Acute quadriplegic myopathy: analysis of myosin isoforms and evidence for calpain-mediated proteolysis.

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: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

abstract：:To evaluate the effect of SMN2 copy number on disease severity in spinal muscular atrophy (SMA), we stratified 45 adult SMA patients based on SMN2 copy number (3 vs. 4 copies). Patients with 3 copies had an earlier age of onset and lower spinal muscular atrophy functional rating scale (SMAFRS) scores and were more lik...

journal_title：Muscle & nerve

authors： Elsheikh B,Prior T,Zhang X,Miller R,Kolb SJ,Moore D,Bradley W,Barohn R,Bryan W,Gelinas D,Iannaccone S,Leshner R,Mendell JR,Mendoza M,Russman B,Smith S,King W,Kissel JT

更新日期：2009-10-01 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：:Exercise intolerance with myalgia, muscle stiffness, and recurrent rhabdomyolysis due to mutations in the DMD gene can mimic metabolic myopathies leading to delayed or inaccurate diagnoses. In this retrospective chart review, we report 3 unrelated boys with exertional myalgia, muscle stiffness, myoglobinuria, and norm...

journal_title：Muscle & nerve

authors： Veerapandiyan A,Shashi V,Jiang YH,Gallentine WB,Schoch K,Smith EC

更新日期：2010-12-01 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：INTRODUCTION:Pretreatment with pyridostigmine bromide (PB) of human intercostal muscle fibers exposed to the irreversible acetylcholinesterase (AChE) inhibitor soman was investigated. METHODS:Muscles were pretreated with 3 × 10(-6) M PB or saline for 20 minutes, then exposed to 10(-7) M soman for 10 minutes. RESULTS:...

journal_title：Muscle & nerve

authors： Maselli RA,Henderson JD,Ng J,Follette D,Graves G,Wilson BW

更新日期：2011-04-01 00:00:00

abstract：:The ideal electrodiagnostic procedure to assess possible plantar neuropathies continues to elude investigators. Motor studies are rarely abnormal, pure sensory studies may be difficult to obtain, needle electromyography can demonstrate membrane instability in normal feet. Mixed nerve plantar studies may be more diagno...

journal_title：Muscle & nerve

authors： Dumitru D,Kalantri A,Dierschke B

更新日期：1991-07-01 00:00:00

abstract：:We compared the diaphragmatic electromyographic (EMG) recordings from 32 patients with known neuromuscular disease and respiratory symptoms (23 neuropathies, 9 myopathies) to recordings from 23 normal subjects. Turns analysis of 219-ms sections, or epochs, of the EMG demonstrated a significant overlap between diagnost...

journal_title：Muscle & nerve

authors： McKeown MJ,Bolton CF

更新日期：1998-07-01 00:00:00

abstract：INTRODUCTION:Immobilization induced by experimental denervation leads to rapid and progressive alterations in structural and biochemical properties of skeletal muscle. Real-time reverse transcription-polymerase chain reaction (RT-PCR) is a popular method of elucidating the molecular mechanisms involved in muscle atroph...

journal_title：Muscle & nerve

authors： Nakao R,Yamamoto S,Yasumoto Y,Kadota K,Oishi K

更新日期：2015-02-01 00:00:00

abstract：:The purpose of this study was to investigate whether the previously demonstrated heterogeneity of myosin heavy chain (MHC) in type 2B fibers of the superficial portion of the lateral gastrocnemius (SLG) muscle of the rat may be due to presence of type 2D/X fibers. Immunohistochemical identification of MHC heterogeneit...

journal_title：Muscle & nerve

authors： Sawchak JA,Leung B,Shafiq SA

更新日期：1992-12-01 00:00:00

abstract：:With the exception of thymectomy, immune modulatory treatment strategies and clinical trials in myasthenia gravis over the past 50 y were mainly borrowed from experience in other nonneurologic autoimmune disorders. The current experimental therapy paradigm has significantly changed such that treatments directed agains...

