Pediatric laminopathies: Whole-body magnetic resonance imaging fingerprint and comparison with Sepn1 myopathy.

abstract：:The American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM) developed guidelines to formalize the ethical standards that neuromuscular and electrodiagnostic (EDx) physicians should observe in their clinical and scientific activities. Neuromuscular and EDx medicine is a subspecialty of medicine that ...

journal_title：Muscle & nerve

authors： Abel NA,De Sousa EA,Govindarajan R,Mayer MP,Simpson DA

更新日期：2015-12-01 00:00:00

abstract：:There is evidence that growth hormone may be related to the progression of weakness in Duchenne dystrophy. We conducted a 12-month controlled trial of mazindol, a putative growth hormone secretion inhibitor, in 83 boys with Duchenne dystrophy. Muscle strength, contractures, functional ability and pulmonary function we...

journal_title：Muscle & nerve

authors： Griggs RC,Moxley RT 3rd,Mendell JR,Fenichel GM,Brooke MH,Miller PJ,Mandel S,Florence J,Schierbecker J,Kaiser KK

更新日期：1990-12-01 00:00:00

abstract：:Implantation of normal muscle precursor cells (mpc) for treatment for inherited myopathies such as Duchenne muscular dystrophy is in clear need of improvement to become practicable, but few variables have been studied comparatively. Here, we report the first quantitative estimate of the effectiveness of implanting mpc...

journal_title：Muscle & nerve

authors： Morgan JE,Fletcher RM,Partridge TA

更新日期：1996-02-01 00:00:00

abstract：:An electrophysiologic study of peripheral nerve excitability was performed in patients with hyperkalemic periodic paralysis (HPP) and in normal controls. It was found that a marked degree of neural hyperexcitability existed in all patients with HPP who showed cold sensitivity. The results suggest involvement of the ne...

journal_title：Muscle & nerve

authors： Segura RP,Petajan JH

更新日期：1979-07-01 00:00:00

abstract：:Several studies have suggested the presence of central nervous system involvement manifesting as cognitive impairment in diseases traditionally confined to the peripheral nervous system. The aim of this review is to highlight the character of clinical, genetic, neurofunctional, cognitive, and psychiatric deficits in n...

journal_title：Muscle & nerve

authors： D'Angelo MG,Bresolin N

更新日期：2006-07-01 00:00:00

abstract：:Lymphoma occasionally affects the peripheral nervous system. When it does, the diagnosis can be elusive since many patients present without known lymphoma. Most peripheral nerve complications are due to non-Hodgkin's lymphoma (NHL), which infiltrates nerves causing axonal damage. This disorder can affect nerve roots a...

journal_title：Muscle & nerve

authors： Kelly JJ,Karcher DS

更新日期：2005-03-01 00:00:00

abstract：:A low conductance calcium-activated K+ channel is thought to regulate the rate of firing of several excitable cells. In skeletal muscle the expression of this channel is under nerve control. Previously, we reported that axonal flow blockade of rat nerves, induced by colchicine, caused a transient increase in muscle ap...

journal_title：Muscle & nerve

authors： Vergara C,Ramírez B,Behrens MI

更新日期：1993-09-01 00:00:00

abstract：BACKGROUND:Anti-cytosolic 5'-nucleotidase 1A (cN1A) antibodies are commonly detected in patients with sporadic inclusion body myositis (sIBM). However, their pathogenic role has not been established. Moreover, efforts toward identifying sIBM distinct clinicopathologic characteristics associated with these antibodies ha...

journal_title：Muscle & nerve

authors： Paul P,Liewluck T,Ernste FC,Mandrekar J,Milone M

更新日期：2020-12-29 00:00:00

abstract：:Five members of the same family, along three generations, presented with hypertrophic cardiomyopathy. Neurological examination, muscle strength, electromyography, and serum creatine kinase were normal. Skeletal muscle biopsy showed abnormal lipid accumulation and carnitine deficiency. In three patients the cardiac sym...

journal_title：Muscle & nerve

authors： Bautista J,Rafel E,Martinez A,Sainz I,Herrera J,Segura L,Chinchon I

更新日期：1990-03-01 00:00:00

abstract：:Absence of α-actinin-3, encoded by the ACTN3 "speed gene," is associated with poorer sprinting performance in athletes and a slowing of relaxation in fast-twitch muscles of Actn3 knockout (KO) mice. Our first aim was to investigate, at the individual-fiber level, possible mechanisms for this slowed relaxation. Our sec...

