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Parkinsonism Relat Disord. 2018 Nov 29. pii: S1353-8020(18)30490-5. doi: 10.1016/j.parkreldis.2018.11.009. [Epub ahead of print]

Clinical and genetic characteristics of late-onset Huntington's disease.

Collaborators (1184)

Bachoud-Lévi AC, Bentivoglio AR, Biunno I, Bonelli RM, Bronzova J, Burgunder JM, Dunnett SB, Ferreira JJ, Frich J, Giuliano J, Handley OJ, Heiberg A, Illarioshkin S, Illmann T, Klempir J, Landwehrmeyer GB, Levey J, McLean T, Nielsen JE, Koivisto SP, Päivärinta M, Pålhagen S, Quarrell O, Ramos-Arroyo M, Roos RAC, Saft C, Sebastián AR, Tabrizi SJ, Vandenberghe W, Verellen-Dumoulin C, Uhrova T, Wahlström J, Zaremba J, Formerly Rödig VB, Barth K, Garde MB, Becanovic K, Bernard T, Betz S, Bos R, Come A, Guedes LC, Callaghan J, Capodarca S, Charpentier S, Vieira da Silva W, Di Renzo M, Ecker D, Finisterra AM, Fullam R, Genoves C, Gilling M, Handley OJ, Horta A, Hvalstedt C, Held C, Hussain H, Koppers K, Lamanna C, Laurà M, Descals AM, Martinez-Horta S, Mestre T, Minster S, Monza D, Münkel K, Mütze L, Oehmen M, Padieu H, Paterski L, Peppa N, Koivisto SP, Rindal B, Rogers D, Røren Formerly Heinonen N, Salgueiro A, Šašinková P, Seliverstov Y, Taylor C, Timewell E, Townhill J, Cubillo PT, van Walsem MR, Witjes-Ané MN, Witkowski G, Wright A, Yudina E, Zielonka D, Zielonka E, Zinzi P, Bonelli RM, Hecht K, Herranhof B, Holl Formerly Hödl A, Kapfhammer HP, Koppitz M, Lilek S, Magnet M, Müller N, Otti D, Painold A, Reisinger K, Scheibl M, Schöggl H, Ullah J, Braunwarth EM, Brugger F, Buratti L, Hametner EM, Hepperger C, Holas C, Hotter A, Hussl A, Larcher B, Mahlknecht P, Müller C, Pinter B, Poewe W, Reiter EM, Seppi K, Sprenger F, Wenning G, Bonelli RM, Ladurner G, Lilek S, Sinadinosa D, Staffen W, Walleczek AM, Linder C, Pirker W, Liessens D, Calmeyn G, Somers N, Delvaux I, Boogaerts A, Flamez A, de Raedt S, Alaerts N, Slama H, Supiot F, Constant E, Gillardin AF, Léonard MC, Verellen-Dumoulin C, van de Wyngaerde F, Dupuis M, Minet C, Ribaï P, Van Paemel D, Verellen-Dumoulin C, Boogaerts A, Vandenberghe W, van Reijen D, Weckx P, Kaiserova M, Šenkárová Z, Bezdíček O, Klempíř J, Klempířová O, Majerová-Ibarburu V, Nikolai T, Roth J, Stárková I, Madsen LH, Møller AT, Hjermind L, Jacobsen O, Larsen IU, Lindquist S, Nielsen J, Regeur L, Roos P, Stockholm J, Vangsted-Hansen C, Vinther-Jensen T, Lolk A, Lundsgaard M, Wermuth L, Andersson C, Nyberg C, Sundblom J, Peippo M, Sipponen M, Bruun A, Hartikainen P, Mäkipää S, Ollokainen M, Åman J, Kärppä M, Ignatius J, Jääskeläinen O, Kajula O, Moilanen J, Mustonen A, Santala M, Eklund P, Hiivola H, Hyppönen H, Martikainen K, Ojala M, Tähkäpää S, Tuuha K, Allain P, Bonneau D, Bost M, Gohier B, Guérid MA, Olivier A, Prouzet J, Prundean A, Scherer-Gagou C, Verny C, Babiloni B, Bled D, Debruxelles S, Duché C, Fraisse