journal_title：Muscle & nerve

authors： Habib AA,Ahmadi Jazi G,Mozaffar T

更新日期：2020-11-01 00:00:00

abstract：INTRODUCTION:The objective of this study was to determine whether patients with carpal tunnel syndrome (CTS) manifest changes in early-stage motor function and to investigate the utility of a gyrosensor for quantitative evaluation of motor function. METHODS:Angular velocity signal was measured during finger tapping in...

journal_title：Muscle & nerve

authors： Seok HY,Kim JW,Kim YH,Park MH,Kwon DY

更新日期：2019-04-01 00:00:00

abstract：:The dystrophin protein complex (DPC), composed of at least 10 proteins that associate with dystrophin, is critical for the maintenance of normal muscle fiber structure and physiology. In this study, we used immunohistochemistry and confocal microscopy to examine the relative abundance and distribution of several of th...

journal_title：Muscle & nerve

authors： Draviam R,Billington L,Senchak A,Hoffman EP,Watkins SC

更新日期：2001-02-01 00:00:00

abstract：:Two brothers with the childhood variant of type II glycogenosis (GSD-IIb) treated with nutrition and exercise therapy (NET) from a young age showed an unusually benign course. Muscle biopsy from the older brother, which showed characteristic vacuolar glycogen accumulation at age 2, had reverted to normal by age 16. A ...

journal_title：Muscle & nerve

authors： Slonim AE,Bulone L,Minikes J,Hays AP,Shanske S,Tsujino S,DiMauro S

更新日期：2006-04-01 00:00:00

abstract：:Mechanical deformation of a peripheral nerve can evoke action potentials in sensory and motor axons. The generation of these impulses with brief stimuli (<0.5 s) and their relationship to the deformation conditions have not been systematically studied in human subjects. Controlled compression stimuli over a range of a...

journal_title：Muscle & nerve

authors： Clarke EC,McNulty PA,Macefield VG,Bilston LE

更新日期：2007-03-01 00:00:00

abstract：INTRODUCTION:We investigated the possible role of intercostal surgical neurolysis in relieving chronic neuropathic pain refractory to other nonsurgical treatments in patients with postsurgical thoracic pain. METHODS:We retrospectively collected clinical data on patients referred to the Neurosurgery Unit of Policlinic ...

journal_title：Muscle & nerve

authors： Cappellari AM,Tiberio F,Alicandro G,Spagnoli D,Grimoldi N

更新日期：2018-11-01 00:00:00

abstract：:In spite of extensive studies it is unclear whether impaired fasting glucose (IFG) or impaired glucose tolerance (IGT), i.e., impaired glucose metabolism (IGM), causes diabetic sensorimotor polyneuropathy (DSPN) or chronic idiopathic axonal polyneuropathy (CIAP); the results and conclusions vary considerably in differ...

journal_title：Muscle & nerve

authors： Dyck PJ,Dyck PJ,Klein CJ,Weigand SD

更新日期：2007-10-01 00:00:00

abstract：:To investigate the role of the thymus in the pathogenesis of myasthenia gravis (MG), immunohistochemical expression of CD44, CD45R0, B7-1, and IL-2 was studied in: (1) hyperplastic thymuses of patients with MG whose symptoms markedly improved after thymectomy, (2) remnant thymuses of patients with MG whose symptoms di...

journal_title：Muscle & nerve

authors： Utsugisawa K,Nagane Y,Tohgi H

更新日期：2000-04-01 00:00:00

abstract：INTRODUCTION:In this study we investigated the interaction between adipose tissue-derived stem cells (ASCs) and myoblasts in co-culture experiments. METHODS:Specific inductive media were used to differentiate ASCs in vitro into a Schwann cell-like phenotype (differentiated adipose tissue-derived stem cells, or dASCs) ...

journal_title：Muscle & nerve

authors： El-Habta R,Kingham PJ,Backman LJ

更新日期：2018-02-01 00:00:00

abstract：:The American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM) developed guidelines to formalize the ethical standards that neuromuscular and electrodiagnostic (EDx) physicians should observe in their clinical and scientific activities. Neuromuscular and EDx medicine is a subspecialty of medicine that ...

