Critical review of gangliosides and thyrotropin-releasing hormone in peripheral neuromuscular diseases.

abstract：:Nemaline myopathy is a rare disorder of varying severity and genetic etiology. We present two cases, a father and son, with a novel missense mutation in the alpha actin gene. Both have a history of early motor impairment, with the son's course being considerably more severe. This pair illustrates the clinical variabil...

journal_title：Muscle & nerve

authors： Bouldin AA,Parisi MA,Laing N,Patterson K,Gospe SM Jr

更新日期：2007-02-01 00:00:00

abstract：:Protein synthesis in soleus and extensor digitorum longus (EDL) muscles was measured in vitro to test the hypothesis that the lack of muscle protein accumulation in dystrophic conditions could be caused by a reduced sensitivity to insulin. We demonstrate that physiological insulin concentrations stimulate protein synt...

journal_title：Muscle & nerve

authors： Ballard FJ,Nield MK,Tomas FM

更新日期：1983-09-01 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：:The aim of this work was to determine the role of peripheral facial muscle reinnervation in the central reorganization of the blink reflex (BR) after hypoglossal-facial anastomosis (HFA). An electrophysiological study was performed on seven patients who underwent HFA after facial nerve transection during surgery for a...

journal_title：Muscle & nerve

authors： Bernat I,Vitte E,Lamas G,Soudant J,Willer JC,Tankéré F

更新日期：2006-03-01 00:00:00

abstract：:Danon disease is a rare X-linked dominant disorder caused by lysosomal-associated membrane protein 2 (LAMP2) deficiency and is characterized by hypertrophic cardiomyopathy, cardiac conduction abnormalities, skeletal vacuolar myopathy, variable degree of mental retardation, and peripheral pigmentary retinopathy. We des...

journal_title：Muscle & nerve

authors： Nadeau A,Therrien C,Karpati G,Sinnreich M

更新日期：2008-03-01 00:00:00

abstract：:Peripheral nerve disorders are commonly encountered in clinical practice. Electrodiagnostic studies remain the cornerstone of the evaluation of nerve disorders. More recently, ultrasound has played an increasing complementary role in the neuromuscular clinic. Ultrasound elastography is a technique that measures the el...

journal_title：Muscle & nerve

authors： Wee TC,Simon NG

更新日期：2019-11-01 00:00:00

abstract：:A treatment has been developed to alleviate muscle weakness in murine dystrophy. Cultured myoblasts from genetically normal mouse embryos were injected into the right soleus of 20-day-old normal or dystrophic mice. Hosts and donors were immunocompatible but exhibited different genotype markers. Donor cells produced GP...

journal_title：Muscle & nerve

authors： Law PK,Goodwin TG,Wang MG

更新日期：1988-06-01 00:00:00

abstract：INTRODUCTION:Ulnar nerve displacement at the elbow causes overestimation of nerve conduction velocity. We hypothesized that this overestimation may cause false-negative results when the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM) diagnostic criteria are used. METHODS:We investigated 9 ...

journal_title：Muscle & nerve

authors： Won SJ,Yoon JS,Kim JY,Kim SJ,Jeong JS

更新日期：2011-10-01 00:00:00

abstract：INTRODUCTION:Nerve enlargement (NE) is described in inflammatory and inherited neuropathies. It is commonly multifocal and moderate in the former and homogeneous and generalized in the latter. We describe 4 cases of massive NE in inflammatory neuropathies. METHODS:Patients presented with symptoms of polyneuropathy tha...

journal_title：Muscle & nerve

authors： Grimm A,Schubert V,Axer H,Ziemann U

更新日期：2017-02-01 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：:We used Southern blot analysis and the polymerase chain reaction to analyze the tissue distribution of multiple mitochondrial DNA (mtDNA) deletions in a 45-year-old man with familial mitochondrial myopathy-Southern blots showed two major types of abnormal mtDNA with approximately 4- and 8-kilobase deletions in the ske...

