Reduced Cancer Incidence in Huntington's Disease: Analysis in the Registry Study

Background: People with Huntington’s disease (HD) have been observed to have lower rates of cancers. Objective: To investigate the relationship between age of onset of HD, CAG repeat length, and cancer diagnosis. Methods: Data were obtained from the European Huntington’s disease network REGISTRY study for 6540 subjects. Population cancer incidence was ascertained from the GLOBOCAN database to obtain standardised incidence ratios of cancers in the REGISTRY subjects. Results: 173/6528 HD REGISTRY subjects had had a cancer diagnosis. The age-standardised incidence rate of all cancers in the REGISTRY HD population was 0.26 (CI 0.22–0.30). Individual cancers showed a lower age-standardised incidence rate compared with the control population with prostate and colorectal cancers showing the lowest rates. There was no effect of CAG length on the likelihood of cancer, but a cancer diagnosis within the last year was associated with a greatly increased rate of HD onset (Hazard Ratio 18.94, p < 0.001). Conclusions: Cancer is less common than expected in the HD population, confirming previous reports. However, this does not appear to be related to CAG length in HTT. A recent diagnosis of cancer increases the risk of HD onset at any age, likely due to increased investigation following a cancer diagnosis

Crystal structure of ribosomal protein L4 shows RNA-binding sites for ribosome incorporation and feedback control of the S10 operon

Ribosomal protein L4 resides near the peptidyl transferase center of the bacterial ribosome and may, together with rRNA and proteins L2 and L3, actively participate in the catalysis of peptide bond formation. Escherichia coli L4 is also an autogenous feedback regulator of transcription and translation of the 11 gene S10 operon. The crystal structure of L4 from Thermotoga maritima at 1.7 Å resolution shows the protein with an alternating α/β fold and a large disordered loop region. Two separate binding sites for RNA are discernible. The N–terminal site, responsible for binding to rRNA, consists of the disordered loop with flanking α–helices. The C–terminal site, a prime candidate for the interaction with the leader sequence of the S10 mRNA, involves two non-consecutive α–helices. The structure also suggests a C–terminal protein-binding interface, through which L4 could be interacting with protein components of the transcriptional and/or translational machineries

