Enzyme replacement therapy in adult-onset glycogenosis II: is quantitative muscle MRI helpful?

abstract：:Weakness is common in both hyper- and hypothyroidism, and skeletal muscle L-carnitine may play a role in this regard, as suggested by studies indicating abnormal levels of carnitine in serum and urine of patients with thyroid dysfunction. Skeletal muscle samples were obtained for carnitine analysis from control subjec...

journal_title：Muscle & nerve

authors： Sinclair C,Gilchrist JM,Hennessey JV,Kandula M

更新日期：2005-09-01 00:00:00

abstract：INTRODUCTION:We studied neurodevelopmental and behavioral/emotional symptoms in patients with Duchenne muscular dystrophy (DMD). METHODS:Retrospective case series of neurodevelopmental and behavioral/emotional symptoms obtained through review of systems of 700 DMD patients in relation to dystrophin gene mutations. RE...

journal_title：Muscle & nerve

authors： Darmahkasih AJ,Rybalsky I,Tian C,Shellenbarger KC,Horn PS,Lambert JT,Wong BL

更新日期：2020-04-01 00:00:00

abstract：:Injection of low-dose arachidonic acid into the rat femoral artery occludes the vasa nervorum of the tibial nerve and produces focal and generalized ischemia with transient effects on nerve conduction. Across a severely ischemic segment of the proximal tibial nerve there is a marked fall in amplitude of the compound m...

journal_title：Muscle & nerve

authors： Parry GJ,Linn DJ

更新日期：1986-05-01 00:00:00

abstract：:Sialorrhea is frequently a socially disabling symptom in patients with bulbar amyotrophic lateral sclerosis (ALS). In this open-label prospective study, we report the effect of botulinum toxin A (Botox) injection into the parotid glands in 10 patients with bulbar ALS and socially disabling sialorrhea. We applied three...

journal_title：Muscle & nerve

authors： Verma A,Steele J

更新日期：2006-08-01 00:00:00

abstract：:There is a paucity of validated tests to quantify hand function impairment due to carpal tunnel syndrome (CTS). The aim of this study was to test the validity and reliability of the Purdue Pegboard Test (PPT) in CTS patients. We compared 190 CTS patients with 122 healthy, age-matched controls. CTS severity was determi...

journal_title：Muscle & nerve

authors： Amirjani N,Ashworth NL,Olson JL,Morhart M,Chan KM

更新日期：2011-02-01 00:00:00

abstract：:To investigate whether transcranial magnetic stimulation (TMS) has an effect on isometric muscle force elicited by maximal voluntary contractions (MVC) and the ability to activate a muscle voluntarily (VA, as a percentage of full muscle activation), a twitch-interpolation technique was applied on the quadriceps femori...

journal_title：Muscle & nerve

authors： Urbach D,Awiszus F

更新日期：2000-07-01 00:00:00

abstract：:Recurrent idiopathic lumbosacral plexopathy has rarely been reported in the literature. The present report describes a 59-year-old man presenting with recurrent episodes of acute leg pain, followed by weakness. After each episode, symptoms progressed for several months before peaking. Thereafter, gradual recovery ensu...

journal_title：Muscle & nerve

authors： Yee T

更新日期：2000-09-01 00:00:00

abstract：INTRODUCTION:In this study we present a reproducible technique to assess motor recovery after nerve injury via neuromuscular junction (NMJ) immunostaining and electrodiagnostic testing. METHODS:Wild-type mice underwent sciatic nerve transection with repair. Hindlimb muscles were collected for microscopy up to 30 weeks...

journal_title：Muscle & nerve

authors： Vannucci B,Santosa KB,Keane AM,Jablonka-Shariff A,Lu CY,Yan Y,MacEwan M,Snyder-Warwick AK

更新日期：2019-11-01 00:00:00

abstract：INTRODUCTION:The morphological characteristics of skeletal muscles innervated caudal to a spinal cord injury (SCI) undergo dramatic phenotypic and microvascular changes. METHOD:Female Sprague-Dawley rats received a severe contusion at thoracic level 9/10 and were randomly assigned to locomotor training, epidural stimu...

journal_title：Muscle & nerve

authors： Kissane RWP,Wright O,Al'Joboori YD,Marczak P,Ichiyama RM,Egginton S

更新日期：2019-03-01 00:00:00

abstract：:To determine the role of personal variables as risk factors for carpal tunnel syndrome (CTS) and their relationship to severity of nerve conduction abnormality, we studied 210 consecutive symptomatic CTS patients and 320 controls subjects without CTS symptomatology or known systemic disorders. The CTS group was classi...

journal_title：Muscle & nerve

authors： Kouyoumdjian JA,Zanetta DM,Morita MP

更新日期：2002-01-01 00:00:00

abstract：:Motor unit (MU) number and size estimates were obtained from the human flexor digiti minimi muscle using histological methods. Ten adult fresh cadavers (33-74 years old) were used. The number of MUs was 130 +/- 15 and the MU size was 108 +/- 10. These values are similar to those reported for other hypothenar muscles, ...

journal_title：Muscle & nerve

authors： Santo Neto H,de Carvalho VC,Marques MJ

更新日期：1998-01-01 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：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：INTRODUCTION:In this study we aimed to clarify whether muscle ultrasound (US) of the forearm can be used to differentiate between patients with sporadic inclusion body myositis (s-IBM) and those with s-IBM-mimicking diseases. METHODS:We compared the echo intensity (EI) of the flexor digitorum profundus (FDP) muscle an...