journal_title：Muscle & nerve

authors： Kawashima S,Ohta S,Kagawa Y,Yoshida M,Nishizawa M

更新日期：1994-07-01 00:00:00

abstract：:The clinical efficacy of functional electrical stimulation (FES) is limited by the rapid onset of fatigue. Functional electrical stimulation applications typically stimulate skeletal muscles with constant-frequency trains (CFTs). Our laboratory has identified trains that we call doublet-frequency trains (DFTs) and tha...

journal_title：Muscle & nerve

authors： Scott WB,Lee SC,Johnston TE,Binder-Macleod SA

更新日期：2005-05-01 00:00:00

abstract：:The utilization of viable biological nerve graft substitutes and nerve allografts raises the problem of nerve storage. To clarify this, rat sciatic nerve segments were harvested and stored in Dulbecco's modified eagle medium. The segments were divided into three groups. In the first group, no cryoprotectant was added,...

journal_title：Muscle & nerve

authors： Fansa H,Lassner F,Kook PH,Keilhoff G,Schneider W

更新日期：2000-08-01 00:00:00

abstract：:Measurements were done on the biceps muscles of 6 healthy volunteers to record simultaneously the surface electromyogram (EMG) and vibromyogram (VMG) by means of a piezoelectric device (accelerometer). The VMG is generated by mechanical waves due to the contraction mechanism and often measured as sound. The frequency ...

journal_title：Muscle & nerve

authors： Zwarts MJ,Keidel M

更新日期：1991-08-01 00:00:00

abstract：:Isometric contractile properties of human elbow flexor muscles were examined in 27 normal subjects (7 females and 20 males). Contractions of elbow flexors were elicited by application of electrical pulses to the biceps motor points. The tension development was recorded at the wrist with the elbow angle fixed at 90 deg...

journal_title：Muscle & nerve

authors： Ismail HM,Ranatunga KW

更新日期：1981-05-01 00:00:00

abstract：:This study aimed to clarify the morphologic variations of the Martin-Gruber anastomosis (MGA) by tracing the anastomotic fascicles. We used 102 upper limbs, and MGA was found in 39.2%. Among 12 instances of MGA between the branches innervating the flexor digitorum profundus muscle, eight anastomotic branches solely in...

journal_title：Muscle & nerve

authors： Lee KS,Oh CS,Chung IH,Sunwoo IN

更新日期：2005-01-01 00:00:00

abstract：:Denervation induces muscle fiber atrophy and changes in the gene expression rates of skeletal muscle. Electrical stimulation (ES) is a procedure generally used to treat denervated muscles in humans. This study evaluated the effect of ES based on chronaxie and rheobase on the expression of the myoD and atrogin-1 genes ...

journal_title：Muscle & nerve

authors： Russo TL,Peviani SM,Freria CM,Gigo-Benato D,Geuna S,Salvini TF

更新日期：2007-01-01 00:00:00

abstract：:Serum creatine kinase (CK) activity, calcium (Ca) and magnesium (Mg) contents of skeletal muscle and isolated mitochondria, as well as oxidative phosphorylation of X-linked muscular dystrophic (mdx) mice were compared with normal control animals at ages 5, 10, and 23 weeks. Serum CK is elevated in mdx mice at all ages...

journal_title：Muscle & nerve

authors： Glesby MJ,Rosenmann E,Nylen EG,Wrogemann K

更新日期：1988-08-01 00:00:00

abstract：INTRODUCTION:Previous studies have reported higher wrist ratios (WR) related to carpal tunnel syndrome (CTS) but have not assessed effect modification by obesity and may have inadequately controlled for confounders. METHODS:Baseline data of a multicenter prospective cohort study were analyzed. CTS was defined by nerve...

journal_title：Muscle & nerve

authors： Thiese MS,Merryweather A,Koric A,Ott U,Wood EM,Kapellusch J,Foster J,Garg A,Deckow-Schaefer G,Tomich S,Kendall R,Drury DL,Wertsch J,Hegmann KT,Wistah Study Team.

