Charcot-Marie-Tooth disease with intermediate conduction velocities caused by a novel mutation in the MPZ gene.

abstract：INTRODUCTION:In compressive neuropathies, large myelinated nerve fibers are generally thought to be more susceptible. In this study, we investigated small myelinated Aδ and unmyelinated C fiber function in patients with mild, moderate, and severe carpal tunnel syndrome. METHODS:Forty-four healthy controls and 81 carpa...

journal_title：Muscle & nerve

authors： Clarke C,Christensen C,Curran MWT,Chan KM

更新日期：2017-10-01 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：: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：:To investigate whether various myelin markers could detect pathological changes in myelination, the activity of 2'-3'-cyclic nucleotide 3'-phosphodiesterase (CNP) and the level of PO protein were compared in the peripheral nervous system (PNS) of the dystrophic mouse and chicken and their phenotypically normal sibling...

journal_title：Muscle & nerve

authors： Lewis LN,Mezei C

更新日期：1985-02-01 00:00:00

abstract：INTRODUCTION:Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are considered part of the same pathological spectrum. There is an increased risk of ALS in patients who have had melanoma. The risk of FTLD in melanoma (or cancer) patients is unknown. We aimed to study if C9ORF72 expansion i...

journal_title：Muscle & nerve

authors： Tábuas-Pereira M,Almendra L,Almeida MR,Durães J,Pinho A,Matos A,Negrão L,Geraldo A,Santana I

更新日期：2019-03-01 00:00:00

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：: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：: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：:Intraoperative electrophysiological monitoring of the spinal cord has traditionally been done by recording somatosensory evoked potentials (SEP). There is a risk that SEPs can be unaltered when significant injury to the anterior spinal cord has occurred. The purpose of this report is to describe a simple technique for...

journal_title：Muscle & nerve

authors： Phillips LH 2nd,Blanco JS,Sussman MD

更新日期：1995-03-01 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：:We report a patient who experienced progressive diplopia and distal weakness of the upper limbs. Magnetic resonance imaging of the brain showed extensive white matter lesions and analysis of cerebrospinal fluid revealed acute human T-lymphotropic virus type I (HTLV-I) infection. Myasthenia gravis (MG) was evidenced by...

journal_title：Muscle & nerve

authors： Lalive PH,Allali G,Truffert A

更新日期：2007-04-01 00:00:00

abstract：:Hindlimb ischemia for 4 h, followed by reperfusion, resulted in necrosis of most soleus muscle in euthyroid rats, whereas only slight damage occurred in hypothyroid rats. Muscle repair after transection of the tibialis anterior muscle of hypothyroid rats showed delayed debris removal, initial retardation of myotube fo...

journal_title：Muscle & nerve

authors： Ozawa J,Kawamata S,Kurosaki T,Iwamizu Y,Matsuura N,Abiko S,Kai S

更新日期：2003-05-01 00:00:00

abstract：:A mathematical model is presented that predicts the electric field induced in the arm during magnetic stimulation of a peripheral nerve. The arm is represented as a homogeneous, cylindrical volume conductor. The electric field arises from two sources: the time-varying magnetic field and the accumulation of charge on t...

journal_title：Muscle & nerve

authors： Roth BJ,Cohen LG,Hallett M,Friauf W,Basser PJ

更新日期：1990-08-01 00:00:00

abstract：INTRODUCTION:Patients with DMD have low bone mineral density (BMD) and are at high risk for fractures. We examined changes in BMD and the effects of alendronate in DMD patients treated at our institution in the last decade. METHODS:Retrospective cohort study of 39 DMD patients. RESULTS:Patients had screening dual ene...

journal_title：Muscle & nerve

authors： Houston C,Mathews K,Shibli-Rahhal A

更新日期：2014-04-01 00:00:00

abstract：INTRODUCTION:Repetitive loading of the low back tissues induces tension relaxation with a corresponding variation in the myoelectric response of the neuromuscular system, which may influence low back health. The purpose of this study was to observe trunk muscle activities before and after a passive cyclic trunk flexion...

journal_title：Muscle & nerve

authors： Olson MW

更新日期：2011-11-01 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：: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：:In muscle of whole animals, pituitary growth hormone, the thyroid hormones, and insulin are major growth-promoting hormones, and the glucocorticoids have significant catabolic actions. At the cellular level the primary anabolic hormones for cultured myoblasts are the somatomedins (insulin-like growth factors) and fibr...

journal_title：Muscle & nerve

authors： Florini JR

更新日期：1987-09-01 00:00:00

abstract：:Congenital myasthenic syndromes (CMS) stem from defects in presynaptic, synaptic basal lamina, and postsynaptic proteins. The presynaptic CMS are associated with defects that curtail the evoked release of acetylcholine (ACh) quanta or ACh resynthesis. Defects in ACh resynthesis have now been traced to mutations in cho...

