Background: The frequency of late-onset Huntington's disease (>59 years) is assumed to be low and the clinical course milder. However, previous literature on late-onset disease is scarce and inconclusive. Objective: Our aim is to study clinical characteristics of late-onset compared to common-onset HD patients in a large cohort of HD patients from the Registry database. Methods: Participants with late- and common-onset (30–50 years)were compared for first clinical symptoms, disease progression, CAG repeat size and family history. Participants with a missing CAG repeat size, a repeat size of ≤35 or a UHDRS motor score of ≤5 were excluded. Results: Of 6007 eligible participants, 687 had late-onset (11.4%) and 3216 (53.5%) common-onset HD. Late-onset (n = 577) had significantly more gait and balance problems as first symptom compared to common-onset (n = 2408) (P <.001). Overall motor and cognitive performance (P <.001) were worse, however only disease motor progression was slower (coefficient, −0.58; SE 0.16; P <.001) compared to the common-onset group. Repeat size was significantly lower in the late-onset (n = 40.8; SD 1.6) compared to common-onset (n = 44.4; SD 2.8) (P <.001). Fewer late-onset patients (n = 451) had a positive family history compared to common-onset (n = 2940) (P <.001). Conclusions: Late-onset patients present more frequently with gait and balance problems as first symptom, and disease progression is not milder compared to common-onset HD patients apart from motor progression. The family history is likely to be negative, which might make diagnosing HD more difficult in this population. However, the balance and gait problems might be helpful in diagnosing HD in elderly patients.
Clinical and genetic characteristics of late-onset Huntington's disease / Oosterloo, Mayke; Bijlsma, Emilia K.; van Kuijk, Sander MJ.; Minkels, Floor; de Die-Smulders, Christine EM.; Bachoud-Lévi, Anne-Catherine; Bentivoglio, Anna-Rita; Biunno, Ida; Bonelli, Raphael M.; Bronzova, Juliana; Burgunder, Jean-Marc; Dunnett, Stephen B.; Ferreira, Joaquim J.; Frich, Jan; Giuliano, Joe; Handley, Olivia J.; Heiberg, Arvid; Illarioshkin, Sergey; Illmann, Torsten; Klempir, Jiri; Landwehrmeyer, G. Bernhard; Levey, Jamie; McLean, Tim; Nielsen, Jørgen E.; Koivisto, Susana Pro; Päivärinta, Markku; Pålhagen, Sven; Quarrell, Oliver; Ramos-Arroyo, Maria; Roos, Raymund A.C.; Saft, Carsten; Sebastián, Ana Rojo; Tabrizi, Sarah J.; Vandenberghe, Wim; Verellen-Dumoulin, Christine; Uhrova, Tereza; Wahlström+, Jan; Zaremba, Jacek; (formerly Rödig, Verena Baake; Barth, Katrin; Garde, Monica Bascuñana; Becanovic, Kristina; Bernard, Tomáš; Betz, Sabrina; Bos, Reineke; Come, Adrien; Guedes, Leonor Correia; Callaghan, Jenny; Capodarca, Selene; Charpentier, Sébastien; Vieira da Silva, Wildson; Di Renzo, Martina; Ecker, Daniel; Finisterra, Ana Maria; Fullam, Ruth; Genoves, Camille; Gilling, Mette; Handley, Olivia