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2. FcγRIIa and FcγRIIIb polymorphisms were not associated with meningococcal disease in Western Norway

Author

Alfred Halstensen, Ingrid Smith, and C. Vedeler

Subjects
Adult, Male, Adolescent, Genotype, Epidemiology, Population, Disease, GPI-Linked Proteins, Meningococcal disease, Antigens, CD, medicine, Humans, Prospective Studies, education, Allele frequency, Genotyping, Alleles, education.field_of_study, Polymorphism, Genetic, Norway, business.industry, Incidence (epidemiology), Receptors, IgG, Middle Aged, Complement deficiency, medicine.disease, Meningococcal Infections, Infectious Diseases, Case-Control Studies, Immunology, Female, business, Research Article
Abstract

Fcgamma-receptor (FcyR) polymorphisms have been associated with acquisition and severity of invasive meningococcal disease. We studied FcgammaR polymorphisms in a population with a high incidence of meningococcal disease. Fifty meningococcal disease patients aged 14-60 years, with bacteriologically confirmed disease and without detected complement deficiency, together with 100 healthy adult controls were included in the study. Clinical and bacteriological data were collected prior to FcgammaRIIa and FcgammaRIIb genotyping, which was performed by polymerase chain reaction. The distribution of the FcgammaRIIa and FcgammaRIIIb allotypes and their allele frequencies were not significantly different amongst the patients and the controls. The combination FcgammaRIIa-R/R and FcgammaRIIb-Na2/Na2 was less common among patients than controls (OR = 0.11, Fisher's exact P = 0.05). No significant association was found between the two FcgammaRs and severity of disease, meningococcal serogroup, age groups or gender. In contrast to previous findings, our study indicates that in Norwegian teenagers and adults, the FcgammaRIIa and FcgammaRIIIb allotypes are not decisive for the acquisition or for the severity of meningococcal disease.

Published
2003
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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. 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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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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. 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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
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5. Complications after LP related to needle type: pencil-point versus Quincke

Author

C. Vedeler and A. Aamodt

Subjects
medicine.medical_specialty, medicine.diagnostic_test, Post-dural-puncture headache, Needle Shape, Lumbar puncture, business.industry, Diagnostic lumbar puncture, Significant difference, Needle type, General Medicine, Surgery, Pencil (optics), Neurology, Anesthesia, medicine, Neurology (clinical), medicine.symptom, Complication, business
Abstract

Objectives - We studied the incidence of complications after diagnostic lumbar puncture (LP) related to needle type. Material and methods - A 5 months' observational study of routine diagnostic LP in 83 patients was conducted. Results - Significantly more headache was observed after LP using thicker cutting needles (20G Quincke) compared with thinner cutting or non-cutting needles (22G Quincke or pencil-point). No significant difference in complications after LP was found between the 22G Quincke and pencil-point needles. Conclusion - The size of the needle and not the needle shape seems to be the main determinant for post-dural puncture headache (PDPH).

Published
2001
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6. Onset and course of chronic inflammatory demyelinating polyneuropathy

Author

C. Vedeler, Åse Mygland, and P. Monstad

Subjects
Adult, Male, medicine.medical_specialty, Physiology, Neural Conduction, Chronic inflammatory demyelinating polyneuropathy, Gastroenterology, Cellular and Molecular Neuroscience, Relapsing course, Predictive Value of Tests, Recurrence, Physiology (medical), Internal medicine, medicine, Humans, Peripheral Nerves, Age of Onset, Muscle, Skeletal, Aged, Immunosuppressive treatment, Muscle Weakness, business.industry, Clinical course, Middle Aged, medicine.disease, Prognosis, Surgery, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, Acute Disease, Disease Progression, Proximal weakness, Female, Neurology (clinical), Remission rate, business, Nerve conduction, Subacute onset, Follow-Up Studies
Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is clinically heterogeneous. Our purpose was to determine whether initial progression time, clinical features, and distribution of nerve conduction slowing at presentation correlate with clinical course and prognosis. We examined how findings at presentation related to clinical course during an average follow-up time of 4.0 (range 1.0-9.0) years in 44 patients with CIDP. We calculated terminal latency index (TLI), a measure of differential slowing in distal relative to more proximal nerve segments. Patients with acute or subacute onset (progression over less than 8 weeks) had a higher remission rate (P = 0.012) than patients with chronic onset (progression over more than 8 weeks). Patients with proximal weakness had a higher remission rate than patients with the distal phenotype (P < 0.001). All 5 patients with a relapsing course had subacute onset. They had lower TLIs, suggesting a more distal pattern of demyelination, than patients with a monophasic or chronic course. In conclusion, subacute onset and presence of proximal weakness are good prognostic signs that correlate with a high rate of recovery to normal in CIDP. Distal accentuation of conduction slowing at presentation correlates with subacute onset and a relapsing course.

Published
2005

7. [Peripheral neuropathy in cancer]

Author

A, Storstein, C, Vedeler, and D C, Johannesen

Subjects
Neoplasms, Lumbosacral Plexus, Humans, Peripheral Nervous System Diseases, Antineoplastic Agents, Neoplasm Metastasis, Radiation Injuries, Magnetic Resonance Imaging
Abstract

Neuropathy is a quite common finding in cancer patients. If the cancer is known, the main clinical problem is often whether the symptoms and signs are caused by metastasis or recurrence, or if the neuropathy can be related to effects of the oncological treatment. In addition to the clinical findings, radiological and neurophysiological examination can often be of considerable diagnostic help. Peripheral neuropathy may also be the first manifestation of the cancer, as in paraneoplastic neuropathies. Detecting the tumor in these cases may improve the oncological prognosis, as the neuropathy often precedes tumor symptoms by several years. A determined approach in searching for an occult cancer is necessary. The neuropathic symptoms are seldom life-threatening. However, the neuropathy may cause distressing symptoms, and symptomatic treatment and rehabilitation is often required.

Published
2001

8. Complications after LP related to needle type: pencil-point versus Quincke

Author

A, Aamodt and C, Vedeler

Subjects
Adult, Male, Back Pain, Needles, Incidence, Headache, Humans, Female, Middle Aged, Spinal Puncture
Abstract

We studied the incidence of complications after diagnostic lumbar puncture (LP) related to needle type.A 5 months' observational study of routine diagnostic LP in 83 patients was conducted.Significantly more headache was observed after LP using thicker cutting needles (20G Quincke) compared with thinner cutting or non-cutting needles (22G Quincke or pencil-point). No significant difference in complications after LP was found between the 22G Quincke and pencil-point needles.The size of the needle and not the needle shape seems to be the main determinant for post-dural puncture headache (PDPH).

Published
2001

9. [Hereditary neuropathy with pressure palsies]

Author

I O, Gjerde, N, Aarskog, and C, Vedeler

Subjects
Adult, Male, Blotting, Southern, Charcot-Marie-Tooth Disease, Pressure, Humans, Paralysis, Female, Hereditary Sensory and Autonomic Neuropathies, Myelin P0 Protein, Ulnar Nerve
Abstract

Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant polyneuropathy usually caused by a deletion in the gene coding for the peripheral nerve myelin protein 22 (PMP22). The patients usually get relapsing and remitting focal nerve symptoms due to mechanical factors like pressure or minor trauma that normal nerves tolerate.Two patients from different families have been examined clinically, neurophysiologically and genetically by Southern blot and PCR techniques.The clinical and neurophysiological findings were typical of this disorder, and the DNA tests showed deletions in the PMP22 gene.We discuss clinical, neurophysiological and molecular diagnostics, pathomechanisms, treatment and secondary prevention. Early diagnosis may be important for optimal management of the patients.

