly turned his head to the right while his shoulders were pulled down by heavy hand-held objects. Electrophysiologic studies demonstrated partial axonotmesis of the spinal accessory nerve branches innervating the sternocleidomastoid and upper and mi...

journal_title：Muscle & nerve

authors： Logigian EL,McInnes JM,Berger AR,Busis NA,Lehrich JR,Shahani BT

更新日期：1988-02-01 00:00:00

abstract：:Body weight-supported treadmill (BWST) training has been shown to improve ambulatory capacity in persons with a spinal cord injury (SCI); however, the effect that BWST training has on skeletal muscle phenotype is unknown. We aimed to determine whether 6 months (three sessions/week) of BWST training in neurologically s...

journal_title：Muscle & nerve

authors： Stewart BG,Tarnopolsky MA,Hicks AL,McCartney N,Mahoney DJ,Staron RS,Phillips SM

更新日期：2004-07-01 00:00:00

abstract：:Forty patients with polymyositis or dermatomyositis underwent detailed electromyographic evaluation. The paraspinal muscles of all patients were examined, as were several extremity muscles. The distribution of fibrillation potentials (FPs) in different muscles is discussed. FPs were most frequent in paraspinal muscles...

journal_title：Muscle & nerve

authors： Streib EW,Wilbourn AJ,Mitsumoto H

更新日期：1979-01-01 00:00:00

abstract：:Recurrent idiopathic lumbosacral plexopathy has rarely been reported in the literature. The present report describes a 59-year-old man presenting with recurrent episodes of acute leg pain, followed by weakness. After each episode, symptoms progressed for several months before peaking. Thereafter, gradual recovery ensu...

journal_title：Muscle & nerve

authors： Yee T

更新日期：2000-09-01 00:00:00

abstract：:An enzyme- and immunohistochemical study has been performed on human masseter muscle spindles. Antibodies selective for different myosin heavy chain (MHC) isoforms and M-band proteins (M-protein, myomesin, and MM-CK) were used. The expression of these proteins was determined in the different intrafusal fiber types. Nu...

journal_title：Muscle & nerve

authors： Eriksson PO,Butler-Browne GS,Thornell LE

更新日期：1994-01-01 00:00:00

abstract：INTRODUCTION:In preparation for clinical trials we examine the validity, reliability, and patient understanding of the Myotonic Dystrophy Health Index (MDHI). METHODS:Initially we partnered with 278 myotonic dystrophy type-1 (DM1) patients and identified the most relevant questions for the MDHI. Next, we used factor a...

journal_title：Muscle & nerve

authors： Heatwole C,Bode R,Johnson N,Dekdebrun J,Dilek N,Heatwole M,Hilbert JE,Luebbe E,Martens W,Mcdermott MP,Rothrock N,Thornton C,Vickrey BG,Victorson D,Moxley R 3rd

更新日期：2014-06-01 00:00:00

abstract：:In this study some particular and puzzling aspects of amyotrophic lateral sclerosis (ALS) are discussed, with an emphasis on the role of neocortical evolution. Other issues explored include the nature of the clinical deficit in ALS; anatomical and evolutionary aspects of the neocortex, motor cortex, and corpus callosu...

journal_title：Muscle & nerve

authors： Eisen A

更新日期：2009-08-01 00:00:00

abstract：:Myasthenia gravis (MG) and its animal model experimental autoimmune myasthenia gravis (EAMG) are caused by autoantibodies against nicotinic acetylcholine receptor (AChR) in skeletal muscle. The production of anti-AChR antibodies is mediated by cytokines produced by CD4+ and CD8+ T helper (Th) cells. Emerging investiga...

journal_title：Muscle & nerve

authors： Zhang GX,Navikas V,Link H

更新日期：1997-05-01 00:00:00

abstract：:Inflammatory myopathies (IMs) occur relatively frequently in dogs, and, with the exception of masticatory muscle myositis (MMM), have not been characterized. This study analyzed the distribution and types of cellular infiltrates in 21 cases of generalized IM, 3 cases of focal IM (MMM), and 1 case with features of both...

journal_title：Muscle & nerve

authors： Pumarola M,Moore PF,Shelton GD

更新日期：2004-06-01 00:00:00