Search Results - "GRIGGS, R. C"

The non-dystrophic myotonias: molecular pathogenesis, diagnosis and treatment by Matthews, E., Fialho, D., Tan, S. V., Venance, S. L., Cannon, S. C., Sternberg, D., Fontaine, B., Amato, A. A., Barohn, R. J., Griggs, R. C., Hanna, M. G.

“…The non-dystrophic myotonias are an important group of skeletal muscle channelopathies electrophysiologically characterized by altered membrane excitability…”
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The primary periodic paralyses: diagnosis, pathogenesis and treatment by Venance, S. L., Cannon, S. C., Fialho, D., Fontaine, B., Hanna, M. G., Ptacek, L. J., Tristani-Firouzi, M., Tawil, R., Griggs, R. C.

“…Periodic paralyses (PPs) are rare inherited channelopathies that manifest as abnormal, often potassium (K)-sensitive, muscle membrane excitability leading to…”
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Primary episodic ataxias: diagnosis, pathogenesis and treatment by Jen, J.C., Graves, T.D., Hess, E.J., Hanna, M.G., Griggs, R.C., Baloh, R.W.

“…Primary episodic ataxias are autosomal dominant channelopathies that manifest as attacks of imbalance and incoordination. Mutations in two genes, KCNA1 and…”
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Acetazolamide efficacy in hypokalemic periodic paralysis and the predictive role of genotype by MATTHEWS, E, PORTARO, S, KE, Q, SUD, R, HAWORTH, A, DAVIS, M. B, GRIGGS, R. C, HANNA, M. G

“…Acetazolamide has been the most commonly used treatment for hypokalemic periodic paralysis since 1968. However, its mechanism of efficacy is not fully…”
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Correlating phenotype and genotype in the periodic paralyses by MILLER, T. M, DIAS DA SILVA, M. R, PETAJAN, J, DALAKAS, M. C, RANUM, L. P. W, FU, Y. H, PTACEK, L. J, MILLER, H. A, KWIECINSKI, H, MENDELL, J. R, TAWIL, R, MCMANIS, P, GRIGGS, R. C, ANGELINI, C, SERVIDEI, S

“…Periodic paralyses and paramyotonia congenita are rare disorders causing disabling weakness and myotonia. Mutations in sodium, calcium, and potassium channels…”
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Randomized, double-blind, placebo-controlled trial of albuterol in facioscapulohumeral dystrophy by KISSEL, J. T, MCDERMOTT, M. P, MENDELL, J. R, KING, W. M, PANDYA, S, GRIGGS, R. C, TAWIL, R

“…Animal and human studies suggest that beta(2)-adrenergic agonists exert anabolic effects on muscles, inducing and preventing atrophy after a variety of…”
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Andersen's syndrome: a distinct periodic paralysis by Sansone, V, Griggs, R C, Meola, G, Ptácek, L J, Barohn, R, Iannaccone, S, Bryan, W, Baker, N, Janas, S J, Scott, W, Ririe, D, Tawil, R

“…A previous study of 4 patients defined Andersen's syndrome (AS) as a triad of potassium-sensitive periodic paralysis, ventricular dysrhythmias, and dysmorphic…”
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Inclusion body myositis and myopathies by Griggs, R C, Askanas, V, DiMauro, S, Engel, A, Karpati, G, Mendell, J R, Rowland, L P

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A randomized efficacy and safety trial of oxandrolone in the treatment of Duchenne dystrophy by FENICHEL, G. M, GRIGGS, R. C, PANDYA, S, ROBISON, V. D, WANG, H, KISSEL, J, KRAMER, T. I, MENDELL, J. R, MOXLEY, R. T, PESTRONK, A, SHENG, K, FLORENCE, J, KING, W. M

“…A pilot study suggested that oxandrolone, an anabolic steroid, improved strength in boys with Duchenne dystrophy (DD) and indicated the need for a more…”
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A prospective natural history study of inclusion body myositis: implications for clinical trials by ROSE, M. R, MCDERMOTT, M. P, THORNTON, C. A, PALENSKI, C, MARTENS, W. B, GRIGGS, R. C

“…Eleven patients with untreated inclusion body myositis (IBM) were prospectively studied during a 6-month period that included muscle strength, lean body mass,…”
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Early and late losses of motor units after poliomyelitis by MCCOMAS, A. J, QUARTLY, C, GRIGGS, R. C

