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1. The V471A polymorphism in autophagy-related gene ATG7 modifies age at onset specifically in Italian Huntington disease patients

Author

Metzger S, Walter C, Riess O, Roos RA, Nielsen JE, Craufurd D, Nguyen HP, REGISTRY Investigators of the European Huntington’s Disease Network:Bachoud Lévi AC, Bentivoglio AR, Biunno I, Bonelli RM, Burgunder JM, Dunnett SB, Ferreira JJ, Handley OJ, Heiberg A, Illmann T, Landwehrmeyer G, Levey J, Ramos Arroyo MA, Nielsen J, Koivisto SP, Päivärinta M, Sebastián AR, Tabrizi S, Vandenberghe W, Verellen Dumoulin C, Zaremba J, Uhrová T, Wahlström J, Barth K, Correia Guedes L, Finisterra AM, Garde MB, Bos R, Betz S, Callaghan J, Fullam R, Ecker D, Nielsen MG, Hvalstedt C, Held C, Koppers K, Laurà M, Horta SM, Descals AM, Díaz MF, Mestre T, Minster S, Monza D, Mütze L, Oehmen M, Townhill J, Orth M, Padieu H, Paterski L, Peppa N, Roedig V, Rialland A, Røren N, Šašinková P, Seliverstov Y, Cubillo PT, van Walsem MR, Wright A, Silva WV, Witjes Anné MN, Yudina E, Zielonka D, Zielonka E, Zinzi P, Herranhof B, Holl A, Kapfhammer HP, Koppitz M, Magnet M, Otti D, Painold A, Reisinger K, Scheibl M, Hecht K, Lilek S, Müller N, Schöggl H, Ullah J, Ribaï P, Boogaerts A, van Reijen D, Klempíř J, Majerová V, Roth J, Hjermind L, Jacobsen O, Vinthev Jensen T, Larsen IU, Stockholm J, Hiivola H, Martikainen K, Tuuha K, Ignatius J, Kärppä M, Åman J, Mustonen A, Kajula O, Santala M, Allain P, Guérid MA, Gohier B, Olivier A, Prundean A, Scherer Gagou C, Verny C, Bost M, Babiloni B, Debruxelles S, Duché C, Goizet C, Lafoucrière D, Jameau L, Spampinato U, De Bruycker C, Cabaret M, Carette AS, Defebvre L, Decorte E, Delval A, Delliaux M, Destee A, Dujardin K, Peter M, Plomhouse L, Sablonnière B, Simonin C, Lemaire MH, Manouvrier S, Thibault Tanchou S, Vuillaume I, Krystkowiak P, Duru C, Roussel M, Wannepain S, Berrissoul H, Bellonet M, Courtin F, Mantaux B, Fasquel V, Godefroy O, Azulay JP, Fluchère F, Delfini M, Eusebio A, Mundler L, Longato N, Rudolf G, Steinmetz G, Tranchant C, Wagner C, Zimmermann MA, Marcel C, Andrich J, Ellrichmann G, Hoffmann R, Kaminski B, Saft C, Stamm C, Boelmans K, Ganos C, Goerendt I, Hidding U, Lewerenz J, Münchau A, Schmalfeld J, Stubbe L, Zittel S, Bürk K, Möller JC, Rissling I, Cormio C, Sciruicchio V, Serpino C, de Tommaso M, CAPELLARI, SABINA, CORTELLI, PIETRO, Gallassi R, PODA, ROBERTO, RIZZO, GIOVANNI, Scaglione C, Abbruzzese G, di Poggio MB, Di Maria E, Ferrandes G, Mandich P, Marchese R, Albanese A, Di Bella D, Di Donato S, Gellera C, Genitrini S, Mariotti C, Nanetti L, Paridi D, Soliveri P, Tomasello C, Squitieri F, Elifani F, Maglione V, Di Pardo A, Alberti S, Griguoli A, Amico E, Martino T, Petrollini M, Catalli C, Di Giacopo R, Fasano A, Frontali M, Guidubaldi A, Ialongo T, Jacopini G, Loria G, Piano C, Chiara P, Quaranta D, Romano S, Soleti F, Spadaro M, van Hout MS, van Vugt JP, de Weert A, Bolwijn JJ, Dekker M, Leenders KL, Dumas EM, van den Bogaard SJ, 't Hart EP, van Duijn E, Kremer B, Verstappen CC, Blinkenberg EØ, Hauge E, Tyvoll H, Frich J, Aaserud O, Wehus R, Bjørgo K, Fannemel M, Gørvell P, Lorentzen E, Retterstøl L, Overland T, Stokke B, Bjørnevoll I, Sando SB, Dziadkiewicz A, Nowak M, Robowski P, Sitek E, Slawek J, Soltan W, Szinwelski M, Blaszcyk M, Boczarska Jedynak M, Ciach Wysocka E, Gorzkowska A, Jasinska Myga B, Opala G, Kłodowska Duda G, Stompel D, Banaszkiewicz K, Boćwińska D, Szczudlik A, Rudzinska M, Wójcik M, Dec M, Krawczyk M, Bojakowska Jaremek K, Szczygieł E, Stenwak A, Wasielewska A, Bryl A, Ciesielska A, Klimberg A, Marcinkowski J, Sempołowicz J, Samara H, Wiśniewski B, Janik P, Gogol A, Kwiecinski H, Jamrozik Z, Kaminska A, Antczak J, Jachinska K, Rakowicz M, Richter P, Rola R, Ryglewicz D, Sienkiewicz Jarosz H, Stępniak I, Witkowski G, Zdzienicka E, Sułek A, Krysa W, Stepniak I, Zieora Jakutowicz K, Júlio F, Januário C, Coelho M, Mendes T, Valadas A, Andrade C, Gago M, Garrett C, Guerra MR, Lima J, Massano J, Meireles J, Herrera CD, Garcia PM, Barrero F, Morales B, Cubo E, Mariscal N, Sánchez J, Alonso Frech F, Perez MR, Fenollar M, García RG, Pin Quiroga P, Vázquez Rivera S, Villanueva C, Alegre J, Bascuñana M, Caldentey JG, Ventura MF, Ribas GG, de Yébenes JG, Moreno JL, Ruíz PJ, Martínez Descals A, Artiga MJ, Sánchez V, Guerrero R, Bárcenas AH, Perea MF, Fortuna L, Torres MM, Reinante G, Moreau LV, Barbera MA, Guia DB, Hernanz LC, Catena JL, Ferrer PQ, Carruesco GT, Bas J, Busquets N, Calopa M, Elorza MD, López CD, Durán Sindreu Terol S, Robert MF, Ruíz BG, Casado AG, Martínez IH, Viladrich CM, Cárdenas RP, Roca E, Llesoy JR, Idiago JM, Vergara MR, García SS, Riballo AV, González SG, Guisasola LM, Salvador C, Martín ES, González M, Gorospe A, Legarda I, Arques PN, Rodríguez MJ, Vives B, Gaston I, Martinez Jaurrieta MD, Moreno JM, Peña JC, Avarvarei LD, Bastida AM, Recio MF, Vergé LR, Sánchez VS, Carrillo F, Cáceres MT, Mir P, Suarez MJ, Loutfi G, Olofsson C, Stattin EL, Westman L, Wikström B, Pålhagen SE, Paucar M, Svenningsson P, Reza Soltani TW, Höglund A, Sandström B, Høsterey Ugander U, Fredlund G, Constantinescu R, Neleborn Lingefjärd L, Stebler Y, Kaelin A, Romero I, Schüpbach M, Zaugg SW, Miedzybrodzka Z, Rae D, Downie L, Simpson S, Summers F, Ure A, Jack R, Matheson K, Akhtar S, Crooks J, Curtis A, de Souza J, Rickards H, Wright J, Barker RA, O' Keefe D, Di Pietro A, Fisher K, Goodman A, Hill S, Mason S, Swain R, Guzman NV, Bisson J, Busse M, Butcher C, Clenaghan C, Dunnett S, Handley O, Hunt S, Hughes A, Johnstone C, Jones L, Jones U, Khalil H, Owen M, Price K, Rose LE, Rosser A, Porteous M, Edwards M, Ho C, McGill M, Pearson P, Brockie P, Foster J, Johns N, McKenzie S, Rothery J, Thomas G, Yates S, Burrows L, Fletcher A, Harding A, Laver F, Silva M, Thomson A, Rowett L, Gallantrae D, Longthorpe M, Markova I, Raman A, Hamer S, Yarduiman P, Chu C, Kraus A, Wild S, Musgrave H, Toscano J, Jamieson S, Hobson E, Clayton C, Dipple H, Middleton J, Freire Patino D, Andrews T, Dougherty A, Kavalier F, Golding C, Laing H, Lashwood A, Robertson D, Ruddy D, Whaite A, Santhouse A, Patton M, Peterson M, Rose S, Bruno S, Chu E, Doherty K, Haider S, Hensman D, Lahiri N, Lewis M, Novak M, Patel A, Robertson N, Rosser E, Taylor R, Warner T, Wild E, Howard L, Sollom A, Snowden J, Thompson J, Jones M, Murphy H, Trender Gerhard I, Rogers D, Bek J, Oughton E, Johnson L, Hare M, Arran N, Verstraelen N, Partington Jones L, Huson S, Stopford C, Westmoreland L, Davidson J, Morgan K, Savage L, Singh B, Komati S, Nemeth AH, Armstrong R, Valentine R, Siuda G, Harrison D, Hughes M, Parkinson A, Soltysiak B, Bandmann O, Bradbury A, Gill P, Fairtlough H, Fillingham K, Foustanos I, Kazoka M, O' Donovan K, Taylor C, Tidswell K, Quarrell O, Metzger S, Walter C, Riess O, Roos RA, Nielsen JE, Craufurd D, Nguyen HP, REGISTRY Investigators of the European Huntington’s Disease Network:Bachoud-Lévi AC, Bentivoglio AR, Biunno I, Bonelli RM, Burgunder JM, Dunnett SB, Ferreira JJ, Handley OJ, Heiberg A, Illmann T, Landwehrmeyer G, Levey J, Ramos-Arroyo MA, Nielsen J, Koivisto SP, Päivärinta M, Sebastián AR, Tabrizi S, Vandenberghe W, Verellen-Dumoulin C, Zaremba J, Uhrová T, Wahlström J, Barth K, Correia-Guedes L, Finisterra AM, Garde MB, Bos R, Betz S, Callaghan J, Fullam R, Ecker D, Nielsen MG, Hvalstedt C, Held C, Koppers K, Laurà M, Horta SM, Descals AM, Díaz MF, Mestre T, Minster S, Monza D, Mütze L, Oehmen M, Townhill J, Orth M, Padieu H, Paterski L, Peppa N, Roedig V, Rialland A, Røren N, Šašinková P, Seliverstov Y, Cubillo PT, van Walsem MR, Wright A, Silva WV, Witjes-Anné MN, Yudina E, Zielonka D, Zielonka E, Zinzi P, Herranhof B, Holl A, Kapfhammer HP, Koppitz M, Magnet M, Otti D, Painold A, Reisinger K, Scheibl M, Hecht K, Lilek S, Müller N, Schöggl H, Ullah J, Ribaï P, Boogaerts A, van Reijen D, Klempíř J, Majerová V, Roth J, Hjermind L, Jacobsen O, Vinthev-Jensen T, Larsen IU, Stockholm J, Hiivola H, Martikainen K, Tuuha K, Ignatius J, Kärppä M, Åman J, Mustonen A, Kajula O, Santala M, Allain P, Guérid MA, Gohier B, Olivier A, Prundean A, Scherer-Gagou C, Verny C, Bost M, Babiloni B, Debruxelles S, Duché C, Goizet C, Lafoucrière D, Jameau L, Spampinato U, De Bruycker C, Cabaret M, Carette AS, Defebvre L, Decorte E, Delval A, Delliaux M, Destee A, Dujardin K, Peter M, Plomhouse L, Sablonnière B, Simonin C, Lemaire MH, Manouvrier S, Thibault-Tanchou S, Vuillaume I, Krystkowiak P, Duru C, Roussel M, Wannepain S, Berrissoul H, Bellonet M, Courtin F, Mantaux B, Fasquel V, Godefroy O, Azulay JP, Fluchère F, Delfini M, Eusebio A, Mundler L, Longato N, Rudolf G, Steinmetz G, Tranchant C, Wagner C, Zimmermann MA, Marcel C, Andrich J, Ellrichmann G, Hoffmann R, Kaminski B, Saft C, Stamm C, Boelmans K, Ganos C, Goerendt I, Hidding U, Lewerenz J, Münchau A, Schmalfeld J, Stubbe L, Zittel S, Bürk K, Möller JC, Rissling I, Cormio C, Sciruicchio V, Serpino C, de Tommaso M, Capellari S, Cortelli P, Gallassi R, Poda R, Rizzo G, Scaglione C, Abbruzzese G, di Poggio MB, Di Maria E, Ferrandes G, Mandich P, Marchese R, Albanese A, Di Bella D, Di Donato S, Gellera C, Genitrini S, Mariotti C, Nanetti L, Paridi D, Soliveri P, Tomasello C, Squitieri F, Elifani F, Maglione V, Di Pardo A, Alberti S, Griguoli A, Amico E, Martino T, Petrollini M, Catalli C, Di Giacopo R, Fasano A, Frontali M, Guidubaldi A, Ialongo T, Jacopini G, Loria G, Piano C, Chiara P, Quaranta D, Romano S, Soleti F, Spadaro M, van Hout MS, van Vugt JP, de Weert A, Bolwijn JJ, Dekker M, Leenders KL, Dumas EM, van den Bogaard SJ, 't Hart EP, van Duijn E, Kremer B, Verstappen CC, Blinkenberg EØ, Hauge E, Tyvoll H, Frich J, Aaserud O, Wehus R, Bjørgo K, Fannemel M, Gørvell P, Lorentzen E, Retterstøl L, Overland T, Stokke B, Bjørnevoll I, Sando SB, Dziadkiewicz A, Nowak M, Robowski P, Sitek E, Slawek J, Soltan W, Szinwelski M, Blaszcyk M, Boczarska-Jedynak M, Ciach-Wysocka E, Gorzkowska A, Jasinska-Myga B, Opala G, Kłodowska-Duda G, Stompel D, Banaszkiewicz K, Boćwińska D, Szczudlik A, Rudzinska M, Wójcik M, Dec M, Krawczyk M, Bojakowska-Jaremek K, Szczygieł E, Stenwak A, Wasielewska A, Bryl A, Ciesielska A, Klimberg A, Marcinkowski J, Sempołowicz J, Samara H, Wiśniewski B, Janik P, Gogol A, Kwiecinski H, Jamrozik Z, Kaminska A, Antczak J, Jachinska K, Rakowicz M, Richter P, Rola R, Ryglewicz D, Sienkiewicz-Jarosz H, Stępniak I, Witkowski G, Zdzienicka E, Sułek A, Krysa W, Stepniak I, Zieora-Jakutowicz K, Júlio F, Januário C, Coelho M, Mendes T, Valadas A, Andrade C, Gago M, Garrett C, Guerra MR, Lima J, Massano J, Meireles J, Herrera CD, Garcia PM, Barrero F, Morales B, Cubo E, Mariscal N, Sánchez J, Alonso-Frech F, Perez MR, Fenollar M, García RG, Pin Quiroga P, Vázquez Rivera S, Villanueva C, Alegre J, Bascuñana M, Caldentey JG, Ventura MF, Ribas GG, de Yébenes JG, Moreno JL, Ruíz PJ, Martínez-Descals A, Artiga MJ, Sánchez V, Guerrero R, Bárcenas AH, Perea MF, Fortuna L, Torres MM, Reinante G, Moreau LV, Barbera MA, Guia DB, Hernanz LC, Catena JL, Ferrer PQ, Carruesco GT, Bas J, Busquets N, Calopa M, Elorza MD, López CD, Durán-Sindreu Terol S, Robert MF, Ruíz BG, Casado AG, Martínez IH, Viladrich CM, Cárdenas RP, Roca E, Llesoy JR, Idiago JM, Vergara MR, García SS, Riballo AV, González SG, Guisasola LM, Salvador C, Martín ES, González M, Gorospe A, Legarda I, Arques PN, Rodríguez MJ, Vives B, Gaston I, Martinez-Jaurrieta MD, Moreno JM, Peña JC, Avarvarei LD, Bastida AM, Recio MF, Vergé LR, Sánchez VS, Carrillo F, Cáceres MT, Mir P, Suarez MJ, Loutfi G, Olofsson C, Stattin EL, Westman L, Wikström B, Pålhagen SE, Paucar M, Svenningsson P, Reza-Soltani TW, Höglund A, Sandström B, Høsterey-Ugander U, Fredlund G, Constantinescu R, Neleborn-Lingefjärd L, Stebler Y, Kaelin A, Romero I, Schüpbach M, Zaugg SW, Miedzybrodzka Z, Rae D, Downie L, Simpson S, Summers F, Ure A, Jack R, Matheson K, Akhtar S, Crooks J, Curtis A, de Souza J, Rickards H, Wright J, Barker RA, O' Keefe D, Di Pietro A, Fisher K, Goodman A, Hill S, Mason S, Swain R, Guzman NV, Bisson J, Busse M, Butcher C, Clenaghan C, Dunnett S, Handley O, Hunt S, Hughes A, Johnstone C, Jones L, Jones U, Khalil H, Owen M, Price K, Rose LE, Rosser A, Porteous M, Edwards M, Ho C, McGill M, Pearson P, Brockie P, Foster J, Johns N, McKenzie S, Rothery J, Thomas G, Yates S, Burrows L, Fletcher A, Harding A, Laver F, Silva M, Thomson A, Rowett L, Gallantrae D, Longthorpe M, Markova I, Raman A, Hamer S, Yarduiman P, Chu C, Kraus A, Wild S, Musgrave H, Toscano J, Jamieson S, Hobson E, Clayton C, Dipple H, Middleton J, Freire-Patino D, Andrews T, Dougherty A, Kavalier F, Golding C, Laing H, Lashwood A, Robertson D, Ruddy D, Whaite A, Santhouse A, Patton M, Peterson M, Rose S, Bruno S, Chu E, Doherty K, Haider S, Hensman D, Lahiri N, Lewis M, Novak M, Patel A, Robertson N, Rosser E, Taylor R, Warner T, Wild E, Howard L, Sollom A, Snowden J, Thompson J, Jones M, Murphy H, Trender-Gerhard I, Rogers D, Bek J, Oughton E, Johnson L, Hare M, Arran N, Verstraelen N, Partington-Jones L, Huson S, Stopford C, Westmoreland L, Davidson J, Morgan K, Savage L, Singh B, Komati S, Nemeth AH, Armstrong R, Valentine R, Siuda G, Harrison D, Hughes M, Parkinson A, Soltysiak B, Bandmann O, Bradbury A, Gill P, Fairtlough H, Fillingham K, Foustanos I, Kazoka M, O' Donovan K, Taylor C, Tidswell K, and Quarrell O

