Dual-energy X-ray absorptiometry in neuromuscular diseases.

abstract：:To clarify the molecular nature of the pathogenesis in X-linked Emery-Dreifuss muscular dystrophy (EDMD), we monitored the expression of 2400 genes in control and EDMD fibroblasts by using complementary DNA (cDNA) microarray techniques. A total of 60 genes whose expression was altered in EDMD fibroblasts when compared...

journal_title：Muscle & nerve

authors： Tsukahara T,Tsujino S,Arahata K

更新日期：2002-06-01 00:00:00

abstract：INTRODUCTION:Hereditary transthyretin (hATTR) amyloidosis is a progressive, degenerative disease, with peripheral neuropathy, cardiomyopathy, and other clinical manifestations. In this study we examine the impact of hATTR amyloidosis on quality of life (QOL). METHODS:Neuropathy-specific QOL, measured with the Norfolk ...

journal_title：Muscle & nerve

authors： Yarlas A,Gertz MA,Dasgupta NR,Obici L,Pollock M,Ackermann EJ,Lovley A,Kessler AS,Patel PA,White MK,Guthrie SD

更新日期：2019-08-01 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：:The lack of effective therapy for many of the chronic neuromuscular diseases such as amyotrophic lateral sclerosis, hereditary motor sensory neuropathy (Charcot-Marie-Tooth disease), spinocerebellar degenerations and idiopathic polyneuropathy has led to a search for substances that may stimulate peripheral nerve regen...

journal_title：Muscle & nerve

authors： Bradley WG

更新日期：1990-09-01 00:00:00

abstract：INTRODUCTION:The distribution of clinical and neurophysiological abnormalities in patients with early amyotrophic lateral sclerosis (ALS) was investigated in an attempt to delineate patterns of disease spread. METHODS:Clinical and electrodiagnostic data were collected from 150 ALS patients and analyzed based on the cl...

journal_title：Muscle & nerve

authors： Simon NG,Lomen-Hoerth C,Kiernan MC

更新日期：2014-12-01 00:00:00

abstract：INTRODUCTION:Skeletal muscle oxidative capacity decreases and fatigability increases after spinal cord injury. Transcription factor peroxisome proliferator-activated receptor δ (PPARδ) promotes a more oxidative phenotype. METHODS:We asked whether PPARδ overexpression could ameliorate these deficits in the medial gastr...

journal_title：Muscle & nerve

authors： Kim JA,Roy RR,Zhong H,Alaynick WA,Embler E,Jang C,Gomez G,Sonoda T,Evans RM,Edgerton VR

更新日期：2016-02-01 00:00:00

abstract：:Technical and instrumentation factors play an important role in obtaining reliable information during electrodiagnostic studies. With contemporary electrodiagnostic equipment, neurophysiologic potentials are detected using a variety of electrodes and undergo differential amplification, filtering, conversion to digital...

journal_title：Muscle & nerve

authors： Gitter AJ,Stolov WC

更新日期：1995-08-01 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：:We previously reviewed the presentation, initial clinical course, and electrodiagnostic features of children with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We now report the long-term follow-up of 12 children with idiopathic CIDP, and compare these to 62 adults with idiopathic CIDP. Children of...

journal_title：Muscle & nerve

authors： Simmons Z,Wald JJ,Albers JW

更新日期：1997-12-01 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：:Genetic testing is rapidly becoming an increasingly significant part of the diagnostic armamentarium of neuromuscular clinicians. Although technically easy to order, the results of such testing, whether positive or negative, have potentially enormous consequences for the individual tested and for family members. As a ...

journal_title：Muscle & nerve

authors： Su X,Kang PB,Russell JA,Simmons Z

更新日期：2016-12-01 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：:Amyotrophic lateral sclerosis (ALS) is a fatal disease with limited treatment options. Controlled studies are a necessary part of Phase II and Phase III assessments of proposed therapies. Due to the relatively small number of patients with ALS, several study designs have been proposed to improve the efficiency of Phas...

journal_title：Muscle & nerve

authors： Simmons Z

更新日期：2009-06-01 00:00:00

abstract：:Hereditary neuropathy with liability to pressure palsy (HNPP) is typified as isolated nerve palsies caused by trivial compression or trauma. It rarely presents in two extremities and even more infrequently affects all four limbs simultaneously. We present a patient who concurrently experienced right shoulder, left han...

journal_title：Muscle & nerve

authors： Crum BA,Sorenson EJ,Abad GA,Dyck PJ

更新日期：2000-06-01 00:00:00

abstract：:The aim of this investigation was to define the boundaries of compound motor action potential (CMAP) dispersion, amplitude decay, and area decay in a control population and determine their dependence on external variables such as age and interelectrode distance. Measurements were made from median, ulnar, and common pe...

journal_title：Muscle & nerve

authors： Taylor PK

更新日期：1993-11-01 00:00:00

abstract：INTRODUCTION:Accuracy of needle electromyography is typically ensured by use of anatomical landmarks and auditory feedback related to voluntary activation of the targeted muscle; however, in certain clinical situations, landmarks may not be palpable, auditory feedback may be limited or not present, and targeting a spec...