S, Goizet C, Jameau L, Lafoucrière D, Spampinato U, Couttier J, Debilly B, Delaigue C, Derost P, Durif F, Germain V, Legendre P, Loiseau S, Marques A, Ulla M, Vidal T, Bachoud-Lévi AC, Badei F, Boissé MF, Boudali L, Cleret de Langavant L, Lemoine L, Morgado G, Youssov K, Annic A, Barthélémy R, De Bruycker C, Cabaret M, Carette AS, Carrière N, Decorte E, Defebvre L, Delliaux M, Delval A, Depelchin A, Destee A, Dewulf-Pasz N, Dondaine T, Dugauquier F, Dujardin K, Hopes L, Krystkowiak P, Lemaire MH, Manouvrier S, Mutez E, Peter M, Plomhause L, Sablonnière B, Simonin C, Tard C, Thibault-Tanchou S, Vuillaume I, Bellonet M, Blin S, Chen S, Krystkowiak P, Masmoudi K, Morin G, Roussel M, Tir M, Schüler B, Wannepain S, Zouitina Y, Azulay JP, Delfini M, Eusebio A, Fluchere F, Guenam A, Mundler L, Nguyen K, Benaich S, Brice A, Boster S, Charles P, Durr A, Ewenczyk C, Francisque H, Jauffret C, Justo D, Kassar A, Klebe S, Lesne F, Milani P, Monin ML, Monnier T, Roze E, Tataru A, Tchikviladzé M, Bioux S, Bliaux E, Girard C, Guyant-Maréchal L, Hannequin D, Hannier V, Jourdain S, Maltête D, Pouliquen D, Anheim M, Barun N, Lagha-Boukbiza O, Longato N, Marcel C, Phillipps C, Rudolf G, Steinmetz G, Tranchant C, Wagner C, Zimmermann MA, Blondeau L, Calvas F, Cheriet S, Delabaere H, Demonet JF, Marquine L, Pariente J, Pierre M, Pomies E, Rolland S, Souyris C, Kosinski CM, Milkereit E, Probst D, Reetz K, Sass C, Schiefer J, Schlangen C, Werner CJ, Beuth M, Gelderblom H, Priller J, Prüß H, Spruth E, Thiel S, Andrich J, Ellrichmann G, Herrmann L, Hoffmann R, Kaminski B, Kraus P, Saft C, Stamm C, Ganos C, Stubbe L, Tadic V, Tübing J, Lange H, Bosredon C, Hunger U, Löhle M, Maass A, Ossig C, Schmidt S, Storch A, Wolz A, Wolz M, Kohl Z, Kozay C, Ullah J, Winkler J, Bergmann U, Böringer R, Capetian P, Kammel G, Lambeck J, Mächtel M, Meier S, Rijntjes M, Zucker B, Boelmans K, Ganos C, Goerendt I, Heinicke W, Hidding U, Lewerenz J, Münchau A, Orth M, Schmalfeld J, Stubbe L, Zittel S, Diercks G, Dressler D, Francis F, Gayde-Stephan S, Gorzolla H, Kramer B, Minschke R, Schrader C, Tacik P, Ribbat M, Longinus B, Möller C, Bürk K, Lüsebrink A, Mühlau M, Peinemann A, Städtler M, Weindl A, Winkelmann J, Ziegler C, Bechtel N, Beckmann H, Bohlen S, Göpfert N, Hölzner E, Lange H, Reilmann R, Rohm S, Rumpf S, Sass C, Schepers S, Weber N, Bachmeier M, Dose M, Hofstetter N, Marquard R, Mühlbäck A, Barth K, Buck A, Connemann J, Ecker D, Geitner C, Held C, Kesse A, Landwehrmeyer B, Lezius F, Lewerenz J, Nepper S, Niess A, Orth M, Schneider A, Schwenk D, Süssmuth S, Trautmann S, Vogel M, Weydt P, Klebe S, Musacchio T, Leypold C, Nöth K, Cormio C, Difruscolo O, Franco G, Nuzzi A, Sciruicchio V, Serpino C, de Tommaso M, Calandra-Buonaura G, Capellari S, Cortelli P, Gallassi R, Poda R, Scaglione C, Agosti C, Barlati S, Compostella S, Marchina E, Padovani