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216 results on '"Z, Argov"'

1. Statins and the neuromuscular system: a neurologist's perspective

Author

Z. Argov

Subjects
myalgia, medicine.medical_specialty, Statin, business.industry, medicine.drug_class, Perspective (graphical), Neuromuscular Diseases, System a, Toxic neuropathy, Muscular Diseases, Neurology, Physical therapy, Humans, Medicine, Neurology (clinical), Hydroxymethylglutaryl-CoA Reductase Inhibitors, medicine.symptom, Genetic risk, business, Intensive care medicine, Myopathy
Abstract

Statins intolerance is mainly due to their side effects on the neuromuscular system (primarily muscle). It has become an important issue because of the major cardiovascular risk reduction of this class of drugs. However, the facts related to these side effects are sometimes under-recognized or controversial. A literature review of the recent developments in the field is given. The clinical definition of statin myopathy and its presentation are not suitable for the myology field. Management and prevention are not validated. More genetic risk factors need to be established. Neurologists should become more involved in statin intolerance evaluation and management.

Published
2014

2. EFNS/ENS Guidelines for the treatment of ocular myasthenia

Author

Z. Argov, Emilia Kerty, Amelia Evoli, Ahmed Elsais, and Nils Erik Gilhus

Subjects
Pediatrics, medicine.medical_specialty, Thymoma, Databases, Factual, Ocular myasthenia, medicine.medical_treatment, Guidelines as Topic, Azathioprine, Humans, Immunologic Factors, Medicine, Statistics & numerical data, myasthenia gravis, acetylcholine receptor, business.industry, Thymectomy, medicine.disease, Myasthenia gravis, Settore MED/26 - NEUROLOGIA, Regimen, Systematic review, Neurology, Physical therapy, Cholinesterase Inhibitors, Neurology (clinical), business, medicine.drug
Abstract

Background and purpose: The symptoms of acquired autoimmune ocular myasthenia are restricted to the extrinsic eye muscles, causing double vision and drooping eyelids. These guidelines are designed to provide advice about best clinical practice based on the current state of clinical and scientific knowledge and the consensus of an expert panel. Search strategy: Evidence for these guidelines was collected by searches in the MEDLINE and Cochrane databases. The task force working group reviewed evidence from original articles and systematic reviews. The evidence was classified (I, II, III, IV) and consensus recommendation graded (A, B or C) according to the EFNS guidance. Where there was a lack of evidence but clear consensus, good practice points are provided. Conclusions: The treatment of ocular myasthenia should initially be started with pyridostigmine (good practice point). If this is not successful in relieving symptoms, oral corticosteroids should be used on an alternate-day regimen (recommendation level C). If steroid treatment does not result in good control of the symptoms or if it is necessary to use high steroid doses, steroid-sparing treatment with azathioprine should be started (recommendation level C). If ocular myasthenia gravis is associated with thymoma, thymectomy is indicated. Otherwise, the role of thymectomy in ocular myasthenia is controversial. Steroids and thymectomy may modify the course of ocular myasthenia and prevent myasthenia gravis generalization (good practice point).

Published
2014

3. Dysferlinopathy in the Jews of the Caucasus: A frequent mutation in the dysferlin gene

Author

Esther Leshinsky-Silver, Z. Argov, Menachem Sadeh, Ron Dabby, Y. Wirguin, L. Rozenboim, Sarit Cohen, Y. Cohen, Dorit Lev, and Z. Tzofi

Subjects
Adult, Male, Heterozygote, Dysferlinopathy, Adolescent, DNA Mutational Analysis, Population, Muscle Proteins, Chromosome Disorders, Genes, Recessive, Frameshift mutation, Dysferlin, Muscular Diseases, Asia, Western, medicine, Humans, Genetic Predisposition to Disease, Genetic Testing, Muscular dystrophy, Frameshift Mutation, Myopathy, education, Genetics (clinical), Genetics, education.field_of_study, Geography, biology, business.industry, Membrane Proteins, medicine.disease, Founder Effect, Neurology, Jews, Mutation, Pediatrics, Perinatology and Child Health, biology.protein, Female, Neurology (clinical), medicine.symptom, business, Gene Deletion, Founder effect, Limb-girdle muscular dystrophy
Abstract

Dysferlin encoding gene (DYS) is mutated in the autosomal recessive disorders Miyoshi myopathy, Limb Girdle Muscular Dystrophy type 2B (LGMD2B) and distal anterior compartment myopathy, causing dysferlin deficiency in muscle biopsy. Three ethnic clusters have previously been described in Dysferlinopathy: the Libyan Jewish population originating in the area of Tripoli, Italian and Spanish populations. We report another cluster of this muscular dystrophy in Israel among Jews of the Caucasus region. A genomic analysis of the dysferlin coding sequence performed in patients from this ethnic group, who demonstrated an absence of dysferlin expression in muscle biopsy, revealed a homozygous frameshift mutation of G deletion at codon 927 (2779delG) predicting a truncated protein and a complete loss of functional protein. The possible existence of a founder effect is strengthened by our finding of a 4% carrier frequency in this community. These findings are important for genetic counseling and also enable a molecular diagnosis of LGMD2B in Jews of the Caucasus region.