Subjects
Adult, Adolescent, Genotype, Huntington, Ubiquitin-Activating Enzymes, Autophagy-Related Protein 7, Polymorphism, Single Nucleotide, Cohort Studies, Young Adult, Gene Frequency, V471A polymorphism, Genetics, Autophagy, Humans, Age of Onset, Child, Biology, Genetic Association Studies, Aged, Clinical Genetics, Evolutionary Biology, Computational Biology, Human Genetics, Middle Aged, Huntington Disease, Neurology, Italy, Autosomal Dominant, Child, Preschool, Genetic Polymorphism, Medicine, Population Genetics, gene ATG7, Research Article
Abstract

The cause of Huntington disease (HD) is a polyglutamine repeat expansion of more than 36 units in the huntingtin protein, which is inversely correlated with the age at onset of the disease. However, additional genetic factors are believed to modify the course and the age at onset of HD. Recently, we identified the V471A polymorphism in the autophagy-related gene ATG7, a key component of the autophagy pathway that plays an important role in HD pathogenesis, to be associated with the age at onset in a large group of European Huntington disease patients. To confirm this association in a second independent patient cohort, we analysed the ATG7 V471A polymorphism in additional 1,464 European HD patients of the "REGISTRY" cohort from the European Huntington Disease Network (EHDN). In the entire REGISTRY cohort we could not confirm a modifying effect of the ATG7 V471A polymorphism. However, analysing a modifying effect of ATG7 in these REGISTRY patients and in patients of our previous HD cohort according to their ethnic origin, we identified a significant effect of the ATG7 V471A polymorphism on the HD age at onset only in the Italian population (327 patients). In these Italian patients, the polymorphism is associated with a 6-years earlier disease onset and thus seems to have an aggravating effect. We could specify the role of ATG7 as a genetic modifier for HD particularly in the Italian population. This result affirms the modifying influence of the autophagic pathway on the course of HD, but also suggests population-specific modifying mechanisms in HD pathogenesis.