journal_title：Muscle & nerve

authors： Boon AJ,Oney-Marlow TM,Murthy NS,Harper CM,McNamara TR,Smith J

更新日期：2011-07-01 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：:Ultrasound (US) technique was applied to measure the thickness, cross-sectional area (CSA), and internal structure of the quadriceps muscle in 70- to 80-year-old endurance and power athletes and untrained men. Isometric knee extension strength was higher in the power athletes than in the other groups. The mean values ...

journal_title：Muscle & nerve

authors： Sipilä S,Suominen H

更新日期：1991-06-01 00:00:00

abstract：:We present the case of a 37-year-old Afghani man with a history of childhood diphtheria, who was diagnosed with bilateral vocal cord paralysis at age 15 years. At about this time he developed progressive muscular wasting and distally predominant weakness, and subsequently developed respiratory insufficiency, necessita...

journal_title：Muscle & nerve

authors： Reichler BD,Scelsa SN,Simpson DM

更新日期：2000-01-01 00:00:00

abstract：INTRODUCTION:Polyneuropathy signs (Neuropathy Impairment Score, NIS), neurophysiologic tests (m+7Ionis ), disability, and health scores were assessed in baseline evaluations of 100 patients entered into an oligonucleotide familial amyloidotic polyneuropathy (FAP) trial. METHODS:We assessed: (1) Proficiency of grading ...

journal_title：Muscle & nerve

authors： Dyck PJ,Kincaid JC,Dyck PJB,Chaudhry V,Goyal NA,Alves C,Salhi H,Wiesman JF,Labeyrie C,Robinson-Papp J,Cardoso M,Laura M,Ruzhansky K,Cortese A,Brannagan TH 3rd,Khoury J,Khella S,Waddington-Cruz M,Ferreira J,Wang AK,

更新日期：2017-11-01 00:00:00

abstract：:We have identified a novel missense mutation in the myophosphorylase gene in a Spanish patient with McArdle's disease. The patient was homozygous for a T-to-C transition at codon 115 (L115P) in exon 3, which changed an encoded leucine (CUG) to a proline (CCG). This is the first mutation to be described in exon 3 and i...

journal_title：Muscle & nerve

authors： Gamez J,Fernandez R,Bruno C,Andreu AL,Cervera C,Navarro C,Schwartz S,Dimauro S

更新日期：1999-08-01 00:00:00

abstract：INTRODUCTION:Immune-mediated myopathies are a heterogeneous group of chronic autoimmune disorders. Autoantibodies associated with this disease complex are classified into myositis-associated and myositis-specific. Anti-tRNA synthetase antibodies are the most well known of the myositis-specific antibodies. Previous repo...

journal_title：Muscle & nerve

authors： Mehndiratta P,Mehta S,Manjila SV,Kammer GM,Cohen ML,Preston DC

更新日期：2012-08-01 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：:EDL muscles from normal and dystrophic (dy2j) mice of various ages were examined. Muscles were divided into three groups according to age: 7 to 14 days postnatal, 16 to 21 days postnatal, and 6 months old, to assess age and/or phenotype related differences in the muscle response to caffeine or high K+. The response of...

journal_title：Muscle & nerve

authors： Dangain J,Neering IR

更新日期：1993-01-01 00:00:00

abstract：:It is important to know the effects of prolonged repetitive nerve stimulation (RNS) when it is used in neurophysiological studies. RNS with up to 100 supramaximal stimuli was given to the median, ulnar, and peroneal nerves of normal subjects and the ulnar nerves of subjects with early amyotrophic lateral sclerosis (AL...

journal_title：Muscle & nerve

authors： Baumann F,Henderson RD,Tremayne F,Hutchinson N,McCombe PA

更新日期：2010-06-01 00:00:00

abstract：INTRODUCTION:Meralgia paresthetica is a common clinical complaint for which some patients ultimately undergo surgical treatment. The lateral femoral cutaneous nerve (LFCN) has been difficult to reliably test electrophysiologically, likely due to anatomic variability and lack of responses in asymptomatic obese subjects....

journal_title：Muscle & nerve

authors： Boon AJ,Bailey PW,Smith J,Sorenson EJ,Harper CM,Hurdle MF

更新日期：2011-10-01 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：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：:The pattern of an abnormal median-normal sural (AMNS) sensory response is associated with acute and chronic inflammatory demyelinating polyradiculoneuropathy (AIDP and CIDP) and considered unusual in other types of neuropathy, although specificity and sensitivity of this pattern have not been evaluated. We compared se...

journal_title：Muscle & nerve

authors： Bromberg MB,Albers JW

更新日期：1993-03-01 00:00:00

abstract：BACKGROUND:A new technique to obtain the sensory nerve action potential (SNAP) of the medial femoral cutaneous nerve is described. METHODS:SNAPs were recorded from 104 subjects with a bar recording electrode 10 cm proximal to the patella along an imaginary line drawn between the medial edge of the patella and the femo...

journal_title：Muscle & nerve

authors： Geney-Castro DE,Vanegas-Muñóz J,Plata-Contreras J,Salinas-Duran F

更新日期：2020-03-01 00:00:00