Published
2007

4. Fourth meeting of the European Neurological Society 25–29 June 1994 Barcelona, Spain

Author

H. Hattig, C. Delli Pizzi, M. C. Addonizio, Michelle Davis, A. R. Giovagnoli, L. Florensa, M. Roth, J. de Kruijk, Francisco Lacruz, Ph. Dewailly, A. Toygar, C. Avendano, P.P. De Deyn, J. F. Hurtevent, F. Lomeila, T. W. Wong, Gordon T. Plant, M. Bud, H. J. Willison, DH Miller, D. W. Langdon, R. Cioni, J. Servan, A. Kaygisiz, E. Racadot, D. B. Schens, E. Picciola, L. Falip, C. Bouchard, J. Jotova, A. Jorge-Santamaria, P. Misra, A. Dufour, C. P. Panagopoulos, A. Venneri, B. Sredni, B. Angelard, M. Janelidze, M. Carreno, J. Obenberger, J. Pouget, H. W. Moser, R. Kaufmann, J. A. Molina, D. Linden, A. Martin Urda, E. Uvestad, A. Krone, J. P. Cochin, J. Mallecourt, A. Cambon-Thomsen, K. Violleau, P. Osschmann, A. M. Durocher, E. Bussaglia, D. M. Danielle, H. Efendi, C. Van Broeckhoven, K. G. Jordan, W. Rautenberg, C. Iniguez, J. M. Delgado, Graham Watson, M. Lawden, Gareth J. Barker, K. Stiasny, James T. Becker, G. Campanella, E. Peghi, A. Poli, A. Haddad, T. Yamawaki, Giacomo P. Comi, S. Sotgiu, B. Ersmark, A. Pomes, M. Ziegler, P. Ferrante, P. Ruppi, H. KuÇukoglu, R. Bouton, U. K. Rinne, P. Vieregge, M. Dary, P. Giunti, Peter J. Goadsby, S. Jung, E. Secor, A. Steinberg, N. Vila, M. A. Hernandez, M. Cursi, A. Enqelhardt, A. Engelhardt, J. Veitch, F. Di Silverio, F. Arnaud, B. Neundörfer, R. Brucher, Dominique Caparros-Lefebvre, B. Meyer, Marianne Dieterich, M. H. Snidaro, R. Gomez, R. Cerbo, M. Ragno, J. M. Vance, S. Nemni, A. Caliskan, F. Barros, I. Velcheva, D. Ceballos-Baumann, V. Barak, A. Avila, N. Antonova, F. Resche, S. Pappata, L. Varela, S. R. Silveira Santos, A. Cammarota, L. Naccache, Y. Nara, E. Tournier-Lasserves, R. Mobner, T. Chase, A. Ensenyat, J. Ulrich, G. Giegerich, M. Rother, M. Revilla, N. Nitschke, K. Honczarenko, E. Basart Tarrats, J. Blin, B. Jacob, J. Santamaria, S. Knezevic, J. L. Castillo, M. Antem, J. Colomer, O. Busse, Didier Hannequin, S. Carrier, J. B. Ruidavets, C. Rozman, J. Bogoussslavsky, J. Pascual Calvet, E. Monros, J. M. Polo, M. Zucconl, Javier Muruzabal, R. R. Allen, R. Rivolta, K. Haugaard, A. Nespolo, K. Hoang-Xuang, G. Bussone, T. Avramidis, E. Corsini, Christiana Franke, T. Vinogradova, H. Boot, K. Vestergaard, G. H. Jansen, N. Argentino, M. Raltzig, W. Linssen, Mark B. Pepys, P. Roblot, L. Lauritzen, E. Fainardi, D. Morin, T. X. Arbizu Urdiain, J. Wollenhaupt, S. Bostantjopoulou, G. Pavesi, A. D. Forman, Giovanni Fabbrini, D. Jean, J. J. Archelos, M. I. Blanchs, M. Del Gobbo, Anna Carla Turconi, Ch. Derouesné, Elio Scarpini, A. Visbeck, P. Castejon, J. P. Renou, F. Mounier-Vehier, G. Potagas, Ch. Duyckaerts, A. Filla, R. Schneider, G. Ronen, K. Nagata, J. P. Vedel, A. Henneberg, G. van Melle, C. Baratti, H. Knott, M. C. Prevett, A. Bes, B. Metin, Jos V. Reempts, L. Martorell, Mefkure Eraksoy, H. O. Handwerker, D. S. Younger, O. Oktem, D. Frongillo, C. Soriano-Soriano, L. Niehaus, F. Zipp, A. Tartaro, S Newman, R. H. Browne, P. Davous, R. Sanchez, M. Muros, M. E. Kornhuber, A. Lavarone, M. Mohr, M. R. Garcia, S. Russell, H. Kellar-Wood, M. R. Tola, B. Ostermeyer, Ch. Tzekov, K. Sartor, E. B. Ringelstein, P. P. Gazzaniga, Paul Krack, H. Fidaner, H. Rico, T. Dbaiss, F. Alameda, E. Torchiana, L. Rumbach, I. Charques, J. M. Bogaard, C. D. Frith, L. J. Rappelle, R. Brenner, A. Joutel, K. Fuxe, G. HÄcker, M. J. Blaser, J. Valls-SolÇ, G. Ulm, M. Alberdi, A. Bock, F. W. Bertelsmann, U. Wieshmann, J. Visa, J. R. Lupski, D. D'Amico, L. M. P. Ramos, A. A. Vanderbark, R. Horn, M. Warmuth, Dietmar Kühne, Mark S. Palmer, C. Ehrenheim, E. Canga, S. Viola, O. Scarpino, P. Naldi, R. Almeida, A. A. Raymond, J. Gamez, Stephan Arnold, A. DiGiovanni, J. Dalmau, C. C. Chari, H. F. Beer, J. C. Koetsier, J. Iriarte, E. Yunis, J. Casadevall, E. Le Guern, E. Stenager, S. R. Benbadis, J. M. Warter, F. Burklin, I. Theodorou, L. Johannesen, G. A. Graveland, X. Leclerc, I. Vecchio, L. Ozelius, G. Nicoletti, R. K. Gherardi, E. Esperet, M. L. Delodovici, F. Cattin, F. Paiau, Giorgio Sacilotto, C. A. J. Broere, D. Chavdarov, J. P. Willmer, C. H. Hawkes, Th. Naegele, E. Ellie, E. Dartigues, M. J. Guardiola, S. Hesse, Z. Levic, Marco Rovaris, P. Saugeir-Veber, B. A. Yaqub, H. F. Durwen, R. Larumbe, J. Ballabrina, M. Sendtner, J. Röther, M. Horstink, C. Kluglein, M.P. Montesi, H. Apaydin, J. Montoya, E. Waubant, Ch. Verellen-Dunoulin, A. Nicolai, J. Lopez-Delval, R. Lemon, G. Cantinho, E. Granieri, A. Zeviani, Wolfgang H. Oertel, U. Ficola, V. Di Piero, V. Fragola, K. Sabev, M. V. Guitera, I. Turki, F. Bolgert, P. Ingrand, J. M. Gobernado, L. M. E. Grimaldi, S. Baybas, B. Eymard, Y. Rolland, Y. Robitaille, Ta. Pampols, P. J. Koehler, A. Carroacedo, J. Vilchez, S. Di Vittorio, I. R. Rise, T. Nagy, M. Kuffner, E. Palazzini, A. Ott, J. Pruim, T. X. Arbizu, E. Manetti, C. Cervera, S. Felber, G. Gursoy, J. Scholz, G. A. Buscaino, M. S. Chen, A. Pascual, J. Hazan, J. U. Gajda, J. G. Cea, G. Bottini, G. Damalik, F. Le Doze, G. Bonaldi, J. M. Hew, C. Messina, A. M. Kennedy, J. M. Carney, N. M. F. Murray, M. Parent, M. Koepp, V. Dimova, D. De Leo, K. Jellinger, G. Salemi, S. Mientus, M. L. Hansen, F. Mazzucchelli, J. Vieth, M. Mauri, E. Bartels, L. Johannsen, C. Humphreys, J. Emile, D. N. Landon, E. Kansu, R. Sanchez-Pernaute, Rsj Frackowiak, M. Gonzalez Torres, L. Oller, C. Machedo, J. Kother, M. Billiard, H. Durak, T. Schindler, A. Frank, A. Uncini, A. Sbriccoli, C. Farinas, D. W. Paty, N. Fast, A. T. Zangaladze, A. Kerkhofs, J. M. Pino Garcia, I. De la Fuente, B. Marini, L. Gomez, I. Rubio, Alessandra Bardoni, C. Brodie, P. Acin, U. Sliwka, S. A. Hawkins, S. Tardieu, F. Vitullo, J. M. Pereira Monteino, R. Gagliardi, T. Jezewski, A. Cano, T. Lempert, F. Abad Alegria, G. Rotondo, D. Ince, C. Martinez Parra, Y. Huang, H. Luders, Y. Steinvil, F. G. A. Van Der Meche, R. Bianchi, A. Sanchez, T. Sevilla, J. M. Ketelslegers, A. Domzal-Stryga, M. Pandolfo, M. O. Josse, K. W. Neff, I. Blanco, G. W. Bruyn, O. W. Witte, J. L. Thibault, G. Andersen, J. Pariset, A. Marcone, R. J. M. Lane, A. Hofman, M. Verin, T. Matilla, P. Bedoucha, J. Roche, M. Lai, M. Collard, A. Ugarte, F. Gallecho, D. Silbersweig, C. Kennard, J. P. Azulay, T. W. Ho, P. L. I. Dellemijn, R. Girardello, F. Baas, B. Voss, F. Rozenberg, E. M. Brocker, V. Stanev, A. A. J. Soeterboek, A. Marra, A. Rey, E. Ertem, M. Sawradewicz-Rybak, J. De Keyser, P. Cavallari, F. Proust, Y. Chevalier, H. C. Hansen, D. Leys, C. A. Davie, K. Hoang-Xuan, C. Bairati, H. van Crevel, Thomas T. Warner, B. Bompais, A. Dobbeleir, T Campbell, C. Macko, C. J. M. Klijn, M. Dussallant, T. P. Berlit, W. Rozenbaum, M. J. van den Bent, W. A. Rocca, M. Muller, H. Hundemer, U. Zifko, M. Campera, F. Drislane, D. Ranoux, T. M. Kloss, Anil Kumar, I. Ruolt, C. Bargnani, B. Marescau, N. A. Losseff, S. Notermans, B. Kint, E. T. Burke, C. Aykut, J. Matias Guiu, P. Maquet, T. Drogendijk, M. Leone, K. von Ammon, M. Pepeliarska, C. Prados, L. DiGiamberardino, T. Logtenberg, G. Lenoir, I. Castaldo, Damhaut, M. Radionova, G. Sirabian, R. Navon, Giovanni Antonini, K. Al Moutaery, E. Chamas, R. Schönhuber, M. Giannini, B. Debilly, I. Labatut, H. Henon, J. A. Egido, M. Baudrimont, J. N. Lorenzo, J. E. C. Bromberg, R. Antonacci, J. J. Vilchez, T. Moulin, B. Rautenstrauss, Giovanni Meola, J. Noth, S Mammi, P. Laforet, F. Lopez, C. Gehring, S. Bort, G. Rancurel, D. Decamps, S. Kostadinova, Y. Shapira, B. Neundoerfer, D. Chavrot, M. Solimena, J. P. Salier, W. Deberdt, R. Hoff-Jörgensen, A. Messina, S. Meairs, G. Rosoklija, E. Nelis, I. Bertran, C. Ertekin, J. Lohmeyer, Mitermayer Galvao dos Reis, L. Calo, E. Maccagnano, A. P. Hays, J. Verlooy, M. G. Forno, T. Blanco, L. Bail, Gabriella Silvestri, J. Montero, F. Bertrand, R. T. Ghnassia, C. Besses, T. Sereghy, F. Shalit, G. Bogliun, S. Braghi, St. Baykouchev, C. Franke, A. Lasa, L. C. Archard, J. Kriebel, S. Shaunak, M. Nocito, Alexander Tsiskaridze, E. Manfredini, T. Seigal, David G. Gadian, M. Barlas, J. D. Degos, C. Seeber, J. Caemert, J. L. Mas, R. B. Pepinsky, M. G. D'Angelo, N. Baumann, S. Yorifuji, H. P. Endtz, M. A. Cassatella, R. A. C. Hughes, V. Golzi, A. Bittencourt, A. Ferreira, M. Sanson, C. Alper, M. Vermeulen, M. A. A. van Walderveen, E. Alexiou, C. H. Lucas, M. Fiorelli, Y. N. Debbink, R. Gil, S. Congia, T. Banerjee, J. M. Bouchard, A. N. Pinto, A. Ceballos-Baumann, G. Grollier, P. I. M. Schmitz, M. D. Catata, N. Lahat, N. S. Rao, P. Papathanasopoulos, J. Valls-Solé, D. Claus, G. Schroter, A. Castro, C. Videbaek, R. Martinez Dreke, A. D. Platts, M. Hermesl, A. C. PeÇanha-Martins, M. Cardoso Silva, P. Masnou, M. J. A. Tanner, Ch. Confavreux, B. Mishu, H. Rasmussen, L. Valenciano, Carlo Pozzilli, S. W. Li, V. Salzman, Y. Vashtang, Massimo Franceschi, M. Severo, G. Deuschl, S. Setien, G. Mariani, A. Protti, J. Castillo, M. J. B. Taphoorn, M. Frontali, I. Milonas, D. Decoq, J. A. Navarro, S. Castellvi-Pel, C. Ertikin, M. Urtasun, Y. Lajat, B. E. Kendall, E. Verdu, B. Gueguen, E. Boisen, R. Couderc, A Danek, JM Stevens, F. Nicoli, L. Feltri, M. L. Vazquez-Andre, J. A. Morgan-Hughes, L. D'Angelo, F. Y. Liew, L. F. Pascual, J. Patrignani Ochoa, Vittorio Martinelli, J. Cophignon, L. Zhang, S. Martin, J. F. Meder, H. C. Buschmann, L. Bertin, J. van Gijn, A. Barreiro, A. Cools, C. Leon, A. Berod, E. A. Anllo, E. Zanette, L. Petrov, R. Barona, B. Gallicchio, P. J. Cozzone, N. Diederich, G. Cancel, L. Schelosky, P. Orizaola, K. Yulug, S. Ozer, Valeria A. Sansone, B. Guiraud-Chaumeil, K. Voigt, P. Labauge, M. Eoli, J. Zhu, J. Aguirre, M. Ferrarini, B. Zyluk, E. Planas, A. Cadilha, C. Tortorella, H. Bismuth, C. E. Counsell, A. Laun, A. Ferlini, Rio J. Montalban, N. Biary, L. Becker, M. Fardeau, M. Poloni, V. M. S. de Bruin, C. Fornada, J. Barros, E. Ganzmann, E. Touze, D. Wallach, J. Peila, H. Fujimura, M. T. Iba-Zizen, G. Macchi, C. Villoslada, R. Gouider, Ph. Rondepierre, P. Grummich, P. Chiodi, C. Conte, M. Michels, P. Annunziata, G. Semana, C. Sommer, J. Vajsar, D. Zekin, J. Kulisevsky, David G. Munoz, B. Jacotot, M. Magoni, A. Luxen, T. Garcia-Silva, S. Di Cesare, Christophe Tzourio, M. Gomori, I. Picomell, L. Santoro, F. Villa, Giovanni Pennisi, T. Ribalta, J. M. Molto, L. Marzorati, P. Loiseau, F. Gemignani, A. Gironell, J. Wissel, A. Prusinski, F. Cailloux, P. Villanueva-Hemandez, P. Cozzone, T. Del Ser, J. Sans-Sabrafen, M. Zappia, P. W. A. Willems, G. Tchernia, D. Gardeur, R. Bauer, F. Palomo, H. Metz, S. Lamoureux, C. Chastang, I. Reinhard, A. Goldfarb, S. Harder, Jordi Río, C. Ozkara, E. Tekinsoy, P. Vontobell, J. De Recondo, M. Rabasa, L. Lacomblez, F. Boon, Dgt Thomas, V. Palma, Renato Mantegazza, A. Dervis, M. Nueckel, B. YalÇinerner, I. Duran, G. Dalla Volta, A. Zubimendi, J. Pinheiro, A. Marbini, Xavier Montalban, H. Wekerle, X. Pereira Monteino, F. Crespo, F. Koskas, N. Battistini, C. Ruiz, H. Offner, J. de Pommery, P. Kanovsky, J. Y. Barnett, J. Pardo, G. Tomei, R. Rene, H. M. Lokhorst, P. Thajeb, H. Bilgin, D. McGehee, R. Fahsold, L. Morgante, Katie Sidle, C. Delwaide, M. N. Diaye, P. H. Rice, A. Creange, C. Sabev, K. Stephan, K. WeilBenborn, G. Magnani, L. Grymonprez, F. Cardellach, M. Kaps, N. G. Meco, F. Vega, V. Bonifati, A. Desomer, M. Baldy-Moulinier, G. Kvale, F. J. Authier, B. Yegen, T. Ho, J. M. Rozet, E. A. Cabanis, L. Bruce, L. Ambrosoli, M. A. Petrella, M. Hernandez, P. Timmings, H. B. van der Worp, F. Mahieux, A. Urbano-Marquez, D. A. Krendel, A. A. Garcia, R. Divari, R. Michalowicz, M. R. Piedmonte, M. Bondavalli, M. Zanca, P. F. Ippel, Onofre Combarros, B. Tavitian, E. Hirsch, I. Anastasopoulos, A. Roses, A. Köhler, P. Vienna, V. Timmerman, P. Sergi, F. Cornelio, A. Di Pasquale, R. Verleger, S. Castellvirel, J. Proano, B. van Moll, F. Rubio, W. Hacke, I. Lavenu, L. Zetta, M. W. Tas, N. Bittmann, M. Bonamini, O. R. Hommes, V. Dousset, N. Afsar, S. Belal, R. R. Myers, J. Goes, Giuseppe Vita, E. Clementi, V. G. Karepov, M. Jueptner, A Vincent, P. Emmrich, Th. Heb, A. Caballo, J. Gallego, T. Mokrusch, C. Perla, L. Gebuhrer, O. Titlbach, Alessandro Prelle, A. Czlonkowska, M. Russo, D. Hadjiev, T. S. Chkhikvishvili, M. Oehlschlager, G. Becker, I. Günther, E. N. Stenager, J. Garcia Agundez, J. Casademont, J. Batlle, S. Podobnik-Sarkanji, C. Alonso-Villaverde, B. Delaguillaume, B. Genc, B. Mazoyer, A. Rodriguez-Al-barino, Ch. Hilger, B. Ferrero, R. Price, W. Grisold, L. Fuhry, D. Oulbani, D. Ewing, A. Petkov, W. Walther, A. Gokyigit, John Newsom-Davis, J. Tayot, D. Seliak, G. Pelliccioni, D. Campagne, K. Kessler, F. Boureau, D. Perani, J. P. N'Guyen, N. Tchalucova, B. A. Antin-Ozerkis, C. Lacroix, B. D. Aronovich, I. H. Jenkins, E. A. dos Reis, M. Hortells, H. M. Meinck, H. Ch. Buschmann, S. C. J. M. Jacobs, T. Wetter, P. Creissard, N. Martinez, J. Weidenfeldl, H. J. Sturenburg, G. Damlacik, V. Gracia, J. C. Turpin, A. Pou-Serradell, J. P. Vincent, T. Gagoshidze, U. Ozkutlu, M. McLeod, K. Siegfried, I. Tchaoussoglou, J. Hildebrand, S. Kowalska, M. C. Picot, G. Galardin, L. Crevits, F. Andreetta, S. Larumbe-Lobalde, G. de la Sierra, J. C. Alvarez-Cermeno, R. J. Seitz, P. L. Oey, L. Ptacek, A. M. J. Paans, A. Wirrwar, A. Schmied, J. Uilchez, H. Tounsi, D. Hipola, V. Avoledo, Y. Hirata, P. Vermersch, T. M. Aisonobe, J. Valls-SoIè, H. Staunton, J. Dichgans, R. Karabudak, I. Dones, G. Porta, E. Janssens, Maria Martinez, J. M. Fernandez-Real, R. Villagra, Y. Yoshino, C. Kabus, K. Schimrigk, I. Girard-Buttaz, F. Piccoli, F. Aichner, P. Zuchegna, S. M. Al Deeb, F. Bono, N. Busquets, A. Jobert, Patrizia Ciscato, M. Martin, L. Polman, S. Darbra, V. Le Cam-Duchez, F. Baldissera, B. Baykan-Kurt, D. Guez, M. Bratoeva, H. Matsui, M. Mila, H. Perron, L. Bjorge, G. Husby, Steven T. DeKosky, D. R. Cornblath, J. M. Gabriel, J. J. Poza, Y. Wu, A. Toscano, R. P. Kleyweg, J. Kuhnen, S. O. Confort-Gouny, A. Barcelo, A. M. Conti, C. Fiol, C. Steichen-Wiehn, J. Rodes, M. Cavenaile, C. Vedeler, M. Drlicek, C. Argentino, M. L. Peris, A. Cervello, A. Z. GinaÏ, S. Yancheva, D. Passingham, S. Aoba, D. L. 