Published
2013

2. Observing Huntington's disease: the European Huntington's Disease Network's REGISTRY

Author

Orth, M, Handley, Oj, Schwenke, C, Dunnett, S, Wild, Ej, Tabrizi, Sj, Landwehrmeyer, Gb, Bachoud Lévi AC, Bentivoglio, Ar, Biunno, I, Bonelli, R, Burgunder, Jm, Dunnett, Sb, Ferreira, Jj, Giuliano, J, Heiberg, A, Illmann, T, van Kammen, D, Landwehrmeye, Gb, Levey, J, Nielsen, Je, Päivärinta, M, Roos, Ra, Sebastián, Ar, Vandenberghe, W, Verellen Dumoulin, C, Zaremba, J, Uhrova, T, Wahlström, J, Wallner, M, Barth, K, Guedes, Lc, Finisterra, Am, Garde, Mb, Bos, R, Burg, S, Ecker, D, Held, C, Koppers, K, Laurà, M, Descals, Am, Mclean, T, Mestre, T, Minster, S, Monza, D, Townhill, J, Padieu, H, Paterski, L, Peppa, N, Koivisto, Sp, Rialland, A, Røren, N, Sasinková, P, Cubillo, Pt, Tritsch, C, van Walsem MR, Witjes Ané MN, Yudina, E, Zielonka, D, Zielonka, E, Zinzi, P, Bonelli, Rm, Herranhof, B, Holl, A, Kapfhammer, Hp, Koppitz, M, Magnet, M, Otti, D, Painold, A, Reisinger, K, Scheibl, M, Hecht, K, Lilek, S, Müller, N, Schöggl, H, Ullah, J, Brugger, F, Hepperger, C, Hotter, A, Mahlknecht, P, Nocker, M, Seppi, K, Wenning, G, Buratti, L, Hametner, Em, Holas, C, Hussl, A, Mair, K, Poewe, W, Wolf, E, Zangerl, A, Braunwarth, Em, Sinadinosa, D, Walleczek, Am, Ladurner, G, Staffen, W, Ribaï, P, Flamez, A, Morez, V, de Raedt, S, Boogaerts, A, van Reijen, D, Klempíř, J, Kucharík, M, Roth, J, Šenkárová, Z, Hasholt, L, Hjermind, Le, Jakobsen, O, Nørremølle, A, Sørensen, Sa, Stokholm, J, Nielsen, J, Hiivola, H, Martikainen, K, Tuuha, K, Peippo, M, Sipponen, M, Ignatius, J, Kärppä, M, Åman, J, Santala, M, Allain, P, Guérid, Ma, Gohier, B, Olivier, A, Prundean, A, Scherer Gagou, C, Verny, C, Babiloni, B, Debruxelles, S, Goizet, C, Lafoucrière, D, De Bruycker, C, Carette, As, Decorte, E, Delval, A, Delliaux, M, Dujardin, K, Peter, M, Plomhouse, L, Simonin, C, Thibault Tanchou, S, Bellonet, M, Duru, C, Krystkowiak, P, Roussel, M, Wannepain, S, Azulay, Jp, Chabot, C, Delphini, M, Eusebio, A, Grosjean, H, Mundler, L, Nowak, M, Rudolf, G, Steinmetz, G, Tranchant, C, Wagner, C, Zimmermann, Ma, Calvas, F, Cheriet, S, Démonet, Jf, Galitzky, M, Kosinski, Cm, Milkereit, E, Probst, D, Sass, C, Schiefer, J, Schlangen, C, Werner, Cj, Gelderblom, H, Priller, J, Prüss, H, Spruth, Ej, Andrich, J, Hoffmann, R, Kraus, Ph, Muth, S, Prehn, C, Saft, C, Salmen, S, Stamm, C, Steiner, T, Strassburger, K, Lange, H, Friedrich, A, Hunger, U, Löhle, M, Schmidt, S, Storch, A, Wolz, A, Wolz, M, Lambeck, J, Zucker, B, Boelmans, K, Ganos, C, Hidding, U, Lewerenz, J, Münchau, A, Schmalfeld, J, Stubbe, L, Zittel, S, Diercks, G, Gorzolla, H, Schrader, C, Heinicke, W, Ribbat, M, Longinus, B, Bürk, K, Möller, Jc, Rissling, I, Peinemann, A, Städtler, M, Weindl, A, Bechtel, N, Beckmann, H, Bohlen, S, Hölzner, E, Reilmann, R, Rohm, S, Rumpf, S, Schepers, S, Beister, A, Dose, M, Hammer, K, Kieni, J, Leythaeuser, G, Marquard, R, Raab, T, Richter, S, Selimbegovic Turkovic, A, Schrenk, C, Schuierer, M, Wiedemann, A, Buck, A, Connemann, J, Eschenbach, C, Landwehrmeyer, B, Lezius, F, Nepper, S, Niess, A, Süssmuth, S, Trautmann, S, Weydt, P, Cormio, C, Difruscolo, O, Sciruicchio, V, Serpino, C, de Tommaso, M, Capellari, S, Cortelli, P, Gallassi, R, Poda, R, Rizzo, G, Scaglione, C, Bertini, E, Ghelli, E, Ginestroni, A, Massaro, F, Mechi, C, Paganini, M, Piacentini, S, Pradella, S, Romoli, Am, Sorbi, S, Abbruzzese, G, di Poggio MB, Di Maria, E, Ferrandes, G, Mandich, P, Marchese, R, Albanese, A, Di Bella, D, Di Donato, S, Gellera, C, Genitrini, S, Mariotti, C, Nanetti, L, Paridi, D, Soliveri, P, Tomasello, C, De Michele, G, Di Maio, L, Rinaldi, C, Russo, Cv, Salvatore, E, Tucci, T, Cannella, M, Codella, V, De Gregorio, F, De Nicola, N, Martino, T, Simonelli, M, Squitieri, F, Catalli, C, Di Giacopo, R, Fasano, A, Frontali, M, Guidubaldi, A, Ialongo, T, Jacopini, G, Loria, G, Modoni, A, Piano, C, Chiara, P, Quaranta, D, Romano, Silvia, Soleti, F, Spadaro, M, van Hout MS, van Vugt JP, de Weert AM, Bolwijn, Jj, Dekker, M, Leenders, Kl, van Oostrom JC, Dumas, Em, Jurgens, Ck, van den Bogaard SJ, 't Hart EP, Kremer, B, Verstappen, Cc, Frich, J, Wehus, R, Aaserud, O, Borgerød, N, Bjørgo, K, Fannemel, M, Gørvell, P, Pro Koivisto, S, Retterstøl, L, Overland, T, Stokke, B, Bjørnevoll, I, Sando, Sb, Blinkenberg, Eø, Hauge, E, Tyvoll, H, Sitek, E, Slawek, J, Soltan, W, Boczarska Jedynak, M, Jasinska Myga, B, Opala, G, Kłodowska Duda, G, Banaszkiewicz, K, Szczudlik, A, Rudzińska, M, Wójcik, M, Dec, M, Krawczyk, M, Bryl, A, Ciesielska, A, Klimberg, A, Marcinkowski, J, Sempołowicz, J, Samara, H, Janik, P, Kalbarczyk, A, Kwiecinski, H, Jamrozik, Z, Antczak, J, Jachinska, K, Rakowicz, M, Richter, P, Ryglewicz, D, Witkowski, G, Zdzienicka, E, Sułek, A, Krysa, W, Júlio, F, Januário, C, Guedes, L, Coelho, M, Mendes, T, Valadas, A, Timóteo, Â, Costa, C, Vale, J, Cavaco, S, Damásio, J, Magalhães, M, Gago, M, Garrett, C, Guerra, Mr, Solis, P, Herrera, Cd, Garcia, Pm, Barrero, F, Morales, B, Cubo, E, Mariscal, N, Alonso Frech, F, Perez, Mr, Fenollar, M, García, Rg, Quiroga, Pp, Rivera, Sv, Villanueva, C, Bascuñana, M, Ventura, Mf, Ribas, Gg, de Yébenes JG, Moreno, Jl, Ruíz, Pj, Martínez Descals, A, Artiga, Mj, Sánchez, V, Perea, Mf, Lorenza, F, Torres, Mm, Reinante, G, Moreau, Lv, Barbera, Ma, Guia, Db, Hernanz, Lc, Catena, Jl, Ferrer, Pq, Carruesco, Gt, Bas, J, Busquets, N, Calopa, M, Buongiorno, Mt, Muñoz, E, Elorza, Md, López, Cd, Terol, Sd, Robert, Mf, Ruíz, Bg, Casado, Ag, Martínez, Ih, Viladrich, Cm, Càrdenas R, Pons i., Roca, E, Llesoy, Jr, Idiago, Jm, Vergara, Mr, García, Ss, Villa Riballo, A, González, Sg, Guisasola, Lm, Salvador, C, San Martín ES, Gorospe, A, Legarda, I, Arques, Pn, Rodríguez, Mj, Vives, B, Gaston, I, Ramos Arroyo MA, Moreno, Jm, Peña, Jc, Avarvarei, Ld, Bastida, Am, Recio, Mf, Vergé, Lr, Sánchez, Vs, Carrillo, F, Cáceres, Mt, Mir, P, Suarez, Mj, Bosca, M, Burguera, Ja, Garcia, Ac, Martínez, Lm, del Val JL, Loutfi, G, Olofsson, C, Stattin, El, Westman, L, Wikström, B, Höglund, A, Pålhagen, Se, Paucar, M, Sandström, B, Soltani, R, Svenningsson, 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European Huntington's Disease, Network, Handley, Oj, Schwenke, C, Dunnett, S, Wild, Ej, Tabrizi, Sj, Bachoud Lévi AC, Landwehrmeyer G. B., Bentivoglio, Ar, Biunno, I, Bonelli, R, Burgunder, Jm, Dunnett, Sb, Ferreira, Jj, Giuliano, J, Heiberg, A, Illmann, T, van Kammen, D, Landwehrmeye, Gb, Levey, J, Nielsen, Je, Päivärinta, M, Roos, Ra, Sebastián, Ar, Vandenberghe, W, Verellen Dumoulin, C, Zaremba, J, Uhrova, T, Wahlström, J, Wallner, M, Barth, K, Guedes, Lc, Finisterra, Am, Garde, Mb, Bos, R, Burg, S, Ecker, D, Held, C, Koppers, K, Laurà, M, Descals, Am, Mclean, T, Mestre, T, Minster, S, Monza, D, Townhill, J, Padieu, H, Paterski, L, Peppa, N, Koivisto, Sp, Rialland, A, Røren, N, Sasinková, P, Cubillo, Pt, Tritsch, C, van Walsem, Mr, Witjes Ané, Mn, Yudina, E, Zielonka, D, Zielonka, E, Zinzi, P, Bonelli, Rm, Herranhof, B, Holl, A, Kapfhammer, Hp, Koppitz, M, Magnet, M, Otti, D, Painold, A, Reisinger, K, Scheibl, M, Hecht, K, Lilek, S, Müller, N, Schöggl, H, Ullah, J, Brugger, F, Hepperger, C, Hotter, A, Mahlknecht, P, Nocker, M, Seppi, K, Wenning, G, Buratti, L, Hametner, Em, Holas, C, Hussl, A, Mair, K, Poewe, W, Wolf, E, Zangerl, A, Braunwarth, Em, Sinadinosa, D, Walleczek, Am, Ladurner, G, Staffen, W, Ribaï, P, Flamez, A, Morez, V, de