Published
2001

10. EFNS task force report: a questionnaire-based survey on the service provision and quality assurance for determination of diagnostic autoantibody tests in European neuroimmunology centres. European Federation of Neurological Societies

Author

H J, Willison, W, Ang, N E, Gilhus, F, Graus, R, Liblau, C, Vedeler, and A, Vincent

Subjects
Europe, Immunoassay, Quality Assurance, Health Care, Paraneoplastic Syndromes, Health Care Surveys, Surveys and Questionnaires, Myasthenia Gravis, Humans, Receptors, Cholinergic, Nervous System Diseases, Autoantibodies, Autoimmune Diseases
Abstract

Autoantibodies to a wide variety of neural components are frequently sought in the sera of patients with neurological diseases suspected to have an antibody-associated autoimmune basis. Variations in assay methodology and availability are likely to exist throughout European diagnostic immunology centres, and interlaboratory discrepancies in performance for some assays have been reported. The availability of quality assurance is largely unknown. In this questionnaire-based EFNS task force, all 18 national representatives of the Neuroimmunology Panel within the EFNS were invited to estimate the service provision within their country; 12 panel members responded. From these responses, it emerged that a range of assays are being performed throughout European centres, involving over 20 separate antigens, using a broad array of immunodetection techniques. With the exception of the estimation of anti-AChR antibodies for the diagnosis of myasthenia gravis, no systematic quality assurance schemes are available, this being conducted on an ad hoc basis, or not at all. Since quality is a central component of assay sensitivity and specificity, we conclude that there is an urgent need to introduce pan-European quality assurance schemes, based on provision of positive and negative test sera from a central source, and in which all neuroimmunology laboratories should participate.

Published
2001

11. Complement activation by titin and ryanodine receptor autoantibodies in myasthenia gravis. A study of IgG subclasses and clinical correlations

Author

F, Romi, G O, Skeie, C, Vedeler, J A, Aarli, F, Zorzato, and N E, Gilhus

Subjects
Adult, Male, Thymoma, Muscle Proteins, Ryanodine Receptor Calcium Release Channel, Middle Aged, Immunoglobulin G, Myasthenia Gravis, Humans, Connectin, Female, Receptors, Cholinergic, Longitudinal Studies, Age of Onset, Complement Activation, Protein Kinases, Aged, Autoantibodies
Abstract

To elucidate the mechanism of immune damage caused by titin and ryanodine receptor (RyR) autoantibodies in myasthenia gravis (MG), we studied the complement-activating capacity and the IgG subclass distribution of these autoantibodies in sera from 49 MG patients. Complement activation occurred in 38 out of 49 titin antibody positive sera, and in 14 out of 21 RyR antibody positive sera. The titin antibodies occurred only in the IgG 1 and IgG 4 subclasses, whereas the RyR antibodies occurred in all four IgG subclasses but with IgG 1 predominance. Complement-activating RyR antibodies occurred with higher frequency in sera of thymoma MG than of late-onset MG. RyR IgG 1 antibodies occurred more often in severe MG than in mild and moderate disease groups. Mean total IgG and IgG 1 titin and RyR antibody titers fell during long-time patient observation together with an improvement of the MG symptoms.

Published
2000

12. [Neurological diseases associated with celiac disease]

Author

E M, Hagen, I O, Gjerde, C, Vedeler, and N, Hovdenak

Subjects
Adult, Male, Celiac Disease, Polyneuropathies, Dermatitis Herpetiformis, Humans, Spinocerebellar Ataxias, Female, Middle Aged, Nervous System Diseases, Prognosis, Spinal Cord Diseases, Aged
Abstract

During the period from May 1997 to October 1998, eight patients with coeliac disease or dermatitis herpetiformis and neurological disorders were admitted to the Department of Neurology, University Hospital of Bergen. The most frequent conditions were polyneuropathy (seven patients) and spinocerebellar ataxia (three patients). Other conditions were lower motor neuron disease, myelopathy, epilepsy and encephalopathy. The patients used various degrees of gluten-free diet at the time of admission. It remains unclear whether there is a shared common pathogenetic mechanism or the neurological disorder is a complication to the coeliac disease. Both vitamin depletion and immunological mechanisms may cause neurological disorder. Neurological manifestations may occur before the gastrointestinal symptoms. With reference to our patients and available literature we discuss prevalence, clinical picture, pathogenesis, treatment and prognosis. Neurologists, gastroenterologists and general practitioners should be aware that coeliac disease can cause neurological diseases, especially polyneuropathy, cerebellar ataxia and encephalopathy.

Published
2000

13. Interferon-alpha2a reduces MRI disease activity in relapsing-remitting multiple sclerosis. Norwegian Study Group on Interferon-alpha in Multiple Sclerosis

Author

K M, Myhr, T, Riise, F E, Green Lilleås, T G, Beiske, E G, Celius, A, Edland, D, Jensen, J P, Larsen, R, Nilsen, M W, Nortvedt, A I, Smievoll, C, Vedeler, and H I, Nyland

Subjects
Adult, Male, Multiple Sclerosis, Time Factors, Interferon-alpha, Interferon alpha-2, Middle Aged, Magnetic Resonance Imaging, Recombinant Proteins, Treatment Outcome, Double-Blind Method, Quality of Life, Humans, Female
Abstract

To evaluate the efficacy and safety of interferon-alpha2a (IFN-alpha2a) in relapsing-remitting MS (RRMS).Several immune-modulating therapy regimens of IFN-alpha have shown varying results in MS. A recent pilot study suggested benefits from IFN-alpha2a.Ninety-seven patients were randomized to receive subcutaneous injections of placebo (33 patients) or 4.5 million international units (mIU) (32 patients) or 9.0 mIU (32 patients) of IFN-alpha2a three times weekly for 6 months, with a further 6 months of follow-up. Monthly gadodiamide-enhanced MRI was the primary method of evaluating efficacy.IFN-alpha2a treatment resulted in fewer new MRI lesions during the treatment period (p0.003). The probability of no new lesions during treatment was2.5 times higher with 9.0 mIU IFN-alpha2a than with placebo (p0.005). The median number of lesions at the end of treatment was lower with IFN-alpha2a treatment than with placebo (p = 0.0004), but the difference disappeared during follow-up. The total number of lesions (mean) increased by 4.78 with placebo, 0.86 with 4.5 mIU IFN-alpha2a, and 0.28 with 9.0 mIU IFN-alpha2a during treatment (p = 0.030). No treatment effect on exacerbation rate, progression of disability, or quality of life was detected. Nine patients discontinued treatment, five because of adverse events.IFN-alpha2a treatment significantly reduced disease activity as measured by MRI, but the efficacy disappeared within 6 months after discontinuation of treatment. A long-term study of more patients using disability as a primary outcome measure is needed to evaluate the clinical impact.

Published
1999

14. [The POEMS syndrome--a rare multiorgan disease]

Author

A, Storstein, I O, Gjerde, C, Vedeler, and I, Nesthus

Subjects
Adult, Diagnosis, Differential, Male, Fatal Outcome, POEMS Syndrome, Humans, Middle Aged
Abstract

POEMS syndrome is a rare multisystemic disorder characterized by a variable constellation of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. The syndrome is believed to be immune-mediated and is probably related to the pathological monoclonal component caused by a myeloma or a plasmocytoma. The treatment is aimed at the underlying plasma cell dyscrasia. We have studied three patients with this rare syndrome. This article presents the clinical picture of each one and a survey of the literature.

Published
1998

15. [Cerebral amyloid angiopathy]

Author

T, Almås, C, Vedeler, G, Moen, and S, Mørk

Subjects
Adult, Diagnosis, Differential, Male, Cerebral Amyloid Angiopathy, Humans, Female, Middle Aged, Tomography, X-Ray Computed, Magnetic Resonance Imaging, Immunosuppressive Agents, Aged
Abstract

Cerebral amyloid angiopathy affects the cerebral vasculature selectively, and there is no systemic amyloidosis. Amyloid is deposited in small and medium-sized vessels of the cortex and leptomeninges. Cerebral amyloid angiopathy is a common cause of spontaneous lobar haemorrhage in elderly patients. However, cerebral amyloid angiopathy may have atypical clinical and radiological presentations. We report on five patients (three males and two females, aged 43-77 years) with histologically verified cerebral amyloid angiopathy. One patient experienced an acute headache attack and classical lobar haemorrhage. The other patients had various neurological symptoms and signs, such as seizure, disturbed vision, pareses, aphasia, and dementia that were initially diagnosed as cerebral infarction or tumour. Two patients with cerebral amyloid angiopathy and granulomatous angiitis responded to immunosuppressive treatment.