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Rosenberg, J. Patrignani, R. Vichi, Martin R. Farlow, J. Roquer, L. Krols, M. Pimenta, C. Bucka, U. Klose, M. Roberts, J. Salas-Puig, R. Ghnassia, A. Mercuri, C. Maltempo, I. Tournev, P. Homeyer, D. Caparros-Lefevre, E. P. O. Sullivan, T. Vashadze, Ph. Lyrer, A. Deltoro, H. Kondo, M. Steinling, A. Graham, G. C. Miescher, A. Pace, D. Branca, G. Avello, H. H. Kornhuber, D. Fernandes, H. Friedrich, R. Chorao, H. O. Lüders, R. T. Bax, J. A. Macias, N. Yilmaz, J. Veroust, M. Miller, S. Confort-Gouny, J. L. Sastre, D. Servello, G. Boysen, S. Koeppen, V. Planté-Bordeneuve, H. Albrecht, R. H. M. King, G. Orkodashili, R. Doornbos, H. Toyooka, V. Larrue, M. Sabatelli, K. Williams, M. Stevens, V. Maria, M. Comabella, C. Lammers, R. M. L. Poublon, E. Tizzano, P. Pazzaglia, F. Zoeller, M. B. Delisle, J. P. Goument, J. M. Minderhoud, A. Sghirlanzoni, V. Meininger, M. Al Deeb, C. Bertelt, A. Cagni, A. Algra, F. Morales, K. A. Flugel, M. Maidani, M. Noya, Z. Seidl, U. Roelcke, D. Cannata, E. 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Henderson, R. Massa, A. Cruz Martinez, U. Liska, F. Hecht, Ernst Holler, V. S. de Bruin, B. B. Sheitman, S. M. Bentzen, C. Bayindir, F. Pallesta, P. E. Roland, J. Parrilla, P. Zunker, L. F. Burchinskaya, G. Mellino, S. Ben Ayed, D. Bonneau, P. Nowacki, M. Goncalves, P. Riederer, N. Mavroudakis, J. Togores, L. Rozewicz, S. Robeck, Y. Perez Gilabert, L. Rampello, A. Rogopoulos, S. Martinez, F. Schildermans, C. Radder, P. B. Hedlund, J. Cambier, M. Aabed, G. D. Jackson, P. Gasparini, P. Santacruz, J. Vandevivere, H. Dural, A. Mantel, W. Dorndorf, N. Ediboglu, A. Lofgren, J. Bogousslavsky, P. Thierauf, L. Goullard, R. Maserati, B. Moering, M. Ryba, J. Serra, G. G. Govan, A. Pascual-Leone, S. Schaeffer, M. R. Rosenfeld, A. P. Correia, K. Ray Chaudhuri, L. Campbell, R. Spreafico, B. Genetet, A. M. Tantot, R. A. G. Hughes, J. A. Vidal, G. Erkol, J. Y. Delattre, B. Yaqub, B. K. Hecht, E. Mayayo, Ph. Scheltens, J. Corral, M. Calaf, L. Henderson, C. Y. Li, U. Bogdahn, R. Sanchez-Roy, M. Navasa, J. Ballabriga, G. Broggi, T. Gudeva, C. Rose, J. Vion-Dury, J. A. Gastaut, J. Pniewski, Nicola J. Robertson, G. Kohncke, M. Billot, S. Gok, E. Castellli, F. Denktas, P. Bazzi, F. Spinelli, I. F. Moseley, C. D. Mardsen, B. Barbiroli, O. M. Koriech, A. Miller, Hiroaki Yoshikawa, F. X. Borruat, J. Zielasek, P. Le Coz, J. Pascual, A. Drouet, L. T. Giron, F. Schondube, R. Midgard, M. Alizadeh, M. Liguori, Lionel Ginsberg, L. Harms, C. Tilgner, G. Tognoni, F. Molteni, Mar Tintoré, M. Psylla, C. Goulon-Goeau, M. V. Aguilar, Massimo Filippi, K. H. Mauritz, Thomas V. Fernandez, C. Basset, S. Rossi, P. Meneses, B. Jandolo, T. Locatelli, D. Shechtcr, C. Magnani, R. Ferri, Bruno Dubois, J. M. Warier, S. Berges, F. Idiman, M. Schabet, R. R. Diehl, P. D'aurelio, M. Musior, Reinhard Hohlfeld, P. Smeyers, M. Olivé, A. Riva, C. A. Broere, N. Egund, S. Franceschetti, V. Bonavita, Nicola Canal, E. Timmermans, M. Ruiz, S. Barrandon, G. Vasilaski, B. Deweer, L. Galiano, S. F. T. M. de Bruijn, L. Masana, A. Goossens, B. Heye, K. Lauer, Heinz Gregor Wieser, Stephen R. Williams, B. Garavaglia, A. P. Sempere, F. Grigoletto, P. Poindron, R. Lopez-Pajares, I. Leite, T. A. McNell, C. Caucheteur, J. M. Giron, A. D. Collins, P. Freger, J. Sanhez Del Rio, D. A. Harn, K. Lindner, S. S. Scherer, G. Serve, M. Juncadella, X. Estivill, R. Binkhorst, M. Anderson, B. Tekinsoy, C. Sagan, T. Anastopoulos, G. Japaridze, S. Guillou, F. Erminio, Jon Sussman, P. G. Oomes, D. S. Rust, S. Mascheroni, O. Berger, M. Peresson, K. V. Toyka, T. W. Polder, M. Huberman, B. Arpaci, H. Ramtami, I. Martinez, Ph. Violon, P. P. Gazzaniga Pozzill, R. Ruda, P. Auzou, J. Parker, S. P. Morrissey, Jiahong Zhu, F. Rotondi, P. Baron, W. Schmid, P. Doneda, M. Spadaro, M. C. Nargeot, I. Banchs, J.S.P. van den Berg, R. Ferrai, M. Robotti, M. Fredj, Pedro M. Rodríguez Cruz, B. Erne, D. G. Piepgras, M. C. Arne-Bes, J. Escudero, C. Goetz, A. R. Naylor, M. Hallett, O. Abramsky, E. Bonifacio, L. E. Larsson, R. Pellikka, P. Valalentino, D. Guidetti, B. Buchwald, C. H. Lücking, D. Gauvreau, F. Pfaff, A. Ben Younes-Chennoufi, R. Kiefer, R. Massot, K. A. Hossmann, L. Werdelin, P. J. Baxter, U. Ziflo, S. Allaria, C. D. Marsden, M. Cabaret, S. P. Mueller, E. Calabrese, R. Colao, S. I. Bekkelund, M. Yilmaz, O. Oktem-Tanor, R. Gine, M. E. Scheulen, J. Beuuer, A. Melo, Z. Gulay, M. D. Have, C. Frith, D. Liberati, J. Gozlan, P. Rondot, Ch. Brunholzl, M. Pocchiari, J. Pena, L. Moiola, C. Salvadori, A. Cabello, T. Catarci, S. Webb, C. Dettmers, N. A. Gregson, Alexandra Durr, F. Iglesias, U. Knorr, L. Ferrini-Strambi, F. Kruggel, P. Allard, A. Coquerel, P. Genet, F. Vinuels, C. Oberwittler, A. Torbicki, P. Leffers, B. Renault, B. Fauser, C. Ciano, G. Uziel, J. M. Gibson, F. Anaya, C. Derouesné, C. N. Anagnostou, M. Kaido, W. Eickhoff, G. Talerico, M. L. Berthier, A. Capdevila, M. Alons, D. Rezek, E. Wondrusch, U. Kauerz, D. Mateo, M. A. Chornet, Holon, N. Pinsard, I. Doganer, E. Paoino, H. Strenge, C. Diaz, J. R. Brasic, W. Heide, I. Santilli, W. M. Korn, D. Selcuki, M. J. Barrett, D. Krieger, T. Leon, T. Houallah, M. Tournilhac, C. Nos, D. Chavot, F. Barbieri, F. J. Jimenez-Jimenez, J. Muruzabal, K. Poeck, A. Sennlaub, L. M. Iriarte, L. G. Lazzarino, C. Sanz, P. A. Fischer, S. D. Shorvon, R. Hoermann, F. Delecluse, M. Krams, O. Corabianu, F. H. Hochberg, Christopher J. Mathias, B. Debachy, C. M. Poser, L. Delodovici, A. Jimenez-Escrig, F. Baruzzi, F. Godenberg, D. Cucinotta, P. J. Garcia Ruiz, K. Maier-Hauff, P. R. Bar, R. Mezt, R. Jochens, S. Karakaneva, C. Roberti, E. Caballero, Joseph E. Parisi, M. Zamboni, T. Lacasa, B. Baklan, J. C. Gautier, J. A. Martinez-Matos, W. Pollmann, G. Thomas, L. Verze, E. Chleide, R. Alvarez Sala, I. Noel, E. Albuisson, O. Kastrup, S. I. Rapoport, H. J. Braune, H. Lörler, M. Le Merrer, A. Biraben, S. Soler, S. J. Taagholt, U. Meyding-Lamadé, K. Bleasdale-Barr, Isabella Moroni, Y. Campos, J. Matias-Guiu, G. Edan, M. G. Bousser, John B. Clark, J. Garcia de Yebenes, N. K. Olsen, P. Hitzenberger, S. Einius, Aj Thompson, Ch. J. Vecht, T. Crepin-Leblond, Klaus L. Leenders, A. Di Muzio, L. Georgieva, René Spiegel, K. Sabey, D. Ménégalli, J. Meulstee, U. Liszka, P. Giral, C. Sunol, J. M. Espadaler, A. D. Crockar, K. Varli, G. Giraud, P. J. Hülser, A. Benazzouz, A. Reggio, M. Salvatore, K. Genc, M. Kushnir, S. Barbieri, J. Ph. Azulay, M. Gianelli, N. Bathien, A. AlMemar, F. Hentati, I. Ragueneau, F. Chiarotti, R. C. F. Smits, A. K. Asbury, F. Lacruz, B. Muller, Alan J. Thompson, Gordon Smith, K. Schmidt, C. Daems Monpeun, Juergen Weber, A. Arboix, G. R. Fink, A. M. Cobo, M. Ait Kaci Ahmed, E. Gencheva, Israel-Biet, G. Schlaug, P. De Jonghe, Philip Scheltens, K. Toyka, P. Gonzalez-Porque, A. Cila, J. M. Fernandez, P. Augustin, J. Siclia, S. Medaglini, D. E. Ziogas, A. Feve, L. Kater, G. J. E. Rinkel, D. Leppert, Rüdiger J. Seitz, S. Ried, C. Turc-Carel, G. Smeyers, F. Godinho, M. Czygan, M. Rijntjes, E. Aversa, M. Frigo, Leif Østergaard, J. L. Munoz Blanco, A. Cruz-Matinez, J. De Reuck, C. Theillet, T. Barroso, V. Oikonen, Florence Lebert, M. Kilinc, C. Cordon-Cardon, G. Stoll, E. Thiery, F. Pulcinelli, J. Solski, M. Schmiegelow, L. J. Polman, P. Fernandez-Calle, C. Wikkelso, M. Ben Hamida, M. Laska, E. Kott, W. Sulkowski, C. Lucas, N. M. Bornstein, D. Schmitz, M. W. Lammers, A. de Louw, R. J. S. Wise, P. A. van Darn, C. Antozzi, P. Villanueva, P. H. E. Hilkens, C. Constantin, W. Ricart, A. Wolf, M. Gamba, P. Maguire, Alessandro Padovani, B. M. Patten, Marie Sarazin, H. Ackermann, L. Durelli, S. Timsit, Sebastian Jander, B. W. Scheithauer, G. Demir, J. P. Neau, P. Barbanti, A. Brand, N. AraÇ, V. Fischer-Gagnepain, R. Marchioli, G. Serratrice, C. Maugard-Louboutin, G. T. Spencer, D. Lücke, G. Mainardi, K. Harmant Van Rijckevorsel, G. B. Creel, R. Manzanares, Francesco Fortunato, A. May, J. Workman, K. Johkura, E. Fernandez, Carlo Colosimo, L. Calliauw, L. Bet, Félix F. Cruz-Sánchez, M. Dhib, H. Meinardi, F. Carrara, J. Kuehnen, C. Peiro, H. Lassmann, K. Skovgaard Olsen, A. McDonald, L. Sciulli, A. Cobo, A. Monticelli, B. Conrad, J. Bagunya, J. Benitez, V. Desnizza, B. Dupont, O. Delrieu, D. Moraes, J. J. Heimans, F. Garcia Rio, M. Matsumto, A. Fernandez, R. Nermni, R. Chalmers, M. J. Marchau, F. Aguado, P. Velupillai, P. J. Martin, P. Tassan, V. Demarin, A. Engelien, T. Gerriets, Comar, J. L. Carrasco, J. P. Pruvo, A. Lopez de Munain, D. Pavitt, J. Alarcon, Chris H. Polman, B. Guldin, N. Yeni, Hartmut Brückmann, N. Wilczak, H. Szwed, R. Causaran, G. Kyriazis, M. E. Westarp, M. Gasparini, N. Pecora, J. M. Roda, E. Lang, V. Scaioli, David R. Fish, D. Caputo, O. Gratzl, R. Mercelis, A. Perretti, G. Steimetz, I. Link, C. Rigoletto, A. Catafau, G. Lucotte, M. Buti, G. Fagiolari, A. Piqueras, C. Godinot, J. C. Meurice, Erodriguez J. Dominigo, F. Lionnet, H. Grzelec, David J. Brooks, P. M. G. Munro, F. X. Weilbach, M. Maiwald, W. Split, B. Widjaja-Cramer, V. Ozturk, J. Colas, E. Brizioli, J. Calleja, L. Publio, M. Desi, R. Soffietti, P. Cortinovis-Tourniaire, E. F. Gonano, G. Cavaletti, S. Uselli, K. Westerlind, H. Betuel, C. O. Dhiver, H. Guggenheim, M. Hamon, R. Fazio, P. Lehikoinen, A. Esser, B. Sadzot, G. Fink, Angelo Antonini, D. Bendahan, V. Di Carlo, G. Galardi, A. F. Boller, M. Aksenova, Del Fiore, V. de la Sayette, H. Chabriat, A. Nicoletti, A. Dilouya, M. L. Harpin, E. Rouillet, J. Stam, A. Wolters, M. R. Delgado, Eduardo Tolosa, G. Said, A. J. Lees, L. Rinaldi, A. Schulze-Bonhage, MA Ron, C. Lefebvre, E. W. Radü, R. Alvarez, M. L. Bots, P. Reganati, S. Palazzi, A. Poggi, N. J. Scolding, V. Sazdovitch, T. Moreau, E. Maes, M. A. Estelies, P. Petkova, Jose-Felix Marti-Masso, G De La Meilleure, N. Mullatti, M. Rodegher, N. C. Notermans, T. A. T. Warner, S. Aktan, J. P. Louboutin, L. Volpe, C. Scheidt, W. Aust, C. M. Wiles, U. Schneider, S. K. Braekken, W. R. Willems, K. Usuku, Peter M. Rothwell, C. Talamon, M. L. Sacchetti, A. Codina, M. H. Marion, A. Santoro, J. Roda, A. Bordoni, D. J. Taylor, S. Ertas, H. H. Emmen, J. Vichez, V. BesanÇon, R. E. Passingham, M. L. Malosio, A. Vérier, M. Bamberg, A. W. Hansen, E. Mostacero, G. Gaudriault, Marie Vidailhet, B. Birebent, K. Strijckmans, F. Giannini, T. Kammer, I. Araujo, J. Nowicki, E. Nikolov, A. Hutzelmann, R. Gherardi, J. Verroust, L. Austoni, A. Scheller, A. Vazquez, S. Matheron, H. Holthausen, J. M. Gerard, M. Bataillard, S. Dethy, V. H. Patterson, V. Ivanez, N. P. Hirsch, F. Ozer, M. Sutter, C. Jacomet, M. Mora, Bruno Colombo, A. Sarropoulos, T. H. Papapetropoulos, M. Schwarz, D. S. Dinner, N. Acarin, B. Iandolo, J. O. Riis, P. R. J. Barnes, F. Taroni, J. Kazenwadel, L. Torre, A. Lugaresi, I. L. Henriques, S. Pauli, S. Alfonso, Pedro Quesada, A. S. T. Planting, J. M. Castilla, Thomas Gasser, M. Van der Linden, A. Alfaro, E. Nobile-Orazio, G. Popova, W. Vaalburg, F. G. A. van der Mech, L. Williams, F. Medina, J. P. Vernant, J. Yaouanq, B. Storch-Hagenlocher, A. Potemkowski, R. Riva, M. H. Mahagne, M. Ozturk, Ve. Drory, N. Konic, C. Jungreis, A. Pou Serradell, J. L. Gauvrit, G. J. Chelune, S. Hermandez, T. Dingus, L. Hewer, Ch. Koch, M. N. Metz-Lutz, G. Parlato, M. Sinaki, Charles Pierrot-Deseilligny, H. C. Diener, J. Broeckx, J. Weill-Fulazza, M. L. Villar, M. Rizzo, O. Ganslandt, C. Duran, N. A. Fletcher, G. Di Giovacchino, Susan T. Iannaccone, C. Kolig, N. Fabre, H. A. Crockard, Rita Bella, M. Tazir, E. Papagiannuli, K. Overgaard, Emma Ciafaloni, I. Lorenzetti, F. Viader, P. A. H. Millac, I. Montiel, L. H. Visser, M. Palomar, P. L. Murgia, H. Pedersen, Rafael Blesa, S. Seddigh, W. O. Renier, I. Lemahieu, H. M. L. Jansen, L. Rosin, J. Galofre, K. Mattos, M. Pondal, G. M. Hadjigeorgiou, D. Francis, L. Cantin, D. Stegeman, M. Rango, A. B. M. F. Karim, S. Schraff, B. Castellotti, I. Iriarte, E. Laborde, T. J. Tjan, R. Mutani, D. Toni, B. Bergaasco, J. G. Young, C. Klotzsch, A. Zincone, X. Ducrocq, M. Uchuya, O. J. Kolar, A. Quattrone, T. Bauermann, Nereo Bresolin, J. Vallée, B. C. Jacobs, A. Campos, Werner Poewe, J. A. Villanueva, A. W. Kornhuber, A. Malafosse, E. Diez-Tejedor, G. Jungreia, M. J. A. Puchner, A. Komiyama, O. Saribas, V. Volpini, L. Geremia, S. Bressi, A. Nibbio, Timothy E. Bates, T. z. Tzonev, E. Ideman, G. A. Damlacik, G. Martino, G. Crepaldi, T. Martino, Kjell Någren, E. Idiman, D. Samuel, J. M. Perez Trullen, Y. van der Graaf, J. O. Thorell, M. J. M. Dupuis, E. Sieber, R. D'Alessandro, C. Cazzaniga, J. Faiss, A. Tanguy, A. Schick, I. Hoksergen, A. Cardozo, R. Shakarishvili, G. K. Wennlng, J. L. Marti-Vilalta, J. Weissenbach, I. L. Simone, Amalia C. Bruni, Darius J. Adams, C. Weiller, A. Pietrangeli, F. Croria, C. Vigo-Pelfrey, Patricia Limousin, A. Ducros, G. Conti, O. Lindvall, E. Richter, M. Zuffi, A. Nappo, T. Riise, J. Wijdenes, M. J. Fernandez, J. Rosell, P. Vermersh, S. Servidei, M. S. C. Verdugo, F. Gouttiere, W. Solbach, M. Malbezin, I. S. Watanabe, A. Tumac, W. I. McDonald, D. A. Butterfield, P. P. Costa, F. deRino, F. Bamonti, J. M. Cesar, C. H. Lahoz, I. Mosely, M. Starck, M. H. Lemaitre, K. M. Stephan, S. Tex, R. Bokonjic, I. Mollee, L. Pastena, M. Gutierrez, F. Boiler, M. C. Martinez-Para, M. Velicogna, O. Obuz, A. Grinspan, M. Guarino, L. M. Cartier, E. Ruiz, D. Gambi, S. Messina, M. Villa, Michael G. Hanna, J. Valk, Leone Pascual, M. Clanet, Z. Argov, B. Ryniewicz, E. Magni, B. Berlanga, K. S. Wong, C. Gellera, C. Prevost, F. Gonzalez-Huix, R. Petraroli, J. E. G. Benedikz, I. Kojder, C. Bommelaer, L. Perusse, M. R. Bangioanni, Guy M. McKhann, A. Molina, C. Fresquet, E. Sindern, Florence Pasquier, M. J. Rosas, M. Altieri, O. Simoncini, M. Koutroumanidis, C. A. F. Tulleken, M. Dary-Auriol, S. Oueslati, H. Kruyer, I. Nishisho, C. R. Horning, A. Vital, G. V. Czettritz, J. Ph. Neau, B. Mihout, A. Ameri, M. Francis, S. Quasthoff, D. Taussig, S. Blunt, P. Valentin, C. Y. Gao, O. Heinzlef, H. d'Allens, C. Coudero, M. Erfas, G. Borghero, P. J. Modrego Pardo, M. C. Patrosso, N. L. Gershfeld, P. A. J. M. Boon, O. Sabouraud, M. Lara, J. Svennevig, G. L. Lenzi, A. Barrio, H. Villaroya, JosÇ M. Manubens, O. Boespflug-Tanguy, M. Carreras, D. A. Costiga, J. P. Breux, S. Lynn, C. Oliveras Ley, A. G. Herbaut, J. Nos, C. Tornali, Y. A. Hekster, J. L. Chopard, J. M. Manubens, P. Chemouilli, A. Jovicic, F. Dworzak, S. Smirne, S. E. Soudain, B. Gallano, D. Lubach, G. Masullo, G. Izquierdo, A. Pascual Leone Pascual, A. Sessa, V. Freitas, O. Crambes, L. Ouss, G. W. Van Dijk, P. Marchettini, P. Confalonieri, M. Donaghy, A. Munnich, M. Corbo, and M. E. L. van der Burg