Raedt, S, Boogaerts, A, van Reijen, D, Klempíř, J, Kucharík, M, Roth, J, Šenkárová, Z, Hasholt, L, Hjermind, Le, Jakobsen, O, Nørremølle, A, Sørensen, Sa, Stokholm, J, Nielsen, J, Hiivola, H, Martikainen, K, Tuuha, K, Peippo, M, Sipponen, M, Ignatius, J, Kärppä, M, Åman, J, Santala, M, Allain, P, Guérid, Ma, Gohier, B, Olivier, A, Prundean, A, Scherer Gagou, C, Verny, C, Babiloni, B, Debruxelles, S, Goizet, C, Lafoucrière, D, De Bruycker, C, Carette, A, Decorte, E, Delval, A, Delliaux, M, Dujardin, K, Peter, M, Plomhouse, L, Simonin, C, Thibault Tanchou, S, Bellonet, M, Duru, C, Krystkowiak, P, Roussel, M, Wannepain, S, Azulay, Jp, Chabot, C, Delphini, M, Eusebio, A, Grosjean, H, Mundler, L, Nowak, M, Rudolf, G, Steinmetz, G, Tranchant, C, Wagner, C, Zimmermann, Ma, Calvas, F, Cheriet, S, Démonet, Jf, Galitzky, M, Kosinski, Cm, Milkereit, E, Probst, D, Sass, C, Schiefer, J, Schlangen, C, Werner, Cj, Gelderblom, H, Priller, J, Prüss, H, Spruth, Ej, Andrich, J, Hoffmann, R, Kraus, Ph, Muth, S, Prehn, C, Saft, C, Salmen, S, Stamm, C, Steiner, T, Strassburger, K, Lange, H, Friedrich, A, Hunger, U, Löhle, M, Schmidt, S, Storch, A, Wolz, A, Wolz, M, Lambeck, J, Zucker, B, Boelmans, K, Ganos, C, Hidding, U, Lewerenz, J, Münchau, A, Schmalfeld, J, Stubbe, L, Zittel, S, Diercks, G, Gorzolla, H, Schrader, C, Heinicke, W, Ribbat, M, Longinus, B, Bürk, K, Möller, Jc, Rissling, I, Peinemann, A, Städtler, M, Weindl, A, Bechtel, N, Beckmann, H, Bohlen, S, Hölzner, E, Reilmann, R, Rohm, S, Rumpf, S, Schepers, S, Beister, A, Dose, M, Hammer, K, Kieni, J, Leythaeuser, G, Marquard, R, Raab, T, Richter, S, Selimbegovic Turkovic, A, Schrenk, C, Schuierer, M, Wiedemann, A, Buck, A, Connemann, J, Eschenbach, C, Landwehrmeyer, B, Lezius, F, Nepper, S, Niess, A, Süssmuth, S, Trautmann, S, Weydt, P, Cormio, C, Difruscolo, O, Sciruicchio, V, Serpino, C, de Tommaso, M, Capellari, S, Cortelli, P, Gallassi, R, Poda, R, Rizzo, G, Scaglione, C, Bertini, E, Ghelli, E, Ginestroni, A, Massaro, F, Mechi, C, Paganini, M, Piacentini, S, Pradella, S, Romoli, Am, Sorbi, S, Abbruzzese, G, di Poggio, Mb, Di Maria, E, Ferrandes, G, Mandich, P, Marchese, R, Albanese, A, Di Bella, D, Di Donato, S, Gellera, C, Genitrini, S, Mariotti, C, Nanetti, L, Paridi, D, Soliveri, P, Tomasello, C, DE MICHELE, Giuseppe, Di Maio, L, Rinaldi, Carlo, Russo, Cv, Salvatore, Elena, Tucci, T, Cannella, M, Codella, V, De Gregorio, F, De Nicola, N, Martino, T, Simonelli, M, Squitieri, F, Catalli, C, Di Giacopo, R, Fasano, A, Frontali, M, Guidubaldi, A, Ialongo, T, Jacopini, G, Loria, G, Modoni, A, Piano, C, Chiara, P, Quaranta, D, Romano, S, Soleti, F, Spadaro, M, van Hout, M, van Vugt, Jp, de Weert, Am, Bolwijn, Jj, Dekker, M, Leenders, Kl, van Oostrom, Jc, Dumas, Em, Jurgens, Ck, van den Bogaard, Sj, 't Hart, Ep, Kremer, B, Verstappen, Cc, Frich, J, Wehus, R, Aaserud, O, Borgerød, N, Bjørgo, K, Fannemel, M, Gørvell, P, Pro Koivisto, S, Retterstøl, L, Overland, T, Stokke, B, Bjørnevoll, I, Sando, Sb, Blinkenberg, Eø, Hauge, E, Tyvoll, H, Sitek, E, Slawek, J, Soltan, W, Boczarska Jedynak, M, Jasinska Myga, B, Opala, G, Kłodowska Duda, G, Banaszkiewicz, K, Szczudlik, A, Rudzińska, M, Wójcik, M, Dec, M, Krawczyk, M, Bryl, A, Ciesielska, A, Klimberg, A, Marcinkowski, J, Sempołowicz, J, Samara, H, Janik, P, Kalbarczyk, A, Kwiecinski, H, Jamrozik, Z, Antczak, J, Jachinska, K, Rakowicz, M, Richter, P, Ryglewicz, D, Witkowski, G, Zdzienicka, E, Sułek, A, Krysa, W, Júlio, F, Januário, C, Guedes, L, Coelho, M, Mendes, T, Valadas, A, Timóteo, Â, Costa, C, Vale, J, Cavaco, S, Damásio, J, Magalhães, M, Gago, M, Garrett, C, Guerra, Mr, Solis, P, Herrera, Cd, Garcia, Pm, Barrero, F, Morales, B, Cubo, E, Mariscal, N, Alonso Frech, F, Perez, Mr, Fenollar, M, García, Rg, Quiroga, Pp, Rivera, Sv, Villanueva, C, Bascuñana, M, Ventura, Mf, Ribas, Gg, de Yébenes, Jg, Moreno, Jl, Ruíz, Pj, Martínez Descals, A, Artiga, Mj, Sánchez, V, Perea, Mf, Lorenza, F, Torres, Mm, Reinante, G, Moreau, Lv, Barbera, Ma, Guia, Db, Hernanz, Lc, Catena, Jl, Ferrer, Pq, Carruesco, Gt, Bas, J, Busquets, N, Calopa, M, Buongiorno, Mt, Muñoz, E, Elorza, Md, López, Cd, Terol, Sd, Robert, Mf, Ruíz, Bg, Casado, Ag, Martínez, Ih, Viladrich, Cm, Pons i., Càrdenas R, Roca, E, Llesoy, Jr, Idiago, Jm, Vergara, Mr, García, S, Villa Riballo, A, González, Sg, Guisasola, Lm, Salvador, C, San Martín, E, Gorospe, A, Legarda, I, Arques, Pn, Rodríguez, Mj, Vives, B, Gaston, I, Ramos Arroyo, Ma, Moreno, Jm, Peña, Jc, Avarvarei, Ld, Bastida, Am, Recio, Mf, Vergé, Lr, Carrillo, F, Cáceres, Mt, Mir, P, Suarez, Mj, Bosca, M, Burguera, Ja, Garcia, Ac, Martínez, Lm, del Val, Jl, Loutfi, G, Olofsson, C, Stattin, El, Westman, L, Wikström, B, Höglund, A, Pålhagen, Se, Paucar, M, Sandström, B, Soltani, R, Svenningsson, P, Reza Soltani, Tw, Constantinescu, R, Fredlund, G, Høsterey Ugander, U, Neleborn Lingefjärd, L, Esmaeilzadeh, M, Tedroff, J, Winnberg, E, Björn, Y, Ekwall, C, Gøller, Ml, Johansson, A, Wiklund, L, Petersen, Å, Reimer, J, Widner, H, Burgunder, Y, Stebler, Y, Kaelin, A, Romero, I, Schüpbach, M, Zaugg, Sw, Jack, R, Matheson, K, Miedzybrodzka, Z, Rae, D, Simpson, S, Summers, F, Ure, A, Crooks, J, Curtis, A, de Souza Keylock, J, Rickards, H, Wright, J, Hayward, B, Sieradzan, K, Wright, A, Barker, Ra, Di Pietro, A, Fisher, K, Goodman, A, Hill, S, Kershaw, A, Mason, S, Paterson, N, Raymond, L, Bisson, J, Busse, M, Clenaghan, C, Ellison Rose, L, Handley, O, Hunt, S, Price, K, Rosser, A, Edwards, M, Hughes, T, Mcgill, M, Pearson, P, Porteous, M, Smith, P, Zeman, A, Causley, A, Harrower, T, Howcroft, D, Lambord, N, Rankin, J, Brockie, P, Foster, J, Johns, N, Mckenzie, S, Rothery, J, Thomas, G, Yates, S, Miller, J, Ritchie, S, Burrows, L, Fletcher, A, Harding, A, Laver, F, Silva, M, Thomson, A, Burns, P, Chu, C, Evans, C, Hamer, S, Markova, I, Raman, A, Barnes, K, Hobson, E, Jamieson, S, Thomson, J, Toscano, J, Wild, S, Yardumian, P, Bourne, C, Clayton, C, Dipple, H, Clapton, J, Grant, D, Hallam, C, Middleton, J, Murch, A, Patino, D, Bate, L, Pate, L, Andrews, T, Dougherty, A, Kavalier, F, Golding, C, Lashwood, A, Robertson, D, Ruddy, D, Whaite, A, Patton, M, Peterson, M, Rose, S, Bruno, S, Chu, E, Doherty, K, Henley, S, Lahiri, N, Novak, M, Patel, A, Read, J, Rosser, E, Say, M, Tabrizi, S, Taylor, R, Warner, T, Wild, E, Arran, N, Callaghan, J, Craufurd, D, Fullam, R, Howard, L, Huson, S, Oughton, E, Partington Jones, L, Snowden, J, Sollom, A, Stopford, C, Thompson, J, Trender Gerhad, I, Verstraelen, N, Westmoreland, L, Nemeth, Ah, Siuda, G, Harrison, D, Hughes, M, Parkinson, A, Soltysiak, B, Burn, J, Coleman, C, Majeed, T, Verstraelen Ritchie, N, Bandmann, O, Bradbury, A, Gill, P, Fairtlough, H, Fillingham, K, Foustanos, I, O'Donovan, K, Tidswell, K, Quarrell, O., Faculty of Economic and Social Sciences and Solvay Business School, Neurology, Clinical sciences, Neuroprotection & Neuromodulation, Orth M., European Huntington's Disease Network, Handley O.J., Schwenke C., Dunnet S., Wild E.J., Tabrizi S.J., Landwehrmeyer G.B., Capellari S., Cortelli P., Gallassi R., Poda R., Rizzo G., and Scaglione C.

