Management of end stage respiratory failure in Duchenne muscular dystrophy.

abstract：:This study attempts to gain a better understanding of the primary cause of doxorubicin myotoxicity to aid in the development of a protocol to increase its effectiveness in treating muscle spasm diseases. The time course of acute injury to the orbicularis oculi muscle after injection of doxorubicin into the eyelids of ...

journal_title：Muscle & nerve

authors： McLoon LK,Luo XX,Wirtschafter J

更新日期：1993-07-01 00:00:00

abstract：:The electrical and mechanical properties of paralyzed human thenar muscles were measured in response to supramaximal stimulation of the median nerve in individuals with chronic cervical spinal cord injury. These data were compared to those recorded from control muscles. Spontaneous motor unit activity was common in pa...

journal_title：Muscle & nerve

authors： Thomas CK

更新日期：1997-07-01 00:00:00

abstract：:Peripheral nerve lesions are sometimes associated with focal dystonia. We diagnosed ulnar neuropathy in 28 of 73 (40%) cases of occupational cramp in musicians. Focal slowing of ulnar conduction across the elbow was identified in 15 of 19 (79%) patients using the near nerve technique and in 5 of 17 (29%) patients usin...

journal_title：Muscle & nerve

authors： Charness ME,Ross MH,Shefner JM

更新日期：1996-04-01 00:00:00

abstract：:The protein compositions of subcellular fractions of muscle obtained from 17 Duchenne dystrophy patients, 15 disease controls (10 different primary myopathies, 5 spinal muscular atrophy patients), and 10 normals were examined by polyacrylamide gel electrophoresis. The gels were stained with Coomassie Brilliant Blue an...

journal_title：Muscle & nerve

authors： Niebroj-Dobosz I,Kornguth S,Schutta H,Siegel FL,Hausmanowa-Petrusewicz I

更新日期：1989-04-01 00:00:00

abstract：:The neuromuscular junction (NMJ) is a complex structure that serves to efficiently communicate the electrical impulse from the motor neuron to the skeletal muscle to signal contraction. Over the last 200 years, technological advances in microscopy allowed visualization of the existence of a gap between the motor neuro...

journal_title：Muscle & nerve

authors： Hughes BW,Kusner LL,Kaminski HJ

更新日期：2006-04-01 00:00:00

abstract：:Bacterial beta-galactosidase cDNA was injected without lipofectin into 41 sites in dog muscle and expression was seen in 22 of them. The cDNA and lipofectin was injected into 35 similar sites and expression was seen in 21. Expression was seen in a maximum of 2.5% of muscle fibers and 23.21% of nonmuscle cells. A total...

journal_title：Muscle & nerve

authors： Howell JM,Fletcher S,O'Hara A,Johnsen RD,Lloyd F,Kakulas BA

更新日期：1998-02-01 00:00:00

abstract：:In acquired polyneuropathies, symptoms and signs are typically distal and symmetrical, more prominent in the lower limbs than the upper limbs. This study was undertaken to measure the extent of the decrease in excitability produced by single impulses and by impulse trains in cutaneous afferents in the median and sural...

journal_title：Muscle & nerve

authors： Lin CS,Mogyoros I,Burke D

更新日期：2000-05-01 00:00:00

abstract：:Because of their central role in muscle development and maintenance, MEF2 family members represent excellent candidate effectors of the muscle pathology in myotonic dystrophy (DM). We investigated the expression and alternative splicing of all four MEF2 genes in muscle from neuromuscular disorder (NMD) patients, inclu...

journal_title：Muscle & nerve

authors： Bachinski LL,Sirito M,Böhme M,Baggerly KA,Udd B,Krahe R

更新日期：2010-12-01 00:00:00

abstract：:Frog muscles bathed in solutions equilibrated with 0.5%-2.0% halothane develop large contractures when cooled to 2-4 degrees C. These halothane-cooling contractures (HCC) are dose dependent and reproducible; they are potentiated by lidocaine (5 mM), abolished by procaine (5 mM), and partially inhibited by procainamide...

journal_title：Muscle & nerve

authors： Suarez-Kurtz G,Sudo RT

更新日期：1986-01-01 00:00:00

abstract：:Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and membranous glomerulonephritis (MGN) are both autoimmune disorders that are rarely observed concurrently. We describe a patient who developed MGN nearly 20 years after the onset of CIDP, resulting in a secondary progression of his neuropathy. He respo...

journal_title：Muscle & nerve

authors： Smyth S,Menkes DL

更新日期：2008-01-01 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：:There are several pathogenic mechanisms of peripheral nerve involvement in patients with monoclonal dysglobulinemia. Intranervous proliferation of malignant cells, immunoglobulin, or amyloid deposits in the endoneurial space can only be determined by examination of nerve biopsy specimens. We present clinical, electrop...

journal_title：Muscle & nerve

authors： Vallat JM,Magy L,Richard L,Piaser M,Sindou P,Calvo J,Ghorab K,Cros D

更新日期：2008-07-01 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：:We have developed an algorithm, called multi-motor unit action potential analysis (MMA), to aid quantification in routine needle EMG examination. In only 5-8 min, it was possible to extract, analyze, and validate 20 motor unit action potentials (MUAPs). In the biceps muscle of normal subjects, the MUAP measurements ar...

journal_title：Muscle & nerve

authors： Nandedkar SD,Barkhaus PE,Charles A

更新日期：1995-10-01 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：: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：:Focal myositis is a rare inflammatory pseudotumor of skeletal muscle which usually has a benign course. We report a 56-year-old woman with a painful mass in the left arm with a radial nerve palsy. Magnetic resonance imaging (MRI) of the left arm showed a mass in the triceps muscle that was suggestive of a soft-tissue ...