Subjects
Neurology, business.industry, Media studies, Library science, Medicine, Neurology (clinical), business
Published
1994

5. Apparent absence of glycogen branching enzyme activity in phosphofructokinase deficiency

Author

V. Barash, S. Lilling, Z. Argov, and R. Fischer

Subjects
Adult, medicine.medical_specialty, Phosphofructokinase-1, Glycogen branching enzyme activity, Glycogen debranching enzyme, chemistry.chemical_compound, 1,4-alpha-Glucan Branching Enzyme, Internal medicine, Genetics, medicine, Glycogen branching enzyme, Humans, False Positive Reactions, Centrifugation, Phosphofructokinase 1, Genetics (clinical), chemistry.chemical_classification, biology, Glycogen, Chemistry, Muscles, medicine.disease, Phosphofructokinase deficiency, Kinetics, Enzyme, Endocrinology, Biochemistry, biology.protein, Female
Abstract

A 30-year-old woman with clinical features and biochemical findings of muscle phosphofructokinase deficiency was found to have a very low level of alpha-1,4-glucan:alpha-1,4-glucan-6-transglucosylase (branching enzyme, EC 2.4.1.18) activity in muscle. In contrast, branching enzyme activity in the leukocytes was in the range of control values. After sedimentation of the glycogen from muscle homogenates by centrifugation at 105,000 g, branching enzyme activity in muscle of the patient was similar to that of control subjects. This patient illustrates the possibility of falsely diagnosing branching enzyme deficiency when muscle glycogen content is elevated. It is likely that such an artefact may also cause a false positive diagnosis of branching enzyme deficiency in other metabolic diseases associated with glycogen accumulation.