A, Figorilli M, Marrosu F, Muroni A, Piras V, Vacca M, Bertini E, Bartoli C, Fortunato F, Ghelli E, Ginestroni A, Mechi C, Paganini M, Piacentini S, Pradella S, Romoli AM, Sorbi S, Abbruzzese G, Bandettini di Poggio M, Ferrandes G, Mandich P, Marchese R, Di Maria E, Tamburini T, Albanese A, Castagliuolo S, Castaldo A, Di Donato S, Di Bella D, Gellera C, Genitrini S, Mariotti C, Monza D, Nanetti L, Panzeri M, Paridi D, Soliveri P, Spagnolo F, Taroni F, Tomasello C, De Michele G, Di Maio L, Rinaldi C, Massarelli M, Peluso S, Roca A, Russo CV, Salvatore E, Sorrentino P, Tucci T, Cannella M, Codella V, De Gregorio F, De Nicola A, Elifani F, Esposito C, Martino T, Mazzante I, Petrollini M, Simonelli M, Vezza M, Squitieri F, D'Alessio B, Lovo F, Bentivoglio AR, Bove F, Catalli C, Di Giacopo R, Fasano A, Frontali M, Guidubaldi A, Ialongo T, Jacopini G, Loria G, Modoni A, Petracca M, Piano C, Chiara P, Quaranta D, Romano S, Soleti F, Solito M, Spadaro M, Torlizzi F, Zinzi P, Coarelli G, Ferraldeschi M, Frontali M, Jacopini G, Ristori G, Romano S, Zinzi P, van Hout MSE, van Vugt JPP, Marit de Weert A, Verhoeven M, Dekker M, Klooster J, Leenders N, van Oostrom J, Kremer B, Baake V, van den Bogaard SJA, Bos R, Dumas EM, T Hart EP, Hogenboom M, Jacobs M, Jurgens C, Kampstra A, Roos RAC, Schoonderbeek A, Witjes-Ané MN, Duits A, Oosterloo M, Waber M, Kremer B, Verstappen C, Blinkenberg EØ, Hauge E, Tyvoll H, Aaserud O, Aanonsen NO, Bjørgo K, Borgerød N, Dramstad E, Fannemel M, Frich JC, Gørvell PF, Haggag K, Johannessen CH, Heiberg A, Retterstøl L, Røsby O, Rummel J, Sikiric A, Stokke B, van Walsem M, Wehus R, Arntsen V, Bjørnevoll I, Sando SB, Haug MG, Størseth HH, Østern R, Paulsen J, Dziadkiewicz A, Konkel A, Narożańska E, Nowak M, Robowski P, Sitek E, Slawek J, Soltan W, Szinwelski M, Arkuszewski M, Błaszczyk M, Boczarska-Jedynak M, Ciach-Wysocka E, Gorzkowska A, Jasińska-Myga B, Kaczmarczyk A, Kłodowska-Duda G, Opala G, Rudzińska M, Stompel D, Banaszkiewicz K, Boćwińska D, Bojakowska-Jaremek K, Dec M, Grabska N, Krawczyk M, Kubowicz E, Malec-Litwinowicz M, Rudzińska M, Stenwak A, Szczudlik A, Szczygieł E, Wójcik M, Wasielewska A, Anna Bryl JA, Ciesielska A, Klimberg A, Marcinkowski J, Samara H, Sempołowicz J, Wiśniewski B, Zielonka D, Gogol Formerly Kalbarczyk A, Janik P, Jamrozik Z, Kaminska A, Kwiecinski H, Antczak J, Jachinska K, Krysa W, Rakowicz M, Rola R, Ryglewicz D, Sienkiewicz-Jarosz H, Stępniak I, Sułek A, Witkowski G, Zaremba J, Zdzienicka E, Ziora-Jakutowicz K, Januário C, Júlio F, Almeida M, Calado A, Dias M, Morgado J, Semedo C, Guedes LC, Coelho M, Ferreira JJ, Magalhães A, Mestre T, Mendes T, Neutel D, Rodrigues F, Valadas A, Costa C, Cardoso H, Mendes T, Santos M, Cação G, Cavaco S, Damásio J, Fernandes J, Gonçalves A, Loureiro R, Moreira I, Magalhães M, Salgado P, Andrade C, Costa A, Garrett C, Gago