journal_title：Muscle & nerve

authors： Chan S,Seto JT,Houweling PJ,Yang N,North KN,Head SI

更新日期：2011-01-01 00:00:00

abstract：:Bcl-2 and Bax immunohistochemistry was examined in the skeletal muscle of rats after cutting the sciatic nerve, as a model of denervation and reinnervation, and in the anterior tibialis muscle of rats after an intramuscular injection of metoclopramide, as a model of necrosis and regeneration of muscle fibers, to bette...

journal_title：Muscle & nerve

authors： Olivé M,Ferrer I

更新日期：2000-12-01 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

abstract：INTRODUCTION:Few data exist on the feasibility and reliability of measuring muscular atrophy in 2 dimensions (2D) by ultrasonography (US) and elasticity with shear wave elastography (SWE) in spastic muscles. METHODS:Fourteen patients with chronic stroke took part in 2 intersession reliability experiments performed wit...

journal_title：Muscle & nerve

authors： Mathevon L,Michel F,Aubry S,Testa R,Lapole T,Arnaudeau LF,Fernandez B,Parratte B,Calmels P

更新日期：2018-02-01 00:00:00

abstract：:The objective of this study was to investigate different methods of estimating muscle inactivation, derived from single and multiple voluntary contractions. Ten subjects performed maximal and submaximal leg extensor contractions to determine an interpolation (IT) or central activation ratio (CAR). A superimposed evoke...

journal_title：Muscle & nerve

authors： Behm D,Power K,Drinkwater E

更新日期：2001-07-01 00:00:00

abstract：:Dystrophic changes of Schwann cells and demyelination occurred in rats with chronic nerve edema induced by feeding a galactose-rich diet for two years. The mechanism for edema is the sorbitol pathway which generates osmotically active polyols from galactose or glucose. The blood-nerve barrier impedes diffusion of macr...

journal_title：Muscle & nerve

authors： Powell HC,Myers RR

更新日期：1983-03-01 00:00:00

abstract：:Evaluation of the precise molecular dynamics of endplate maintenance and reorganization has been limited by the lack of available in vitro preparations. We describe an organ culture preparation of mouse diaphragm muscle which permits long-term maintenance of muscle viability. Spontaneous fibrillations, increased level...

journal_title：Muscle & nerve

authors： Wetzel DM,Salpeter MM

更新日期：1991-10-01 00:00:00

abstract：:Loss of cells from the motor system occurs during the normal aging process, leading to reduction in the complement of motor neurons and muscle fibers. The latter age-related decrease in muscle mass has been termed "sarcopenia" and is often combined with the detrimental effects of a sedentary lifestyle in older adults,...

journal_title：Muscle & nerve

authors： Vandervoort AA

更新日期：2002-01-01 00:00:00

abstract：:The aim of this study was to investigate the fasciculation potentials (FPs) in the small-peripheral muscles of the foot and hand and the possible associated factors, in a healthy population. One hundred-twenty-two normal individuals (65 men and 57 women), aged 17-67 years (mean 39.96, SD=12.76) participated in the stu...

journal_title：Muscle & nerve

authors： Mitsikostas DD,Karandreas N,Coutsopetras P,Piperos P,Lygidakis C,Papageorgiou C

更新日期：1998-04-01 00:00:00

abstract：:The cerebrospinal fluid (CSF) of patients with demyelinating neurological disease, such as Guillain-Barré syndrome or multiple sclerosis, contains factors that inhibit the excitatory Na+ current. Such antiexcitatory factors are occasionally also detectable in CSF from patients with other neurological diseases but were...

journal_title：Muscle & nerve

authors： Brinkmeier H,Seewald MJ,Wollinsky KH,Rüdel R

更新日期：1996-01-01 00:00:00

abstract：INTRODUCTION:Symptoms often return in patients with carpal tunnel syndrome (CTS) who are treated with a local corticosteroid injection. We aimed to determine prognostic factors for a successful long-term outcome. METHODS:We prospectively followed 113 patients who received an injection for CTS. Clinical, electrophysiol...

journal_title：Muscle & nerve

authors： Meys V,Thissen S,Rozeman S,Beekman R

更新日期：2011-11-01 00:00:00

abstract：INTRODUCTION:We determined whether instrumenting timed functional tasks with wireless inertial motion sensors were responsive to facioscapulohumeral muscular dystrophy (FSHD) progression and movement pattern changes. METHODS:Ten individuals who were clinically affected with genetically confirmed FSHD, mean age 54 year...