journal_title：Muscle & nerve

authors： Engel AG,Ohno K,Sine SM

更新日期：2003-01-01 00:00:00

abstract：INTRODUCTION:Duchenne muscular dystrophy (DMD) is characterized by progressive muscle degeneration leading to immobility, respiratory failure, and premature death. As chronic inflammation and stress are implicated in DMD pathology, the efficacy of an anti-inflammatory and anti-stress intervention strategy in ameliorati...

journal_title：Muscle & nerve

authors： Manning J,Buckley MM,O'Halloran KD,O'Malley D

更新日期：2017-12-01 00:00:00

abstract：:At least 50 disease-causing mutations in the skeletal muscle voltage-gated chloride channel gene (CLCN1), almost all of which originate from Caucasian families, have been identified. We investigated a Japanese family with Thomsen's myotonia congenita that included 16 affected individuals (8 men and 8 women) through fi...

journal_title：Muscle & nerve

authors： Sasaki R,Ito N,Shimamura M,Murakami T,Kuzuhara S,Uchino M,Uyama E

更新日期：2001-03-01 00:00:00

abstract：:Inhibitory reflex responses to electrical stimulation of the mental nerve were studied in recordings from single motor units (SMU) in the human masseter muscle. A new analytical technique for spike train data was used. Electrical stimuli were delivered to the mental nerve when the SMU fired with two consecutive inter-...

journal_title：Muscle & nerve

authors： Graven-Nielsen T,Svensson P,McMillan AS,Arendt-Nielsen L

更新日期：2000-02-01 00:00:00

abstract：:The effects of altering (cross-reinnervation, CR) and/or eliminating (spinal isolation, SI) the activation-dependent neural input to the cat soleus for 8 months on its mechanical and biochemical properties were determined. The percent fast fibers was 0, 27, 38, and 54% for normal control (NC), NC-CR, SI, and SI-CR cat...

journal_title：Muscle & nerve

authors： Roy RR,Eldridge L,Baldwin KM,Edgerton VR

更新日期：1996-06-01 00:00:00

abstract：:The muscle-specific helix-loop-helix (HLH) transcription factors myoD, myogenin, MRF4, and myf-5 are called the muscle regulatory factor family (MRF). Levels of MRFs are strongly regulated by muscle electrical activity and are thought to control downstream genes that are important for muscle phenotype such as the acet...

journal_title：Muscle & nerve

authors： Carlsen H,Gundersen K

更新日期：2000-09-01 00:00:00

abstract：:Direct infection of muscle fibers by human T-lymphotropic virus type I (HTLV-I) has recently been reported in a patient with polymyositis infected with both HTLV-I and human immunodeficiency virus (HIV). Coinfections of these viruses are frequently found in the United States. In Kagoshima, Japan, patients with polymyo...

journal_title：Muscle & nerve

authors： Higuchi I,Nerenberg M,Yoshimine K,Yoshida M,Fukunaga H,Tajima K,Osame M

更新日期：1992-01-01 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

abstract：:A patient in perfect health prior to electrical shock developed an ALS-like syndrome after the shock. Onset of the disease occurred in the limb through which the shock entered, and subsequently followed a course well-described in previous case reports. Possible mechanisms of disease are discussed. ...

journal_title：Muscle & nerve

authors： Sirdofsky MD,Hawley RJ,Manz H

更新日期：1991-10-01 00:00:00

abstract：INTRODUCTION:The purpose of this study was to determine the impact of an in-home expiratory muscle strength training (EMST) program on pulmonary, swallow, and cough function in individuals with amyotrophic lateral sclerosis (ALS). METHODS:EMST was tested in a prospective, single-center, double-blind, randomized, contr...

journal_title：Muscle & nerve

authors： Plowman EK,Tabor-Gray L,Rosado KM,Vasilopoulos T,Robison R,Chapin JL,Gaziano J,Vu T,Gooch C

更新日期：2019-01-01 00:00:00

abstract：:This article describes nerve conduction studies of the deep temporal nerve (DTN) and the mylohyoid nerve (MHN) motor branches of the trigeminal nerve. These nerves were stimulated intraorally with a pediatric surface stimulator. Compound muscle action potentials were recorded over the temporalis and mylohyoid muscles ...

journal_title：Muscle & nerve

authors： Dillingham TR,Spellman NT,Chang AS

更新日期：1996-03-01 00:00:00

abstract：INTRODUCTION:The aim of this study was to compare ultrasound echo intensity (EI) with high-resolution T1 -weighted MRI and to establish calibration equations to estimate percent intramuscular fat from EI. METHODS:Thirty-one participants underwent both ultrasound and MRI testing of 4 muscles: rectus femoris (RF); bicep...

journal_title：Muscle & nerve

authors： Young HJ,Jenkins NT,Zhao Q,Mccully KK

更新日期：2015-12-01 00:00:00