更新日期：2017-12-01 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：INTRODUCTION:Previous studies of limb-girdle muscular dystrophy type 2A (LGMD2A) patients in many countries have suggested a heterogeneous genetic and clinical spectrum, but the genotypes and phenotypes of Chinese LGMD2A patients remain unclear. METHODS:We directly screened calpain-3 (CAPN3) in 18 Chinese Han subjects...

journal_title：Muscle & nerve

authors： Luo SS,Xi JY,Zhu WH,Zhao CB,Lu JH,Lin J,Wang Y,Lu J,Qiao K

更新日期：2012-11-01 00:00:00

abstract：:Intramuscular injection of botulinum toxin A is used to treat focal dystonias. Because immunoresistance has been documented in some patients, other molecular forms of the toxin have been evaluated clinically. The present investigation compared the time course and extent of neuromuscular blockade and recovery of botuli...

journal_title：Muscle & nerve

authors： Billante CR,Zealear DL,Billante M,Reyes JH,Sant'Anna G,Rodriguez R,Stone RE Jr

更新日期：2002-09-01 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：INTRODUCTION:We sought to determine whether electrical impedance myography (EIM) could serve as a diagnostic procedure for evaluation of radiculopathy. METHODS:Twenty-seven patients with clinically and radiologically diagnosed cervical or lumbosacral radiculopathy who met a "gold standard" definition underwent EIM and...

journal_title：Muscle & nerve

authors： Spieker AJ,Narayanaswami P,Fleming L,Keel JC,Muzin SC,Rutkove SB

更新日期：2013-11-01 00:00:00

abstract：:Cardiotrophin-1 (CT-1) has potent survival-promoting effects on motor neurons in vitro and in vivo and may be effective in treating motor neuron diseases (MND). We investigated the effects of CT-1 treatment in wobbler mouse MND. Wobbler mice were randomly assigned to receive subcutaneously injected CT-1 (1 mg/kg, n = ...

journal_title：Muscle & nerve

authors： Mitsumoto H,Klinkosz B,Pioro EP,Tsuzaka K,Ishiyama T,O'Leary RM,Pennica D

更新日期：2001-06-01 00:00:00

abstract：:The clinical, pathologic, and genetic findings of a boy with intranuclear nemaline rod myopathy are described. Serial muscle biopsies revealed myocyte nuclei containing inclusions that were immunoreactive for alpha-actinin and increased with age. Genetic analysis revealed a Val163Leu ACTA1 mutation previously associat...

journal_title：Muscle & nerve

authors： Kaimaktchiev V,Goebel H,Laing N,Narus M,Weeks D,Nixon R

更新日期：2006-09-01 00:00:00

abstract：INTRODUCTION:POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes) syndrome may be mistaken for chronic inflammatory demyelinating polyneuropathy (CIDP). Differentiating the 2 entities is crucial, as there are major treatment implications. METHODS:We compared platelet ...

journal_title：Muscle & nerve

authors： Naddaf E,Dispenzieri A,Mandrekar J,Mauermann ML

更新日期：2015-10-01 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：:Toxoplasma gondii encysts in skeletal muscle. Although only rarely found at muscle biopsy, this parasite has previously been regarded as a possible cause of polymyositis. We report a case of biopsy-proven toxoplasmic myositis in a non-HIV-infected patient that led to recognition of idiopathic CD4 lymphocytopenia (ICL)...

journal_title：Muscle & nerve

authors： Plonquet A,Bassez G,Authier FJ,Dray JM,Farcet JP,Gherardi RK

更新日期：2003-06-01 00:00:00

abstract：INTRODUCTION:The risk of cancer in patients diagnosed with myotonic dystrophy (DM) is reported for the homogeneous Utah population. METHODS:Clinical data accessed from the largest Utah healthcare providers have been record-linked to the Utah Population Database, a population-based resource also linked to the Utah Canc...

journal_title：Muscle & nerve

authors： Abbott D,Johnson NE,Cannon-Albright LA

更新日期：2016-10-01 00:00:00