J.; Horta, Andrea; Hvalstedt, Carina; Held, Christine; Hussain, Hasina; Koppers, Kerstin; Lamanna, Claudia; Laurà, Matilde; Descals, Asunción Martínez; Martinez-Horta, Saul; Mestre, Tiago; Minster, Sara; Monza, Daniela; Münkel, Kristina; Mütze, Lisanne; Oehmen, Martin; Padieu, Helene; Paterski, Laurent; Peppa, Nadia; Koivisto, Susana Pro; Rindal, Beate; Rogers, Dawn; Røren (formerly Heinonen), Niini; Salgueiro, Ana; Šašinková, Pavla; Seliverstov, Yury; Taylor, Catherine; Timewell, Erika; Townhill, Jenny; Cubillo, Patricia Trigo; van Walsem, Marleen R.; Witjes-Ané, Marie-Noelle; Witkowski, Grzegorz; Wright, Abigail; Yudina, Elizaveta; Zielonka, Daniel; Zielonka, Eugeniusz; Zinzi, Paola; Bonelli, Raphael M.; Hecht, Karen; Herranhof, Brigitte; Holl (formerly Hödl), Anna; Kapfhammer, Hans-Peter; Koppitz, Michael; Lilek, Sabine; Magnet, Markus; Müller, Nicole; Otti, Daniela; Painold, Annamaria; Reisinger, Karin; Scheibl, Monika; Schöggl, Helmut; Ullah, Jasmin; Braunwarth, Eva-Maria; Brugger, Florian; Buratti, Lisa; Hametner, Eva-Maria; Hepperger, Caroline; Holas, Christiane; Hotter, Anna; Hussl, Anna; Larcher, Barbara; Mahlknecht, Philipp; Müller, Christoph; Pinter, Bernadette; Poewe, Werner; Reiter, Eva-Magdalena; Seppi, Klaus; Sprenger, Fabienne; Wenning, Gregor; Bonelli, Raphael M.; Ladurner, Gunther; Lilek, Stefan; Sinadinosa, Daniela; Staffen, Wolfgang; Walleczek, Anna Maria; Linder, Christoph; Pirker, Walter; Liessens, Dirk; Calmeyn, Godelinde; Somers, Nele; Delvaux, Isabelle; Boogaerts, Andrea; Flamez, Anja; de Raedt, Sylvie; Alaerts, Nick; Slama, Hichem; Supiot, Frédéric; Constant, Eric; Gillardin, Anne-Françoise; Léonard, Marie-Claude; Verellen-Dumoulin, Christine; van de Wyngaerde, Françoise; Dupuis, Michel; Minet, Cécile; Ribaï, Pascale; Van Paemel, Dominique; Verellen-Dumoulin, Christine; Boogaerts, Andrea; Vandenberghe, Wim; van Reijen, Dimphna; Weckx, Petra; Kaiserova, Michaela; Šenkárová, Zuzana; Bezdíček, Ondřej; Klempíř, Jiří; Klempířová, Olga; Majerová-Ibarburu, Veronika; Nikolai, Tomáš; Roth, Jan; Stárková, Irena; Madsen, Louise Hasselstrøm; Møller, Anette Torvin; Hjermind, Lena; Jacobsen, Oda; Larsen, Ida Unmack; Lindquist, Suzanne; Nielsen, Jørgen; Regeur, Lisbeth; Roos, Peter; Stockholm, Jette; Vangsted-Hansen, Christina; Vinther-Jensen, Tua; Lolk, Annette; Lundsgaard, Marianne; Wermuth, Lene; Andersson, Christian; Nyberg, Clara; Sundblom, Jimmy; Peippo, Maarit; Sipponen, Marjatta; Bruun, Anu; Hartikainen, Paivi; Mäkipää, Seija; Ollokainen, Mari; Åman, Jaana; Kärppä, Mikko; Ignatius, Jaakko; Jääskeläinen, Outi; Kajula, Outi; Moilanen, Jukka; Mustonen, Aki; Santala, Maire; Eklund, Pia; Hiivola, Heli; Hyppönen, Hannele; Martikainen, Kirsti; Ojala, Marjut; Tähkäpää, Sirkku; Tuuha, Katri; Allain, Philippe; Bonneau, Dominique; Bost, Marie; Gohier, Bénédicte; Guérid, Marie-Anne; Olivier, Audrey; Prouzet, Julie; Prundean, Adriana; Scherer-Gagou, Clarisse; Verny, Christophe; Babiloni, Blandine; Bled, Déborah; Debruxelles, Sabrina; Duché, Charlotte; Fraisse, Sonia; Goizet, Cyril; Jameau, Laetitia; Lafoucrière, Danielle; Spampinato, Umberto; Couttier, Julien; Debilly, Bérengère; Delaigue, Christine; Derost, Philippe; Durif, Franck; Germain, Véronique; Legendre, Perrine; Loiseau, Sylvie; Marques, Ana; Ulla, Miguel; Vidal, Tiphaine; Bachoud-Lévi, Anne-Catherine; Badei, Farideh; Boissé, Marie-Françoise; Boudali, Lotfi; Cleret de Langavant, Laurent; Lemoine, Laurie; Morgado, Graca; Youssov, Katia; Annic, Agnès; Barthélémy, Recka; De Bruycker, Christelle; Cabaret, Maryline; Carette, Anne-Sophie; Carrière, Nicolas; Decorte, Eric; Defebvre, Luc; Delliaux, Marie; Delval, Arnaud; Depelchin, Alizé; Destee, Alain; Dewulf-Pasz, Nelly; Dondaine, Thibaut; Dugauquier, Florence; Dujardin, Kathy; Hopes, Lucie; Krystkowiak, Pierre; Lemaire, Marie-Hélène; Manouvrier, Sylvie; Mutez, Eugénie; Peter, Mireille; Plomhause, Lucie; Sablonnière, Bernard; Simonin, Clémence; Tard, Céline; Thibault-Tanchou, Stéphanie; Vuillaume, Isabelle; Bellonet, Marcellin; Blin, Stéphanie; Chen, Simone; Krystkowiak, Pierre; Masmoudi, Kamel; Morin, Gilles; Roussel, Martine; Tir, Mélissa; Schüler, Béatrice; Wannepain, Sandrine; Zouitina, Yassine; Azulay, Jean-Philippe; Delfini, Marie; Eusebio, Alexandre; Fluchere, Frédérique; Guenam, Aicha; Mundler, Laura; Nguyen, Karine; Benaich, Sandra; Brice, Alexis; Boster, Sarah; Charles, Perrine; Durr, Alexandra; Ewenczyk, Claire; Francisque, Hélène; Jauffret, Céline; Justo, Damian; Kassar, Abdulrahman; Klebe, Stephan; Lesne, Fabien; Milani, Paolo; Monin, Marie-Lorraine; Monnier, Tiffany; Roze, Emmanuel; Tataru, Alina; Tchikviladzé, Maya; Bioux, Sandrine; Bliaux, Evangeline; Girard, Carole; Guyant-Maréchal, Lucie; Hannequin, Didier; Hannier, Véronique; Jourdain, Séverine; Maltête, David; Pouliquen, Dorothée; Anheim, Mathieu; Barun, Nadia; Lagha-Boukbiza, Ouhaid; Longato, Nadine; Marcel, Christophe; Phillipps, Clélie; Rudolf, Gabrielle; Steinmetz, Gisèle; Tranchant, Christine; Wagner, Caroline; Zimmermann, Marie-Agathe; Blondeau, Leily; Calvas, Fabienne; Cheriet, Samia; Delabaere, Helène; Demonet, Jean-François; Marquine, Laurent; Pariente, Jérémie; Pierre, Michèle; Pomies, Elsa; Rolland, Sandrine; Souyris, Corinne; Kosinski, Christoph Michael; Milkereit, Eva; Probst, Daniela; Reetz, Kathrin; Sass, Christian; Schiefer, Johannes; Schlangen, Christiane; Werner, Cornelius J.; Beuth, Markus; Gelderblom, Harald; Priller, Josef; Prüß, Harald; Spruth, Eike; Thiel, Silvia; Andrich, Jürgen; Ellrichmann, Gisa; Herrmann, Lennard; Hoffmann, Rainer; Kaminski, Barbara; Kraus, Peter; Saft, Carsten; Stamm, Christiane; Ganos, Christos; Stubbe, Lars; Tadic, Vera; Tübing, Jennifer; Lange, Herwig; Bosredon, Cecile; Hunger, Ulrike; Löhle, Matthias; Maass, Antonia; Ossig, Christiana; Schmidt, Simone; Storch, Alexander; Wolz, Annett; Wolz, Martin; Kohl, Zacharias; Kozay, Christina; Ullah, Jasmin