“…Motor unit number estimation was employed to assess muscle innervation in 76 patients with prior poliomyelitis. Of the 68 patients who were < 70 years of age,…”
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Pilot trial of albuterol in facioscapulohumeral muscular dystrophy by KISSEL, J. T, MCDERMOTT, M. P, NATARAJAN, R, MENDELL, J. R, PANDYA, S, KING, W. M, GRIGGS, R. C, TAWIL, R

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Multiple mitochondrial DNA deletions in sporadic inclusion body myositis: a study of 56 patients by Santorelli, F M, Sciacco, M, Tanji, K, Shanske, S, Vu, T H, Golzi, V, Griggs, R C, Mendell, J R, Hays, A P, Bertorini, T E, Pestronk, A, Bonilla, E, DiMauro, S

“…Inclusion body myositis, a chronic inflammatory disorder, is the most common cause of myopathy in adults over the age of 50. Diagnosis is based on clinical…”
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Effect of testosterone on muscle mass and muscle protein synthesis by Griggs, R C, Kingston, W, Jozefowicz, R F, Herr, B E, Forbes, G, Halliday, D

“…We have studied the effect of a pharmacological dose of testosterone enanthate (3 mg.kg-1.wk-1 for 12 wk) on muscle mass and total-body potassium and on…”
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Sodium channel mutations in acetazolamide-responsive myotonia congenita, paramyotonia congenita, and hyperkalemic periodic paralysis by PTACEK, L. J, TAWIL, R, LEPPERT, M. F, GRIGGS, R. C, MEOLA, G, MCMANIS, P, BAROHN, R. J, MENDELL, J. R, HARRIS, C, SPITZER, R, SANTIAGO, F

“…Hyperkalemic periodic paralysis (hyperKPP) and paramyotonia congenita (PC) are genetic muscle disorders sharing the common features of myotonia and episodic…”
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Improving adherence to dementia guidelines through education and opinion leaders : A Randomized, controlled trial by GIFFORD, D. R, HOLLOWAY, R. G, FRANKEL, M. R, ALBRIGHT, C. L, MEYERSON, R, GRIGGS, R. C, VICKREY, B. G

“…Educational methods that encourage physicians to adopt practice guidelines are needed. To evaluate an educational strategy to increase neurologists' adherence…”
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Andersen's syndrome: potassium-sensitive periodic paralysis, ventricular ectopy, and dysmorphic features by Tawil, R, Ptacek, L J, Pavlakis, S G, DeVivo, D C, Penn, A S, Ozdemir, C, Griggs, R C

“…Andersen's syndrome is a clinically distinct form of potassium-sensitive periodic paralysis associated with cardiac dysrhythmias. The subtle nature of the…”
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Mutations in the human skeletal muscle chloride channel gene (CLCN1) associated with dominant and recessive myotonia congenita by ZHANG, J, GEORGE, A. L, GRIGGS, R. C, FOUAD, G. T, ROBERTS, J, KWIECINSKI, H, CONNOLLY, A. M, PTACEK, L. J

“…Myotonia, defined as delayed relaxation of muscle after contraction, is seen in a group of genetic disorders that includes autosomal dominant myotonia…”
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End-plate acetylcholine receptor deficiency due to nonsense mutations in the epsilon subunit by Engel, A G, Ohno, K, Bouzat, C, Sine, S M, Griggs, R C

“…We describe a congenital myasthenic syndrome associated with severe end-plate (EP) acetylcholine receptor (AChR) deficiency not associated with an EP myopathy,…”
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Duchenne dystrophy : randomized, controlled trial of prednisone (18 months) and azathioprine (12 months) by GRIGGS, R. C, MOXLEY, R. T, KING, W, SIGNORE, L, SCHIERBECKER, J, FLORENCE, J, MATHESON-BURDEN, N, WILSON, B, MENDELL, J. R, FENICHEL, G. M, BROOKE, M. H, PESTRONK, A, MILLER, J. P, CWIK, V. A, PANDYA, S, ROBISON, J

“…Prednisone has been shown to improve strength in Duchenne dystrophy. Azathioprine often benefits corticosteroid-responsive diseases and can reduce the dose of…”
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