Published
1998

16. FcgammaRIIA and FcgammaRIIIB polymorphisms in myasthenia gravis

Author

G, Raknes, G O, Skeie, N E, Gilhus, S, Aadland, and C, Vedeler

Subjects
Polymorphism, Genetic, Gene Frequency, Genotype, Thymoma, Myasthenia Gravis, Receptors, IgG, Humans, Disease Susceptibility, Thymus Neoplasms, Age of Onset, Alleles, Autoimmune Diseases
Abstract

The Fcgamma receptors, FcgammaRIIA and FcgammaRIIIB contain polymorphisms with different capacity for IgG binding and phagocytosis. Thirty myasthenia gravis (MG) patients and 49 healthy controls were genotyped for the FcgammaRIIA and FcgammaRIIIB polymorphisms using polymerase chain reaction. The frequency of the FcgammaRIIA-H/H genotype was increased in thymoma MG patients compared to other MG patients (P = 0.05) and controls (P = 0.02). The distribution of FcgammaRIIIB alleles in MG patients did not differ from the controls, but MG patients with the NA1/NA1 genotype had the most severe MG (P = 0.01). Levels of AChR-antibodies and frequency of titin or ryanodine receptor antibodies were not associated with the FcgammaRIIA or FcgammaRIIIB genotypes. The results suggest different pathogenetic mechanisms in paraneoplastic and non-paraneoplastic autoimmune MG.

Published
1998

17. [Paraneoplastic cerebellar degeneration. A case report]

Author

A, Storstein, C, Vedeler, B, Krossnes, and S, Mørk

Subjects
Ovarian Neoplasms, Purkinje Cells, Antigens, Neoplasm, Paraneoplastic Syndromes, Cerebellum, Biomarkers, Tumor, Humans, Female, Adenocarcinoma, Cerebellar Neoplasms, Aged
Abstract

Paraneoplastic cerebellar degeneration is a rare remote effect of ovarian and breast carcinoma especially, and is characterised clinically by rapidly evolving pancerebellar symptoms. A woman aged 83 developed progressive vertigo, cerebellar ataxia, nystagmus and dysarthria. The cerebrospinal fluid showed slight mononuclear pleocytosis, elevated total protein and IgG concentrations, and oligoclonal bands. A magnetic resonance investigation performed within the first month of symptoms was normal. A left pelvic mass was found, possibly a carcinoma of the colon or the left ovary. Cancer antigen 125 was elevated in the serum and antibodies against Purkinje cells (anti-Yo antibodies) were demonstrated in the serum and cerebrospinal fluid. These results suggested a carcinoma of the ovary as primary site of cancer. Autopsy revealed a left ovarian adenocarcinoma and marked loss of Purkinje cells in the cerebellum. The case illustrates that anti-Yo antibodies may serve as a marker not only for paraneoplastic cerebellar degeneration, but also for the nature of the neoplasm that caused it.

Published
1997

18. [Guillain-Barré syndrome. Variation on the theme]

Author

B, Karlsen and C, Vedeler

Subjects
Adult, Diagnosis, Differential, Polyradiculoneuropathy, Humans, Female, Middle Aged, Prognosis
Abstract

Guillain-Barré syndrome, or acute inflammatory demyelinating polyneuropathy, is a frequent cause of acute onset of flaccid paresis and areflexia. Electrophysiological studies show demyelination, sometimes with varying degrees of axonal degeneration. In some cases axonal degeneration apparently develops rapidly, without signs of primary demyelination. However, it is still a matter of discussion whether a pure axonal form of Guillain-Barré syndrome exists, or whether the axonal degeneration is secondary to demyelination. We report on clinical and electrophysiological findings in three patients with variants of Guillain-Barré syndrome. These include pure demyelination, combined demyelination and axonal degeneration and possible primary axonal degeneration. Electrophysiological studies can differentiate between the variants of Guillain-Barré syndrome, and thus give indications of pathogenesis and prognosis.

Published
1996

19. [Unusual intracranial infections]

Author

E, Dietrichs and C, Vedeler

Subjects
Travel, Norway, Immunologic Deficiency Syndromes, Encephalitis, Humans, Encephalitis, Viral, Emigration and Immigration
Abstract

The frequency of intracranial infections caused by microorganisms which have been uncommon in Norway is increasing. Contributing factors are travel, immigration and acquired immune deficiency, either as the result of disease or medical treatment. This article presents some of these infections, and also briefly reviews some other unusual infections which should be considered as differential diagnoses in unclear disease of the central nervous system.

Published
1995

20. The expression of CD59 in normal human nervous tissue

Author

C, Vedeler, E, Ulvestad, L, Bjørge, G, Conti, K, Williams, S, Mørk, and R, Matre

Subjects
Adult, Central Nervous System, Membrane Glycoproteins, Blotting, Western, Brain, chemical and pharmacologic phenomena, CD59 Antigens, Sciatic Nerve, Immunoenzyme Techniques, Molecular Weight, nervous system, Antigens, CD, Ganglia, Spinal, Peripheral Nervous System, Humans, Schwann Cells, Cells, Cultured, Research Article
Abstract

The expression of CD59, a complement regulator of the formation and function of the terminal cytolytic membrane attack complex, was studied in human normal nervous tissue by immunohistochemical markers using two monoclonal antibodies 1F5 and MEM43. CD59 was present on Schwann cells, neurons and endothelial cells in the peripheral nervous system (PNS), and on Schwann cells in culture. In the central nervous system (CNS) CD59 was found predominantly on endothelial cells. There was also a diffuse staining of white and grey matter of the spinal cord and brain, presumably of microglia, oligodendrocytes, astrocytes and neurons, as these cells were CD59 positive in culture. Furthermore, CD59 was detected in the cerebrospinal fluid (CSF) of healthy individuals. CD59 in the PNS and CNS was glycosyl-phosphatidylinositol linked and had a molecular weight of 19,000-25,000. The presence of CD59 on various cells of the nervous system and in the CSF suggests that regulation of complement activation by this protein is important in neural host defence mechanisms.

Published
1994

24. The Genesis of the Toleration Reforms in Bavaria under Montgelas

Author

Harold C. Vedeler

Subjects
History, Government, State (polity), Dismissal, media_common.quotation_subject, Political science, Economic history, Ethnology, Revolution from above, Toleration, Accession, media_common
Abstract

HAROLD C. VEDELER F nROM the accession of Maximilian IV Joseph on February 16, 1799, until the dismissal of Montgelas eighteen years later, the government of the last elector and the first king enacted a series of reforms which made Bavaria a modern state. The minister who inspired the policies underlying these changes and guided the fortunes of the state through the stormy era of the Napoleonic wars was Count Montgelas. For this reason his name has come to designate an epoch in Bavarian history. The content of the Montgelas reforms has been analyzed in detail; the motives and circumstances impelling the Bavarian government to this revolution from above have attracted far less attention.2 Since there are but few studies dealing with this latter subiect in its own rilht,3 it is impossible at the present

Published
1938
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25. [Poliomyelitis--an acute disease only?]