; Winkler, Jürgen; Bergmann, Ulrike; Böringer, Regina; Capetian, Philipp; Kammel, Gerit; Lambeck, Johann; Mächtel, Miriam; Meier, Simone; Rijntjes, Michel; Zucker, Birgit; Boelmans, Kai; Ganos, Christos; Goerendt, Ines; Heinicke, Walburgis; Hidding, Ute; Lewerenz, Jan; Münchau, Alexander; Orth, Michael; Schmalfeld, Jenny; Stubbe, Lars; Zittel, Simone; Diercks, Gabriele; Dressler, Dirk; Francis, Flverly; Gayde-Stephan, Sabine; Gorzolla, Heike; Kramer, Bianca; Minschke, Rebecca; Schrader, Christoph; Tacik, Pawel; Ribbat+, Michael; Longinus, Bernhard; Möller, Carsten; Bürk, Katrin; Lüsebrink, Antje; Mühlau, Mark; Peinemann, Alexander; Städtler, Michael; Weindl, Adolf; Winkelmann, Juliane; Ziegler, Cornelia; Bechtel, Natalie; Beckmann, Heike; Bohlen, Stefan; Göpfert, Nicole; Hölzner, Eva; Lange, Herwig; Reilmann, Ralf; Rohm, Stefanie; Rumpf, Silke; Sass, Christian; Schepers, Sigrun; Weber, Nathalia; Bachmeier, Michael; Dose, Matthias; Hofstetter, Nina; Marquard, Ralf; Mühlbäck, Alzbeta; Barth, Katrin; Buck, Andrea; Connemann, Julia; Ecker, Daniel; Geitner, Carolin; Held, Christine; Kesse, Andrea; Landwehrmeyer, Bernhard; Lezius, Franziska; Lewerenz, Jan; Nepper, Solveig; Niess, Anke; Orth, Michael; Schneider, Ariane; Schwenk, Daniela; Süssmuth, Sigurd; Trautmann, Sonja; Vogel, Melanie; Weydt, Patrick; Klebe, Stephan; Musacchio, Thomas; Leypold, Christine; Nöth, Kerstin; Cormio, Claudia; Difruscolo, Olimpia; Franco, Giovanni; Nuzzi, Angela; Sciruicchio, Vittorio; Serpino, Claudia; de Tommaso, Marina; Calandra-Buonaura, Giovanna; Capellari, Sabina; Cortelli, Pietro; Gallassi, Roberto; Poda, Roberto; Scaglione, Cesa; Agosti, Chiara; Barlati, Sergio; Compostella, Silvia; Marchina, Eleonora; Padovani, Alessando; Figorilli, Michela; Marrosu, Francesco; Muroni, Antonella; Piras, Valeria; Vacca, Melisa; Bertini, Elisabetta; Bartoli, Caterina; Fortunato, Fernanda; Ghelli, Elena; Ginestroni, Andrea; Mechi, Claudia; Paganini, Marco; Piacentini, Silvia; Pradella, Silvia; Romoli, Anna Maria; Sorbi, Sandro; Abbruzzese, Giovanni; Bandettini di Poggio, Monica; Ferrandes, Giovanna; Mandich, Paola; Marchese, Roberta; Di Maria, Emilio; Tamburini, Tiziano; Albanese, Alberto; Castagliuolo, Simona; Castaldo, Anna; Di Donato, Stefano; Di Bella, Daniela; Gellera, Cinzia; Genitrini, Silvia; Mariotti, Caterina; Monza, Daniela; Nanetti, Lorenzo; Panzeri, Marta; Paridi, Dominga; Soliveri, Paola; Spagnolo, Francesca; Taroni, Franco; Tomasello, Chiara; De Michele, Giuseppe; Di Maio, Luigi; Rinaldi, Carlo; Massarelli, Marco; Peluso, Silvio; Roca, Alessandro; Russo, Cinzia Valeria; Salvatore, Elena; Sorrentino, Pierpaolo; Tucci, Tecla; Cannella, Milena; Codella, Valentina; De Gregorio, Francesca; De Nicola, Annunziata; Elifani, Francesca; Esposito, Chiara; Martino, Tiziana; Mazzante, Irene; Petrollini, Martina; Simonelli, Maria; Vezza, Maurizio; Squitieri, Ferdinando; D'Alessio, Barbara; Lovo, Francesca; Bentivoglio, Anna