Published
1991

7. Characterization of Blood Glucose & Glycosuria following IV Trehalose (Cabaletta) administration for OPMD

Author

I. Gliko-Kabir, H. Vornovitzki, S. Blotnick, Yoseph Caraco, and Z. Argov

Subjects
Pharmacology, Glycosuria, medicine.medical_specialty, Hexokinase, business.industry, Urinary system, Therapy Trial, Urine, Trehalose, chemistry.chemical_compound, Endocrinology, Biochemistry, chemistry, Internal medicine, medicine, Pharmacology (medical), Trehalase, medicine.symptom, Urine sample, business
Abstract

CharaCTerizaTioN of Blood GluCoSe & GlyCoSuria followiNG iV TrehaloSe (CaBaleTTa) admiNiSTraTioN for oPmd H. Vornovitzki; S. Blotnick; I. Gliko-Kabir; Z. Argov; and Y. Caraco Clinical Pharmacology Unit, Hadassah University Hospital, Jerusalem, Israel; and Bioblast-Pharma Ltd., Tel-Aviv, Israel Background: Trehalose is a naturally occurring disaccharide that is currently being evaluated for the treatment of Oculopharyngeal Muscular Dystrophy (OPMD) and spinocerebellar ataxia 3. Objective: The purpose of this sub-study (part of OPMD phase 2 therapy trial) was to evaluate the extent of increase in blood glucose and the extent and duration of glycosuria associated with the intravenous administration of Trehalose. Methods: Fourteen OPMD patients received weekly intravenous 30 gr trehalose over a period of approximately 80 minutes. Just prior to and following the administration of the 3rd dose, blood were taken periodically over 5 hours for the evaluation of plasma concentration of trehalose using a validated high performance liquid chromatographic tandem mass spectrometry. On a different dosing day, plasma and urine samples were taken periodically over 8 hours for the evaluation of glucose concentration using hexokinase enzyme method on a chemistry analyzer. Results: Plasma concentration of trehalose exhibited more than 2-fold variation with AUC0→ 5 ranging from 2698 to 6103 μg*h/mL. A mild rise in plasma glucose was noted in 11 patients reaching a peak of (mean ± SD) 11.28 ± 7.27 mg% within 1-2 hours following Trehalose infusion. The rise in blood glucose was transient and it reverted back to pre-Trehalose concentrations within 1-4 hours (2.10 ± 1.29 hours). Some degree of glycosuria was noted in all patients. Maximal glucose urinary concentration varied ranging from 42 mg% and up to 1031 mg% (358 ± 341 mg%). Peak glycosuria was noted within 1-3 hours and it was evident only in 1 patient in the urine sample taken after 8 hours. Conclusions: The intravenous administration of 30 gr trehalose (Cabaletta) is associated with a subtle rise in blood glucose concentration and transient glycosuria. The presence of urinary glucose represents most probably in-situ renal activity of trehalase.

Published
2015

8. Insights into muscle diseases gained by phosphorus magnetic resonance spectroscopy

Author

Z, Argov, M, Löfberg, and D L, Arnold

Subjects
Magnetic Resonance Spectroscopy, Muscular Diseases, Humans, Phosphorus, Energy Metabolism, Exercise
Abstract

Phosphorus magnetic resonance spectroscopy (P-MRS) has now been used in the investigation of muscle energy metabolism in health and disease for over 15 years. The present review describes the basics of the metabolic observations made by P-MRS including the assumptions and problems associated with the use of this technique. Extramuscular factors, which may affect the P-MRS results, are detailed. The important P-MRS observations in patients with mitochondrial myopathies, including the monitoring of experimental therapies, are emphasized. The findings in other metabolic myopathies (those associated with glycolytic defects or endocrine disturbances) and in the destructive myopathies (the dystrophies and the inflammatory myopathies) are also described. Observations made in normal and abnormal fatigue, fibromyalgia, and malignant hyperthermia are considered. Finally, a summary of the possible diagnostic use of P-MRS in exercise intolerance is provided.

Published
2000

9. Muscular dystrophy due to dysferlin deficiency in Libyan Jews. Clinical and genetic features

Author

Z, Argov, M, Sadeh, K, Mazor, D, Soffer, E, Kahana, I, Eisenberg, S, Mitrani-Rosenbaum, I, Richard, J, Beckmann, S, Keers, R, Bashir, K, Bushby, and H, Rosenmann

Subjects
Adult, Family Health, Male, Electromyography, Biopsy, DNA Mutational Analysis, Homozygote, Membrane Proteins, Muscle Proteins, Hypertrophy, Libya, Middle Aged, Muscular Dystrophies, Pedigree, Jews, Humans, Female, Muscle, Skeletal, Dysferlin, Polymorphism, Single-Stranded Conformational
Abstract

The cluster in Jews of Libyan origin of limb-girdle muscular dystrophy type 2B due to a dysferlin 1624delG mutation is described. The carrier frequency of this mutation is calculated to be approximately 10% in this population, in which the disease prevalence is at least 1 per 1300 adults. Twenty-nine patients from 12 families were all homozygous for the same mutation. However, clinical features were heterogeneous even within the same family: in half of the patients onset was in the distal muscles of the legs, which is similar to Miyoshi myopathy, while in others onset was in the proximal musculature, which is similar to other forms of limb-girdle dystrophies. Age at onset varied from 12 to 28 years (mean 20.3 +/- 5.5 years). One patient was presymptomatic at age 28 years. Progression was slow regardless of age of onset, patients remaining ambulatory until at least 33 years. Five patients described subacute, painful enlarged calves as an early, unusual feature. The variable features in this ethnic cluster contribute to the definition of the clinical spectrum of dysferlinopathies in general. The cause of the observed heterogeneity remains unclear.

Published
2000

10. Muscle phosphorus magnetic resonance spectroscopy oxidative indices correlate with physical activity

Author

M C, Tartaglia, J T, Chen, Z, Caramanos, T, Taivassalo, D L, Arnold, and Z, Argov

Subjects
Adult, Male, Magnetic Resonance Spectroscopy, Phosphocreatine, Rest, Phosphorus, Middle Aged, Mitochondria, Muscle, Phosphates, Adenosine Diphosphate, Physical Fitness, Humans, Female, Muscle, Skeletal, Exercise, Oxidation-Reduction, Phosphorus Radioisotopes
Abstract

The purpose of this study was to assess the effect of physical deconditioning on skeletal muscle's oxidative metabolism as evaluated by phosphorus-31 magnetic resonance spectroscopy ((31)P MRS). Twenty-seven subjects without muscle disease, representing a wide range of fitness levels, were evaluated with (31)P MRS. Spectra were obtained at rest and during recovery from in-magnet exercise. The data show a significant correlation between maximum resting metabolic equivalent (MET) score and the following (31)P MRS recovery indices: adenosine diphosphate and phosphocreatine recovery half-time; initial phosphocreatine resynthesis rate; calculated estimation of mitochondrial capacity; pH at end of exercise; and phosphocreatine depletion. In addition, significant differences between the deconditioned and conditioned group were found for all of the aforementioned recovery indices. At rest, only the inorganic phosphate concentration was significantly different between the two groups. These data indicate that physical activity level should be taken into account when assessing patients' oxidative metabolism with (31)P MRS.

Published
2000

11. Fitting cytosolic ADP recovery after exercise with a step response function

Author

J T, Chen, Z, Argov, R E, Kearney, and D L, Arnold

Subjects
Adult, Male, Magnetic Resonance Spectroscopy, Time Factors, Physical Exertion, Mitochondrial Myopathies, Phosphorus Isotopes, Middle Aged, Mitochondria, Muscle, Adenosine Diphosphate, Cytosol, Oxygen Consumption, Ischemia, Regional Blood Flow, Linear Models, Humans, Female, Muscle, Skeletal, Oxidation-Reduction, Algorithms
Abstract

Phosphorus magnetic resonance (MR) spectroscopy was used to measure the recovery kinetics of calculated cytoplasmic metabolically active adenosine diphosphate (ADP) after exercise in normal subjects and patients with mitochondrial myopathies. These kinetics have previously been fitted with a single exponential function, despite a complex time-dependent undershoot in many subjects. By considering the transition from ischemic-exercise to perfused-recovery as a step function input, a second-order linear system was developed yielding a step response function to fit the ADP recovery. Using this method, an average improvement in fit of 23% resulted in a significant improvement in the characterization of ADP recovery for all normal subjects with substantial undershoot. The patient group had a comparable improvement in fit of 11%. Fitting the ADP recovery with a second-order step response function can provide significantly better characterization of muscle oxidative metabolism in vivo.

Published
1999

12. Intracellular phosphates in inclusion body myositis - a 31P magnetic resonance spectroscopy study

Author

Z, Argov, T, Taivassalo, N, De Stefano, A, Genge, G, Karpati, and D L, Arnold

Subjects
Aged, 80 and over, Inclusion Bodies, inorganic phosphate, Physical Exertion, Middle Aged, magnetic resonance spectroscopy, Oxidative Phosphorylation, Mitochondria, Myositis, Inclusion Body, Phosphates, inclusion body myopathy, magnetic resonance spectroscopy, mitochondrial disorders, oxidative metabolism, inorganic phosphate, mitochondrial disorders, inclusion body myopathy, oxidative metabolism, Muscle Fatigue, Humans, Muscle, Skeletal, Radionuclide Imaging, Phosphorus Radioisotopes, Aged
Abstract

Muscle phosphorus magnetic resonance spectroscopy was used to study oxidative metabolism at rest and during recovery from exercise in 7 patients with sporadic inclusion body myositis (s-IBM), compared with normal controls (n=8) and mitochondrial myopathies (n=20). At rest, 6/7 patients had elevated inorganic phosphates. Recovery parameters were not different from controls, in contrast with mitochondrial myopathies, who showed abnormal rest and recovery. The normal recovery suggests that mitochondrial oxidative capacity is not impaired in s-IBM.

Published
1998

13. Biochemical and genetic studies in a family with mitochondrial myopathy

Author

T D, Heiman-Patterson, Z, Argov, J M, Chavin, B, Kalman, H, Alder, S, DiMauro, W, Bank, and A J, Tahmoush

Subjects
Adult, Gene Rearrangement, Male, Polymorphism, Genetic, Adolescent, Muscles, Mitochondrial Myopathies, Middle Aged, DNA, Mitochondrial, Methylprednisolone, Pedigree, Microscopy, Electron, Child, Preschool, Mutation, Humans, Female, Child, Glucocorticoids
Abstract

We present a family with severe exercise intolerance, progressive proximal weakness, and lactic acidemia. Fifteen of 24 family members in five generations were affected. Since the affected males do not have offspring at this time, the family pedigree is consistent with either maternal or autosomal dominant inheritance. Muscle histochemistry showed ragged-red fibers and electron microscopy showed globular mitochondrial inclusions. Biochemical analysis showed reduced muscle activities of mitochondrial NADH-cytochrome c reductase (1 of 2 patients), succinate-cytochrome c reductase (2 patients), and cytochrome c oxidase (2 patients). For 1 patient, sequence analysis of 44% of the muscle mitochondrial DNA including all 22 transfer RNA regions showed no point mutation with pathogenic significance. Southern blot analysis showed no deletion. Six affected members of the family were treated with methylprednisolone (0.25 mg/kg) for 3 months. Muscle strength, serum lactate, and energy metabolism at rest (measured by 31P magnetic resonance spectroscopy) significantly improved with treatment.

Published
1997

14. Abnormal oxidative metabolism in exercise intolerance of undetermined origin

Author

Z, Argov, N, De Stefano, T, Taivassalo, J, Chen, G, Karpati, and D L, Arnold

Subjects
Adult, Male, myalgia, Adolescent, exercise, mitochondrial myopathies, Middle Aged, phosphorus magnetic resonance spectroscopy, Mitochondria, Adenosine Diphosphate, Muscular Diseases, metabolic myopathies, phosphorus magnetic resonance spectroscopy, exercise, mitochondrial myopathies, myalgia, Humans, Female, metabolic myopathies
Abstract

Twenty-four patients with exercise intolerance of undetermined origin were examined by muscle phosphorus magnetic resonance spectroscopy (31P-MRS) to test for a possible underlying defect in oxidative metabolism. Results were compared to those of 37 normal controls and 22 patients with well-defined mitochondrial disorders. In 17 (71%) patients with exercise intolerance, ADP recovery after exercise, an index of mitochondrial function, was abnormally slow. The energy state of phosphate-containing metabolites at rest was abnormal in 33% of patients. In 17/22 patients with well-defined mitochondrial disorders, ADP recovery was similarly slow. Abnormalities at rest were slightly more prevalent (50%) in this group of patients. Other 31P-MRS measurements did not add to the overall sensitivity in detecting abnormalities in either of these groups. We suggest that many patients with exercise intolerance of undetermined cause may have impaired muscle oxidative metabolism, that is an important causative factor in the pathophysiology of their symptoms.