journal_title：Muscle & nerve

authors： Alzagatiti BI,Bertorini TE,Horner LH,Maccarino VS,O'Brien T

更新日期：1999-07-01 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：:The mdx mouse, an animal model used to study Duchenne muscular dystrophy, has a nonsense mutation in exon 23 of the dystrophin gene which should result in a truncated protein that cannot be correctly localized at the sarcolemma of the muscle fibers. Immunohistochemical staining with antidystrophin antibodies has shown...

journal_title：Muscle & nerve

authors： Wilton SD,Dye DE,Laing NG

更新日期：1997-06-01 00:00:00

abstract：:Ultrastructural, biochemical, and electrophysiological analyses were done on 12-14-month-old mice deficient for myelin-associated glycoprotein (MAG) to further characterize the neuropathy that develops as they age. Electron microscopy demonstrated normal myelin compaction and axonal degeneration in a large number of m...

journal_title：Muscle & nerve

authors： Weiss MD,Luciano CA,Quarles RH

更新日期：2001-10-01 00:00:00

abstract：:We previously reviewed the presentation, initial clinical course, and electrodiagnostic features of children with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We now report the long-term follow-up of 12 children with idiopathic CIDP, and compare these to 62 adults with idiopathic CIDP. Children of...

journal_title：Muscle & nerve

authors： Simmons Z,Wald JJ,Albers JW

更新日期：1997-12-01 00:00:00

abstract：:Acetylcholine receptor (AChR) metabolism was studied in muscle from juvenile and adult dogs with congenital myasthenia gravis (CMG) and their unaffected littermates. Although the amount of AChR in the junctional region of innervated CMG muscle fibers was 25% of normal, or less, denervation of CMG fibers resulted in th...

journal_title：Muscle & nerve

authors： Oda K,Lennon VA,Lambert EH,Palmer AC

更新日期：1984-11-01 00:00:00

abstract：:Hereditary neuropathy with liability to pressure palsy (HNPP) is typified as isolated nerve palsies caused by trivial compression or trauma. It rarely presents in two extremities and even more infrequently affects all four limbs simultaneously. We present a patient who concurrently experienced right shoulder, left han...

journal_title：Muscle & nerve

authors： Crum BA,Sorenson EJ,Abad GA,Dyck PJ

更新日期：2000-06-01 00:00:00

abstract：:The cerebrospinal fluid (CSF) of patients with demyelinating neurological disease, such as Guillain-Barré syndrome or multiple sclerosis, contains factors that inhibit the excitatory Na+ current. Such antiexcitatory factors are occasionally also detectable in CSF from patients with other neurological diseases but were...

journal_title：Muscle & nerve

authors： Brinkmeier H,Seewald MJ,Wollinsky KH,Rüdel R

更新日期：1996-01-01 00:00:00

abstract：:The F-wave was used to estimate the fastest motor nerve conduction velocity (MNCV) along the proximal segment (spinal cord to knee) of the axons of the tibial nerve in 65 control subjects, 8 patients with diabetic polyneuropathy, and 4 patients with Charcot-Marie-Tooth disease. Terminal latencies and MNCV along the di...

journal_title：Muscle & nerve

authors： Panayiotopoulos CP,Scarpalezos S,Nastas PE

更新日期：1978-05-01 00:00:00

abstract：INTRODUCTION:We describe a novel, clinically applicable conduction study of the laryngeal nerves. METHODS:Seventeen normal volunteer subjects were included. Activation of the sensory territory of the superior laryngeal nerve was performed by administration of low level brief electrical stimuli. The laryngeal closure r...

journal_title：Muscle & nerve

authors： Carey B,Sulica L,Wu A,Branski R

更新日期：2013-03-01 00:00:00

abstract：INTRODUCTION:The aim of this study was to evaluate the ultrasound (US) morphological changes of sural nerves (SNs) of uremic patients on hemodialysis. METHODS:Sixty-six SNs in 33 uremic patients were examined by 22-MHZ high-frequency US and routine nerve conduction studies (NCS), and 76 SNs in 38 controls were also ex...

journal_title：Muscle & nerve

authors： Wei M,Zhu J,Wang N,Xue Q,Liu F,Hu B,Fang J,Zhang Y

更新日期：2012-12-01 00:00:00

abstract：:A randomized double-blind therapeutic trial of isaxonine was completed over a 2-year period for 20 ambulant boys with Duchenne muscular dystrophy aged 5 1/2-10 years. The effect of the drug was monitored by measurement of walking times over 28 and 150 ft, motor ability score, MRC score based on 32 muscle groups, and m...

journal_title：Muscle & nerve

authors： Heckmatt JZ,Hyde SA,Gabain A,Dubowitz V

更新日期：1988-08-01 00:00:00

abstract：:Muscle weakness, studied in 4 patients with multiple sclerosis (MS), was compared with values from normal subjects. Twitch occlusion showed that normal subjects could activate their muscles maximally, but patients rarely achieved greater than 60% activation. In both groups, motoneuron firing rates increased linearly w...

journal_title：Muscle & nerve

authors： Rice CL,Vollmer TL,Bigland-Ritchie B

更新日期：1992-10-01 00:00:00

abstract：:Clinicopathologic findings from two golden retriever dogs with an inherited, progressive, degenerative muscle disease that were studied until 27 and 40 months of age are described. Initial clinical signs included stilted gait and simultaneous advancement of their pelvic limbs. Further gait restriction and muscle hyper...

journal_title：Muscle & nerve

authors： Kornegay JN,Tuler SM,Miller DM,Levesque DC

更新日期：1988-10-01 00:00:00