Rita; Bove, Francesco; Catalli, Claudio; Di Giacopo, Raffaella; Fasano, Alfonso; Frontali, Marina; Guidubaldi, Arianna; Ialongo, Tamara; Jacopini, Gioia; Loria, Giovanna; Modoni, Anna; Petracca, Martina; Piano, Carla; Chiara, Piccininni; Quaranta, Davide; Romano, Silvia; Soleti, Francesco; Solito, Marcella; Spadaro, Maria; Torlizzi, Flavia; Zinzi, Paola; Coarelli, Giulia; Ferraldeschi, Michela; Frontali, Marina; Jacopini, Gioia; Ristori, Giovanni; Romano, Silvia; Zinzi, Paola; van Hout, Monique S.E.; van Vugt, Jeroen P.P.; Marit de Weert, A.; Verhoeven, Marloes; Dekker, Meike; Klooster, Jesper; Leenders, Nico; van Oostrom, Joost; Kremer, Berry; Baake, Verena; van den Bogaard, Simon J.A.; Bos, Reineke; Dumas, Eve M.; t Hart, Ellen P.; Hogenboom, Marye; Jacobs, Milou; Jurgens, Caroline; Kampstra, Anne; Roos, Raymund A.C.; Schoonderbeek, Anne; Witjes-Ané, Marie-Noëlle; Duits, Annelien; Oosterloo, Mayke; Waber, Mirella; Kremer, Berry; Verstappen, Carla; Blinkenberg, Ellen Økland; Hauge, Erik; Tyvoll, Hilde; Aaserud, Olaf; Aanonsen, Nils Olaf; Bjørgo, Kathrine; Borgerød, Nancy; Dramstad, Elisabeth; Fannemel, Madeleine; Frich, Jan C.; Gørvell, Per F.; Haggag, Kathrine; Johannessen, Cecilie Haggag; Heiberg, Arvid; Retterstøl, Lars; Røsby, Oddveig; Rummel, Jutta; Sikiric, Alma; Stokke, Bodil; van Walsem, Marleen; Wehus, Ragnhild; Arntsen, Vibeke; Bjørnevoll, Inga; Sando, Sigrid Botne; Haug, Marte Gjøl; Størseth, Hanna Haugan; Østern, Rune; Paulsen, Julie; Dziadkiewicz, Artur; Konkel, Agnieszka; Narożańska, Ewa; Nowak, Malgorzata; Robowski, Piotr; Sitek, Emilia; Slawek, Jaroslaw; Soltan, Witold; Szinwelski, Michal; Arkuszewski, Michał; Błaszczyk, Magdalena; Boczarska-Jedynak, Magdalena; Ciach-Wysocka, Ewelina; Gorzkowska, Agnieszka; Jasińska-Myga, Barbara; Kaczmarczyk, Aleksandra; Kłodowska – Duda, Gabriela; Opala, Grzegorz; Rudzińska, Monika; Stompel, Daniel; Banaszkiewicz, Krzysztof; Boćwińska, Dorota; Bojakowska-Jaremek, Kamila; Dec, Małgorzata; Grabska, Natalia; Krawczyk, Malgorzata; Kubowicz, Ewelina; Malec-Litwinowicz, Michalina; Rudzińska, Monika; Stenwak, Agata; Szczudlik, Andrzej; Szczygieł, Elżbieta; Wójcik, Magdalena; Wasielewska, Anna; Anna Bryl, Jacek Anioła; Ciesielska, Anna; Klimberg, Aneta; Marcinkowski, Jerzy; Samara, Husam; Sempołowicz, Justyna; Wiśniewski, Bartłomiej; Zielonka, Daniel; Gogol (formerly Kalbarczyk), Anna; Janik, Piotr; Jamrozik, Zygmunt; Kaminska, Anna; Kwiecinski+, Hubert; Antczak, Jakub; Jachinska, Katarzyna; Krysa, Wioletta; Rakowicz, Maryla; Rola, Rafal; Ryglewicz, Danuta; Sienkiewicz-Jarosz, Halina; Stępniak, Iwona; Sułek, Anna; Witkowski, Grzegorz; Zaremba, Jacek; Zdzienicka, Elzbieta; Ziora-Jakutowicz, Karolina; Januário, Cristina; Júlio, Filipa; Almeida, Manuel; Calado, Ana; Dias, Margarida; Morgado, Joana; Semedo, Cristina; Guedes, Leonor Correia; Coelho, Miguel; Ferreira, Joaquim