Published
1997

15. Transient neurological events during dipyridamole stress test: an arterial steal phenomenon?

Author

D, Schechter, M, Bocher, Y, Berlatzky, H, Anner, Z, Argov, G, Beer, Y, Krausz, M S, Gotsman, and R, Chisin

Subjects
Male, Cerebrovascular Disorders, Thallium Radioisotopes, Hand Strength, Humans, Coronary Disease, Heart, Dipyridamole, Middle Aged, Nervous System Diseases, Radionuclide Imaging, Exercise, Aged
Abstract

Dipyridamole-associated adverse neurological side effects have not been extensively described. We present two cases of dipyridamole-associated transient motor neurological events with no evidence of residual neurological deficits detected clinically or by head CT. The patients showed no evidence of significant extracranial (internal carotid) artery disease. We propose the presence of a regional cerebral perfusion disturbance due to an intracranial vascular steal phenomenon as the mechanism for the above side effects of dipyridamole.

Published
1994

16. Common mutations in the phosphofructokinase-M gene in Ashkenazi Jewish patients with glycogenesis VII--and their population frequency

Author

J B, Sherman, N, Raben, C, Nicastri, Z, Argov, H, Nakajima, E M, Adams, C M, Eng, T M, Cowan, and P H, Plotz

Subjects
Adult, Male, Phosphofructokinase-1, DNA Mutational Analysis, Molecular Sequence Data, Russia, Gene Frequency, Humans, Point Mutation, Amino Acid Sequence, Frameshift Mutation, Aged, Glycogen Storage Disease Type VII, Base Sequence, Genetic Carrier Screening, Nucleic Acid Heteroduplexes, Exons, Middle Aged, Blotting, Northern, Introns, United States, Isoenzymes, Jews, Mutation, Electrophoresis, Polyacrylamide Gel, Female, Poland, Research Article
Abstract

Phosphofructokinase (PFK) catalyzes the rate-limiting step of glycolysis. Deficiency of the muscle enzyme is manifested by exercise intolerance and a compensated hemolytic anemia. Case reports of this autosomal recessive disease suggest a predominance in Ashkenazi Jews in the United States. We have explored the genetic basis for this illness in nine affected families and surveyed the normal Ashkenazi population for the mutations we have found. Genomic DNA was amplified using PCR, and denaturing gradient-gel electrophoresis was used to localize exons with possible mutations. The polymorphic exons were sequenced or digested with restriction enzymes. A previously described splicing mutation, delta 5, accounted for 11 (61%) of 18 abnormal alleles in the nine families. A single base deletion leading to a frameshift mutation in exon 22 (delta C-22) was found in six of seven alleles. A third mutation, resulting in a nonconservative amino acid substitution in exon 4, accounted for the remaining allele. Thus, three mutations could account for all illness in this group, and two mutations could account for 17 of 18 alleles. In screening 250 normal Ashkenazi individuals for all three mutations, we found only one delta 5 allele. Clinical data revealed no correlation between the particular mutations and symptoms, but male patients were more symptomatic than females, and only males had frank hemolysis and hyperuricemia. Because PFK deficiency in Ashkenazi Jews is caused by a limited number of mutations, screening genomic DNA from peripheral blood for the described mutations in this population should enable rapid diagnosis without muscle biopsy.

Published
1994

17. Werner Schmid, 1930–2002

Author

Z. Liu, H. Starke, C.L. Li, A. Rupprecht, S.L. Ewart, L. Fröhlich, R. Tomasini, D. Gallagher, N. Gmachl, N. Ghaffari-Tabrizi, N.A. Jenkins, T. Leeb, M. Grosz, S. Izraeli, H. Akutsu, C. Steinlein, I. Eisenberg, C. Drögemüller, M. Satoh, W. Engel, I.M. Adham, D.J. Gilbert, K. Ying, H.P. Klinger, Y. Ma, N. Coleman, J. Nowak, T. Yagi, R.L. Prueitt, A. Bernheim, N.B. Atkin, H. Kikuchi, R. Tang, M. Bina, L. Ziercher, T. Osada, N. Dusetti, J. Taylor, E. Antoniou, E.I. Pares-Matos, C. Knorr, D.R. Beier, A. Kuechler, H. Chen, T. Haaf, Y. Mao, W. Feichtinger, D.B. Zimonjic, A. Heller, S.K. Swanson, D.K. Griffin, P. Quignon, B. Bjerkehagen, M. Muenke, M. Shibazaki, N.G. Copeland, H. Kuiper, J.C. Phillips, G. Cao, E. Nacheva, L.A. Azizi Samir, H.-U.G. Weier, M.-G. Mattei, M. Wills-Karp, H. Hochner, D.C. Shing, T.P. Yang, H. Cheng, R. Shamsadin, A.M. Simckes, E. Roessler, D.M. Shubitowski, K. Mrasek, R. Fries, B. Lanske, I. Artner, M.-J. Pébusque, S. Kollers, C.A. Morley-Jacob, A. Dutra, S. Mitrani-Rosenbaum, N. Kleiter, R.A. White, P. Coullin, F. Micci, I. Roberts, A.R. Zinn, N.C. Popescu, S. Heim, R. Yanagimachi, F. Galibert, A. Weise, A. Srisodsai, C. Auffray, S.C. Harvey, B. Brenig, I. Kirsch, X. Ni, Selena Davis, C. Ji, C.L. Keck-Waggoner, B. Perbal, Z.H. Shan, J. Young, Y. Xie, B. Hong, T. Niimi, H. Kaiser, S. Fukushige, D.J. Penman, L.A.P. Carrasco, R.I. Barnes, W.H. Brooks, N.R. Bromage, J. Masabanda, J.-L. Iovanna, G.A. Rohrer, O. Distl, S.G. Rak, M. Schmid, Y. Shiratori, K. Kratochwil, M. Sadeh, H. Kusakabe, U. Claussen, M. Jiang, M.R. Teixeira, Z. Argov, T. Liehr, R. Zoorob, W.H. Reeves, J. Womack, J.D. Karkera, S. Kimura, J.-C. Dagorn, and L. Guo

Subjects
Psychoanalysis, Genetics, Biology, Molecular Biology, Genetics (clinical)
Published
2002

19. Effects of intra-arterial epinephrine on energy metabolism in exercising rabbit gastrocnemius muscle, studied by in vivo phosphorus nuclear magnetic resonance

Author

Z, Argov, S, Nioka, S, Eleff, and B, Chance

Subjects
Magnetic Resonance Spectroscopy, Epinephrine, Injections, Intra-Arterial, Animals, Muscle, Smooth, Phosphorus, Rabbits, Energy Metabolism, Glycolysis
Abstract

Epinephrine has an inotropic effect on skeletal muscle, especially on glycolytic type 2 fibers. The mechanism of this effect is not completely clear and its association with a change in oxidative metabolism or glycolytic activation was not fully investigated. Epinephrine's effects on muscle bioenergetics were studied by in vivo 31P nuclear magnetic resonance to find if mitochondrial metabolism is changed during the inotropic action and if the known glycolytic activation by epinephrine is operative during muscle twitch. The study was also used as a model for the application of in vivo 31P nuclear magnetic resonance in the evaluation of short-term acting drugs. When injected intra-arterially, epinephrine (1 micrograms/kg) augmented the twitch tension of indirectly stimulated, continuously working rabbit gastrocnemius muscle by 15.4 + 6.5%. This increase in work was associated with reduction of phosphocreatine to inorganic phosphate ratio (PCr/Pi) from 3.4 to 2.1 without change in ATP levels. Intracellular pH was reduced from 6.9 to 6.75, but no accumulation of glycolytic intermediates could be observed. The increase in work was not associated with a rise in ADP. All these changes occurred for a few minutes only. The findings suggest that epinephrine's inotropic action is not mediated by a change in mitochondrial metabolism. Glycolytic activation by epinephrine occurs even during twitch and contributes partly to the energy demands of the augmented force. Epinephrine's inotropic effect is, however, not primarily due to changes in bioenergetic kinetics, but to effects on force generating mechanisms, with secondary reduction in energy state.

Published
1991

20. Brief Gene Mapping Reports A / B / C / D / E / F / G

Author

Y. Mao, M. Grosz, S. Izraeli, J.D. Karkera, S. Kimura, I.M. Adham, A.R. Zinn, Y. Ma, T. Yagi, T. Haaf, S. Mitrani-Rosenbaum, N. Coleman, N. Ghaffari-Tabrizi, J. Taylor, J. Nowak, A. Srisodsai, W. Feichtinger, J.-L. Iovanna, C.L. Li, A. Rupprecht, N.A. Jenkins, P. Coullin, M. Muenke, Selena Davis, C. Ji, D.B. Zimonjic, A. Heller, J. Masabanda, J.-C. Dagorn, Y. Xie, B. Hong, H. Kuiper, R.I. Barnes, W.H. Brooks, A.M. Simckes, T. Leeb, D.J. Gilbert, I. Eisenberg, D.M. Shubitowski, R. Fries, B. Lanske, R. Zoorob, G. Cao, W.H. Reeves, M. Satoh, E. Roessler, S.G. Rak, W. Engel, H. Akutsu, L. Guo, H. Kusakabe, S.K. Swanson, D.R. Beier, K. Mrasek, N. Dusetti, M. Shibazaki, C. Steinlein, L. Fröhlich, S.L. Ewart, H. Starke, S. Kollers, C.A. Morley-Jacob, B. Bjerkehagen, N. Kleiter, R.A. White, T.P. Yang, T. Niimi, L.A.P. Carrasco, H. Kikuchi, A. Weise, M. Jiang, R. Tomasini, M.R. Teixeira, L.A. Azizi Samir, C. Auffray, A. Kuechler, H. Cheng, S. Heim, H.-U.G. Weier, M.-J. Pébusque, Z. Liu, S.C. Harvey, D. Gallagher, J.C. Phillips, F. Micci, H. Hochner, N. Gmachl, X. Ni, M.-G. Mattei, A. Dutra, E. Nacheva, C. Drögemüller, R.L. Prueitt, J. Womack, Z.H. Shan, N.C. Popescu, R. Tang, H. Kaiser, G.A. Rohrer, N.G. Copeland, O. Distl, N.R. Bromage, M. Schmid, A. Bernheim, K. Kratochwil, F. Galibert, M. Bina, R. Shamsadin, B. Brenig, Y. Shiratori, E. Antoniou, H. Chen, R. Yanagimachi, M. Sadeh, S. Fukushige, U. Claussen, I. Kirsch, N.B. Atkin, B. Perbal, C.L. Keck-Waggoner, H.P. Klinger, T. Osada, E.I. Pares-Matos, J. Young, C. Knorr, D.J. Penman, K. Ying, L. Ziercher, P. Quignon, M. Wills-Karp, I. Artner, I. Roberts, D.K. Griffin, T. Liehr, D.C. Shing, and Z. Argov

Subjects
Genetics, Gene mapping, Biology, Molecular Biology, Genetics (clinical)
Published
2002

21. Orthostatic Hypotension Induced by Vitamin B12Deficiency

Author

Z. Argov and A. Lossos

Subjects
Male, Vascular disease, business.industry, Arterial hypotension, Vitamin B 12 Deficiency, medicine.disease, Hypotension, Orthostatic, Orthostatic vital signs, Anesthesia, medicine, Humans, Vitamin B12, Cyanocobalamin, Geriatrics and Gerontology, business, Aged
Published
1991

22. Epidural Anesthesia

Author

I. Steiner, Z. Argov, and O. Abramsky

Subjects
Neurology (clinical)
Published
1997

24. Effects of cholinergic blockers on auditory brain-stem evoked potentials in rats

Author

A. Ashkenazi, Sharon Freeman, and Z. Argov

Subjects
Atropine, Male, medicine.medical_treatment, Tubocurarine, Muscarinic Antagonists, Nicotinic Antagonists, Cholinergic Antagonists, Muscarinic acetylcholine receptor, Evoked Potentials, Auditory, Brain Stem, Reaction Time, medicine, Animals, Efferent Pathway, Saline, business.industry, General Neuroscience, Rats, Inbred Strains, Rats, Nicotinic agonist, Neurology, Mechanism of action, Cerebral ventricle, Cholinergic, Neurology (clinical), medicine.symptom, business, Neuroscience, medicine.drug
Abstract

The pharmacology of auditory brain-stem evoked potentials (ABEP) pathways is poorly understood. There are anecdotal reports on the involvement of various neurotransmitters but they were not investigated systematically. The aim of this study was to investigate the effects on ABEP of muscarinic and nicotinic blockers, administered into the cerebral ventricles. Atropine sulfate, d-Tubocurarine and saline were injected stereotactically into the lateral cerebral ventricle of anesthetized male rats. Auditory clicks were given at a rate of 20 s −1 . ABEP recording was performed before and 30 min after injection. Pre- and post-injection peak latencies and peak-to-peak amplitudes of positive waves were compared for each animal. Atropine reduced the amplitudes of waves P1, P3 and P4 and increased mildly the brain stem transmission time. d-Tubocurarine reduced the amplitudes of P1 and P4 with no significant effect on the peak latencies. Saline injection had no effect on any of the parameters. These results show that both cholinergic systems are involved in ABEP generation or transmission. Mechanism of action could be either direct inhibition of afferent pathways or indirect effect, via modulating efferent pathways.

