BACKGROUND: The primary aim of the present study was to determine the survival rates and identify predictors of disease duration in a cohort of Huntington's disease (HD) patients from Southern Italy. METHODS: All medical records of HD patients followed between 1977 and 2008 at the Department of Neurological Sciences of Federico II University in Naples were retrospectively reviewed and 135 patients were enrolled in the analysis. At the time of data collection, 41 patients were deceased (19 males and 22 females) with a mean ± SD age at death of 56.6 ± 14.9 years (range 18-83). RESULTS: The median survival time was 20 years (95% CI: 18.3-21.7). Cox regression analysis showed that the number of CAG in the expanded allele (HR 1.09 for 1 point triplet increase, p=0.002) and age of onset (HR 1.05 for 1 point year increase, p=0.002) were independent and significant predictors of lower survival rates. CONCLUSIONS: We believe that these findings are important for a better understanding of the natural history of the disease and may be relevant in designing future therapeutic trials.
BACKGROUND: The primary aim of the present study was to determine the survival rates and identify predictors of disease duration in a cohort of Huntington's disease (HD) patients from Southern Italy. METHODS: All medical records of HDpatients followed between 1977 and 2008 at the Department of Neurological Sciences of Federico II University in Naples were retrospectively reviewed and 135 patients were enrolled in the analysis. At the time of data collection, 41 patients were deceased (19 males and 22 females) with a mean ± SD age at death of 56.6 ± 14.9 years (range 18-83). RESULTS: The median survival time was 20 years (95% CI: 18.3-21.7). Cox regression analysis showed that the number of CAG in the expanded allele (HR 1.09 for 1 point triplet increase, p=0.002) and age of onset (HR 1.05 for 1 point year increase, p=0.002) were independent and significant predictors of lower survival rates. CONCLUSIONS: We believe that these findings are important for a better understanding of the natural history of the disease and may be relevant in designing future therapeutic trials.
Authors: Björn Oskarsson; Victoria Wheelock; Michael Benatar; J Paul Taylor; Nanette Joyce; David Chesak; Lee-Way Jin Journal: Amyotroph Lateral Scler Frontotemporal Degener Date: 2014-09-10 Impact factor: 4.092
Authors: Branduff McAllister; James F Gusella; G Bernhard Landwehrmeyer; Jong-Min Lee; Marcy E MacDonald; Michael Orth; Anne E Rosser; Nigel M Williams; Peter Holmans; Lesley Jones; Thomas H Massey Journal: Neurology Date: 2021-03-25 Impact factor: 11.800
Authors: Jesús de Pedro-Cuesta; Alberto Rábano; Pablo Martínez-Martín; María Ruiz-Tovar; Enrique Alcalde-Cabero; Javier Almazán-Isla; Fuencisla Avellanal; Miguel Calero Journal: PLoS One Date: 2015-09-03 Impact factor: 3.240