Subjects
Gerontology, medicine.medical_specialty, Neurology, International Cooperation, Disease, Irritability, Data Collection/methods, Huntington's disease, medicine, Humans, Registries, Psychiatry, Suicidal ideation, Disease burden, business.industry, Data Collection, medicine.disease, Europe, Huntington Disease, Psychiatry and Mental health, European Huntington's Disease Network, Surgery, Observational study, Neurology (clinical), medicine.symptom, business
Abstract

BACKGROUND: Huntington's disease (HD) is a rare triplet repeat (CAG) disorder. Advanced, multi-centre, multi-national research frameworks are needed to study simultaneously multiple complementary aspects of HD. This includes the natural history of HD, its management and the collection of clinical information and biosamples for research. METHODS: We report on cross-sectional data of the first 1766 participants in REGISTRY, the European Huntington's Disease Network's (EHDN), multi-lingual, multi-national prospective observational study of HD in Europe. Data collection (demographics, phenotype, genotype, medication, co-morbidities, biosamples) followed a standard protocol. RESULTS: Phenotype, and the HD genotype, of manifest HD participants across different European regions was similar. Motor onset was most common (48%) with a non-motor onset in more than a third of participants. Motor signs increased, and cognitive abilities and functional capacity declined as the disease burden (CAGn-35.5) X age) increased. A life-time history of behavioural symptoms was common, but the behavioural score was not related to disease burden. One fifth of participants had severe psychiatric problems, e.g. suicidal ideation and attempts, and/or irritability/aggression, with psychosis being less common. Participants on anti-dyskinetic medication had a higher motor and lower cognitive score, were older, and more prone to physical trauma. A higher motor and a lower cognitive score predicted more advanced disease. CONCLUSIONS: The unparalleled collection of clinical data and biomaterials within the EHDN's REGISTRY can expedite the search for disease modifiers (genetic and environmental) of age at onset and disease progression that could be harnessed for the development of novel treatments.

Published
2011

3. Tolerability profile of Aripiprazole in adult patients with Tourette syndrome

Author

Selvini C, Cavanna A, Termine C, Luoni C, Eddy C, Rickards H, Selvini, C, Cavanna, A, Termine, C, Luoni, C, Eddy, C, and Rickards, H

Subjects
Psychiatry, Neurology, Surgery, Neuroscience
Abstract

Aims Little is known about the tolerability profile of Aripiprazole in the TS population. We set out to evaluate the prevalence of psychiatric and non-psychiatric adverse events (PAE and nPAE) of Aripiprazole through a retrospective chart review of adult patients seen at a specialist TS clinic. Methods We reviewed the clinical files of 29 patients (22 males; mean age 29.7±13.0) who had been started on Aripiprazole (median starting dose: 5.0 mg; median maintenance dose: 10.0 mg). Results Twenty-one patients (72.4%) developed adverse effects: 6 (20.7%) PAE (most common: anxiety); 17 (58.6%) nPAE (most common: sedation). Thirteen patients (44.8%) discontinued the Aripiprazole, 6 (20.7%) solely because of the severity of specific adverse effects (sedation, sleep problems, weight gain, nausea, lost appetite, tic worsening, hot flushes). When comparing patients with and without adverse effects, we found no differences in socio-demographic or clinical variables. However, there was a trend towards a statistically significant difference in Aripiprazole starting dose (p=0.07). Conclusions The most commonly reported adverse effects of Aripiprazole treatment in TS were sedation and sleep problems. In the majority of cases, adverse effects were not severe. In our clinical sample there were no predictors for poor tolerability. Slow titration is recommended in order to minimise the occurrence of adverse effects.