Published
1995

26. Lateonset muscular weakness in phosphofructokinase deficiency due to exon 5intron 5 junction point mutation

Author

MD, Z. Argov, PhD, V. Barash, MD, D. Soffer, Sherman, J., and Raben, N.

Abstract

Late-onset muscle weakness is rare in glycolytic disorders. There are two reports in the literature of phosphofructokinase (PFK)-deficient Ashkenazi Jews with severe vacuolar myopathy manifesting in late adulthood. The genetic abnormality in these patients is unknown. We report a third patient with a similar syndrome: early-onset exercise intolerance in young childhood and progressive weakness in a limb-girdle distribution appearing at 57 years of age, leading to severe incapacity. Muscle histology showed diffuse vacuolar changes, and muscle fibers contained excess glycogen-like material. Muscle biochemistry was diagnostic for PFK deficiency. DNA analysis from the patient and his family showed that he was homozygous for a recently identified point mutation at the exon 5/intron 5 junction (a G-to-A change); two other family members were heterozygous for this mutation. It is not clear whether late-onset weakness is the natural course for all PFK-deficient patients or whether the exon 5 mutation carries increased risk for this severe myopathy.

Published
1994

27. Magnetic resonance spectroscopy of normal and diseased muscles

Author

B. Chance, D. P. Younkin, R. Kelley, W. J. Bank, H. D. Berkowitz, Z. Argov, E. Donlon, B. Boden, K. McCully, N. M. R. Buist, N. Kennaway, John M. Opitz, and James F. Reynolds

Subjects
Magnetic Resonance Spectroscopy, Phosphocreatine, Phosphofructokinase-1, Physical Exertion, Michaelis–Menten kinetics, Muscular Dystrophies, Phosphates, Immobilization, Nuclear magnetic resonance, Atrophy, Muscular Diseases, Oxidative enzyme, medicine, Animals, Humans, Disease process, Genetics (clinical), chemistry.chemical_classification, Adenine Nucleotides, Muscles, Age Factors, Dystrophy, Nuclear magnetic resonance spectroscopy, medicine.disease, Cytochrome b Group, NAD, Mitochondria, Muscle, Kinetics, Enzyme, Biochemistry, chemistry, Cattle, Steady state (chemistry), Acidosis, Energy Metabolism
Abstract

Phosphorus magnetic resonance spectroscopy (P MRS) affords and innovative approach to the study of the oxidative enzyme content of normal and diseased muscles. Examples of the evaluation of the enzyme content of normal muscles by an exercise protocol are provided. The protocol affords a hyperbolic work/cost profile, the Vmax of which is calculated by the reciprocal plots giving the enzyme content and the "effective Michaelis constant" with an evaluation of the resting metabolism. This steady state protocol clearly illustrates enzyme adaptation, on the one hand, and tissue atrophy particularly in the case of tissue injury, Duchenne's dystrophy, and genetic deletion of specific enzymes, on the other hand. The method is rapid, safe, and affords a quantitative evaluation of the disease process and possibilities for following appropriate therapies. So far, approx 1000 examinations of normal and diseased human limbs have been carried out in our laboratory in over the past four years.

Published
1986

29. [Diabetic neuropathy]

Author

I, Steiner and Z, Argov

Subjects
Diabetic Neuropathies, Humans
Published
1984

32. Phosphorus Magnetic Resonance Spectroscopy (31P NMR) as a Tool for in Vivo Monitoring of Mitochrondrial Muscle Disorders

Author

Z. Argov

Subjects
Normal functioning, Nuclear magnetic resonance, Mitochondrial myopathy, Chemistry, In vivo, Mitochondrial disease, medicine, Energy metabolism, Biochemical reactions, Nuclear magnetic resonance spectroscopy, Muscle disorder, medicine.disease
Abstract

Magnetic resonance spectroscopy is a relatively new technique for the study of in vivo biochemistry. It offers a look into several biochemical reactions in normally functioning intact tissues. This chapter will not describe the basic NMR experiment and the various atoms that can be studied by it. For an introduction to the NMR field the reader is referred to monographs on the subject (e.g. Gadian [1]). The chapter will also only briefly describe the basics of phosphorus nuclear magnetic resonance spectroscopy (31P NMR). I will concentrate on the use of 31P NMR in muscle mitochondrial disorders and related conditions. Findings in other disorders of energy metabolism (e.g. glycolytic defects) will not be described. Data on normal functioning muscle will be presented only if they are relevant to mitochondrial diseases.

Published
1989

34. 'Rimmed vacuole myopathy' sparing the quadriceps. A unique disorder in Iranian Jews

Author

Z, Argov and R, Yarom

Subjects
Adult, Male, Electromyography, Muscles, Neuromuscular Diseases, Syndrome, Iran, Middle Aged, Organ Specificity, Jews, Vacuoles, Humans, Female, Israel
Abstract

Nine cases from 4 different Iranian-Jewish families presented with generalized muscular weakness. Quadriceps muscle was the only leg muscle which retained its normal power and function even in advanced cases. Biopsies from severely involved muscles revealed a typical "rimmed vacuole myopathy" while the quadriceps and deltoid showed minimal changes. CK levels were normal or moderately elevated and the EMG pattern suggested an underlining neurogenic disorder. The possible neurogenic character of this autosomal recessive disorder is discussed with relevance to the rimmed vacuoles.

Published
1984

35. D-Penicillamine-associated myasthenia gravis: immunological and electrophysiological studies

Author

P R, Fawcett, S M, McLachlan, L V, Nicholson, Z, Argov, and F L, Mastaglia

Subjects
Adult, Arthritis, Rheumatoid, Male, Immunoglobulin M, Electromyography, Immunoglobulin G, Myasthenia Gravis, Penicillamine, Humans, Female, Receptors, Cholinergic, Middle Aged, Autoantibodies
Abstract

Immunological and electrophysiological features were investigated in a patient with rheumatoid arthritis who developed myasthenic symptoms after 10 months of treatment with D-penicillamine (D-pen). After an initial rise, acetylcholine receptor (AChR) antibodies gradually fell from 24.4 nmole alpha-bungarotoxin bound/liter to 1.8 nmole/liter following withdrawal of the drug, and this was associated with clinical and electrophysiological improvement. Immunoglobuline synthesis by peripheral blood lymphocytes cultured with pokeweed mitogen and D-pen was significantly increased at D-pen concentrations of 10-100 micrograms/ml in 2 patients with D-pen associated myasthenia gravis and 1 out of 3 normal individuals. Furthermore, lymphocytes from 1 myasthenic patient synthesized detectable AChR antibodies in vitro, and the amount of receptor antibody produced was increased in the presence of the drug. This effect was most marked at a concentration of 10 micrograms/ml which is thought to approximate the concentration in vivo. Our results confirm the in vivo findings of others that D-pen may have different effects on immunoglobulin production in different individuals and may also enhance the snythesis of at least 1 autoantibody, the AChR antibody.

Published
1982

36. [Antibiotic treatment in myasthenia gravis]

Author

Z, Argov and O, Abramsky

Subjects
Male, Sulfonamides, Adolescent, Middle Aged, Infections, Neostigmine, Anti-Bacterial Agents, Aminoglycosides, Chloramphenicol, Myasthenia Gravis, Humans, Ampicillin, Female, Aged
Published
1982

37. Drug therapy: Disorders of neuromuscular transmission caused by drugs

Author

Z, Argov and F L, Mastaglia

Subjects
Psychotropic Drugs, Drug-Related Side Effects and Adverse Reactions, Penicillamine, Neuromuscular Junction, Cardiovascular Agents, Chloroquine, Syndrome, Motor Endplate, Synaptic Transmission, Hormones, Anti-Bacterial Agents, Postoperative Complications, Myasthenia Gravis, Animals, Humans, Anticonvulsants, Respiratory Insufficiency, Aged
Published
1979

38. Prevention of experimental autoimmune myasthenia gravis in rats by fetal alpha-fetoprotein-rich fractions

Author

T, Brenner, A, Zielinski, Z, Argov, and O, Abramsky

Subjects
Immunity, Cellular, Electromyography, Muscles, Radioimmunoassay, Lymphocyte Activation, Torpedo, Muscle Denervation, Rats, Fetus, Rats, Inbred Lew, Acute Disease, Antibody Formation, Chronic Disease, Myasthenia Gravis, Animals, Humans, Female, Receptors, Cholinergic, alpha-Fetoproteins, Immunosuppressive Agents, Autoantibodies
Abstract

Rats immunized with Torpedo acetylcholine receptor (AChR) developed experimental autoimmune myasthenia gravis (EAMG) manifested by an early acute phase and a late chronic phase. In the animals the acute and the chronic diseases were prevented by the administration of alpha-fetoprotein (AFP)-rich fractions obtained from human amniotic fluid and umbilical cord serum. Both the clinical and the electromyographic manifestations of EAMG was affected by AFP treatment. The anti-AChR antibody level as well as the cellular immune response to AChR were also affected by AFP. Rats immunized with Torpedo AChR and treated with AFP-rich fractions showed low levels of antibodies to rat AChR, and an inhibited proliferation response of lymphocytes to the antigen AChR. The addition of AFP in vitro to the lymphocyte culture significantly inhibited the proliferative response to AChR and to the mitogen phytohemagglutinin. AFP is present in high amounts during pregnancy, and therefore the present findings suggest that clinical remissions of myasthenia gravis during the second half of pregnancy may be attributed to the immunosuppressive effect of AFP.

Published
1984

41. Familial progressive neuronal disease and chronic idiopathic intestinal pseudo-obstruction

Author

J. Faber, A. Steinberg, Z. Argov, A. Gilai, I. Steiner, and A. Fich

Subjects
Adult, Male, Pathology, medicine.medical_specialty, Sensorimotor peripheral neuropathy, Adolescent, Hearing loss, business.industry, Eye disease, Intestinal Pseudo-Obstruction, Peripheral Nervous System Diseases, medicine.disease, Neuronal disease, Bowel obstruction, medicine.anatomical_structure, otorhinolaryngologic diseases, medicine, Humans, Chronic Idiopathic Intestinal Pseudo-Obstruction, Female, Neurology (clinical), Neuron, medicine.symptom, business, Polyneuropathy
Abstract

Chronic idiopathic intestinal pseudo-obstruction (CIIP) is characterized by recurrent episodes of bowel obstruction without mechanical cause. In five members of two Jewish-Iranian families, CIIP was associated with progressive neuronal disease, starting before age 30, with ophthalmoplegia, sensorimotor peripheral neuropathy, and hearing loss. There was no evidence of CNS involvement. The pattern suggested autosomal recessive inheritance.

Published
1987

42. The history of the European Neurological Society (1986-2014)-10 years later.

Author

Toyka KV, Krarup C, Steck A, Said G, Argov Z, van Gijn J, Ferro J, Comi G, and Bassetti CLA

Subjects
History, 20th Century, Europe, Humans, History, 21st Century, Neurology history, Societies, Medical history
Abstract

Background and Purpose: The European Academy of Neurology (EAN) was a merger from two parent societies: the European Neurological Association (ENS, founded in 1986) and the European Federation of Neurological Societies (EFNS, founded in 1987)., Methods: This article was written by nine former presidents, three of whom were also founders of the ENS, and is based on recollections and documents. It follows up on a review of the ENS history stored in the EAN archive., Results: The first European society (ENS) was founded by eight individual European academic clinician-neuroscientists aiming at joining with other qualified European neuroscientists on an individual membership basis. After 1990 members were also invited from behind the former Iron Curtain. A principal goal was holding neurology meetings (700 participants in 1988 and over 3000 in 2010), promoting collaborative research projects with exchange of junior neuroscientists, and providing teaching and education independent from nationality. Health politics were not part of the agenda. The executive boards (4-year term) were staffed with academic scientists from all subspecialties of neurology. Numerous bursaries and fellowships were established for junior neurologists. The impact of ENS members on research activities of young investigators was appreciated by academia at large. After years of negotiations ENS and EFNS joint efforts resulted in forming the EAN covering all fields of neurology and neuroscience under one roof., Conclusion: The basic principles of the ENS were successfully integrated into the new EAN in particular documented by the number of individual members rising to over 4000 in 2024., (© 2024 The Author(s). European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Published
2024
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43. Statins in hereditary myopathies: to give or not to give.