journal_title：Muscle & nerve

authors： Statland JM,Karanevich A,Bruetsch A,Huisinga J

更新日期：2019-11-01 00:00:00

abstract：:Diabetic neuropathies are the most common type of neuropathies seen in clinical practice. These neuropathies can range clinically from asymptomatic to manifesting symptoms caused by motor, sensory, and autonomic nerve dysfunction. These neuropathies can affect the peripheral nervous system, pain receptors, cardiovascu...

journal_title：Muscle & nerve

authors： Patel K,Horak H,Tiryaki E

更新日期：2021-01-01 00:00:00

abstract：:The purpose of this long-term, open parallel-group, double-consent study of alternate-day, low-dose prednisone in 2-4-year-old patients with Duchenne muscular dystrophy (DMD) was to determine whether prednisone produces a beneficial effect when given earlier than usual. Muscle function was evaluated by timed tests, an...

journal_title：Muscle & nerve

authors： Merlini L,Cicognani A,Malaspina E,Gennari M,Gnudi S,Talim B,Franzoni E

更新日期：2003-02-01 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：:Eleven patients with previous polio were studied. The concentration of energy-related metabolites and energy charge was measured from the vastus lateralis muscle, as was isometric muscle strength of knee extension. Cross-sectional area of the quadriceps femoris muscle was calculated from magnetic resonance imaging. Re...

journal_title：Muscle & nerve

authors： Nordgren B,Falck B,Stålberg E,Ronquist G,Waldenström A,Ahlström H,Hemmingson A

更新日期：1997-11-01 00:00:00

abstract：INTRODUCTION:We adopted a proteomics-based approach to gain insights into phenotypic differences between A/J and B10.SJL murine dysferlinopathy models. METHODS:We optimized immunoblotting of dysferlin by preparing homogenates of the tibialis anterior (TA) muscle under several different conditions. We compared TA muscl...

journal_title：Muscle & nerve

authors： Mueller AL,Desmond PF,Hsia RC,Roche JA

更新日期：2014-08-01 00:00:00

abstract：:The acronym CANOMAD encompasses chronic ataxic neuropathy combined with ophthalmoplegia, M protein, cold agglutinins, and anti-disialosyl antibodies.Herein we describe 2 patients presenting with progressive ataxic neuropathy who only developed ophthalmoplegia after a significant delay post-presentation, which in 1 cas...

journal_title：Muscle & nerve

authors： Kam C,Balaratnam MS,Purves A,Mills KR,Riordan-Eva P,Pollock S,Bodi I,Munro NA,Bennett DL

更新日期：2011-11-01 00:00:00

abstract：:KANDID is an advanced EMG decision support system dedicated to the support of the clinical neurophysiologist during EMG examinations. It has facilities for test planning, automatized and structured data interpretation, EMG diagnosis, explanation, and reporting. In a prospective European multicenter field trial, the ag...

journal_title：Muscle & nerve

authors： Vingtoft S,Fuglsang-Frederiksen A,Rønager J,Petrera J,Stigsby B,Willison RG,Jarratt JA,Fawcett PR,Schofield IS,Otte G

更新日期：1993-05-01 00:00:00

abstract：:We used peristimulus time histograms (PSTHs) to estimate characteristics of the composite excitatory postsynaptic potentials (EPSPs) generated at the anterior horn cell by a descending cortical volley induced by subthreshold transcranial magnetic stimuli in 11 normal subjects, 9 patients with DNA-confirmed Kennedy's d...

journal_title：Muscle & nerve

authors： Weber M,Eisen A

更新日期：1999-03-01 00:00:00

abstract：:The distinction between chronic demyelinating polyneuropathies associated with IgM paraproteinemia and anti-myelin-associated glycoprotein (MAG) antibodies (MAG-PN) and chronic inflammatory demyelinating polyneuropathies (CIDPs) relies on the anti-MAG antibodies assay. The aim of the study was to identify clinical and...

journal_title：Muscle & nerve

authors： Isoardo G,Migliaretti G,Ciaramitaro P,Rota E,Poglio F,Tavella A,Paolasso I,Cavallo F,Bergamasco B,Cocito D

更新日期：2005-01-01 00:00:00