Chorea-acanthocytosis presenting as motor neuron disease.

abstract：:Ion channels are complex proteins that span the lipid bilayer of the cell membrane, where they orchestrate the electrical signals necessary for normal function of the central nervous system, peripheral nerve, and both skeletal and cardiac muscle. The role of ion channel defects in the pathogenesis of numerous disorder...

journal_title：Muscle & nerve

authors： Kleopa KA,Barchi RL

更新日期：2002-09-01 00:00:00

abstract：INTRODUCTION:Livedoid vasculopathy is a rare dermatological condition characterized by painful ulceration, atrophic scarring, and persistent livedo reticularis. The pathogenesis is unclear. METHODS:We report a patient with biopsy-proven livedoid vasculopathy who developed a progressive sensory ganglionopathy with prof...

journal_title：Muscle & nerve

authors： Alix JJ,Hadjivassiliou M,Ali R,Slater D,Messenger AG,Rao DG

更新日期：2015-02-01 00:00:00

abstract：:The latency fluctuation of single motor unit potentials (MUPH) in the H-reflex is greater than the latency fluctuation of MUPs in the direct (MUPM) and recurrent (MUPF) responses. This has been attributed to the variability in the impulse generation at the site of nerve stimulation, and to the variation in the synapti...

journal_title：Muscle & nerve

authors： Soliven B,Maselli RA

更新日期：1992-06-01 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：UNLABELLED:Contemporary natural history data in Duchenne muscular dystrophy (DMD) is needed to assess care recommendations and aid in planning future trials. METHODS:The Cooperative International Neuromuscular Research Group (CINRG) DMD Natural History Study (DMD-NHS) enrolled 340 individuals, aged 2-28 years, with DM...

journal_title：Muscle & nerve

authors： McDonald CM,Henricson EK,Abresch RT,Han JJ,Escolar DM,Florence JM,Duong T,Arrieta A,Clemens PR,Hoffman EP,Cnaan A,Cinrg Investigators.

更新日期：2013-07-01 00:00:00

abstract：:The purpose of this study was to determine whether initial muscle length influenced the extent of isometric force deficit following 20 in vitro lengthening contractions of the soleus muscle from Fischer 344 rats. Force deficit was evaluated following one of five protocols: (1) lengthening contractions from optimal mus...

journal_title：Muscle & nerve

authors： Gosselin LE,Burton H

更新日期：2002-06-01 00:00:00

abstract：:Present studies evaluated the functions of thick and thin nerve fibers in carpal tunnel syndrome (CTS) to determine whether data of thin nerve fibers can add further information to the diagnosis of CTS. Measurements were performed in 22 patients and 16 age-matched control subjects. Motor and sensory nerve conduction, ...

journal_title：Muscle & nerve

authors： Lang E,Spitzer A,Pfannmüller D,Claus D,Handwerker HO,Neundörfer B

更新日期：1995-02-01 00:00:00

abstract：:L1 radiculopathy is very rare and difficult to diagnose with needle electromyography. A patient presented with pain and hypesthesia on the anterolateral aspect of the left thigh. Nerve conduction studies and needle electromyography were normal, except for the quadratus lumborum and iliopsoas muscles, which showed abno...

journal_title：Muscle & nerve

authors： Yang SN,Kim DH

更新日期：2010-04-01 00:00:00

abstract：:Immersion of rat hemidiaphragms in Ca2+-free Krebs solution (KS) containing Ca2+ chelator in vitro leads to separation of basal lamina from the plasma membrane, as well as transient contracture and rapid loss of twitch response [calcium paradox (CP) phase 1]. Subsequent immersion in regular KS results in necrosis of m...

journal_title：Muscle & nerve

authors： Soza M,Karpati G,Carpenter S

更新日期：1986-03-01 00:00:00

abstract：INTRODUCTION:Skin-derived precursor cells (SKPs) are neural crest progenitor cells that can attain a Schwann cell-like phenotype through in vitro techniques (SKP-SCs). We hypothesized that SKP-SCs could produce mature myelin and, in doing so, facilitate the recovery of a focal demyelination injury. METHODS:We unilater...