Author

N E, Gilhus, K, Todnem, C, Vedeler, and R E, Strandjord

Subjects
Male, Muscular Atrophy, Muscles, Humans, Paralysis, Middle Aged, Poliomyelitis
Published
1986

28. En mann i 70-årene med svekket kraft og følelse i beina.

Author

Nes MS, Søyland J, Haugen M, and Vedeler C

Subjects
Humans, Male, Diagnosis, Differential, Aged, Leg, Immunoglobulins, Intravenous therapeutic use, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating diagnosis, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating drug therapy
Abstract

Background: Over the last decade, antibodies targeting proteins at the node of Ranvier have been described in a subgroup of patients with autoimmune neuropathies, known as nodopathies. These disorders present an important differential diagnosis to chronic inflammatory demyelinating polyneuropathy (CIDP) due to the clinical overlap and the differences in treatment needed., Case Presentation: A previously healthy man in his seventies presented with a two-week history of mild paresis, diffuse sensory reduction and diminished tendon reflexes in the lower limbs. Findings from an MRI scan of the spinal cord were normal, but a spinal tap revealed albuminocytological dissociation. Neurography indicated acute motor sensory axonal neuropathy (AMSAN), leading to the patient being treated with IVIG., Interpretation: Para/nodopathies present important differential diagnoses to CIDP, as they do not respond to conventional treatments. Accurate diagnosis is crucial, as the treatment approach differs but is effective for both groups.

Published
2025
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29. The "Doing" of Compassionate Care in the Context of Childbirth from a Women's Perspective.

Author

Vedeler C, Nilsen ABV, Downe S, and Eri TS

Abstract

Women who are giving birth need to be met with compassion and understanding from healthcare professionals. However, there are growing concerns about the perceived lack of compassion in the delivery of healthcare services in general and maternity care in particular. We conducted 15 qualitative interviews with women who had given birth in Norway within the previous year, asking them to describe their experiences of compassionate care. We aimed to explore what healthcare professionals "do" that is experienced as compassionate. The analysis was informed by Paul Gilbert's theory of compassion and a concept analysis of compassionate midwifery undertaken by Ménage and colleagues. The compassionate caring actions of healthcare professionals that were identified in the women's narratives generated five themes: attuning actions, validating actions, contextualizing actions, empowering actions, and small acts of kindness. The findings build on the prior theoretical concepts used for the study and provide a nuanced account of how women perceive compassionate care from healthcare professionals. They could contribute to understanding more of the meaning and nature of compassionate care during childbirth. The analysis indicates the importance of ensuring that compassionate care is at the very core of maternity care services., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Published
2024
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30. Serum neurofilament light chain associates with symptom burden in Lyme neuroborreliosis patients: a longitudinal cohort study from Norway.

Author

Skarstein I, Ulvestad E, Solheim AM, Vedeler C, Ljøstad U, Mygland Å, Eikeland R, Reiso H, Lorentzen ÅR, and Bos SD

Subjects
Humans, Male, Female, Middle Aged, Norway, Adult, Aged, Longitudinal Studies, Double-Blind Method, Anti-Bacterial Agents administration & dosage, Doxycycline administration & dosage, Cohort Studies, Symptom Burden, Lyme Neuroborreliosis blood, Lyme Neuroborreliosis drug therapy, Lyme Neuroborreliosis diagnosis, Neurofilament Proteins blood, Biomarkers blood
Abstract

Objectives: Serum neurofilament light chain (sNfL), an indicator of neuronal damage, is increasingly recognized as a potential biomarker for disease activity in neurodegenerative disorders. In this study, we wanted to investigate sNfL as a prognostic marker in a large, well-defined population of 90 patients with Lyme neuroborreliosis (LNB). In addition, we sought to explore associations between symptoms and sNfL levels during the acute phase of LNB., Materials and Methods: Patients diagnosed with definite or possible LNB were recruited from a double-blinded, placebo-controlled, multi-center trial, in which the participants were randomly assigned to 2 or 6 weeks of oral doxycycline treatment. The sNfL levels were measured using a single molecule array assay at both diagnosis and 6-month follow-up, and analysed against clinical parameters, variations in symptom burden and long-term complaints as assessed by a composite clinical score., Results: At the time of diagnosis, approximately 60% of the patients had elevated sNfL levels adjusted for age. Notably, mean sNfL levels were significantly higher at diagnosis (52 pg/ml) compared to 6 months after treatment (12 pg/ml, p < 0.001), when sNfL levels had normalized in the majority of patients. Patients with objective signs of spinal radiculitis had significantly higher baseline sNfL levels compared to patients without spinal radiculitis (p = 0.033)., Conclusion: Our findings suggest that sNfL can serve as a biomarker for peripheral nerve tissue involvement in the acute phase of LNB. As found in an earlier study, we confirm normalization of sNfL levels in blood after treatment. We found no prognostic value of acute-phase sNfL levels on patient outcome., (© 2024. The Author(s).)

Published
2024
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31. A cerebellar degeneration-related protein 2-like cell-based assay for anti-Yo detection in patients with paraneoplastic cerebellar degeneration.

Author

Erikstad KI, Herdlevaer I, Peter E, Haugen M, Totland C, and Vedeler C

Subjects
Humans, Autoantibodies, Nerve Tissue Proteins, Recombinant Proteins, Cerebellar Diseases complications, Paraneoplastic Cerebellar Degeneration diagnosis, Paraneoplastic Cerebellar Degeneration complications
Abstract

Background and Purpose: Commercially available tests for Yo antibody detection have low specificity for paraneoplastic cerebellar degeneration (PCD) because these assays use cerebellar degeneration-related protein 2 (CDR2) as the antigen, not CDR2-like (CDR2L). We aimed to test the hypothesis that use of a CDR2L cell-based assay (CBA), as an additional screening technique, would increase the accuracy of Yo-PCD diagnosis., Methods: An in-house CBA to test for anti-CDR2L antibodies was developed and used to screen sera from 48 patients with confirmed anti-Yo-associated PCD. Fifteen non-Yo PCD patients, 22 patients with ovarian cancer without neurological syndromes, 50 healthy blood donors, 10 multiple sclerosis, 15 Parkinson's disease, and five non-paraneoplastic ataxic patients were included as controls. Sera were also tested by western blot analysis using recombinant CDR2 and CDR2L proteins developed in house, by the commercially available line immunoassays from Ravo Diagnostika and Euroimmun, and by the CDR2 CBA from Euroimmun., Results: The CDR2L CBA identified all 48 patients with Yo-PCD. No CDR2L CBA reaction was observed in any of the control sera. The western blot technique had lower sensitivity and specificity as sera from eight and six of the 48 Yo-PCD patients did not react with recombinant CDR2 or CDR2L, respectively., Conclusions: The CDR2L CBA is highly reliable for identification of Yo-PCD. Although our findings indicate that, currently, the combination of CDR2 and CDR2L yields the most reliable test results, it remains to be evaluated if a test for single anti-CDR2L positivity will serve as a sufficient biomarker for Yo-PCD diagnosis., (© 2023 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Published
2023
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32. Women's negative childbirth experiences and socioeconomic factors: Results from the Babies Born Better survey.

Author

Vedeler C, Eri TS, Nilsen RM, Blix E, Downe S, van der Wel KA, and Nilsen ABV

Subjects
Pregnancy, Female, Humans, Delivery, Obstetric methods, Socioeconomic Factors, Parturition psychology, Labor, Obstetric psychology
Abstract

Objective: To investigate the association between women's socioeconomic status and overall childbirth experience and to explore how women reporting an overall negative birth experience describe their experiences of intrapartum care., Methods: We used both quantitative and qualitative data from the Babies Born Better (B3) survey version 2, including a total of 8317 women. First, we performed regression analyses to explore the association between women's socioeconomic status and labour and birth experience, and then a thematic analysis of three open-ended questions from women reporting a negative childbirth experience (n = 917)., Results: In total 11.7% reported an overall negative labour and birth experience. The adjusted odds ratio (OR) of a negative childbirth experience was elevated for women with non-tertiary education, for unemployed, students and not married or cohabiting. Women with lower subjective living standard had an adjusted OR of 1.70 (95% CI 1.44-2.00) for a negative birth experience, compared with those with average subjective living standard. The qualitative analysis generated three themes: 1) Uncompassionate care: lack of sensitivity and empathy, 2) Impersonal care: feeling objectified, and 3) Critical situations: feeling unsafe and loss of control., Conclusion: Important socioeconomic disparities in women's childbirth experiences exist even in the Norwegian setting. Women reporting a negative childbirth experience described disrespect and mistreatment as well as experiences of insufficient attention and lack of awareness of individual and emotional needs during childbirth. The study shows that women with lower socioeconomic status are more exposed to these types of experiences during labour and birth., Tweetable Abstract: Women with lower socioeconomic status are more exposed to negative experiences during labour and birth., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 The Author(s). Published by Elsevier B.V. All rights reserved.)