M, Guimarães J, Massano J, Meireles J, Monteiro A, Khasanova D, Zalyalova Z, Illarioshkin S, Klyushnikov S, Sidorova O, Smirnov O, Yudina E, Seliverstov Y, Antonova V, Kopishinskaya S, Korotysh M, Magzhanov R, Saifullina E, Kurbatov S, Solis P, Herrera CD, Moreno PG, Bas J, Busquets N, Calopa M, Classen SJ, Dedichá NR, Buongiorno MT, María ACS, Muñoz E, Santacruz P, Barbera MA, Sebastián AR, Pardo SA, Guia DB, Calzado N, Hernanz LC, Tartari Díaz-Zorita JP, Catena JL, Ferrer PQ, Carruesco GT, Robert MF, Viladrich CM, Roca E, Ruiz Idiago JM, Riballo AV, Campolongo A, Fernandez de Bobadilla R, Horta A, Bojarsky JK, Martinez-Horta S, Pagonabarraga J, Perez JP, Ribosa R, Villa C, Acera Gil MA, Corrales KB, Gomez Esteban JC, González A, Merino BT, Cubo E, Polo CG, Mariscal N, Sánchez J, Romero SG, Arbelo JM, Malo de Molina R, Martín I, Periañez JM, Udaeta B, Alonso-Frech F, Loarte MDV, Barrero F, Morales B, Frades B, Villanueva MÁ, Zea Sevilla MA, Fenollar MDM, García-Ramos García R, Villanueva C, Bascuñana M, Ventura MF, Caldentey JG, Ribas GG, García de Yébenes J, López-Sendón Moreno JL, Barral VM, Cubillo PT, Feliz C, García Ruíz PJ, García A, Caldentey JG, López RG, Bárcenas AH, Martínez-Descals A, Pueyo AM, Martin VP, Martínez NR, Montojo T, Sainz Artiga MJ, Sánchez V, Alarcón MD, Almagro CA, Diéguez E, Fortuna L, Legaz A, Manzanares S, Muñoz JM, Antequera Torres MM, Perea FN, Vivancos L, González S, Guisasola LM, Prieto MP, Ribacoba R, Salvador C, Lozano PS, Caldentey JG, Ramirez IL, Benito DM, Arques PN, Lopera MR, Pastor BV, Gaston I, Garcia-Amigot F, Martinez-Jaurrieta MD, Ramos-Arroyo MA, Adarmes A, Bernal-Escudero M, Carrillo F, Jesús S, Mir P, Vargas-González L, Hermoso FD, García Moreno JM, Jaramillo JA, Lucena CM, Pacheco Cortegana EM, Peña JC, Redondo L, Sánchez VS, Fernandez CM, Romero Lemos MD, Mata MP, Casado RV, Bosca M, Burguera JA, Brugada FC, Millán Salvador JM, Vilaplana CP, Solís P, Figuerola BJ, Palanca PM, Diago EB, López Del Val J, Martinez LM, López E, Wahlström J, Høsterey-Ugander U, Fredlund G, Constantinescu R, Lewin K, Neleborn-Lingefjärd L, Berglund M, Berglund P, Linnsand P, Petersén Å, Reimer J, Widner H, Esmaeilzadeh M, Tedroff J, Winnberg E, Benaminov S, Björnsson E, Merrick D, Paucar M, Pålhagen S, Svenningsson P, Wallden T, Berglund M, Loutfi G, Olofsson C, Stattin EL, Westman L, Wikström B, Ekwall C, Göller ML, Johansson A, Niemelä V, Nyholm D, Sundblom J, Wiklund L, Burgunder JM, Koehli J, Stebler Y, Kaelin A, Romero I, Schüpbach M, Zaugg SW, Esposito F, Good JM, Paus K, Vingerhoets F, Wider C, Jung HH, Petersen JA, Ligon-Auer M, Mihaylova V, Downie L, Jack R, Matheson K, Miedzybrodzka Z, Rae D, Simpson SA, Summers F, Ure A, Vaughan V, Harrower T, Vernon N, Akhtar S, Crooks J, Curtis A, de Souza Keylock J, Piedad J, Rickards H, Wright J, Haig-Brown D, Craven J, Pallett A, Simpson