journal_title：Muscle & nerve

authors： Grochmal J,Dhaliwal S,Stys PK,van Minnen J,Midha R

更新日期：2014-08-01 00:00:00

abstract：INTRODUCTION:Antimitochondrial antibodies are autoantibodies detected in 90% of primary biliary cirrhosis (PBC) patients. Some PBC cases are complicated by myositis, which is difficult to confirm due to minimal histological evidence of inflammation in limb muscles. METHODS:Our aim was to determine the extent of inflam...

journal_title：Muscle & nerve

authors： Uenaka T,Kowa H,Sekiguchi K,Nagata K,Ohtsuka Y,Kanda F,Toda T

更新日期：2013-05-01 00:00:00

abstract：:We report electrophysiological findings of conduction along peripheral sensory fibers in 64 patients with amyotrophic lateral sclerosis. Distribution of the values of action potential amplitudes and conduction velocities of peripheral afferent fibers were significantly lower than in normal age-matched controls. Sensor...

journal_title：Muscle & nerve

authors： Mondelli M,Rossi A,Passero S,Guazzi GC

更新日期：1993-02-01 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：:Doxorubicin is a chemotherapeutic agent prescribed for a variety of tumors. While undergoing treatment, patients exhibit frequent symptoms that suggest respiratory muscle weakness. Cancer patients can receive doxorubicin chemotherapy through either intravenous (IV) or intraperitoneal (IP) injections. We hypothesized t...

journal_title：Muscle & nerve

authors： Gilliam LA,Moylan JS,Callahan LA,Sumandea MP,Reid MB

更新日期：2011-01-01 00:00:00

abstract：:Ventricular repolarization dysfunction has recently been reported in multiple sclerosis (MS). We evaluated ventricular repolarization dysfunction in 52 MS patients and looked for a relationship between corrected QT (QTc) abnormalities (i.e., abnormalities of QT intervals corrected for rate) and spinal cord magnetic re...

journal_title：Muscle & nerve

authors： de Seze J,Stojkovic T,Gauvrit JY,Saint Michel T,Ayachi M,Pruvo JP,Vermersch P

更新日期：2000-08-01 00:00:00

abstract：INTRODUCTION:Pes cavus often signals the presence of Charcot-Marie-Tooth (CMT) in adult patients, although its prevalence in the general population makes it a finding of unclear significance. METHODS:We undertook a pilot double cohort study to investigate the feasibility of comparing preselected bedside and radiograph...

journal_title：Muscle & nerve

authors： Stino AM,Atway S,Anthony M,Kline D,Kissel JT

更新日期：2019-01-01 00:00:00

abstract：:The purpose of this study was to determine if chronic fatigue syndrome (CSF) is characterized by abnormalities in oxidative muscle metabolism. Patients with CFS according to Centers for Disease Control (CDC) criteria (n = 22) were compared to normal sedentary subjects (n = 15). CFS patients were also tested before and...

journal_title：Muscle & nerve

authors： McCully KK,Natelson BH,Iotti S,Sisto S,Leigh JS Jr

更新日期：1996-05-01 00:00:00

abstract：:As applied to skeletal muscle, stem cell therapy is a reincarnation of myoblast transfer therapy that has resulted from recent advances in the cell biology of skeletal muscle. Both strategies envisage the reconstruction of damaged muscle from its precursors, but stem cell therapy employs precursors that are earlier in...

journal_title：Muscle & nerve

authors： Partridge TA

更新日期：2003-02-01 00:00:00

abstract：INTRODUCTION:The radial nerve and posterior interosseous nerve (PIN) are prone to injury at multiple sites. Electrodiagnostic (EDx) studies may only identify the most proximal lesion. Nerve ultrasound could augment EDx by visualizing additional pathology. METHODS:This investigation was a retrospective examination of u...

journal_title：Muscle & nerve

authors： Dietz AR,Bucelli RC,Pestronk A,Zaidman CM

更新日期：2016-03-01 00:00:00

abstract：INTRODUCTION:Valosin-containing protein (VCP) variants that affect muscle, bone, and the nervous system are termed multisystem proteinopathy. VCP myopathy is manifested as limb-girdle weakness, distal weakness and scapuloperoneal weakness. METHODS:We reviewed clinical, genetic, and muscle biopsy data from 6 members of...