Published
2011

4. Atypical Antipsychotics for Tourette Syndrome

Author

Cavanna A, Pierce A, Rickards H, Cavanna, A, Pierce, A, and Rickards, H

Subjects
Psychiatry, Neurology, Surgery, Neuroscience
Abstract

Aims The aim of this Cochrane review is to evaluate the efficacy and safety of atypical antipsychotics compared with placebo in treating tics in patients with Tourette syndrome (TS). Methods We cross referenced atypical antipsychotics-risperidone, olanzapine, clozapine, amisulpiride, quetiapine, loxapine, ziprasidone, clotiapine, aripiprazole, remoxipride, sertindole, zotepine, sulpiride and their proprietary names with “Tourette Syndrome” and its derivations and searched the Cochrane Movement Disorder Group Trials Register, Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library 2010, issue 3), MEDLINE (1950–2010), EMBASE (1980–2010), PsycINFO(1987–2010), and CINAHL (2010). Grey literature, reference lists, hand searching, clinical trials websites and pharmaceutical company sites were also searched. All relevant randomised, controlled, double blind studies were considered for inclusion in this review. Results The search identified three randomised controlled trials, two comparing risperidone and one comparing ziprasidone with placebo. Risperidone was superior to placebo in one trial although the 95% CIs were very large. Two trials showed no statistical difference between risperidone and ziprasidone against placebo. Methodological quality was rated as “unclear”. Clinical heterogeneity made meta-analysis inappropriate. Conclusions It is difficult to draw clear conclusions from the identified trials in the review as evidence is limited. Placebo controlled trials with longer duration and larger groups are needed to investigate the safety and efficacy of atypical antipsychotics as a pharmaceutical class in TS populations.

Published
2011

5. The prevalence and clinical characteristics of pathological gambling in patients with Parkinson's Disease: an evidence based review

Author

Barns M, Rickards H, Cavanna A, Barns, M, Rickards, H, and Cavanna, A

Subjects
Psychiatry, Neurology, Surgery, Neuroscience
Abstract

Aims To identify the prevalence of pathological gambling (PG) in patients with Parkinson's disease (PD) from studies using comparable methodology to the best general population estimates. Additionally, to collate case-control based observations regarding the clinical characteristics of this phenomenon. Methods MEDLINE, EMBASE and PyscInfo databases were searched for prospective prevalence studies which used systematic screening methods and interview based diagnosis using recognised diagnostic criteria for PD. The same search strategy was used to identify case-control studies examining the clinical characteristics of PG. Results Five prospective studies were included in this review with a total of 1032 patients with PD. 3.2% of all patients fulfilled the diagnostic criteria for PG, compared to 0.43–1% in the general population based on North American surveys. The prevalence in men was 1% higher than in women, although this was not statistically significant. The prevalence of PG among patients on dopamine agonists was 5.7%. Younger age of PD onset, increased novelty seeking behaviours and personal/family history of alcohol misuse were all associated with PG. Psychiatric co-morbidities including other impulse control disorders were commonly reported, although no statistically significant associations have been found. Conclusions Currently available evidence-based data suggest a high prevalence of PG among patients with PD. Dopamine agonist use was strongly associated with PG across all studies. Patients who develop PG present with specific clinical characteristics which are suggestive of underlying susceptibility factors.

Published
2011

6. Social and economic reasoning in Tourette syndrome

Author

Eddy CM, Mitchell IJ, Beck SR, Cavanna A, Rickards H, Eddy, C, Mitchell, I, Beck, S, Cavanna, A, and Rickards, H

Subjects
Psychiatry, Neurology, Surgery, Neuroscience
Abstract

Aims In Tourette Syndrome (TS), striatal dysfunction could affect the functioning of the frontal cortex, leading to changes in cognition and social behaviour. This study investigated social and economic reasoning in patients with TS. Methods 16 patients with TS and 20 neurologically intact controls completed three reasoning tasks that involved making judgements about mental states (Theory of Mind) and an economic decision making task. The tasks used were the “Eyes Test”, a “socially competitive emotions” task, a humourous cartoons task featuring sarcasm and irony, and a version of the Ultimatum Game. Executive functions were assessed using the FAS verbal fluency test and a black and white Stroop task. Results Patients with TS exhibited significant impairments on all four of the tasks selected to assess social and economic reasoning. These difficulties were evident despite the finding that patients did not exhibit significant executive deficits on the verbal fluency or inhibitory measures. Conclusions TS is associated with deficits on a range of tasks involving social and economic reasoning. Impairments on similar tasks have been reported in patients who have dysfunction of ventromedial prefrontal cortex. The observed deficits could imply that patients with TS exhibit dysfunction within frontostriatal pathways involving this region.

Published
2010

7. Refractoriness to pharmacological treatment for tics: A multicentre European audit

Author

Annette M. Hartmann, D. Martino, Mauro Porta, Hugh Rickards, Kirsten R. Müller-Vahl, Francesco Cardona, Renata Rizzo, Mariangela Gulisano, Tammy Hedderly, Irene Neuner, Pieter J. Hoekstra, Nanette Mol Debes, Antonella Macerollo, Andrea E. Cavanna, Veit Roessner, Clinical Cognitive Neuropsychiatry Research Program (CCNP), Macerollo, A, Martino, D, Cavanna, A, Gulisano, M, Hartmann, A, Hoekstra, P, Hedderly, T, Debes, N, Muller-Vahl, K, Neuner, I, Porta, M, Rickards, H, Rizzo, R, Cardona, F, and Roessner, V

Subjects
Adult, medicine.medical_specialty, Tics, Adolescent, Refractory period, Drug Resistance, Medication adherence, Audit, Tourette syndrome, Severity of Illness Index, Tic refractorine, Treatment failure, Pharmacological treatment, Medication Adherence, Tertiary Care Centers, Antipsychotic, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Internal medicine, Surveys and Questionnaires, Medicine, Humans, Antipsychotics, Treatment Failure, Psychiatry, Child, Dose-Response Relationship, Drug, business.industry, Disease Management, antipsychotics, tic refractoriness, neurology, neurology (clinical), medicine.disease, 030227 psychiatry, Europe, Neurology, Multicenter study, Tic Disorders, Neurology (clinical), business, 030217 neurology & neurosurgery, Central Nervous System Agents, Tic refractoriness
Published
2016

8. The assessment of consciousness during partial seizures

Author

Hugh Rickards, Andrea E. Cavanna, Fizzah Ali, Ali, F, Rickards, H, and Cavanna, A

Subjects
Consciousness, Psychometrics, Consciousne, media_common.quotation_subject, Unconsciousness, Assessment, Models, Psychological, Complex partial seizure, Terminology, Developmental psychology, Behavioral Neuroscience, Epilepsy, Seizures, Terminology as Topic, medicine, Humans, In patient, Ictal, Simple partial seizures, media_common, partial seizures, Conscious State, medicine.disease, Neurology, Categorization, Responsivene, Epilepsies, Partial, Neurology (clinical), Psychology, Cognitive psychology
Abstract

A wide range of controversial definitions and dynamic components surround the multi-dimensional concept of consciousness, with important reflections on the phenomenological description of ictal states relevant to epileptic seizures. The inadequacies of terminology, the insufficient emphasis on the subjective nature of consciousness, as well as the intrinsic limitations of the simple versus complex dichotomy for partial seizures, are to be considered in view of a modern definition of consciousness. In this paper, we review the difficulties encountered by clinicians in assessing the ictal conscious state in patients with epilepsy, and illustrate how a more sophisticated bi-dimensional model of consciousness can prove a valuable conceptual tool for the clinical assessment of ictal consciousness and the categorization of seizures.

Published
2012
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9. Treatment strategies for tics in Tourette syndrome

Author

Andrea E. Cavanna, Hugh Rickards, Clare M. Eddy, Eddy, C, Rickards, H, and Cavanna, A

Subjects
tic, medicine.medical_specialty, Deep brain stimulation, Tics, medicine.medical_treatment, Psychological intervention, Reviews, Tourette syndrome, lcsh:RC346-429, Neurodevelopmental disorder, Quality of life (healthcare), mental disorders, Medicine, botulinum toxin, Medical prescription, Psychiatry, lcsh:Neurology. Diseases of the nervous system, Pharmacology, treatment, business.industry, medicine.disease, neuroleptic, antipsychotic, deep brain stimulation, Neurology, Treatment strategy, medication, Neurology (clinical), business
Abstract

Tourette syndrome (TS) is a chronic neurodevelopmental disorder characterized by tics: repetitive, involuntary movements and vocalizations. These symptoms can have a significant impact on patients’ daily functioning across many domains. Tics tend to be most severe in child and adolescent sufferers, so their presence has the potential to impact a period of life that is both critical for learning and is often associated with the experience of greater social tension and self-consciousness than adulthood. Furthermore, control over tics that lead to physical impairment or self-injurious behaviour is of vital importance in maintaining health and quality of life. There are numerous complicating factors in the prescription of treatment for tics, due to both the side effects associated with alleviating agents and patient characteristics, such as age and comorbid conditions. This review summarizes literature pertaining to the efficacy and safety of both traditionally prescribed and more modern medications. We also discuss the merits of behavioural and surgical techniques and highlight newer emerging treatments. Although treatment response is to some extent variable, there are a number of agents that are clearly useful as first-line treatments for TS. Other interventions may be of most benefit to patients exhibiting refractory tics or more specific symptom profiles.