J.; Magalhães, Andreia; Mestre, Tiago; Mendes, Tiago; Neutel, Dulce; Rodrigues, Filipe; Valadas, Anabela; Costa, Cristina; Cardoso, Helena; Mendes, Tiago; Santos, Mariana; Cação, Gonçalo; Cavaco, Sara; Damásio, Joana; Fernandes, Joana; Gonçalves, Alexandra; Loureiro, Rui; Moreira, Inês; Magalhães, Marina; Salgado, Paula; Andrade, Carlos; Costa, Andreia; Garrett, Carolina; Gago, Miguel; Guimarães, Joana; Massano, João; Meireles, Joana; Monteiro, Ana; Khasanova, Diana; Zalyalova, Zuleykha; Illarioshkin, Sergey; Klyushnikov, Sergey; Sidorova, Olga; Smirnov, Oleg; Yudina, Elizaveta; Seliverstov, Yury; Antonova, Victoria; Kopishinskaya, Svetlana; Korotysh, Maria; Magzhanov, Rim; Saifullina, Elena; Kurbatov, Sergey; Solis, Pilar; Herrera, Carmen Durán; Moreno, Patrocinio Garcia; Bas, Jordi; Busquets, Núria; Calopa, Matilde; Classen, Serge Jaumà; Dedichá, Nadia Rodríguez; Buongiorno, María Teresa; María, Andrés de la Cerda Santa; Muñoz, Esteban; Santacruz, Pilar; Barbera, Miquel Aguilar; Sebastián, Ana Rojo; Pardo, Sonia Arribas; Guia, Dolors Badenes; Calzado, Noemi; Hernanz, Laura Casas; Tartari Díaz-Zorita, Juan Pablo; Catena, Judit López; Ferrer, Pilar Quiléz; Carruesco, Gemma Tome; Robert, Misericordia Floriach; Viladrich, Cèlia Mareca; Roca, Elvira; Ruiz Idiago, Jesús Miguel; Riballo, Antonio Villa; Campolongo, Antonia; Fernandez de Bobadilla, Ramon; Horta, Andrea; Bojarsky, Jaime Kulisevsky; Martinez-Horta, Saul; Pagonabarraga, Javier; Perez, Jesus Perez; Ribosa, Roser; Villa, Carolina; Acera Gil, Maria Angeles; Corrales, Koldo Berganzo; Gomez Esteban, Juan Carlos; González, Amaia; Merino, Beatriz Tijero; Cubo, Esther; Polo, Cecilia Gil; Mariscal, Natividad; Sánchez, Jesús; Romero, Sandra Gutierrez; Arbelo, José Matías; Malo de Molina, Rocío; Martín, Idaira; Periañez, Juan Manuel; Udaeta, Beatriz; Alonso-Frech, Fernando; Loarte, María del Valle; Barrero, Francisco; Morales, Blas; Frades, Belén; Villanueva, Marina Ávila; Zea Sevilla, Maria Ascension; Fenollar, María del Mar; García-Ramos García, Rocío; Villanueva, Clara; Bascuñana, Mónica; Ventura, Marta Fatás; Caldentey, Juan García; Ribas, Guillermo García; García de Yébenes, Justo; López-Sendón Moreno, José Luis; Barral, Verónica Mañanes; Cubillo, Patricia Trigo; Feliz, Cici; García Ruíz, Pedro José; García, Ana; Caldentey, Juan García; López, Rosa Guerrero; Bárcenas, Antonio Herranz; Martínez-Descals, Asunción; Pueyo, Angel Martínez; Martin, Veronica Puertas; Martínez, Noelia Rodríguez; Montojo, Teresa; Sainz Artiga, María José; Sánchez, Vicenta; Alarcón, María Dolores; Almagro, Carmen Antúnez; Diéguez, Esther; Fortuna, Lorenza; Legaz, Agustina; Manzanares, Salvadora; Muñoz, Juan Marín; Antequera Torres, María Martirio; Perea, Fuensanta Noguera; Vivancos, Laura; González, Sonia; Guisasola, Luis Menéndez; Prieto, Marta Para; Ribacoba, René; Salvador, Carlos; Lozano, Pablo Sánchez; Caldentey, Juan García; Ramirez, Inés Legarda; Benito, Dolors Moragues; Arques, Penelope Navas; Lopera, Monica