journal_title：Muscle & nerve

authors： Guo X,Zhao Z,Shen H,Qi B,Li N,Hu J

更新日期：2019-03-01 00:00:00

abstract：:This study aimed to clarify the morphologic variations of the Martin-Gruber anastomosis (MGA) by tracing the anastomotic fascicles. We used 102 upper limbs, and MGA was found in 39.2%. Among 12 instances of MGA between the branches innervating the flexor digitorum profundus muscle, eight anastomotic branches solely in...

journal_title：Muscle & nerve

authors： Lee KS,Oh CS,Chung IH,Sunwoo IN

更新日期：2005-01-01 00:00:00

abstract：INTRODUCTION:Motor unit number estimates (MUNEs) provide important information in health, aging, and disease, and can be determined using decomposition-enhanced spike-triggered averaging (DE-STA). Discrimination of surface-detected motor unit potentials (S-MUPs) has been limited to contractile forces of ∽30% maximum vo...

journal_title：Muscle & nerve

authors： Stevens DE,Harwood B,Power GA,Doherty TJ,Rice CL

更新日期：2014-07-01 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：:Daptomycin is a lipopeptide antibiotic with strong bactericidal effects against Gram-positive bacteria and minor side effects on skeletal muscles. The type and magnitude of the early effect of daptomycin on skeletal muscles of rats was quantified by histopathology, examination of contractile properties, Evans Blue Dye...

journal_title：Muscle & nerve

authors： Kostrominova TY,Hassett CA,Rader EP,Davis C,Larkin LM,Coleman S,Oleson FB,Faulkner JA

更新日期：2010-09-01 00:00:00

abstract：INTRODUCTION:Changes in subsynovial connective tissue (SSCT) in carpal tunnel syndrome (CTS) patients may result in altered dynamics; consequently, quantification of these dynamics might support objective diagnosis of CTS. METHODS:We measured and compared longitudinal excursion of the flexor digitorum superficialis an...

journal_title：Muscle & nerve

authors： Korstanje JW,Scheltens-De Boer M,Blok JH,Amadio PC,Hovius SE,Stam HJ,Selles RW

更新日期：2012-05-01 00:00:00

abstract：INTRODUCTION:In this study we noninvasively evaluated skeletal muscle mitochondrial capacity, muscle-specific endurance, and energy/fatigue feelings in persons with Friedreich ataxia (FRDA) and able-bodied controls (AB). METHODS:Forearm mitochondrial capacity was measured in FRDA (n = 16) and AB (n = 10) study partici...

journal_title：Muscle & nerve

authors： Bossie HM,Willingham TB,Schoick RAV,O'Connor PJ,McCully KK

更新日期：2017-10-01 00:00:00

abstract：:Single fiber electromyography ( SFEMG ) of the extensor digitorum communis (EDC) muscle and nerve conduction studies were performed on healthy, active elderly men (66-77 years old) to assess age-related changes in neuromuscular physiology and the effect of long-term increased muscular activity on these changes. The fo...

journal_title：Muscle & nerve

authors： Shields RW Jr,Robbins N,Verrilli AA 3rd

更新日期：1984-05-01 00:00:00

abstract：INTRODUCTION:The p.A1156T mutation alters the function of the voltage-gated sodium channel Nav1.4 on the muscle sarcolemma, causing a channelopathy without overt myotonia or periodic paralysis but with myalgic pain. METHODS:A postal survey was conducted to assess the prevalence and characteristics of pain and related ...

journal_title：Muscle & nerve

authors： Suokas K,Palmio J,Sandell S,Udd B,Hietaharju A

更新日期：2018-06-01 00:00:00

abstract：INTRODUCTION:An open-label trial suggested that valproic acid (VPA) improved strength in adults with spinal muscular atrophy (SMA). We report a 12-month, double-blind, cross-over study of VPA in ambulatory SMA adults. METHODS:There were 33 subjects, aged 20–55 years, included in this investigation. After baseline asse...

journal_title：Muscle & nerve

authors： Kissel JT,Elsheikh B,King WM,Freimer M,Scott CB,Kolb SJ,Reyna SP,Crawford TO,Simard LR,Krosschell KJ,Acsadi G,Schroth MK,D'Anjou G,LaSalle B,Prior TW,Sorenson S,Maczulski JA,Swoboda KJ,Project Cure Spinal Muscular Atr

更新日期：2014-02-01 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