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

none642sinoneOrth 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, Handley OJ, Heiberg A, Illmann T, van Kammen D, Landwehrmeye GB, Levey J, Nielsen JE, Päivärinta M, Roos RA, Sebastián AR, Tabrizi SJ, Vandenberghe W, Verellen-Dumoulin C, Zaremba J, Uhrova T, Wahlström J, Schwenke C, Orth M, Illmann T, Wallner M, Barth K, Guedes LC, Finisterra AM, Garde MB, Bos R, Burg S, Ecker D, Handley OJ, Held C, Koppers K, Laurà M, Descals AM, McLean T, Mestre T, Minster S, Monza D, Townhill J, Orth M, 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, Lilek S, Sinadinosa D, Walleczek AM, Bonelli RM, Ladurner G, Staffen W, Ribaï P, Verellen-Dumoulin C, Flamez A, Morez V, de Raedt S, Boogaerts A, Vandenberghe W, 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, Orth M, 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, Lange H, 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, Barth K, Buck A, Connemann J, Ecker D, Eschenbach C, Held C, Landwehrmeyer B, Lezius F, Nepper S, Niess A, Orth M, 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, Monza D, 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, Bentivoglio AR, 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, Zinzi P, van Hout MS, van Vugt JP, de Weert AM, Bolwijn JJ, Dekker M, Leenders KL, van Oostrom JC, Bos R, Dumas EM, Jurgens CK, van den Bogaard SJ, Roos RA, 't Hart EP, Witjes-Ané MN, Kremer B, Verstappen CC, Heiberg A, van Walsem MR, 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, Zielonka D, Samara H, Janik P, Kalbarczyk A, Kwiecinski H, Jamrozik Z, Antczak J, Jachinska K, Rakowicz M, Richter P, Ryglewicz D, Witkowski G, Zdzienicka E, Zaremba J, Sułek A, Krysa W, Júlio F, Januário C, Mestre T, Guedes L, Coelho M, Mendes T, Valadas A, Ferreira JJ, 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, Cubillo PT, 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, Sebastián AR, 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 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 P, Reza-Soltani TW, Constantinescu R, Fredlund G, Høsterey-Ugander U, Neleborn-Lingefjärd L, Wahlström J, Esmaeilzadeh M, Tedroff J, Winnberg E, Björn Y, Ekwall C, Gøller ML, Johansson A, Wiklund L, Petersen Å, Reimer J, Widner H, Burgunder JM, 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, Townhill J, 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, Miller J, Raman A, Barnes K, Chu C, Hobson E, Jamieson S, Markova I, 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, Andrews T, Bruno S, Chu E, Doherty K, Golding, Fillingham K, Foustanos I, O'Donovan K, Peppa N, Tidswell K, Quarrell O.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; Handley, Oj; Heiberg, A; Illmann, T; van Kammen, D; Landwehrmeye, Gb; Levey, J; Nielsen, Je; Päivärinta, M; Roos, Ra; Sebastián, Ar; Tabrizi, Sj; Vandenberghe, W; Verellen-Dumoulin, C; Zaremba, J; Uhrova, T; Wahlström, J; Schwenke, C; Orth, M; Illmann, T; Wallner, M; Barth, K; Guedes, Lc; Finisterra, Am; Garde, Mb; Bos, R; Burg, S; Ecker, D; Handley, Oj; Held, C; Koppers, K; Laurà, M; Descals, Am; Mclean, T; Mestre, T; Minster, S; Monza, D; Townhill, J; Orth, M; 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; Lilek, S; Sinadinosa, D; Walleczek, Am; Bonelli, Rm; Ladurner, G; Staffen, W; Ribaï, P; Verellen-Dumoulin, C; Flamez, A; Morez, V; de Raedt, S; Boogaerts, A; Vandenberghe, W; 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; Orth, M; 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; Lange, H; 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; Barth, K; Buck, A; Connemann, J; Ecker, D; Eschenbach, C; Held, C; Landwehrmeyer, B; Lezius, F; Nepper, S; Niess, A; Orth, M; 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; Monza, D; 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; Bentivoglio, Ar; 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; Zinzi, P; van Hout, Ms; van Vugt, Jp; de Weert, Am; Bolwijn, Jj; Dekker, M; Leenders, Kl; van Oostrom, Jc; Bos, R; Dumas, Em; Jurgens, Ck; van den Bogaard, Sj; Roos, Ra; 't Hart, Ep; Witjes-Ané, Mn; Kremer, B; Verstappen, Cc; Heiberg, A; van Walsem, Mr; 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; Zielonka, D; Samara, H; Janik, P; Kalbarczyk, A; Kwiecinski, H; Jamrozik, Z; Antczak, J; Jachinska, K; Rakowicz, M; Richter, P; Ryglewicz, D; Witkowski, G; Zdzienicka, E; Zaremba, J; Sułek, A; Krysa, W; Júlio, F; Januário, C; Mestre, T; Guedes, L; Coelho, M; Mendes, T; Valadas, A; Ferreira, Jj; 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; Cubillo, Pt; 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; Sebastián, Ar; 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, 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, P; Reza-Soltani, Tw; Constantinescu, R; Fredlund, G; Høsterey-Ugander, U; Neleborn-Lingefjärd, L; Wahlström, J; Esmaeilzadeh, M; Tedroff, J; Winnberg, E; Björn, Y; Ekwall, C; Gøller, Ml; Johansson, A; Wiklund, L; Petersen, Å; Reimer, J; Widner, H; Burgunder, Jm; 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; Townhill, J; 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; Miller, J; Raman, A; Barnes, K; Chu, C; Hobson, E; Jamieson, S; Markova, I; 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; Andrews, T; Bruno, S; Chu, E; Doherty, K; Golding, ; Fillingham, K; Foustanos, I; O'Donovan, K; Peppa, N; Tidswell, K; Quarrell, O