Rodriguez; Pastor, Barbara Vives; Gaston, Itziar; Garcia-Amigot, Fermin; Martinez-Jaurrieta, Maria Dolores; Ramos-Arroyo, Maria Antonia; Adarmes, Astrid; Bernal-Escudero, Maravilla; Carrillo, Fátima; Jesús, Silvia; Mir, Pablo; Vargas-González, Laura; Hermoso, Fátima Damas; García Moreno, José Manuel; Jaramillo, Javier Abril; Lucena, Carolina Mendez; Pacheco Cortegana, Eva María; Peña, José Chacón; Redondo, Luis; Sánchez, Violeta Sánchez; Fernandez, Cristina Melgar; Romero Lemos, María Dolores; Mata, Maite Paredes; Casado, Rocío Villagrán; Bosca, Maria; Burguera, Juan Andres; Brugada, Francisco Castera; Millán Salvador, Jose Maria; Vilaplana, Carmen Peiró; Solís, Pilar; Figuerola, Begoña Jeweinat; Palanca, Paloma Millan; Diago, Elena Bellosta; López del Val, Javier; Martinez, Laura Martinez; López, Elena; Wahlström+, Jan; Høsterey-Ugander, Ulrika; Fredlund, Gunnel; Constantinescu, Radu; Lewin, Kajsa; Neleborn-Lingefjärd, Liselotte; Berglund, Maria; Berglund, Peter; Linnsand, Petra; Petersén, Åsa; Reimer, Jan; Widner, Håkan; Esmaeilzadeh, Mouna; Tedroff, Joakim; Winnberg, Elisabeth; Benaminov, Stanislav; Björnsson, Elisabeth; Merrick, Daniel; Paucar, Martin; Pålhagen, Sven; Svenningsson, Per; Wallden, Tina; Berglund, Måns; Loutfi, Ghada; Olofsson, Carina; Stattin, Eva-Lena; Westman, Laila; Wikström, Birgitta; Ekwall, Camilla; Göller, Marie-Lousie; Johansson, Anders; Niemelä, Valter; Nyholm, Dag; Sundblom, Jimmy; Wiklund, Leif; Burgunder, Jean-Marc; Koehli, Jessica; Stebler, Yanik; Kaelin, Alain; Romero, Irene; Schüpbach, Michael; Zaugg, Sabine Weber; Esposito, Federica; Good, Jean-Marc; Paus, Karin; Vingerhoets, Francois; Wider+, Christian; Jung, Hans H.; Petersen, Jens A.; Ligon-Auer, Maria; Mihaylova, Violeta; Downie, Lorna; Jack, Roisin; Matheson, Kirsty; Miedzybrodzka, Zosia; Rae, Daniela; Simpson, Sheila A.; Summers, Fiona; Ure, Alexandra; Vaughan, Vivien; Harrower, Timothy; Vernon, Nathan; Akhtar, Shahbana; Crooks, Jenny; Curtis, Adrienne; de Souza (Keylock), Jenny; Piedad, John; Rickards, Hugh; Wright, Jan; Haig-Brown, Diane; Craven, Janet; Pallett, Andrew; Simpson, Steve; Weekes, Rebecca; Coulthard, Elizabeth; Gethin, Louise; Hayward, Beverley; Sieradzan, Kasia; Wright, Abigail; Barker, Roger A.; O'Keefe, Deidre; Gerrtiz (nee Di Pietro), Anna; Fisher, Kate; Goodman, Anna; Hill, Susan; Mason, Sarah; Swain, Rachel; Guzman, Natalie Valle; Busse, Monica; Butcher, Cynthia; Callaghan, Jenny; Dunnett, Stephen; Clenaghan, Catherine; Fullam, Ruth; Hunt, Sarah; Jones, Lesley; Jones, Una; Khalil, Hanan; Minster, Sara; Owen, Michael; Price, Kathleen; Townhill, Jenny; Rosser, Anne; Goudie, David; Buchanan, Lindsay; McFadyen, Paula; Tonner, Alison; Taylor, Anne-Marie; Edwards, Maureen; Ho, Carrie; McGill, Marie; Porteous, Mary; Pearson, Pauline; Harrower, Timothy; Irvine, Sarah; Brockie, Peter; Foster, Jillian; Johns, Nicola; McKenzie, Sue; Rothery, Jean; Thomas, Gareth; Yates, Shona; Neumann, Christian; Patterson, Kirsten; Thomson, David; Deith, Catherine; Ireland, Jane; Ritchie, Stuart; Brown, Pauline; Burrows, Liz; Fletcher, Amy; Harding, Alison; Harrison, Kaye; Laver, Fiona; Silva, Mark; Thomson, Aileen; Chu, Carol; Evans, Carole; Gallentree, Deena; Hamer, Stephanie; Kraus, Alison; Markova, Ivana; Raman, Ashok; Rowett, Liz; Andrew, Alyson; Frost, Julie; Noad, Rupert; Cosgrove, Jeremy; Gallantree, Deena; Hamer, Stephanie; Hobson, Emma; Jamieson, Stuart; Kraus, Alison; Longthorpe, Mandy; Markova, Ivana; Musgrave, Hannah; Peacy, Caroline; Raman, Ashok; Rowett, Liz; Toscano, Jean; Wild, Sue; Yardumian, Pam; Clayton, Carole; Dipple, Heather; Freire-Patino, Dawn; Hallam, Caroline; Middleton, Julia; Alusi, Sundus; Davies, Rhys; Foy, Kevin; Gerrans, Emily; Pate, Louise; Anjum, Uruj; Coebergh, Jan; Eddy, Charlotte; 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[10.1016/j.parkreldis.2018.11.009]
Clinical and genetic characteristics of late-onset Huntington's disease
Calandra-Buonaura, GiovannaMembro del Collaboration Group
;Capellari, SabinaMembro del Collaboration Group
;Cortelli, PietroMembro del Collaboration Group
;
2019
Abstract
Background: The frequency of late-onset Huntington's disease (>59 years) is assumed to be low and the clinical course milder. However, previous literature on late-onset disease is scarce and inconclusive. Objective: Our aim is to study clinical characteristics of late-onset compared to common-onset HD patients in a large cohort of HD patients from the Registry database. Methods: Participants with late- and common-onset (30–50 years)were compared for first clinical symptoms, disease progression, CAG repeat size and family history. Participants with a missing CAG repeat size, a repeat size of ≤35 or a UHDRS motor score of ≤5 were excluded. Results: Of 6007 eligible participants, 687 had late-onset (11.4%) and 3216 (53.5%) common-onset HD. Late-onset (n = 577) had significantly more gait and balance problems as first symptom compared to common-onset (n = 2408) (P <.001). Overall motor and cognitive performance (P <.001) were worse, however only disease motor progression was slower (coefficient, −0.58; SE 0.16; P <.001) compared to the common-onset group. Repeat size was significantly lower in the late-onset (n = 40.8; SD 1.6) compared to common-onset (n = 44.4; SD 2.8) (P <.001). Fewer late-onset patients (n = 451) had a positive family history compared to common-onset (n = 2940) (P <.001). Conclusions: Late-onset patients present more frequently with gait and balance problems as first symptom, and disease progression is not milder compared to common-onset HD patients apart from motor progression. The family history is likely to be negative, which might make diagnosing HD more difficult in this population. However, the balance and gait problems might be helpful in diagnosing HD in elderly patients.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