journal_title：Muscle & nerve

authors： Noto Y,Shiga K,Tsuji Y,Kondo M,Tokuda T,Mizuno T,Nakagawa M

更新日期：2014-05-01 00:00:00

abstract：:We report the case of a 55-year-old man with non-small-cell lung cancer who underwent radiation, chemotherapy with carbotaxol and paclitaxel, and left upper lobe removal 2 years prior to evaluation. He was referred for disabling orthostatic hypotension (113/69 mm Hg supine and 66/47 mm Hg standing after 10 minutes) wi...

journal_title：Muscle & nerve

authors： Peltier AC,Black BK,Raj SR,Donofrio P,Robertson D,Biaggioni I

更新日期：2010-03-01 00:00:00

abstract：:Changes in contractile rate of force development (RFD), measured within a short time interval from contraction initiation, were measured after a period of strength training that led to increases in muscle fascicle length but no measurable change in neuromuscular activity. The relationship between training-induced shif...

journal_title：Muscle & nerve

authors： Blazevich AJ,Cannavan D,Horne S,Coleman DR,Aagaard P

更新日期：2009-04-01 00:00:00

abstract：INTRODUCTION:Our aim in this study was to determine whether intravenous immunoglobulin (IVIg) or plasma exchange (PLEx) for treatment of neurologic disease is a trigger for thrombotic events. METHODS:Using administrative data from 2005 to 2014, we identified index admissions for thrombotic events. We performed case-cr...

journal_title：Muscle & nerve

authors： Jin PH,Shin SC,Dhamoon MS

更新日期：2020-09-01 00:00:00

abstract：INTRODUCTION:Muscle changes in patients with diabetes and lower leg pain due to chronic exertional compartment syndrome (CECS) were investigated before and after fasciotomy. METHODS:The tibialis anterior muscle was analyzed with histochemical and morphological techniques in 7 patients with diabetes and CECS before fas...

journal_title：Muscle & nerve

authors： Edmundsson DS,Toolanen GL,Stål PS

更新日期：2018-02-01 00:00:00

abstract：:We investigated the influence of electrical stimulation of the posterior cruciate ligament (PCL) on the motoneuron pool of the thigh muscle during voluntary static and dynamic muscle contraction. The study group comprised nine young men with no history of injury to the knee joints. Multistranded Teflon-insulated stain...

journal_title：Muscle & nerve

authors： Fischer-Rasmussen T,Krogsgaard M,Jensen DB,Dyhre-Poulsen P

更新日期：2001-11-01 00:00:00

abstract：:Two sisters presented with distal weakness and their muscle biopsy was dystrophic. This distal muscular dystrophy has an autosomal recessive inheritance and its features are somewhat different from the more common autosomal dominant distal muscular dystrophy and include: a) onset in early adult life; b) involvement of...

journal_title：Muscle & nerve

authors： Scoppetta C,Vaccario ML,Casali C,Di Trapani G,Mennuni G

更新日期：1984-07-01 00:00:00

abstract：:Using poststimulus time histograms, it has been reported that stimulation of the median nerve at the elbow produces a monosynaptic EPSP in voluntarily active single motoneurons of the human biceps brachii. The present study was undertaken to: (i) determine whether such stimulation could evoke a reproducible reflex res...

journal_title：Muscle & nerve

authors： Miller TA,Mogyoros I,Burke D

更新日期：1995-06-01 00:00:00

abstract：:The consequences of general hypoxemia (PaO2 = 51 mmHg) on two muscle groups (adductor pollicis and diaphragm) sustaining 80% maximal isometric voluntary contraction were studied in healthy individuals. For adductor pollicis, contractions were also executed after 10-s or 3-min rest ischemia. Compared to control, i.e., ...

journal_title：Muscle & nerve

authors： Badier M,Guillot C,Lagier-Tessonnier F,Jammes Y

更新日期：1994-05-01 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：: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：:We prospectively studied the diagnostic utility of upper limb segmental reflexes in patients with suspected cervical radiculopathy (CR). Fifty-three patients (29 men and 24 women), referred for electrodiagnostic testing, were positive for at least one of four clinical criteria for CR: abnormal (1) history, (2) motor (...

journal_title：Muscle & nerve

authors： Miller TA,Pardo R,Yaworski R

更新日期：1999-08-01 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：INTRODUCTION:Paraneoplastic lower motor neuronopathies have been reported rarely with Hodgkin lymphoma. METHODS:We report a case of rapidly progressive motor neuronopathy preceding the diagnosis of Hodgkin lymphoma. RESULTS:A 31-year-old woman developed subacute rapidly progressive quadriparesis. Electrodiagnostic st...

journal_title：Muscle & nerve

authors： Flanagan EP,Sandroni P,Pittock SJ,Inwards DJ,Jones LK Jr

更新日期：2012-11-01 00:00:00

abstract：INTRODUCTION:The minimal clinically important difference (MCID) is the smallest outcome change that has clinical significance. Its use has not been established in the study of myasthenia gravis (MG). METHODS:Patients from a published intravenous immunoglobulin (IVIg) vs. placebo study were studied. One anchor-based an...

journal_title：Muscle & nerve

authors： Katzberg HD,Barnett C,Merkies IS,Bril V

更新日期：2014-05-01 00:00:00

abstract：:Myasthenia gravis (MG) and its animal model experimental autoimmune myasthenia gravis (EAMG) are caused by autoantibodies against nicotinic acetylcholine receptor (AChR) in skeletal muscle. The production of anti-AChR antibodies is mediated by cytokines produced by CD4+ and CD8+ T helper (Th) cells. Emerging investiga...

journal_title：Muscle & nerve

authors： Zhang GX,Navikas V,Link H

更新日期：1997-05-01 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