Suicidal ideation in a European Huntington's disease population

BACKGROUND: Previous studies indicate increased prevalences of suicidal ideation, suicide attempts, and completed suicide in Huntington's disease (HD) compared with the general population. This study investigates correlates and predictors of suicidal ideation in HD. METHODS: The study cohort consisted of 2106 HD mutation carriers, all participating in the REGISTRY study of the European Huntington's Disease Network. Of the 1937 participants without suicidal ideation at baseline, 945 had one or more follow-up measurements. Participants were assessed for suicidal ideation by the behavioural subscale of the Unified Huntington's Disease Rating Scale (UHDRS). Correlates of suicidal ideation were analyzed using logistic regression analysis and predictors were analyzed using Cox regression analysis. RESULTS: At baseline, 169 (8.0%) mutation carriers endorsed suicidal ideation. Disease duration (odds ratio [OR]=0.96; 95% confidence interval [CI]: 0.9-1.0), anxiety (OR=2.14; 95%CI: 1.4-3.3), aggression (OR=2.41; 95%CI: 1.5-3.8), a previous suicide attempt (OR=3.95; 95%CI: 2.4-6.6), and a depressed mood (OR=13.71; 95%CI: 6.7-28.0) were independently correlated to suicidal ideation at baseline. The 4-year cumulative incidence of suicidal ideation was 9.9%. Longitudinally, the presence of a depressed mood (hazard ratio [HR]=2.05; 95%CI: 1.1-4.0) and use of benzodiazepines (HR=2.44; 95%CI: 1.2-5.0) at baseline were independent predictors of incident suicidal ideation, whereas a previous suicide attempt was not predictive. LIMITATIONS: As suicidal ideation was assessed by only one item, and participants were a selection of all HD mutation carriers, the prevalence of suicidal ideation was likely underestimated. CONCLUSIONS: Suicidal ideation in HD frequently occurs. Assessment of suicidal ideation is a priority in mutation carriers with a depressed mood and in those using benzodiazepines

Suicidal ideation in a European Huntington's disease population

Previous studies indicate increased prevalences of suicidal ideation, suicide attempts, and completed suicide in Huntington's disease (HD) compared with the general population. This study investigates correlates and predictors of suicidal ideation in HD. METHODS: The study cohort consisted of 2106 HD mutation carriers, all participating in the REGISTRY study of the European Huntington's Disease Network. Of the 1937 participants without suicidal ideation at baseline, 945 had one or more follow-up measurements. Participants were assessed for suicidal ideation by the behavioural subscale of the Unified Huntington's Disease Rating Scale (UHDRS). Correlates of suicidal ideation were analyzed using logistic regression analysis and predictors were analyzed using Cox regression analysis. RESULTS: At baseline, 169 (8.0%) mutation carriers endorsed suicidal ideation. Disease duration (odds ratio [OR]=0.96; 95% confidence interval [CI]: 0.9-1.0), anxiety (OR=2.14; 95%CI: 1.4-3.3), aggression (OR=2.41; 95%CI: 1.5-3.8), a previous suicide attempt (OR=3.95; 95%CI: 2.4-6.6), and a depressed mood (OR=13.71; 95%CI: 6.7-28.0) were independently correlated to suicidal ideation at baseline. The 4-year cumulative incidence of suicidal ideation was 9.9%. Longitudinally, the presence of a depressed mood (hazard ratio [HR]=2.05; 95%CI: 1.1-4.0) and use of benzodiazepines (HR=2.44; 95%CI: 1.2-5.0) at baseline were independent predictors of incident suicidal ideation, whereas a previous suicide attempt was not predictive. LIMITATIONS: As suicidal ideation was assessed by only one item, and participants were a selection of all HD mutation carriers, the prevalence of suicidal ideation was likely underestimated. CONCLUSIONS: Suicidal ideation in HD frequently occurs. Assessment of suicidal ideation is a priority in mutation carriers with a depressed mood and in those using benzodiazepines