Author

Argov Z

Subjects
Humans, Creatine Kinase blood, Hydroxymethylglutaryl-CoA Reductase Inhibitors adverse effects, Hydroxymethylglutaryl-CoA Reductase Inhibitors therapeutic use, Muscular Diseases genetics, Muscular Diseases chemically induced, Muscular Diseases drug therapy
Abstract

Hyperlipidemia is not uncommon in patients with hereditary myopathies who get older and also in several conditions in which it is frequently observed. Thus, using the common cholesterol reducing medications of the stains group could be considered. However, the side effects of these drugs include myalgia, myopathy and rhabdomyolysis typically associated with high serum creatine kinase (CK). Because high CK levels are very frequently found in hereditary myopathies, physicians are reluctant to use statins in such patients. Reviewing the literature about statin side effects in hereditary myopathies does not provide a clear evidence about the true risk of these drugs. This review critically describes the reported cases of statin side effects in several genetic myopathies and suggests some guidelines for conditions that are contra indicated for statin usage (particularly in mitochondrial disorders, metabolic myopathies, myotonic dystrophy type 2). Possible solutions to the dilemma of whether to use statins in hereditary myopathies are discussed (prescribing other cholesterol lowering agents and a carefully monitored treatment initiation of statins)., Competing Interests: Declaration of competing interest The Author has no conflict of interests to declare, (Copyright © 2024. Published by Elsevier B.V.)

Published
2024
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44. Induced Muscle and Liver Absence of Gne in Postnatal Mice Does Not Result in Structural or Functional Muscle Impairment.

Author

Harazi A, Yakovlev L, Ilouz N, Selke P, Horstkorte R, Fellig Y, Lahat O, Lifschytz T, Abudi N, Abramovitch R, Argov Z, and Mitrani-Rosenbaum S

Subjects
Animals, Mice, Distal Myopathies genetics, Distal Myopathies metabolism, Carbohydrate Epimerases genetics, Carbohydrate Epimerases metabolism, Multienzyme Complexes genetics, Multienzyme Complexes metabolism, N-Acetylneuraminic Acid metabolism, Muscle, Skeletal metabolism, Liver metabolism, Mice, Knockout, Disease Models, Animal
Abstract

Background: GNE Myopathy is a unique recessive neuromuscular disorder characterized by adult-onset, slowly progressive distal and proximal muscle weakness, caused by mutations in the GNE gene which is a key enzyme in the biosynthesis of sialic acid. To date, the precise pathophysiology of the disease is not well understood and no reliable animal model is available. Gne KO is embryonically lethal in mice., Objective: To gain insights into GNE function in muscle, we have generated an inducible muscle Gne KO mouse. To minimize the contribution of the liver to the availability of sialic acid to muscle via the serum, we have also induced combined Gne KO in liver and muscle., Methods: A mouse carrying loxp sequences flanking Gne exon3 was generated by Crispr/Cas9 and bred with a human skeletal actin (HSA) promoter driven CreERT mouse. Gne muscle knock out was induced by tamoxifen injection of the resulting homozygote GneloxpEx3loxp/HSA Cre mouse. Liver Gne KO was induced by systemic injection of AAV8 vectors carrying the Cre gene driven by the hepatic specific promoter of the thyroxine binding globulin gene., Results: Characterization of these mice for a 12 months period showed no significant changes in their general behaviour, motor performance, muscle mass and structure in spite of a dramatic reduction in sialic acid content in both muscle and liver., Conclusions: We conclude that post weaning lack of Gne and sialic acid in muscle and liver have no pathologic effect in adult mice. These findings could reflect a strong interspecies versatility, but also raise questions about the loss of function hypothesis in Gne Myopathy. If these findings apply to humans they have a major impact on therapeutic strategies.

Published
2024
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45. GNE myopathy: can homozygous asymptomatic subjects give a clue for the identification of protective factors?

Author

Mitrani-Rosenbaum S, Attali R, and Argov Z

Subjects
Aged, Female, Humans, Middle Aged, Muscle, Skeletal, Mutation, Protective Factors, Distal Myopathies genetics, Muscular Diseases diagnosis, Muscular Diseases genetics
Abstract

GNE myopathy is caused by bi allelic recessive mutations in the GNE gene. The largest identified cohort of GNE myopathy patients carries a homozygous mutation- M743T (the "Middle Eastern" mutation). More than 160 such patients in 67 families have been identified by us. Mean onset in this cohort is 30 years (range 17-48) with variable disease severity. However, we have identified two asymptomatic females, homozygous for M743T in two different families, both with affected siblings. The first showed no myopathy when examined at age 76 years. The second has no sign of disease at age 60 years. Since both agreed only for testing of blood, we performed exome and RNA sequencing of their blood and that of their affected siblings. Various filtering layers resulted in 2723 variant loci between symptomatic and asymptomatic individuals, representing 1364 genes. Among those, 39 genes are known to be involved in neuromuscular diseases, and only in two of them the variant is located in the proper exon coding region, resulting in a missense change. Surprisingly, only 27 genes were significantly differentially expressed between the asymptomatic and the GNE myopathy affected individuals, with three overexpressed genes overlapping between exome and RNA sequencing. Although unable to unravel robust candidate genes, mostly because of the very low number of asymptomatic individuals analyzed, and because of the tissue analyzed (blood and not muscle), this study resulted in relatively restricted potential candidate protective genes, emphasizing the power of using polarized phenotypes (completely asymptomatic vs clearly affected individuals) with the same genotype to unmask those genes which could be used as targets for disease course modifiers., Competing Interests: Declaration of Competing Interest The authors declare they have no conflict of interest., (Copyright © 2023. Published by Elsevier B.V.)

Published
2023
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46. Pearls & Oy-sters: Reversible Postpartum Pseudocoma State Associated With Magnesium Therapy: A Report of 2 Cases.

Author

Friedman-Korn T, Lerner Y, Haggiag S, Nama A, Ekstein D, Argov Z, Vaknin-Dembinsky A, and Gotkine M

Subjects
Calcium, Coma, Female, Humans, Neurotransmitter Agents, Postpartum Period, Pregnancy, Magnesium therapeutic use, Pre-Eclampsia
Abstract

Magnesium (Mg) competes with calcium in normal synaptic transmission, inhibiting neurotransmitter release. As a drug, it is usually given as a treatment for eclampsia and preeclampsia. Two eclamptic pregnant women treated with Mg developed a pseudocoma state immediately after emergency Caesarian section. The clinical presentation was flaccid quadriparesis, areflexia, absent respiratory effort and vestibular-ocular reflexes, but with preserved pupillary responses. Decremental responses on repetitive nerve stimulation were found in both women. Recovery was obtained after cessation of Mg. The persistence of pupillary reflexes in the absence of reflexes involving striated muscles was an important clinical clue, indicating neuromuscular junction dysfunction., (© 2022 American Academy of Neurology.)

Published
2022
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48. Pre Clinical Assessment of AAVrh74.MCK.GNE Viral Vector Therapeutic Potential: Robust Activity Despite Lack of Consistent Animal Model for GNE Myopathy.

Author

Mitrani-Rosenbaum S, Yakovlev L, Becker Cohen M, Argov Z, Fellig Y, and Harazi A

Subjects
Animals, Dependovirus, Disease Models, Animal, Humans, Mice, Mice, Transgenic, Muscular Diseases physiopathology, Genetic Therapy methods, Multienzyme Complexes genetics, Muscular Diseases genetics, Muscular Diseases therapy
Abstract

Background: GNE myopathy is a unique adult onset rare neuromuscular disease caused by recessive mutations in the GNE gene. The pathophysiological mechanism of this disorder is not well understood and to date, there is no available therapy for this debilitating disease. We have previously established proof of concept that AAV based gene therapy can effectively deliver the wild type human GNE into cultured muscle cells from human patients and in mice, using a CMV promoter driven human wild type GNE plasmid delivered through an adeno associated virus (AAV8) based platform., Objective: In the present study we have generated a muscle specific GNE construct, driven by the MCK promoter and packaged with the AAVrh74 serotype for efficacy evaluation in an animal model of GNE Myopathy., Methods: The viral vector was systemically delivered at 2 doses to two age groups of a Gne-/- hGNED207V Tg mouse described as a preclinical model of GNE Myopathy, and treatment was monitored for long term efficacy., Results: In spite of the fact that the full described characteristics of the preclinical model could not be reproduced, the systemic injection of the rAAVrh74.MCK.GNE viral vector resulted in a long term presence and expression of human wt GNE in the murine muscles and in some improvements of their mild phenotype. The Gne-/- hGNED207V Tg mice are smaller from birth, but cannot be differentiated from littermates by muscle function (grip strength and Rotarod) and their muscle histology is normal, even at advanced age., Conclusions: The rAAVrh74.MCK.GNE vector is a robust tool for the development of GNE Myopathy therapies that supply the intact GNE. However, there is still no reliable animal model to fully assess its efficacy since the previously developed Gne-/- hGNED207V Tg mice do not present disease characteristics.

Published
2022
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49. Two intronic cis-acting variants in both alleles of the POLR3A gene cause progressive spastic ataxia with hypodontia.

Author

Fellner A, Lossos A, Kogan E, Argov Z, Gonzaga-Jauregui C, Shuldiner AR, Darawshe M, Bazak L, Lidzbarsky G, Shomron N, Basel-Salmon L, and Goldberg Y

Subjects
Alleles, Anodontia complications, Anodontia diagnostic imaging, Anodontia enzymology, DNA Mutational Analysis, Female, Frameshift Mutation, Humans, Intellectual Disability complications, Intellectual Disability diagnostic imaging, Intellectual Disability enzymology, Male, Middle Aged, Muscle Spasticity complications, Muscle Spasticity diagnostic imaging, Muscle Spasticity enzymology, Optic Atrophy complications, Optic Atrophy diagnostic imaging, Optic Atrophy enzymology, Spinocerebellar Ataxias complications, Spinocerebellar Ataxias diagnostic imaging, Spinocerebellar Ataxias enzymology, Anodontia genetics, Intellectual Disability genetics, Introns genetics, Muscle Spasticity genetics, Optic Atrophy genetics, RNA Polymerase III genetics, Spinocerebellar Ataxias genetics
Abstract

POLR3A encodes the largest subunit of the DNA-dependent RNA polymerase III. Pathogenic variants in this gene are associated with dysregulation of tRNA production and other non-coding RNAs. POLR3A-related disorders include variable phenotypes. The genotype-phenotype correlation is still unclear. Phenotypic analysis and exome sequencing were performed in four affected siblings diagnosed clinically with hereditary spastic ataxia, two healthy siblings and their unaffected mother. All four affected siblings (ages 46-55) had similar clinical features of early childhood-onset hypodontia and adolescent-onset progressive spastic ataxia. None had progeria, gonadal dysfunction or dysmorphism. All affected individuals had biallelic POLR3A pathogenic variants composed by two cis-acting intronic splicing-altering variants, c.1909 + 22G > A and c.3337-11 T > C. The two healthy siblings had wild-type alleles. The mother and another unaffected sibling were heterozygous for the allele containing both variants. This is the first report addressing the clinical consequence associated with homozygosity for a unique pathogenic intronic allele in the POLR3A gene. This allele was previously reported in compound heterozygous combinations in patients with Wiedemann-Rautenstrauch syndrome, a severe progeroid POLR3A-associated phenotype. We show that homozygosity for this allele is associated with spastic ataxia with hypodontia, and not with progeroid features. These findings contribute to the characterization of genotype-phenotype correlation in POLR3A-related disorders., (© 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Published
2021
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50. Dysphagia in adult myopathies.

Author

Argov Z and de Visser M

Subjects
Deglutition physiology, Esophagus, Humans, Mouth, Pharynx, Quality of Life, Deglutition Disorders complications, Muscular Diseases complications
Abstract

Dysphagia (impaired swallowing) is not a rare problem in various neuromuscular disorders, both in the pediatric and the adult patient population. On many occasions such patients are first presented to other medical specialists or health professionals. Disorders of deglutition are probably underrecognized in patients with a neuromuscular disease as a result of patient's and doctor's delay. This review will focus on dysphagia in adults suffering from a myopathy. Dysphagia in myopathies usually affects the oropharyngeal phases which rely mostly on voluntary muscle activity of the mouth, pharynx and upper esophageal sphincter. Dysphagia is known to contribute to a reduction of quality of life and may also lead to increased morbidity and mortality. The review includes an overview on symptomatology and tools of assessments, and elaborates on dysphagia in specific hereditary and acquired myopathies., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Published
2021
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