Published
2023
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33. Giving birth and becoming a parent during the COVID-19 pandemic: A qualitative analysis of 806 women's responses to three open-ended questions in an online survey.

Author

Eri TS, Blix E, Downe S, Vedeler C, and Nilsen ABV

Subjects
Female, Humans, Infant, Newborn, Pandemics, Parents, Parturition, Pregnancy, COVID-19, Maternal Health Services
Abstract

Background: When Europe was hit by the COVID-19 pandemic, changes were made in maternity care to reduce infections. In Norway, hospital maternity wards, postnatal wards, and neonatal units' companions and visitors were restricted. We aimed to explore the experiences of being pregnant, giving birth and becoming a parent in Norway during the COVID-19 pandemic., Methods: The study is based on the responses from women who provided in-depth qualitative accounts to the ongoing Babies Born Better survey version 3 during the first year of the COVID-19 pandemic. The responses were analysed with inductive thematic analysis., Results: In all, 806 women were included, regardless of parity and mode of birth. They gave birth in 42 of 45 available birthing units across Norway. The analysis resulted in four themes: 1) Pregnancy as a stressful waiting period; 2) Feeling lonely, isolated, and disempowered without their partner; 3) Sharing experiences and becoming a family; and 4) Busy postnatal care without compassion., Conclusion: The COVID-19 pandemic seems to have affected women's experiences of giving birth and becoming a parent in Norway. The restrictions placed on companionship by the healthcare facilities varied between hospitals. However, the restrictions seem to have affected a range of aspects related to women's experiences of late pregnancy, early labour and birth and the early postpartum period. Postnatal care was already poor, and the pandemic has highlighted the shortcomings, especially where companionship was banned., Competing Interests: Declaration of Competing Interest The authors report no conflicts of interest., (Copyright © 2022 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Published
2022
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34. What women emphasise as important aspects of care in childbirth - an online survey.

Author

Vedeler C, Nilsen A, Blix E, Downe S, and Eri TS

Subjects
Empathy, Female, Humans, Norway, Parturition, Patient Satisfaction, Pregnancy, Prenatal Care standards, Qualitative Research, Surveys and Questionnaires, Delivery, Obstetric psychology, Patient Preference, Prenatal Care psychology
Abstract

Objective: To explore and describe what women who have given birth in Norway emphasise as important aspects of care during childbirth., Design: The study is based on data from the Babies Born Better online survey, version 2., Setting: The maternity care system in Norway., Study Population: Women who gave birth in Norway between 2013 and 2018., Method: Descriptive statistics were used to describe sample characteristics and to compare data from the B3 survey with national data from the Medical Birth Registry of Norway. The open-ended questions were analysed with an inductive thematic analysis., Main Outcome Measures: Themes developed from two open-ended questions., Results: The final sample included 8401 women. There were no obvious differences between the sample population and the national population with respect to maternal age, marital status, parity, mode of birth and place of birth, except for the proportion of planned home births. Four themes and one overarching theme were identified; Compassionate and Respectful Care, A Family Focus, Sense of Continuity and Consistency, and Sense of Security. Overarching theme: Coherence in Childbearing., Conclusions: Norwegian women across all birth settings emphasise maternity care that authentically focuses on both socio-cultural and psychological aspects of care, and physical and clinical factors. If the positive aspects of care identified in this study are adopted at all levels of the maternity care system and from all care providers, there is a high chance that most women will have a safe outcome, and a strong sense of coherence related to a positive birth and motherhood experience., Tweetable Abstract: Having a baby is a pivotal life changing experience and not just a clinical event, according to a survey of 8400 women in Norway. Positive birth and motherhood experiences depend on maternity staff who are both skilled and kind., (© 2021 The Authors. BJOG: An International Journal of Obstetrics and Gynaecology published by John Wiley & Sons Ltd.)

Published
2022
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35. Expression of cerebellar degeneration-related proteins CDR2 and CDR2L in human and rat brain tissue.

Author

Raspotnig M, Kråkenes T, Herdlevær I, Haugen M, and Vedeler C

Subjects
Animals, Ependyma metabolism, Humans, Muscle, Smooth, Vascular metabolism, Purkinje Cells metabolism, Rats, Rats, Wistar, Autoantigens metabolism, Cerebellum metabolism, Nerve Tissue Proteins metabolism
Abstract

Patients with ovarian cancer and paraneoplastic cerebellar degeneration, a cancer-related immune disorder, often have anti-Yo antibody. Here we studied the distributions of anti-Yo antigens CDR2L and CDR2 in rat and human brain using immunohistochemistry and western blot. CDR2L localized mainly to the Purkinje cells and large neurons scattered in the brain stem. CDR2 was detected in vascular smooth muscle cells of rat and human and in cells lining the ventricle system in rats. The observed distribution of CDR2L is compatible with the hypothesis that this antigen is the major target of anti-Yo. CDR2 and CDR2L are expressed by different cell subtypes., (Copyright © 2021. Published by Elsevier B.V.)

Published
2022
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36. CRMP5 Antibodies-Diagnostic Challenges.

Author

Totland C, Haugen M, and Vedeler C

Abstract

CRMP5-associated paraneoplastic neurological syndromes (PNS) are rare, and only few studies describe larger cohorts of patients with CRMP5 antibodies. We have included 24 patients with CRMP5 antibodies and compared clinical findings with diagnostic findings from two different line assays (Ravo and Euroimmun), staining of cerebellar sections and results of a newly developed cell-based assay for detection of CRMP5 antibodies, CRMP5-CBA. We found that peripheral neuropathy and cerebellar ataxia together with lung cancer were the most common diagnoses associated with CRMP5 antibodies. CRMP5-CBA was easy to perform, identified all relevant cases for CRMP5-associated PNS and is therefore a valuable add-on for verification of CRMP5 positivity in diagnosis of PNS., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Totland, Haugen and Vedeler.)

Published
2021
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37. Paraneoplastic Cerebellar Degeneration: The Importance of Including CDR2L as a Diagnostic Marker.

Author

Herdlevær I, Haugen M, Mazengia K, Totland C, and Vedeler C

Subjects
Adult, Aged, Animals, Biomarkers blood, Biomarkers cerebrospinal fluid, Female, HEK293 Cells, Humans, Male, Middle Aged, Paraneoplastic Cerebellar Degeneration diagnosis, Rats, Retrospective Studies, Autoantigens blood, Autoantigens cerebrospinal fluid, Nerve Tissue Proteins blood, Nerve Tissue Proteins cerebrospinal fluid, Paraneoplastic Cerebellar Degeneration blood, Paraneoplastic Cerebellar Degeneration cerebrospinal fluid
Abstract

Objective: Investigate the value of including cerebellar degeneration-related protein 2-like (CDR2L) as a marker in commercial diagnostic tests for anti-Yo-associated paraneoplastic cerebellar degeneration (PCD)., Methods: We included sera and CSF samples from 24 patients with suspected PCD (6 of whom had PCD with underlying gynecologic or breast cancer), who were positive for Yo antibodies using the commercially available, paraneoplastic neurologic syndromes (PNS) 14 Line Assay from Ravo Diagnostika. The samples were further evaluated using the EUROLINE PNS 12 Ag Line Assay and a cell-based assay (CBA) from Euroimmun. For confirmation of positive lineblot results, we used indirect immunofluorescence of rat cerebellar sections. We also tested all samples in 2 assays developed in-house: a CBA for CDR2L and a Western blot analysis using recombinant cerebellar degeneration-related protein 2 (CDR2) and CDR2L proteins., Results: In PNS 14 and PNS 12 Ag Line Assays, anti-CDR2 reactivity was observed for 24 (100%) and 20 (83%) of the 24 samples, respectively. Thirteen of 24 subjects (54%) were also positive using the Euroimmun CBA. Rat cerebellar immunofluorescence was the best confirmatory test. In our in-house CBA for CDR2L and Western blot for CDR2 and CDR2L, only the 6 patients with confirmed PCD reacted with CDR2L., Conclusions: Commercially available tests for Yo antibody detection have low specificity for PCD because these assays use CDR2 as antigen. By adding a test for CDR2L, which is the major Yo antigen, the accuracy of PCD diagnosis greatly improved., Classification of Evidence: This study provides Class III evidence that a CBA for CDR2L accurately identifies patients with PCD., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

Published
2021
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39. Candidate Markers for Stratification and Classification in Rheumatoid Arthritis.

Author

Bader L, Gullaksen SE, Blaser N, Brun M, Bringeland GH, Sulen A, Gjesdal CG, Vedeler C, and Gavasso S

Subjects
Adult, Aged, Arthritis, Rheumatoid pathology, Biomarkers metabolism, Female, Humans, Male, Middle Aged, Neutrophils immunology, Proto-Oncogene Proteins c-akt metabolism, Receptors, Tumor Necrosis Factor, Type II metabolism, Arthritis, Rheumatoid classification, Arthritis, Rheumatoid diagnosis, CD4-Positive T-Lymphocytes immunology, Monocytes immunology, Tumor Necrosis Factor-alpha metabolism
Abstract

Rheumatoid arthritis (RA) is a chronic autoimmune, inflammatory disease, characterized by synovitis in small- and medium-sized joints and, if not treated early and efficiently, joint damage, and destruction. RA is a heterogeneous disease with a plethora of treatment options. The pro-inflammatory cytokine tumor necrosis factor (TNF) plays a central role in the pathogenesis of RA, and TNF inhibitors effectively repress inflammatory activity in RA. Currently, treatment decisions are primarily based on empirics and economic considerations. However, the considerable interpatient variability in response to treatment is a challenge. Markers for a more exact patient classification and stratification are lacking. The objective of this study was to identify markers in immune cell populations that distinguish RA patients from healthy donors with an emphasis on TNF signaling. We employed mass cytometry (CyTOF) with a panel of 13 phenotyping and 10 functional markers to explore signaling in unstimulated and TNF-stimulated peripheral blood mononuclear cells from 20 newly diagnosed, untreated RA patients and 20 healthy donors. The resulting high-dimensional data were analyzed in three independent analysis pipelines, characterized by differences in both data clean-up, identification of cell subsets/clustering and statistical approaches. All three analysis pipelines identified p-p38, IkBa, p-cJun, p-NFkB, and CD86 in cells of both the innate arm (myeloid dendritic cells and classical monocytes) and the adaptive arm (memory CD4 + T cells) of the immune system as markers for differentiation between RA patients and healthy donors. Inclusion of the markers p-Akt and CD120b resulted in the correct classification of 18 of 20 RA patients and 17 of 20 healthy donors in regression modeling based on a combined model of basal and TNF-induced signal. Expression patterns in a set of functional markers and specific immune cell subsets were distinct in RA patients compared to healthy individuals. These signatures may support studies of disease pathogenesis, provide candidate markers for response, and non-response to TNF inhibitor treatment, and aid the identification of future therapeutic targets.

Published
2019
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40. Charcot-Marie-Tooth disease type 4C in Norway: Clinical characteristics, mutation spectrum and minimum prevalence.

Author

Arntzen KA, Høyer H, Ørstavik K, Tallaksen C, Vedeler C, Østern R, Nebuchennykh M, Braathen GJ, and Fagerheim T

Subjects
Adolescent, Adult, Charcot-Marie-Tooth Disease epidemiology, Child, Child, Preschool, Female, Humans, Infant, Intracellular Signaling Peptides and Proteins, Male, Middle Aged, Norway epidemiology, Prevalence, Young Adult, Charcot-Marie-Tooth Disease genetics, Mutation, Proteins genetics
Abstract

Autosomal recessive Charcot-Marie-Tooth disease (CMT) is considered rare and phenotypic descriptions are scarce for the different subgroups. Mutations in the SH3TC2 gene, causing recessive demyelinating CMT type 4C have been found in several Norwegian CMT patients over the last years. We aimed to estimate a minimum prevalence and to study the genotypic and phenotypic variability of CMT4C in Norway. Patients were selected from diagnostic registries in medical genetic centers in Norway for cases of CMT4C. All patients were invited to complete a questionnaire and give medical consent to the use of clinical data from medical hospital records. A total of 35 patients from 31 families were found with CMT4C, which gives a minimum prevalence of 0.7/100,000 in Norway. Six new mutations were identified. Most patients had debut in the first decade with foot deformities, distal limb paresis, sensory ataxia and scoliosis. Proximal lower limb paresis and cranial nerve involvement was seen in about half of the patients. CMT4C is the most common recessive CMT in Norway. In addition to the classic distal limb affection, early debut, scoliosis, proximal paresis, cranial nerve affection and sensory ataxia are the most prominent features of CMT4C., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Published
2018
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41. Expression of the onconeural protein CDR1 in cerebellum and ovarian cancer.

Author

Totland C, Kråkenes T, Mazengia K, Haugen M, and Vedeler C

Abstract

Cerebellar degeneration related protein 1 (CDR1) is expressed in the cerebellum, and CDR1 antibodies have been associated with paraneoplastic cerebellar degeneration (PCD). In this study, we examined CDR1 expression in cerebellum and in ovarian and breast tumors, as well as the intracellular localization of CDR1 in cancer cells in culture. CDR1 was strongly expressed in the cytosol and dendrites of Purkinje cells and in interneurons of the molecular layer in cerebellum. CDR1 was also present in ovarian and breast tumors, as well as in ovarian and breast cancer cell lines, but was not present in normal breast or ovarian tissue. In cells overexpressing CDR1, CDR1 localized close to the plasma membrane in a polarized pattern at one edge. CDR1 was strongly expressed on the outer surface, apparently in filopodias or lamellipodias, in cells endogenously expressing CDR1. Overexpression of CDR1 showed a 37 and a 45 kDa band in western blot. The 37-kDa isoform was present in 16 ovarian cancer lysates, while the 45-kDa isoform was only found in three ovarian cancer patients. The presence of CDR1 in ovarian cancer was not associated with PCD. CDR1 antibodies were only found in serum from one patient with PCD and ovarian tumor with metastases. Therefore, CDR1 is probably not a marker for PCD. However, CDR1 may be associated with cell migration and differentiation., Competing Interests: CONFLICTS OF INTEREST The authors have no conflicts of interest to disclose.

Published
2018
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42. Neurofilament light chain predicts disease activity in relapsing-remitting MS.