Published
2010
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10. Ictal consciousness in epilepsy and nonepileptic attack disorder

Author

Doug McCorry, Hugh Rickards, Andrea E. Cavanna, Lyn Greenhill, F Ali, Manny Bagary, Ali, F, Rickards, H, Bagary, M, Greenhill, L, Mccorry, D, and Cavanna, A

Subjects
Adult, Male, medicine.medical_specialty, Consciousness, Consciousne, Neuropsychiatry, Temporal lobe, Young Adult, Nonepileptic attack disorder, Behavioral Neuroscience, Epilepsy, medicine, Humans, Ictal, Generalized epilepsy, Medical diagnosis, Psychiatry, Ictal Consciousness Inventory, Electroencephalography, Middle Aged, medicine.disease, Psychophysiologic Disorders, Neurology, Frontal lobe, Dual diagnosis, Female, Psychogenic nonepileptic seizure, Neurology (clinical), Psychology
Abstract

Exploration of subjective experiences during seizures may enhance knowledge of the differing natures of epilepsy and nonepileptic attack disorder (NEAD). We performed a quantitative evaluation of both the general level of awareness and the specific contents of consciousness during seizures using the Ictal Consciousness Inventory (ICI). Ninety-five adult outpatients attending general neuropsychiatry and epilepsy clinics with established diagnoses of either epilepsy (n = 66) or NEAD (n = 29) completed one ICI for each witnessed seizure recalled. Patients with a dubious/dual diagnosis were excluded. ICI Level (ICI-L) and ICI Content (ICI-L) scores were calculated for the 167 questionnaires generated by patients with epilepsy (n = 119, of which 58 from patients with temporal lobe epilepsy, 14 frontal lobe epilepsy, and 47 idiopathic 30 generalized epilepsy) and patients with NEAD (n = 48). Mann-Whitney U tests revealed statistically significant higher ICI-L and ICI-C scores for patients with NEAD (both P = 0.01). Subjective reports of consciousness experiences varied between epilepsy and NEAD, with patients with NEAD reporting significantly greater levels of general awareness/responsiveness and more vivid subjective experiences during attacks. The ICI is proposed as a potentially useful self-report instrument to supplement clinical and instrumental tests for the differential diagnosis of epilepsy and NEAD. (C) 2010 Elsevier Inc. All rights reserved.

Published
2010
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11. Tourette syndrome is characterised by atypical activation within the mentalizing network

Author

Eddy CM, Hansen PC, Rickards HE, Cavanna A, Eddy, C, Hansen, P, Rickards, H, and Cavanna, A

Subjects
Psychiatry, Neurology, Neuroscience
Abstract

Background: The neuropsychiatric condition Tourette syndrome (TS) frequently involves complex tics with social relevance, including echophenomena (i.e. tics involving imitation) and socially inappropriate behaviors. Recent studies have indicated that these patients can exhibit subtle reasoning differences on tasks involving theory of mind (ToM): the ability to reason about mental states. Objective: We investigated the neural correlates of mentalizing in TS. Methods: Twenty-five adults with TS (but no comorbid disorders) and twenty-five healthy controls underwent fMRI during an established ToM false-belief paradigm. Patients and controls were matched for age and gender and neuropsychological profiles were similar. Tic symptoms, premonitory urges, echophenomena, impulsive and obsessive-compulsive behaviors were assessed for covariate analysis. Results: There were no group differences in behavioral performance on the false-belief task. However, when contrasting neural activation across mentalizing and nonmentalizing conditions and comparing the two groups, patients with TS exhibited abnormalities of up and down-regulation of key nodes within the mentalizing network, including right temporo-parietal junction, right amygdala bordering the temporal pole, and posterior cingulate cortex. Group comparisons collapsed across conditions revealed additional activation differences in the inferior parietal region, part of the human mirror neuron system. In TS, inferior parietal activation covaried with a measure of echophenomena. Conclusion: The observed pattern of altered activation within the mentalizing network may reflect a functional neural biomarker specific to TS, which could underpin the reported differences in reasoning about ToM, and perhaps socially inappropriate behaviors. Furthermore, our findings highlight dysfunction within the mirror neuron system as the likely mechanism driving echophenomena.

Published
2015

12. Pharmacological interventions for depression in people with traumatic brain injury: Systematic review

Author

Andrea E. Cavanna, J Joy, Hugh Rickards, JJ Vattakatuchery, N Lathif, Vattakatuchery, J, Lathif, N, Joy, J, Cavanna, A, and Rickards, H

Subjects
Psychiatry, medicine.medical_specialty, Sertraline, business.industry, Traumatic brain injury, Methylphenidate, Modafinil, Poison control, medicine.disease, Placebo, Psychiatry and Mental health, Neurology, Physical therapy, Medicine, Anxiety, Surgery, Neurology (clinical), medicine.symptom, business, Depression (differential diagnoses), medicine.drug, Neuroscience
Abstract

Objective To undertake a systematic review and meta-analysis of pharmacological interventions for depression in people with traumatic brain injury. Method Searches were undertaken for randomised controlled trials of pharmacological interventions in people with depression and traumatic brain injury. Searches were carried out as per our protocol and studies that fulfilled our inclusion criteria were included in the meta-analysis. Results Four studies were identified that fulfilled our inclusion criteria. Sertraline, desipramine, methylphenidate and modafinil were investigated in these studies. There were 72 participants in total in the intervention arm and 57 participants in the placebo arm. Meta-analysis showed favourable results for sertraline, desipramine and methylphenidate on outcomes measures for depressive symptoms. Only results for methylphenidate were statistically significant. Sertraline, methylphenidate and modafinil showed favourable results on quality of life indicators but results were not statistically significant. Sertraline showed favourable results on outcome measures for anxiety symptoms but results were not statistically significant. Conclusion Some pharmacological interventions appear to improve depressive symptoms, anxiety symptoms and quality of life in people with depression and traumatic brain injury. However, the evidence is limited. There is a paucity of evidence for the effectiveness of other pharmacological interventions used in depression in this particular patient group.

Published
2014

13. The 'imprisoned illness:' Motor tic disorder in Rainer Maria Rilke's Notebooks of Malte Laurids Brigge

Author

Maria Grazia Pattumelli, Hugh Rickards, Andrea E. Cavanna, Fizzah Ali, Tiziana Quarto, Cavanna, A, Pattumelli, M, Quarto, T, Ali, F, and Rickards, H

Subjects
Involuntary movement, Tic, Tic disorder, Psychoanalysis, Tics, Medicine in Literature, Tourette syndrome, Character (symbol), Neurological disorder, medicine.disease, Motor tic disorder, Neurology, Rainer Maria Rilke, Tic Disorders, medicine, Humans, Neurology (clinical), Psychology, The Notebooks of Malte Laurids Brigge
Abstract

Rainer Maria Rilke's novel The Notebooks of Malte Laurids Brigge contains a reference of interest for the catalog of literary portrayals of tiqueurs. In this article, we report his description of a Parisian character displaying multiple motor tic symptoms, along with a brief commentary.

Published
2010
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14. The psychopathological spectrum of Gilles de la Tourette syndrome

Author

Hugh Rickards, Andrea E. Cavanna, Cavanna, A, and Rickards, H

Subjects
medicine.medical_specialty, Gilles de la Tourette syndrome, Obsessive-Compulsive Disorder, Neurology, Tics, Cognitive Neuroscience, Impulse control disorder, Tourette syndrome, Affective disorder, Behavioral Neuroscience, medicine, Humans, Behaviour, Coprophenomena, Vocal tics, Psychiatry, Paliphenomena, Depression (differential diagnoses), Tic, Psychopathology, Mood Disorders, medicine.disease, Echophenomena, Personality disorders, Neuropsychology and Physiological Psychology, Mood disorders, Attention Deficit Disorder with Hyperactivity, Obsessive-compulsive symptom, Attention-deficit and hyperactivity disorder, Psychology, Tourette Syndrome
Abstract

Gilles de la Tourette syndrome (GTS) holds a unique status as quintessentially neuropsychiatric condition at the interface between neurology (movement disorder) and psychiatry (behavioural condition). This is a reflection of the common observation that the vast majority of patients present with behavioural problems in association with the motor and vocal tics which define GTS. The present article focuses on the relationship between GTS and obsessive-compulsive disorder (OCD), attention-deficit and hyperactivity disorder (ADHD), affective disorders (both major depression and bipolar affective disorder), and personality disorders. Over the last decade, converging lines of research have pointed towards the concept of a 'GTS spectrum', encompassing motor phenomena and behavioural symptoms, with important implications for the clinical management of patients.

Published
2012

15. From ipse dixit to evidence-based guidelines: on the optimal management of Tourette syndrome

Author

Andrea E. Cavanna, Veit Roessner, Hugh Rickards, Kerstin J. Plessen, Pieter J. Hoekstra, Rhiannon Worrall, Cavanna, A, Rickards, H, Worrall, R, Hoekstra, P, Plessen, K, and Roessner, V

Subjects
Gilles de la Tourette syndrome, medicine.medical_specialty, drug safety, Psychotherapist, Evidence-based practice, evidence based medicine, Tics, attention deficit disorder, letter, SOCIETY, medical expert, Tourette syndrome, RECOMMENDATIONS, TICS, Medicine, Humans, human, medical society, Psychiatry, prescription, health care organization, physician, business.industry, medical specialist, neurology, practice guideline, consensus development, clinical assessment, General Medicine, medicine.disease, Optimal management, health survey, drug efficacy, comorbidity, drug formulation, priority journal, tardive dyskinesia, EUROPEAN CLINICAL GUIDELINES, drug substitution, drug withdrawal, Pediatrics, Perinatology and Child Health, Ipse dixit, Neurology (clinical), business, drug tolerability, Tourette Syndrome
Published
2012

16. The evolving discipline and services of neuropsychiatry in the United Kingdom

Author

Hugh Rickards, Andrea E. Cavanna, Narayana M. A. Arambepola, Arambepola, N, Rickards, H, and Cavanna, A

Subjects
medicine.medical_specialty, business.industry, Service provision, neurology, Specialty, neuropsychology, The Renaissance, future direction, Neuropsychiatry, psychiatry, clinical practice, Clinical Practice, Psychiatry and Mental health, Scale (social sciences), Medicine, Engineering ethics, neuropsychiatry, business, Psychiatry, Biological Psychiatry
Abstract

Background: The last few decades have seen a renaissance in the development of neuropsychiatric services on a global scale.Methods: This paper reviews the existing literature on the changing role of the clinical neuropsychiatrist and discusses the evolving theory and practice of neuropsychiatry in the United Kingdom.Results: The rapidly evolving specialty of neuropsychiatry is currently facing a number of challenges. These include, but are not limited to, uncertainties about the curricular requirements for clinical neuropsychiatrists and the changing roles within the wider scenario of health-care service provision.Discussion: An informed historical perspective on this multifaceted discipline allows key insights into its future development.

