Age at symptom onset and death and disease duration in genetic frontotemporal dementia: an international retrospective cohort study

KM Moore; J Nicholas; M Grossman; CT McMillan; DJ Irwin; L Massimo; VM Van Deerlin; JD Warren; NC Fox; MN Rossor; S Mead; M Bocchetta; BF Boeve; DS Knopman; NR Graff-Radford; LK Forsberg; R Rademakers; ZK Wszolek; JC van Swieten; LC Jiskoot; LH Meeter; EG Dopper; JM Papma; JS Snowden; J Saxon; M Jones; S Pickering-Brown; I Le Ber; A Camuzat; A Brice; P Caroppo; R Ghidoni; M Pievani; L Benussi; G Binetti; BC Dickerson; D Lucente; S Krivensky; C Graff; L Öijerstedt; M Fallström; H Thonberg; N Ghoshal; JC Morris; B Borroni; A Benussi; A Padovani; D Galimberti; E Scarpini; GG Fumagalli; IR Mackenzie; GYR Hsiung; P Sengdy; AL Boxer; H Rosen; JB Taylor; M Synofzik; C Wilke; P Sulzer; JR Hodges; G Halliday; J Kwok; R Sanchez-Valle; A Lladó; S Borrego-Ecija; I Santana; MR Almeida; M Tábuas-Pereira; F Moreno; M Barandiaran; B Indakoetxea; J Levin; A Danek; JB Rowe; TE Cope; M Otto; S Anderl-Straub; A de Mendonça; C Maruta; M Masellis; SE Black; P Couratier; G Lautrette; ED Huey; S Sorbi; B Nacmias; R Laforce; MPL Tremblay; R Vandenberghe; PV Damme; EJ Rogalski; S Weintraub; A Gerhard; CU Onyike; S Ducharme; SG Papageorgiou; ASL Ng; A Brodtmann; E Finger; R Guerreiro

Journal article

Age at symptom onset and death and disease duration in genetic frontotemporal dementia: an international retrospective cohort study

KM Moore, J Nicholas, M Grossman, CT McMillan, DJ Irwin, L Massimo, VM Van Deerlin, JD Warren, NC Fox, MN Rossor, S Mead, M Bocchetta, BF Boeve, DS Knopman, NR Graff-Radford, LK Forsberg, R Rademakers, ZK Wszolek, JC van Swieten, LC Jiskoot Show all

Lancet Neurology | Published : 2020

DOI: 10.1016/S1474-4422(19)30394-1

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Abstract

Background: Frontotemporal dementia is a heterogenous neurodegenerative disorder, with about a third of cases being genetic. Most of this genetic component is accounted for by mutations in GRN, MAPT, and C9orf72. In this study, we aimed to complement previous phenotypic studies by doing an international study of age at symptom onset, age at death, and disease duration in individuals with mutations in GRN, MAPT, and C9orf72. Methods: In this international, retrospective cohort study, we collected data on age at symptom onset, age at death, and disease duration for patients with pathogenic mutations in the GRN and MAPT genes and pathological expansions in the C9orf72 gene through the Frontotem..

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