Author

Varhaug KN, Barro C, Bjørnevik K, Myhr KM, Torkildsen Ø, Wergeland S, Bindoff LA, Kuhle J, and Vedeler C

Abstract

Objective: To investigate whether serum neurofilament light chain (NF-L) and chitinase 3-like 1 (CHI3L1) predict disease activity in relapsing-remitting MS (RRMS)., Methods: A cohort of 85 patients with RRMS were followed for 2 years (6 months without disease-modifying treatment and 18 months with interferon-beta 1a [IFNB-1a]). Expanded Disability Status Scale was scored at baseline and every 6 months thereafter. MRI was performed at baseline and monthly for 9 months and then at months 12 and 24. Serum samples were collected at baseline and months 3, 6, 12, and 24. We analyzed the serum levels of NF-L using a single-molecule array assay and CHI3L1 by ELISA and estimated the association with clinical and MRI disease activity using mixed-effects models., Results: NF-L levels were significantly higher in patients with new T1 gadolinium-enhancing lesions (37.3 pg/mL, interquartile range [IQR] 25.9-52.4) and new T2 lesions (37.3 pg/mL, IQR 25.1-48.5) compared with those without (28.0 pg/mL, IQR 21.9-36.4, β = 1.258, p < 0.001 and 27.7 pg/mL, IQR 21.8-35.1, β = 1.251, p < 0.001, respectively). NF-L levels were associated with the presence of T1 gadolinium-enhanced lesions up to 2 months before ( p < 0.001) and 1 month after ( p = 0.009) the time of biomarker measurement. NF-L levels fell after initiation of IFNB-1a treatment ( p < 0.001). Changes in CHI3L1 were not associated with clinical or MRI disease activity or interferon-beta 1a treatment., Conclusion: Serum NF-L could be a promising biomarker for subclinical MRI activity and treatment response in RRMS. In clinically stable patients, serum NF-L may offer an alternative to MRI monitoring for subclinical disease activity., Clinicaltrialsgov Identifier: NCT00360906.

Published
2017
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43. Morvan syndrome with Caspr2 antibodies. Clinical and autopsy report.

Author

Sundal C, Vedeler C, Miletic H, and Andersen O

Subjects
Aged, Autoantibodies, Autopsy, Complement C1q metabolism, HLA-DR Antigens metabolism, Humans, Leukocyte Common Antigens metabolism, Magnetic Resonance Imaging, Male, Membrane Proteins immunology, Nerve Tissue Proteins immunology, Syringomyelia blood, Syringomyelia immunology
Published
2017
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44. Multicentre comparison of a diagnostic assay: aquaporin-4 antibodies in neuromyelitis optica.

Author

Waters P, Reindl M, Saiz A, Schanda K, Tuller F, Kral V, Nytrova P, Sobek O, Nielsen HH, Barington T, Lillevang ST, Illes Z, Rentzsch K, Berthele A, Berki T, Granieri L, Bertolotto A, Giometto B, Zuliani L, Hamann D, van Pelt ED, Hintzen R, Höftberger R, Costa C, Comabella M, Montalban X, Tintoré M, Siva A, Altintas A, Deniz G, Woodhall M, Palace J, Paul F, Hartung HP, Aktas O, Jarius S, Wildemann B, Vedeler C, Ruiz A, Leite MI, Trillenberg P, Probst M, Saschenbrecker S, Vincent A, and Marignier R

Subjects
Aquaporin 4 immunology, Autoantibodies immunology, Enzyme-Linked Immunosorbent Assay methods, Humans, Immunohistochemistry methods, Neuromyelitis Optica immunology, Sensitivity and Specificity, Aquaporin 4 blood, Autoantibodies blood, Neuromyelitis Optica blood
Abstract

Objective: Antibodies to cell surface central nervous system proteins help to diagnose conditions which often respond to immunotherapies. The assessment of antibody assays needs to reflect their clinical utility. We report the results of a multicentre study of aquaporin (AQP) 4 antibody (AQP4-Ab) assays in neuromyelitis optica spectrum disorders (NMOSD)., Methods: Coded samples from patients with neuromyelitis optica (NMO) or NMOSD (101) and controls (92) were tested at 15 European diagnostic centres using 21 assays including live (n=3) or fixed cell-based assays (n=10), flow cytometry (n=4), immunohistochemistry (n=3) and ELISA (n=1)., Results: Results of tests on 92 controls identified 12assays as highly specific (0-1 false-positive results). 32 samples from 50 (64%) NMO sera and 34 from 51 (67%) NMOSD sera were positive on at least two of the 12 highly specific assays, leaving 35 patients with seronegative NMO/spectrum disorder (SD). On the basis of a combination of clinical phenotype and the highly specific assays, 66 AQP4-Ab seropositive samples were used to establish the sensitivities (51.5-100%) of all 21 assays. The specificities (85.8-100%) were based on 92 control samples and 35 seronegative NMO/SD patient samples., Conclusions: The cell-based assays were most sensitive and specific overall, but immunohistochemistry or flow cytometry could be equally accurate in specialist centres. Since patients with AQP4-Ab negative NMO/SD require different management, the use of both appropriate control samples and defined seronegative NMOSD samples is essential to evaluate these assays in a clinically meaningful way. The process described here can be applied to the evaluation of other antibody assays in the newly evolving field of autoimmune neurology., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/)

Published
2016
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45. A case of relapsing-remitting facial palsy and ipsilateral brachial plexopathy caused by HSV-1.

Author

Alstadhaug KB, Kvarenes HW, Prytz J, and Vedeler C

Subjects
Acyclovir administration & dosage, Antiviral Agents administration & dosage, Brachial Plexus diagnostic imaging, Brachial Plexus pathology, Chemoprevention methods, DNA, Viral isolation & purification, Female, Humans, Magnetic Resonance Imaging, Mannose-Binding Lectin deficiency, Mouth Mucosa virology, Recurrence, Young Adult, Brachial Plexus Neuropathies etiology, Brachial Plexus Neuropathies pathology, Facial Paralysis etiology, Facial Paralysis pathology, Herpes Simplex complications, Herpesvirus 1, Human isolation & purification
Abstract

The etiologies of Bell's palsy and brachial neuritis remain uncertain, and the conditions rarely co-occur or reoccur. Here we present a woman in her twenties who had several relapsing-remitting episodes with left-sided facial palsy and brachial neuropathy. The episodes always started with painful left-sided oral blisters. Repeat PCRs HSV-1 DNA from oral vesicular lesions were positive. Extensive screening did not reveal any other underlying cause. Findings on MRI T2-weighted brachial plexus STIR images, using a 3.0-Tesla scanner during an episode, were compatible with brachial plexus neuritis. Except a mannose-binding lectin deficiency, a congenital complement deficiency that is frequently found in the general Caucasian population, no other immunodeficiency was demonstrated in our patient. In vitro resistance to acyclovir was tested negative, but despite prophylactic treatment with the drug in high doses, relapses recurred. To our knowledge, this is the first ever reported documentation of relapsing-remitting facial and brachial plexus neuritis caused by HSV-1., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Published
2016
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46. Paraneoplastic syndrome-associated neuronal antibodies in adult ADHD.

Author

Haukanes BI, Hegvik TA, Eichler T, Haavik J, and Vedeler C

Subjects
Adolescent, Adult, Animals, Female, Fluorescent Antibody Technique, Humans, Immunoblotting, Male, Middle Aged, Rats, Young Adult, Attention Deficit Disorder with Hyperactivity blood, Attention Deficit Disorder with Hyperactivity immunology, Autoantibodies blood, Neurons immunology
Abstract

A high seroprevalence of Yo antibodies targeting cerebellar Purkinje cells was recently reported in children with attention deficit/hyperactivity disorder (ADHD). We investigated the presence of 8 paraneoplastic neurological syndrome (PNS)-associated antibodies including anti-Yo in 169 adult ADHD patients. No associations between ADHD and serum Yo antibodies or other antibodies associated with PNS were found. However, 10 out of 48 ADHD patient sera analyzed by immunofluorescence presented antibodies targeting cerebellar Purkinje cells. This reactivity probably represents the presence of low levels of antibodies against multiple cellular hitherto unknown antigens with little to no clinical significance., (Copyright © 2015 The Authors. Published by Elsevier B.V. All rights reserved.)

Published
2015
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47. Paraneoplastic neurological syndromes in lung cancer patients with or without onconeural antibodies.

