Filipe Brogueira Rodrigues1,2, Daisy Abreu1,2, Joana Damásio3, Nilza Goncalves1,2, Leonor Correia-Guedes1,2,4, Miguel Coelho1,2,4, Joaquim J Ferreira1,2,5. 1. Clinical Pharmacology Unit Instituto de Medicina Molecular Lisbon Portugal. 2. Laboratory of Clinical Pharmacology and Therapeutics Faculty of Medicine University of Lisbon Lisbon Portugal. 3. Neurology Department Hospital de Santo António-Centro Hospitalar do Porto Porto Portugal. 4. Department of Neurosciences Neurology Hospital de Santa Maria Centro Hospitalar de Lisboa Norte Lisbon Portugal. 5. Campus Neurológico Senior Torres Vedras Portugal.
Abstract
BACKGROUND: Huntington's disease (HD) is a rare and fatal inherited genetic disorder characterized by progressive motor, cognitive, and behavioral impairment. It leads to premature death, but data regarding advanced-stage disease are scarce. We sought to determine HD-associated survival, mortality, and causes and places of death. METHODS: Data from the European HD Network prospective study (REGISTRY) collected from 2001 through 2013 were used, including the Unified Huntington's Disease Rating Scale and death report forms. Group comparisons were performed using the t test or the χ2 test. Survival analyses were computed through Kaplan-Meier estimates of median survival. All tests were 2-sided with a significance level of P = 0.05. RESULTS: In total, 5164 participants were analyzed. The mean age at diagnosis was 49 years, and the mean age at death was 58 years. At the end of the study period, there were 533 deaths (10.3% of patients). Median survival was 24 years from diagnosis and 35 years from symptom onset. The most frequent causes of death were pneumonia (19.5%), other infections (6.9%), and suicide (6.6%). The most frequent places of death were the hospital (29.8%), the home (23.9%), and nursing houses (19.8%). CONCLUSIONS: Patients with HD tend to die from the same conditions as patients with other neurodegenerative diseases. However, compared with nonhereditary Parkinson's disease and Alzheimer's disease, the median time from onset to death is longer, and the places of death are distinctive.
BACKGROUND: Huntington's disease (HD) is a rare and fatal inherited genetic disorder characterized by progressive motor, cognitive, and behavioral impairment. It leads to premature death, but data regarding advanced-stage disease are scarce. We sought to determine HD-associated survival, mortality, and causes and places of death. METHODS: Data from the European HD Network prospective study (REGISTRY) collected from 2001 through 2013 were used, including the Unified Huntington's Disease Rating Scale and death report forms. Group comparisons were performed using the t test or the χ2 test. Survival analyses were computed through Kaplan-Meier estimates of median survival. All tests were 2-sided with a significance level of P = 0.05. RESULTS: In total, 5164 participants were analyzed. The mean age at diagnosis was 49 years, and the mean age at death was 58 years. At the end of the study period, there were 533 deaths (10.3% of patients). Median survival was 24 years from diagnosis and 35 years from symptom onset. The most frequent causes of death were pneumonia (19.5%), other infections (6.9%), and suicide (6.6%). The most frequent places of death were the hospital (29.8%), the home (23.9%), and nursing houses (19.8%). CONCLUSIONS: Patients with HD tend to die from the same conditions as patients with other neurodegenerative diseases. However, compared with nonhereditary Parkinson's disease and Alzheimer's disease, the median time from onset to death is longer, and the places of death are distinctive.
Entities:
Keywords:
Huntington's disease; cause of death; mortality; survival
Authors: Carlo Rinaldi; Elena Salvatore; Ilaria Giordano; Sara De Matteis; Tecla Tucci; Valeria Russo Cinzia; Fabiana Rossi; Imma Castaldo; Vincenzo Brescia Morra; Luigi Di Maio; Alessandro Filla; Giuseppe De Michele Journal: Can J Neurol Sci Date: 2012-01 Impact factor: 2.104
Authors: Guilherme Borges; Matthew K Nock; Josep M Haro Abad; Irving Hwang; Nancy A Sampson; Jordi Alonso; Laura Helena Andrade; Matthias C Angermeyer; Annette Beautrais; Evelyn Bromet; Ronny Bruffaerts; Giovanni de Girolamo; Silvia Florescu; Oye Gureje; Chiyi Hu; Elie G Karam; Viviane Kovess-Masfety; Sing Lee; Daphna Levinson; Maria Elena Medina-Mora; Johan Ormel; Jose Posada-Villa; Rajesh Sagar; Toma Tomov; Hidenori Uda; David R Williams; Ronald C Kessler Journal: J Clin Psychiatry Date: 2010-08-24 Impact factor: 4.384
Authors: Sarah J Tabrizi; Rachael I Scahill; Gail Owen; Alexandra Durr; Blair R Leavitt; Raymund A Roos; Beth Borowsky; Bernhard Landwehrmeyer; Chris Frost; Hans Johnson; David Craufurd; Ralf Reilmann; Julie C Stout; Douglas R Langbehn Journal: Lancet Neurol Date: 2013-05-09 Impact factor: 44.182
Authors: Michael Orth; Olivia J Handley; Carsten Schwenke; Stephen B Dunnett; David Craufurd; Aileen K Ho; Edward Wild; Sarah J Tabrizi; G Bernhard Landwehrmeyer Journal: PLoS Curr Date: 2010-09-28
Authors: Zhen-Yi Andy Ou; Lauren M Byrne; Filipe B Rodrigues; Rosanna Tortelli; Eileanoir B Johnson; Martha S Foiani; Marzena Arridge; Enrico De Vita; Rachael I Scahill; Amanda Heslegrave; Henrik Zetterberg; Edward J Wild Journal: Sci Rep Date: 2021-02-10 Impact factor: 4.379
Authors: Filipe B Rodrigues; Lauren M Byrne; Enrico De Vita; Eileanoir B Johnson; Nicola Z Hobbs; John S Thornton; Rachael I Scahill; Edward J Wild Journal: Eur J Neurosci Date: 2019-02-19 Impact factor: 3.386
Authors: Leonard L Sokol; Jonathan P Troost; Benzi M Kluger; Allison J Applebaum; Jane S Paulsen; Danny Bega; Samuel Frank; Joshua M Hauser; Nicholas R Boileau; Colin A Depp; David Cella; Noelle E Carlozzi Journal: Ann Clin Transl Neurol Date: 2021-07-20 Impact factor: 4.511