S, Weekes R, Coulthard E, Gethin L, Hayward B, Sieradzan K, Wright A, Barker RA, O'Keefe D, Gerrtiz Nee Di Pietro A, Fisher K, Goodman A, Hill S, Mason S, Swain R, Guzman NV, Busse M, Butcher C, Callaghan J, Dunnett S, Clenaghan C, Fullam R, Hunt S, Jones L, Jones U, Khalil H, Minster S, Owen M, Price K, Townhill J, Rosser A, Goudie D, Buchanan L, McFadyen P, Tonner A, Taylor AM, Edwards M, Ho C, McGill M, Porteous M, Pearson P, Harrower T, Irvine S, Brockie P, Foster J, Johns N, McKenzie S, Rothery J, Thomas G, Yates S, Neumann C, Patterson K, Thomson D, Deith C, Ireland J, Ritchie S, Brown P, Burrows L, Fletcher A, Harding A, Harrison K, Laver F, Silva M, Thomson A, Chu C, Evans C, Gallentree D, Hamer S, Kraus A, Markova I, Raman A, Rowett L, Andrew A, Frost J, Noad R, Cosgrove J, Gallantree D, Hamer S, Hobson E, Jamieson S, Kraus A, Longthorpe M, Markova I, Musgrave H, Peacy C, Raman A, Rowett L, Toscano J, Wild S, Yardumian P, Clayton C, Dipple H, Freire-Patino D, Hallam C, Middleton J, Alusi S, Davies R, Foy K, Gerrans E, Pate L, Anjum U, Coebergh J, Eddy C, Lahiri N, McEntagart M, Patton M, Peterson M, Rose S, Andrews T, Dougherty A, Golding C, Kavalier F, Laing H, Lashwood A, Robertson D, Ruddy D, Santhouse A, Whaite A, Andrews T, Gosling Nee Brown S, Bruno S, Chu E, Doherty K, Golding C, Haider S, Hensman D, Lahiri N, Lewis M, Novak M, Patel A, Robertson N, Rosser E, Tabrizi S, Taylor R, Warner T, Wild E, Ackermann O, Duport S, Scott A, Stoy N, Vaughn J, Arran N, Bek J, Callaghan J, Craufurd D, Fullam R, Hare M, Howard L, Huson S, Johnson L, Jones M, Krishnamoorthy A, Murphy H, Oughton E, Partington-Jones L, Rogers D, Sollom A, Snowden J, Stopford C, Thompson J, Trender-Gerhard I, Verstraelen Formerly Ritchie N, Westmoreland L, Cass G, Davidson L, Davison J, Fullerton N, Holmes K, Komati S, McDonnell S, Mohammed Z, Morgan K, Savage L, Singh B, Wood J, Chu E, Knight C, O'Neill M, Purkayastha DD, Nemeth AH, Siuda G, Valentine R, Dixon K, Armstrong R, Harrison D, Hughes M, Large S, Donovan JO, Palmer A, Parkinson A, Soltysiak B, Timings L, Williams J, Burn J, Weekes R, Craven J, Bailey W, Coleman C, Haig-Brown D, Simpson S, Hare M, Majeed T, Verstraelen Ritchie N, Armstrong R, Dixon K, Barrett W, Ho A, Bandmann O, Bradbury A, Fairtlough H, Fillingham K, Foustanos I, Gill P, Kazoka M, O'Donovan K, Nevitt L, Peppa N, Quarrell O, Taylor C, Tidswell K, Kipps C, MacKinnon L, Agarwal V, Hayward E, Gunner K, Harris K, Anderson M, Heywood M, Keys L, Smalley S, El-Nimr G, Duffell A, Wood S, Kennedy Nee Smith K, Gowers L, Powell K, Bethwaite P, Edwards R, Fuller K, Phillips M, Tan L, Burgunder JM, Lau PN, Pica E, Roos RA.

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.

KEYWORDS:

Age of onset; Huntington's disease; Late-onset Huntington's disease

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