Published
2012

17. The cognitive impact of antiepileptic drugs

Author

Hugh Rickards, Andrea E. Cavanna, Clare M. Eddy, Eddy, C, Rickards, H, and Cavanna, A

Subjects
cognition, medicine.medical_specialty, seizure, neuropsychology, Patient characteristics, Review, lcsh:RC346-429, Treatment and control groups, Epilepsy, antiepileptic drug, medicine, Effective treatment, Cognitive skill, Psychiatry, lcsh:Neurology. Diseases of the nervous system, Pharmacology, business.industry, Neuropsychology, Cognition, medicine.disease, Neurology, Tolerability, epilepsy, medication, Neurology (clinical), business, Clinical psychology
Abstract

Effective treatment of epilepsy depends on medication compliance across a lifetime, and studies indicate that drug tolerability is a significant limiting factor in medication maintenance. Available antiepileptic drugs (AEDs) have the potential to exert detrimental effects on cognitive function and therefore compromise patient wellbeing. On the other hand, some agents may serve to enhance cognitive function. In this review paper, we highlight the range of effects on cognition linked to a variety of newer and older AEDs, encompassing key alterations in both specific executive abilities and broader neuropsychological functions. Importantly, the data reviewed suggest that the effects exerted by an AED could vary depending on both patient characteristics and drug-related variables. However, there are considerable difficulties in evaluating the available evidence. Many studies have failed to investigate the influence of patient and treatment variables on cognitive functioning. Other difficulties include variation across studies in relation to design, treatment group and assessment tools, poor reporting of methodology and poor specification of the cognitive abilities assessed. Focused and rigorous experimental designs including a range of cognitive measures assessing more precisely defined abilities are needed to fill the gaps in our knowledge and follow up reported patterns in the literature. Longitudinal studies are needed to improve our understanding of the influence of factors such as age, tolerance and the stability of cognitive effects. Future trials comparing the effects of commonly prescribed agents across patient subgroups will offer critical insight into the role of patient characteristics in determining the cognitive impact of particular AEDs.

Published
2011

18. Paligraphia and written jocularity in Gilles de la Tourette syndrome

Author

Andrea E. Cavanna, Fizzah Ali, Hugh Rickards, Cavanna, A, Ali, F, and Rickards, H

Subjects
tic, medicine.medical_specialty, Gilles de la Tourette syndrome, letter, paligraphia, Tourette syndrome, involuntary movement, dysgraphia, male, obsessive compulsive disorder, medicine, case report, neuropsychiatry, human, Psychiatry, child, adult, medicine.disease, school child, writing, comorbidity, female, Neurology, priority journal, Neurology (clinical), Psychology
Published
2011

19. The role of impulse control disorders in Tourette syndrome: An exploratory study

Author

Andrea E. Cavanna, Hugh Rickards, Madeleine C. Frank, John Piedad, Frank, M, Piedad, J, Rickards, H, and Cavanna, A

Subjects
Adult, Male, medicine.medical_specialty, Adolescent, Health-related quality of life, Impulse control disorder, Exploratory research, Comorbidity, Impulsivity, Severity of Illness Index, Tourette syndrome, Young Adult, Quality of life, Compulsive buying disorder, medicine, Humans, Psychiatry, Psychiatric Status Rating Scales, Adult patients, Middle Aged, medicine.disease, Impulse control, Disruptive, Impulse Control, and Conduct Disorders, Neurology, Quality of Life, Female, Neurology (clinical), medicine.symptom, Intermittent explosive disorder, Psychology, Clinical psychology
Abstract

Introduction Tourette syndrome (TS) is a neurodevelopmental condition characterised by the presence of multiple motor tics and one or more phonic tics, often associated with co-morbid behavioural problems. Impulse control disorders (ICDs) are a set of disorders where patients have significant difficulties in controlling their urges to perform rewarding behaviours. ICDs are expected to be common in patients with TS, as many problems reported in TS are related to difficulties in controlling impulsivity. Aims This exploratory study aimed to determine the clinical characteristics of ICDs in adult patients with TS, and to investigate the relationship between the presence of ICDs and health-related quality of life (HR-QOL). Methods Thirty-one patients with a diagnosis of TS were screened for ICDs using the Minnesota Impulsive Disorders Interview (MIDI). HR-QOL was assessed using a generic instrument, the Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36) and a disease-specific scale, the Gilles de la Tourette Syndrome-Quality of Life Scale (GTS-QOL). Results Twenty-three out of 31 participants (74.2%) had at least one ICD. The most common ICDs were intermittent explosive disorder (51.6%) and compulsive buying disorder (41.9%). The number of ICDs significantly correlated with reduced HR-QOL ( p =0.011) as measured by the GTS-QOL, but not by the SF-36. Conclusions ICDs are common in patients with TS. HR-QOL measures specific to this patient population show that the presence of co-morbid ICDs results in poorer HR-QOL.

Published
2011

20. The experience of fear in Huntington's disease

Author

Eddy CM, Mitchell IJ, Beck SR, Cavanna A, Rickards HE, Eddy, C, Mitchell, I, Beck, S, Cavanna, A, and Rickards, H

Subjects
Psychiatry, Neurology, Surgery, Neuroscience
Published
2011

21. Altered subjective fear responses in Huntington's disease

Author

Hugh Rickards, Clare M. Eddy, Andrea E. Cavanna, Sarah R. Beck, Ian J. Mitchell, Eddy, C, Mitchell, I, Beck, S, Rickards, H, and Cavanna, A

Subjects
medicine.medical_specialty, media_common.quotation_subject, Audiology, Anger, Neuropsychological Tests, Amygdala, Developmental psychology, Striatum, Huntington's disease, medicine, Humans, Affective Symptoms, International Affective Picture System, media_common, Emotion, Facial expression, Analysis of Variance, Fear, medicine.disease, humanities, Disgust, medicine.anatomical_structure, Huntington Disease, Neurology, Happiness, Neurology (clinical), Geriatrics and Gerontology, Psychology, Cognition Disorders, Mental Status Schedule, Executive dysfunction
Abstract

Patients with Huntington’s disease (HD) have been shown to exhibit impairment in the recognition of facial expressions such as disgust, as well as deficits in disgust responses to olfactory and gustatory stimuli. The present study investigated whether HD is associated with changes in emotional responses to a variety of visual and verbal stimuli selected to elicit core disgust, moral disgust, fear and happiness. Thirteen patients with HD and twelve controls provided emotional ratings after both reading emotion eliciting scenarios and viewing pictures from the International Affective Picture System database. Patients with HD exhibited executive dysfunction. In comparison to controls, they gave similar ratings for happy stimuli and did not differ significantly in response to core disgust or moral disgust stimuli. However, they did exhibit lower fear ratings in response to both sets of fear stimuli (pictures and scenarios), and higher anger ratings than controls in response to fear pictures. These differences in fear response could reflect dysfunction within frontostriatal pathways involving the amygdala. Changes to fear responses in HD may impair decision making and lead to increased risk-taking behaviour with significant personal or social consequences.

Published
2010

22. Gilles de la Tourette: the man behind the syndrome

Author

Hugh Rickards, Andrea E. Cavanna, Rickards, H, and Cavanna, A

Subjects
Gilles de la Tourette syndrome, Paris, Psychoanalysis, Character (symbol), Biography, History, 19th Century, Hysteria, Obituary, medicine.disease, Tourette syndrome, Psychiatry and Mental health, Clinical Psychology, Neurology, Maladie des tics, Georges Gilles de la Tourette, medicine, Henry Meige, Psychology
Abstract

French neuropsychiatrist Georges Gilles de la Tourette first described in 1885 the "Maladie des Tics" which earned him eponymous fame. Both his colleagues at La Salpetriere hospital in Paris and medical historians report that he was a highly intelligent, if irascible, character. The Gilles de la Tourette syndrome was only a very minor contribution of its author, at the time. Gilles de la Tourette's main and continued contributions were on hysteria and hypnotism. This article concentrates on his life and includes previously untranslated passages from authors of the time and, for the first time, a full English translation of his obituary written by Henry Meige.

Published
2009

23. Hassler and Dieckmann's seminal paper on stereotactic thalamotomy for Gilles de la Tourette syndrome: translation and critical reappraisal

Author

Clare Wood, Hugh Rickards, Andrea E. Cavanna, Rickards, H, Wood, C, and Cavanna, A

Subjects
medicine.medical_specialty, Gilles de la Tourette syndrome, Deep brain stimulation, Psychoanalysis, Tics, Thalamotomy, medicine.medical_treatment, History, 20th Century, medicine.disease, Tourette syndrome, Stereotactic thalamotomy, Stereotaxic Techniques, Neurology, Stereotactic neurosurgery, Thalamus, Brain stimulation, medicine, Obsessive-compulsive disorder, Humans, Neurology (clinical), Psychiatry, Psychology, Tourette Syndrome
Abstract

Papers concerning the use of deep brain stimulation to the thalamus for relief of symptoms of Tourette syndrome have frequently cited a paper by Hassler and Dieckmann, published in 1970. We present a translation of this paper and discussion in English.

Published
2008

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