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Predictors of Survival in a Huntington's Disease Population from Southern Italy

Published online by Cambridge University Press:  02 December 2014

Carlo Rinaldi
Affiliation:
Department of Neurological Sciences, IEOS CNR, Università Federico II, Naples
Elena Salvatore
Affiliation:
Department of Neurological Sciences, IEOS CNR, Università Federico II, Naples
Ilaria Giordano
Affiliation:
Department of Neurological Sciences, IEOS CNR, Università Federico II, Naples
Sara De Matteis
Affiliation:
Department of Occupational and Environmental Health, Università degli Studi di Milano
Tecla Tucci
Affiliation:
Department of Neurological Sciences, IEOS CNR, Università Federico II, Naples
Valeria Russo Cinzia
Affiliation:
Department of Neurological Sciences, IEOS CNR, Università Federico II, Naples
Fabiana Rossi
Affiliation:
Department of Neurological Sciences, IEOS CNR, Università Federico II, Naples
Imma Castaldo
Affiliation:
Department of Cellular and Molecular Biology, IEOS CNR, Università Federico II, Naples
Vincenzo Brescia Morra
Affiliation:
Department of Neurological Sciences, IEOS CNR, Università Federico II, Naples
Luigi Di Maio
Affiliation:
Department of Neurological Sciences, IEOS CNR, Università Federico II, Naples
Alessandro Filla
Affiliation:
Department of Neurological Sciences, IEOS CNR, Università Federico II, Naples
Giuseppe De Michele
Affiliation:
Department of Neurological Sciences, IEOS CNR, Università Federico II, Naples
Corresponding
E-mail address:
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Abstract

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=0.022) 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.

Type
Research Article
Copyright
Copyright © The Canadian Journal of Neurological 2012

References

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