Assessing mNIS+7Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial.

abstract：INTRODUCTION:Duchenne muscular dystrophy (DMD) is characterized by absence of the subsarcolemmal protein dystrophin, present in skeletal muscles and cardiomyocytes. We hypothesized that progressive respiratory and left ventricular (LV) insufficiencies in DMD could be parallel and interrelated phenomena. METHODS:We con...

journal_title：Muscle & nerve

authors： Khokhar A,Nair A,Midya V,Kumar A,Sinharoy A,Ahmad TA,Abu-Hasan M,Mondal P

更新日期：2019-09-01 00:00:00

abstract：INTRODUCTION:Because paradoxical sleep deprivation (PSD) induces a catabolic hormone profile, we sought to evaluate the morphology of the tibialis anterior (TA) muscle and testosterone and corticosterone levels of paradoxical sleep-deprived rats. METHODS:Three study groups of rats were established: the first group was...

journal_title：Muscle & nerve

authors： Dattilo M,Antunes HK,Medeiros A,Mônico-Neto M,Souza Hde S,Lee KS,Tufik S,de Mello MT

更新日期：2012-03-01 00:00:00

abstract：:This longitudinal study of 194 patients with sporadic ALS demonstrated that it is possible for physicians to predict the approximate survival time for an individual ALS patient given: (1) the age of the patient, (2) the duration of his weakness and (3) an estimate of his clinical disability (ALS Score). This informati...

journal_title：Muscle & nerve

authors： Jablecki CK,Berry C,Leach J

更新日期：1989-10-01 00:00:00

abstract：:The clinical and neurophysiological features of multifocal motor neuropathy (MMN) indicate selective involvement of motor axons, but pathological abnormalities in sensory axons suggest a more widespread disease process. The present study was undertaken to determine whether the focal abnormalities are associated with w...

journal_title：Muscle & nerve

authors： Cappelen-Smith C,Kuwabara S,Lin CS,Burke D

更新日期：2002-12-01 00:00:00

abstract：BACKGROUND:In adult males, cross-sectional area (CSA) for type II muscle fibers is generally larger than for type I fibers. In this cross-sectional study the aim was to compare sex-related CSAs of various muscle fiber types during childhood-to-adulthood transition. METHODS:Percutaneous biopsy samples were obtained fro...

journal_title：Muscle & nerve

authors： Esbjörnsson ME,Dahlström MS,Gierup JW,Jansson EC

更新日期：2020-12-21 00:00:00

abstract：:The selection of patients who will benefit from peripheral nerve surgery continues to evolve. The evaluation of patients with peripheral nerve problems, and the indications and timing of surgery are reviewed. This includes patients with transections, lesions in continuity, entrapments, tumors, injection injuries, and ...

journal_title：Muscle & nerve

authors： Spinner RJ,Kline DG

更新日期：2000-05-01 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：:Muscle-derived neurotrophins are thought to contribute to the adaptation of skeletal muscle to exercise, but the effects of brief exercise interventions on BDNF, NT-4/5, and trkB are not understood. RNA was extracted for RT-PCR from soleus and medial gastrocnemius of Sprague-Dawley rats exercised on a treadmill at spe...

journal_title：Muscle & nerve

authors： Ogborn DI,Gardiner PF

更新日期：2010-03-01 00:00:00

abstract：:High-density multichannel electromyography (EMG) recordings add spatial information to the temporal information content of the surface EMG (sEMG) signal. This study explores the potential value of such multichannel information at a single motor unit level, in particular for the improvement of motor unit number estimat...

journal_title：Muscle & nerve

authors： Blok JH,Van Dijk JP,Zwarts MJ,Stegeman DF

更新日期：2005-09-01 00:00:00

abstract：INTRODUCTION:This study compares the acute and chronic response of high-load resistance training (HL) to low-load resistance training with low blood flow restriction (LL-BFR) pressure. METHODS:Participants completed elbow flexion with either HL or LL-BFR or nonexercise. In the chronic study, participants in the HL and...

