Painful cramps and giant myotonic discharges in a family with the Nav1.4-G1306A mutation.

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：INTRODUCTION:Duchenne muscular dystrophy (DMD) is a degenerative disease of skeletal, respiratory, and cardiac muscles caused by defects in the dystrophin gene. More recently, brain involvement has been verified. Mitochondrial dysfunction and oxidative stress may underlie the pathophysiology of DMD. In this study we ev...

journal_title：Muscle & nerve

authors： Comim CM,Hoepers A,Ventura L,Freiberger V,Dominguini D,Mina F,Mendonça BP,Scaini G,Vainzof M,Streck EL,Quevedo J

更新日期：2016-01-01 00:00:00

abstract：:When an active muscle is stretched, the force increases due to strain of contractile and noncontractile proteins. We examined this force enhancement in rat extensor digitorum longus (EDL) and soleus muscles, which differ in their composition of these proteins, and their susceptibility to damage. Small stretches were a...

journal_title：Muscle & nerve

authors： Ramsey KA,Bakker AJ,Pinniger GJ

更新日期：2010-11-01 00:00:00

abstract：INTRODUCTION:The aim of this work was to determine the effect of chronic alcohol exposure on peripheral nerves in a nutritionally balanced rat model of alcoholism. METHODS:Three different strains of adult male rats were pair-fed for 8 weeks with isocaloric liquid diets containing 0% or 37% ethanol. Nerve conduction st...

journal_title：Muscle & nerve

authors： Mellion ML,Nguyen V,Tong M,Gilchrist J,De La Monte S

更新日期：2013-08-01 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:Neutral lipid-storage disease with myopathy is caused by mutations in PNPLA2, which produce skeletal and cardiac myopathy. We report a man with multiorgan neutral lipid storage and unusual multisystem clinical involvement, including cognitive impairment. METHODS:Quantitative brain MRI with voxel-based mor...

journal_title：Muscle & nerve

authors： Massa R,Pozzessere S,Rastelli E,Serra L,Terracciano C,Gibellini M,Bozzali M,Arca M

更新日期：2016-04-01 00:00:00

abstract：:If anti-GM1 antibody plays a role in the axonal damage in Guillain-Barré syndrome, the common entrapment sites may be preferentially involved with evidence of axonal dysfunction. To assess this hypothesis, we studied nerve conduction across the cubital tunnel in 44 patients. Abnormal amplitude reduction of compound mu...

journal_title：Muscle & nerve

authors： Kuwabara S,Mori M,Ogawara K,Mizobuchi K,Hattori T,Koga M,Yuki N

更新日期：1999-07-01 00:00:00

abstract：:We describe a consanguineous family that had progressive myopathy with rimmed vacuole (RV) formation and amyloid deposition. Patient 1 is a 71-year-old woman with muscle atrophy in the lumbar girdle and lower extremities. Patient 2 is a 40-year-old man (the son of Patient 1) with fatty changes in the biceps femoris mu...

journal_title：Muscle & nerve

authors： Kojima Y,Sakai K,Ishida C,Asaka T,Hamaguchi T,Nozaki I,Fukushima K,Tsuchiya A,Kametani F,Yazaki M,Okino S,Yamada M

更新日期：2009-09-01 00:00:00

abstract：:This study compared the force output produced by variable frequency, short-duration trains (VFTs) of electrical pulses with the forces produced by constant frequency, short-duration trains (CFTs). Human quadriceps femoris muscle was stimulated with a 300-msec train of pulses once every second for 180 seconds. Each sub...

journal_title：Muscle & nerve

authors： Binder-Macleod SA,Barker CB 3rd

更新日期：1991-09-01 00:00:00

abstract：:A family is described in which five males have late-onset facial weakness, dysarthria, dysphagia, and slowly progressive proximal weakness. Electrodiagnostic studies and muscle biopsy were compatible with spinal muscular atrophy. This family appears quite similar to several previously reported families with late-onset...

journal_title：Muscle & nerve

authors： Ringel SP,Lava NS,Treihaft MM,Lubs ML,Lubs HA

更新日期：1978-07-01 00:00:00

abstract：:Recent years have seen steady progress in the identification of genetic muscle diseases as well as efforts to develop treatment for these diseases. Consequently, sensitive and objective new methods are required to identify and monitor muscle pathology. Magnetic resonance imaging offers multiple potential biomarkers of...

journal_title：Muscle & nerve

authors： Leung DG

更新日期：2019-10-01 00:00:00

abstract：BACKGROUND:Despite use of qualitative laryngeal electromyography (LEMG) guided botulinum toxin A (BoNT-A) injection for treatment of adductor spasmodic dysphonia (AdSD), unsatisfactory injections and complete "misses" remain problematic. We aimed to determine if the quantitative LEMG measure of number of small segments...

journal_title：Muscle & nerve

authors： Dwyer CD,Leclerc AA,Nandedkar SD,Young VN,Rosen CA

更新日期：2020-12-31 00:00:00

abstract：:To study noninvasively multiple descending volleys on alpha motoneurons, we used a 30-50% maximum H-reflex in the wrist flexor muscle conditioned by a subthreshold magnetic stimulus of the contralateral motor cortex as a parameter of motoneuronal excitability in seven neurologically healthy subjects. The time interval...