journal_title：Muscle & nerve

authors： Abel NA,De Sousa EA,Govindarajan R,Mayer MP,Simpson DA

更新日期：2015-12-01 00:00:00

abstract：:The purpose of this long-term, open parallel-group, double-consent study of alternate-day, low-dose prednisone in 2-4-year-old patients with Duchenne muscular dystrophy (DMD) was to determine whether prednisone produces a beneficial effect when given earlier than usual. Muscle function was evaluated by timed tests, an...

journal_title：Muscle & nerve

authors： Merlini L,Cicognani A,Malaspina E,Gennari M,Gnudi S,Talim B,Franzoni E

更新日期：2003-02-01 00:00:00

abstract：:Recent studies on the distribution of labeled endogenous proteins in the experimental neuropathies induced by streoptozotocin diabetes, galactose feeding, zinc pyridinethione, 2,5-hexanedione, acrylamide, and p-bromophenylacetylurea (BPAU) have demonstrated an impaired build up of retrogradely transported material der...

journal_title：Muscle & nerve

authors： Jakobsen J,Brimijoin S,Sidenius P

更新日期：1983-02-01 00:00:00

abstract：:Although the effect of low temperature on the peripheral nervous system has been systematically studied, the effect of high temperature has not. We investigated the effect of elevating limb temperature from 32 degrees C to 42 degrees C by performing sequential motor studies, antidromic sensory studies, and 3-Hz repeti...

journal_title：Muscle & nerve

authors： Rutkove SB,Kothari MJ,Shefner JM

更新日期：1997-04-01 00:00:00

abstract：:A 29-year-old Haitian man had a sore throat with a marked tonsillar exudate followed by the onset of increasingly blurred vision with swallowing difficulties and, then, a diffuse acute demyelinating neuropathy. Cerebrospinal fluid showed moderate pleiocytosis and elevated protein content. Serological testing for dipht...

journal_title：Muscle & nerve

authors： Créange A,Meyrignac C,Roualdes B,Degos JD,Gherardi RK

更新日期：1995-12-01 00:00:00

abstract：INTRODUCTION:Duchenne muscular dystrophy (DMD) subjects ≥5 years with nonsense mutations were followed for 48 weeks in a multicenter, randomized, double-blind, placebo-controlled trial of ataluren. Placebo arm data (N = 57) provided insight into the natural history of the 6-minute walk test (6MWT) and other endpoints. ...

journal_title：Muscle & nerve

authors： McDonald CM,Henricson EK,Abresch RT,Florence JM,Eagle M,Gappmaier E,Glanzman AM,PTC124-GD-007-DMD Study Group.,Spiegel R,Barth J,Elfring G,Reha A,Peltz S

更新日期：2013-09-01 00:00:00

abstract：INTRODUCTION:The purpose of this study was to investigate in vivo median nerve longitudinal mobility in different segments of the carpal tunnel associated with active finger motion in carpal tunnel syndrome (CTS) patients in a comparison with healthy controls. METHODS:Eleven healthy volunteers and 11 CTS patients part...

journal_title：Muscle & nerve

authors： Yao Y,Grandy E,Evans PJ,Seitz WH Jr,Li ZM

更新日期：2020-10-01 00:00:00

abstract：:The loss of contractile protein in Duchenne muscular dystrophy could result from low rates of synthesis, abnormally high rates of protein degradation, or a combination of both. We measured overall protein degradation rates in cultured human muscle cells obtained at biopsy from patients with Duchenne dystrophy or vario...

journal_title：Muscle & nerve

authors： Neville HE,Harrold S

更新日期：1985-03-01 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：:Jitter after axonal microstimulation in the masseter muscle was studied in 30 consecutive patients (12 women) with myasthenia gravis (MG). Patients' mean age was 42.3 (12-75), median disease duration was 3 months (1-72), and onset was ocular (15 cases), oculobulbar (7), bulbar (6), or generalized (2). There were 23 ne...

journal_title：Muscle & nerve

authors： Khuraibet AJ,Rousseff RT,Behbehani R,al-Shubaili AF,Khan RA

更新日期：2008-04-01 00:00:00