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.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).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.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.
Predictors of survival in a Huntington's disease population from southern Italy / C., Rinaldi; Salvatore, Elena; I., Giordano; S. D., Matteis; T., Tucci; V. R., Cinzia; F., Rossi; Castaldo, Imma; BRESCIA MORRA, Vincenzo; L. D., Maio; Filla, Alessandro; DE MICHELE, Giuseppe. - In: CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES. - ISSN 0317-1671. - 39:(2012), pp. 48-51.
Predictors of survival in a Huntington's disease population from southern Italy.
SALVATORE, ELENA;CASTALDO, IMMA;BRESCIA MORRA, VINCENZO;FILLA, ALESSANDRO;DE MICHELE, GIUSEPPE
2012
Abstract
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.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).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.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.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