Author

Raspotnig M, Vedeler C, and Storstein A

Subjects
Aged, Aged, 80 and over, Female, Humans, Lung Neoplasms complications, Male, Middle Aged, Norway, Paraneoplastic Syndromes, Nervous System etiology, Small Cell Lung Carcinoma complications, Antibodies, Neoplasm blood, Lung Neoplasms immunology, Paraneoplastic Syndromes, Nervous System immunology, Registries, Small Cell Lung Carcinoma immunology
Abstract

Background: Paraneoplastic neurological syndromes (PNS) are poorly described in patients without onconeural antibodies and in patients with non-small cell lung cancer (NSCLC). We compared the clinical characteristics of PNS in lung cancer patients with and without onconeural antibodies., Methods: Medical records from patients with lung cancer and neurological symptoms referred for onconeural antibody analysis in the period 1995-2004 were analyzed and well-established diagnostic criteria used for the retrospective diagnosis of PNS. Thirty-one patients were diagnosed with PNS and included in the study. Data from the Cancer Registry of Norway and follow-up medical data were analyzed., Results: Small-cell lung cancer (SCLC) was the most common lung cancer in the 31 PNS patients (77%, P<0.01). Onconeural antibodies were found in 18 of the PNS patients (58%). Paraneoplastic encephalomyelitis (PEM) was the most common PNS among the seropositive patients (11 of 18 patients), of which 10 had SCLC. Various types of PNS were found in the 13 seronegative patients., Conclusion: Approximately 40% of PNS patients with lung cancer do not have onconeural antibodies. PEM was the most common PNS in the seropositive patients. Our results underline the importance of recognizing PNS in patients with NSCLC and those without onconeural antibodies., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Published
2015
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48. Deficient phosphorylation of Stat1 in leukocytes identifies neutralizing antibodies in multiple sclerosis patients treated with interferon-beta.

Author

Gavasso S, Mosleth EF, Marøy T, Jørgensen K, Nakkestad HL, Gjertsen BT, Myhr KM, and Vedeler C

Subjects
Adult, Female, Gene Expression Regulation drug effects, Gene Expression Regulation immunology, Humans, Interferon-beta blood, Interferon-beta pharmacology, Leukocytes drug effects, Male, Middle Aged, Multiple Sclerosis pathology, Phosphoproteins metabolism, Phosphorylation drug effects, Phosphorylation immunology, Signal Transduction drug effects, Signal Transduction immunology, Single-Cell Analysis, Treatment Failure, Antibodies, Neutralizing immunology, Interferon-beta immunology, Interferon-beta therapeutic use, Leukocytes metabolism, Multiple Sclerosis drug therapy, Multiple Sclerosis immunology, STAT1 Transcription Factor metabolism
Abstract

Background: Anti interferon-beta (IFN-β) neutralizing antibodies (NAb) affect efficacy of treatment of multiple sclerosis patients, but exactly when the detrimental effects of NAbs offset therapeutic efficacy is debated. Quantification of intracellular pathway-specific phosphorylation by phospho-specific flow cytometry (phosphoflow) is a promising tool for evaluation of these effects in primary immune cells from treated patients at the single-cell level., Method: Samples for phosphoflow and gene expression changes were collected before administration of IFN-β and at four, six, and eight hours thereafter. Patients were NAb negative (n = 3) or were NAb positive with low/medium (n = 1) or high (n = 2) NAb titers. Levels of phosphorylation of six Stat transcription factors (pStat) in seven cell subtypes and expression levels of 71 pathway-specific genes in whole blood were measured. The data was subjected to principal component analysis (PCA), fifty-fifty MANOVA, ANOVA, and partial least square regression (PLSR)., Results: PCA of pStat levels clustered patients according to NAb class independently of time. PCA of gene expression data clustered patients according to NAb class but was affected by time and treatment. In the fifty-fifty MANOVA, NAb class was significant for both pStat levels and gene expression data. The ANOVA identified pStat1 protein in several cell subtypes as significantly affected by NAb class. The best fitting model for NAb prediction based on PLSR included pStat1 in monocytes, T cells, or lymphocytes and pStat3 in monocytes (r = 0.97). Gene expression data were slightly less predictive of NAb titers., Conclusion: Based on this proof of concept study, we hypothesize that NAb effects can be monitored by evaluation of a single biomarker, pStat1, in either monocytes or T cells by phosphoflow directly after IFN-β administration. The method will significantly reduce cost relative to labor intensive in vitro methods and offers a patient-specific approach to NAb evaluation.

Published
2014
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49. Avidity of onconeural antibodies is of clinical relevance.

Author

Totland C, Ying M, Haugen M, Mazengia K, Storstein A, Aarseth J, Martinez A, and Vedeler C

Subjects
Antibodies, Neoplasm blood, ELAV Proteins immunology, Humans, Hydrolases, Immunoprecipitation, Microtubule-Associated Proteins, Nerve Tissue Proteins immunology, Paraneoplastic Syndromes blood, Sensitivity and Specificity, Surface Plasmon Resonance, Antibodies, Neoplasm immunology, Antibody Affinity, Paraneoplastic Syndromes diagnosis, Paraneoplastic Syndromes immunology
Abstract

Onconeural antibodies are important in the detection of paraneoplastic neurological syndromes (PNS). The avidity of Hu, Yo, and CRMP5 antibodies from 100 patients was determined by immunoprecipitation (IP), and 13 of the Yo positive sera were also tested by surface plasmon resonance (SPR). There was a significant association between the results from IP and SPR. Yo antibodies had higher avidity than Hu and CRMP5 antibodies, and both high- and low-avidity antibodies were associated with tumors and PNS. High-avidity Yo antibodies were mainly associated with ovarian cancer, whereas high-avidity Hu and CRMP5 antibodies were mainly associated with small-cell lung cancer. Low-avidity CRMP5 and Yo antibodies were less often detected by a commercial line blot than high-avidity antibodies. The failure to detect low-avidity onconeural antibodies may result in under diagnosis of PNS.

Published
2013
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50. Progressive striatal necrosis associated with anti-NMDA receptor antibodies.

Author

Tzoulis C, Vedeler C, Haugen M, Storstein A, Tran GT, Gjerde IO, Biermann M, Schwarzlmüller T, and Bindoff LA

Subjects
Adolescent, Brain Diseases drug therapy, Brain Diseases physiopathology, Corpus Striatum diagnostic imaging, Disease Progression, Fluorodeoxyglucose F18, Humans, Immunoglobulins, Intravenous therapeutic use, Immunologic Factors therapeutic use, Male, Necrosis diagnostic imaging, Necrosis pathology, Positron-Emission Tomography, Tomography, X-Ray Computed, Antibodies metabolism, Brain Diseases pathology, Corpus Striatum pathology, Receptors, N-Methyl-D-Aspartate immunology
Abstract

Background: We report a case of childhood onset, generalized dystonia due to slowly progressive bilateral striatal necrosis associated with anti-N-methyl-D-aspartate receptor (NMDAR) antibodies. This clinical phenotype has not been previously associated with NMDA receptor autoimmunity., Case Presentation: An eighteen year old man presented with a history of childhood-onset, progressive generalized dystonia. Clinical examination revealed a pure generalized dystonia with no cognitive or other neurological findings. Magnetic resonance imaging showed bilateral high T2 signal striatal lesions, which were slowly progressive over a period of nine years. New parts of the lesion showed restricted water diffusion suggesting cytotoxic oedema. Positron emission tomography of the brain showed frontal hypermetabolism and cerebellar hypometabolism. Antibodies against the NR1 subunit of the NMDA receptor were detected in the patient's serum and cerebrospinal fluid. There was no neoplasia or preceding infection or vaccination., Conclusion: This is the first report of chronic progressive bilateral striatal necrosis associated with anti-NMDAR antibodies. Our findings expand the clinical spectrum of disease associated with anti-NMDAR antibodies and suggest that these should be included in the work-up of dystonia with striatal necrosis.

Published
2013
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