journal_title：Muscle & nerve

authors： Kim D,Loenneke JP,Ye X,Bemben DA,Beck TW,Larson RD,Bemben MG

更新日期：2017-12-01 00:00:00

abstract：:We report an 18-month-old Charcot-Marie-Tooth type 1A (CMT1A) patient who developed a rapid-onset neuropathy, with proximal and distal weakness, and non-uniform nerve conduction studies. The neuropathy responded well to immunomodulation, confirming the coexistence of an inherited and an inflammatory neuropathy. Unexpe...

journal_title：Muscle & nerve

authors： Marques W Jr,Funayama CA,Secchin JB,Lourenço CM,Gouvêa SP,Marques VD,Bastos PG,Barreira AA

更新日期：2010-10-01 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：:To define numerically the clinical severity of facioscapulohumeral muscular dystrophy (FSHD), we developed a protocol that quantifies muscle weakness by combining the functional evaluation of six muscle groups affected in this disease. To validate reproducibility of the protocol, 69 patients were recruited. Each patie...

journal_title：Muscle & nerve

authors： Lamperti C,Fabbri G,Vercelli L,D'Amico R,Frusciante R,Bonifazi E,Fiorillo C,Borsato C,Cao M,Servida M,Greco F,Di Leo R,Volpi L,Manzoli C,Cudia P,Pastorello E,Ricciardi L,Siciliano G,Galluzzi G,Rodolico C,Santoro L

更新日期：2010-08-01 00:00:00

abstract：INTRODUCTION:Nebulin is a giant actin-binding protein in the thin filament of the skeletal muscle sarcomere. Studies of nebulin interactions are limited by the size, complexity, and poor solubility of the protein. We divided the nebulin super-repeat region into a super-repeat panel, and studied nebulin/actin interactio...

journal_title：Muscle & nerve

authors： Laitila J,Lehtonen J,Lehtokari VL,Sagath L,Wallgren-Pettersson C,Grönholm M,Pelin K

更新日期：2019-01-01 00:00:00

abstract：INTRODUCTION:Psoriasis is a T-cell-mediated skin disorder with uncommon extracutaneous manifestations. Rare patients with psoriasis and myopathy have been reported. METHODS:We conducted a retrospective review of medical records of psoriasis patients seen at the Mayo Clinic during the period from January 1, 1996 to May...

journal_title：Muscle & nerve

authors： Liewluck T,Ernste FC,Tracy JA

更新日期：2013-11-01 00:00:00

abstract：INTRODUCTION:We studied the functional effects of combined strength and aerobic anti-gravity training in severely affected patients with Becker and Limb-Girdle muscular dystrophies. METHODS:Eight patients performed 10-week progressive combined strength (squats, calf raises, lunges) and aerobic (walk/run, jogging in pl...

journal_title：Muscle & nerve

authors： Jensen BR,Berthelsen MP,Husu E,Christensen SB,Prahm KP,Vissing J

更新日期：2016-08-01 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：:The electrical characteristics of myotonia in skeletal muscle appear remarkably similar in a variety of natural disorders and animal models. Recent experimental work has made it clear, however, that the pathophysiologic mechanisms underlying these myotonic discharges can be quite different. A mechanistic classificatio...

journal_title：Muscle & nerve

authors： Barchi RL

更新日期：1982-01-01 00:00:00

abstract：BACKGROUND:Carpal tunnel syndrome (CTS) may be associated with structural lesions or anatomical variations at the wrist, especially in patients whose symptoms are more severe in, or limited to, the nondominant hand. The aims of this study were to identify the type and frequency of structural abnormalities and anatomica...

journal_title：Muscle & nerve

authors： Chompoopong P,Preston DC

更新日期：2020-12-21 00:00:00

abstract：:The loss of muscle strength with aging appears to be greater in the lower than upper limbs, but strength and its neural control have never been compared in the same population of individuals in both upper and lower limbs. The aim of this study was to investigate differences between eight young (20-31 years) and eight ...

journal_title：Muscle & nerve

authors： Bazzucchi I,Felici F,Macaluso A,De Vito G

更新日期：2004-11-01 00:00:00

abstract：INTRODUCTION:Detection of denervation in muscles in the craniobulbar area is important to assure widespread lower motor neuron involvement in the diagnosis of amyotrophic lateral sclerosis (ALS). The value of spontaneous activity analysis in needle electromyography (EMG) of the tongue has been questioned in the recent ...