journal_title：Muscle & nerve

authors： van der Linden C,Bruggeman R

更新日期：1993-04-01 00:00:00

abstract：:Type 1 diabetes mellitus (T1DM) is associated with a peripheral neuropathy that reduces nerve conduction velocity. This may impair high motor-unit discharge frequencies (MUDF), decrease muscle activation, and curtail the ability to sustain repetitive contractile tasks. We examined (1) whether MUDF, the contractile pro...

journal_title：Muscle & nerve

authors： Almeida S,Riddell MC,Cafarelli E

更新日期：2008-02-01 00:00:00

abstract：:Electrophysiologic detection of the first signs of gastrocnemius muscle reinnervation shows that after a single localized freezing of the rat sciatic nerve, isaxonine does not significantly increase the rate of axonal regeneration. However, this drug does significantly enhance axonal sprouting, as it induces an increa...

journal_title：Muscle & nerve

authors： Pecot-Dechavassine M,Mira JC

更新日期：1985-02-01 00:00:00

abstract：:Electrodiagnostic findings of fibrillations and positive sharp waves in the lumbosacral paraspinals in patients without previous back surgery has been generally considered to be abnormal, consistent with posterior rami denervation. In some cases, it is the only abnormality on the electromyographic examination. This st...

journal_title：Muscle & nerve

authors： Date ES,Mar EY,Bugola MR,Teraoka JK

更新日期：1996-03-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is a debilitating neurodegenerative disorder with complex biology and significant clinical heterogeneity. Many preclinical and early phase ALS clinical trials have yielded promising results that could not be replicated in larger phase 3 confirmatory trials. One reason for the lack o...

journal_title：Muscle & nerve

authors： Goyal NA,Berry JD,Windebank A,Staff NP,Maragakis NJ,van den Berg LH,Genge A,Miller R,Baloh RH,Kern R,Gothelf Y,Lebovits C,Cudkowicz M

更新日期：2020-08-01 00:00:00

abstract：:We prospectively evaluated thalidomide-induced neuropathy using electrodiagnostic studies. Sixty-seven men with metastatic androgen-independent prostate cancer in an open-label trial of oral thalidomide underwent neurologic examinations and nerve conduction studies (NCS) prior to and at 3-month intervals during treatm...

journal_title：Muscle & nerve

authors： Molloy FM,Floeter MK,Syed NA,Sandbrink F,Culcea E,Steinberg SM,Dahut W,Pluda J,Kruger EA,Reed E,Figg WD

更新日期：2001-08-01 00:00:00

abstract：:Patients with prior poliomyelitis may experience muscle function deterioration decades after onset of disease. The present study is aimed at describing electromyographic and morphometric evidence of muscular compensation and of on-going muscular instability. Ten subjects 42-62 years of age with onset of polio 25-52 ye...

journal_title：Muscle & nerve

authors： Einarsson G,Grimby G,Stålberg E

更新日期：1990-02-01 00:00:00

abstract：:Mutations in the sarcoglycan (SG) genes cause a subset of limb-girdle muscular dystrophies (LGMD). We report a Spanish patient with progressive LGMD exhibiting an almost isolated loss of gamma-SG and a homozygous Delta521-T mutation in the gamma-SG gene. These results suggest that isolated loss of gamma-SG might remai...

journal_title：Muscle & nerve

authors： Vorgerd M,Gencik M,Mortier J,Epplen JT,Malin JP,Mortier W

更新日期：2001-03-01 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：:Muscle biopsy and computerized tomography indicated that unilateral enlargement of the gastrocnemius muscle in a 12-year-old girl was the result of an infiltrating lipoma. This rare condition may cause diagnostic confusion before EMG and muscle biopsy studies are performed, but it can be readily distinguished from oth...

journal_title：Muscle & nerve

authors： Fetell MR,Duffy PE,Rowland LP

更新日期：1978-01-01 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：: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：:In median nerve lesions of the hand, electrophysiologic evaluation of the second lumbrical muscle can help to differentiate between lesions of the common digital nerves and the proper digital nerves, as illustrated by this case report. Median nerve conduction studies to the second lumbrical muscle can be performed by ...

journal_title：Muscle & nerve

authors： Busis NA,Logigian EL,Shahani BT

更新日期：1986-03-01 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：: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：:C-protein isoform expression in hereditary dystrophic chicken skeletal muscle was compared with that in normal chicken muscle during postnatal development by immunocytochemical and immunoblot methods. In the pectoralis muscle (PM) of both normal and dystrophic chicken, slow- and fast-type C-proteins were coexpressed i...

journal_title：Muscle & nerve

authors： Obinata T,Shinbo K

更新日期：1987-05-01 00:00:00

abstract：:Variable-frequency stimulation trains (VFTs) that take advantage of the catchlike property of skeletal muscle have been shown to augment the force production of fatigued muscles compared with constant-frequency trains (CFTs). The present study is the first to report the force augmentation produced by VFTs after fatigu...

journal_title：Muscle & nerve

authors： Binder-Macleod SA,Lee SC,Russ DW,Kucharski LJ

更新日期：1998-09-01 00:00:00

abstract：:Involuntary muscle contractions are common after spinal cord injury (SCI). Increased sensitivity to Ia muscle afferent input may contribute to the development of these spasms. Since tendon vibration results in a period of postactivation depression of the Ia synapse, we sought to determine whether Achilles tendon vibra...

journal_title：Muscle & nerve

authors： Butler JE,Godfrey S,Thomas CK

更新日期：2006-05-01 00:00:00