journal_title：Muscle & nerve

authors： Tankisi H,Otto M,Pugdahl K,Fuglsang-Frederiksen A

更新日期：2013-08-01 00:00:00

abstract：:Platelet-derived microparticle (PDMP) levels were measured using an enzyme-linked immunosorbent assay (ELISA) to elucidate the role of platelet activation in patients with polymyositis or dermatomyositis (PM/DM). PDMP levels in active PM/DM patients (median 13.3 U/ml, interquartile range 9.9-20.7 U/ml, n = 16) and tho...

journal_title：Muscle & nerve

authors： Shirafuji T,Hamaguchi H,Higuchi M,Kanda F

更新日期：2009-05-01 00:00:00

abstract：INTRODUCTION:Mutations in the guanosine diphosphate-mannose pyrophosphorylase-B gene (GMPPB) have been identified in congenital muscular dystrophies, limb-girdle muscular dystrophy (LGMD2T), and congenital myasthenic syndromes (CMSs); overall, 41 patients have been described. METHODS:Two patients presented with a myas...

journal_title：Muscle & nerve

authors： Montagnese F,Klupp E,Karampinos DC,Biskup S,Gläser D,Kirschke JS,Schoser B

更新日期：2017-08-01 00:00:00

abstract：:We describe a patient with transthyretin amyloidosis who presented with multifocal mononeuropathies with features of demyelination on nerve conduction studies, a constellation of findings not previously described in amyloid polyneuropathy. Genetic testing revealed a valine122isoleucine mutation in the coding region of...

journal_title：Muscle & nerve

authors： Briemberg HR,Amato AA

更新日期：2004-02-01 00:00:00

abstract：INTRODUCTION:Contradictory reports of the myosin heavy chain (MHC) composition of adult human suprahyoid muscles leave unresolved the extent to which these muscles express developmental and unconventional MHC. METHODS:By immunohistochemistry, separation sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PA...

journal_title：Muscle & nerve

authors： Luo Q,Douglas M,Burkholder T,Sokoloff AJ

更新日期：2014-04-01 00:00:00

abstract：:The distribution of collagen types I, III, and V and fibronectin was investigated by means of immunofluorescent techniques in denervated and normal rat skeletal muscle. During a period of 28 days, a distinct atrophy developed in the denervated gastrocnemius muscle and was accompanied by an increase in types I and III ...

journal_title：Muscle & nerve

authors： Salonen V,Lehto M,Kalimo M,Penttinen R,Aro H

更新日期：1985-02-01 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：:Tarsal tunnel syndrome is relatively rare and the diagnosis may be difficult even though the etiologies for the syndrome are multiple. The symptoms are often vague but are usually burning pain and paresthesias in the toes and soles of the feet with nocturnal exacerbations. The physical examination may elicit a sensory...

journal_title：Muscle & nerve

authors： DeLisa JA,Saeed MA

更新日期：1983-11-01 00:00:00

abstract：INTRODUCTION:Peripheral nerve injuries (PNI) are among the leading causes of physical disability in the United States. The majority of injuries occur in the upper extremities, and functional recovery is often limited. Robust animal models are critical first steps for developing effective therapies to restore function a...

journal_title：Muscle & nerve

authors： Meyers EC,Granja R,Solorzano BR,Romero-Ortega M,Kilgard MP,Rennaker RL 2nd,Hays S

更新日期：2017-12-01 00:00:00

abstract：INTRODUCTION:Nemaline myopathy is a rare disorder characterized by skeletal muscle weakness of varying severity and onset, with the presence of nemaline rods on muscle biopsy. Congenital nemaline body myopathy due to mutations in TNNT1 has hitherto only been described as a result of a single founder mutation in patient...

journal_title：Muscle & nerve

authors： Abdulhaq UN,Daana M,Dor T,Fellig Y,Eylon S,Schuelke M,Shaag A,Elpeleg O,Edvardson S

更新日期：2016-04-01 00:00:00