title: Predictors of survival in a Huntington's disease population from southern Italy.
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=0.002) 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.affiliation: Department of Neurological Sciences, Università Federico II, Naples, Italy. rinaldi.ca@gmail.com
authors.forename: Carlo
authors.initials: C
authors.lastname: Rinaldi
authors.forename: Elena
authors.initials: E
authors.lastname: Salvatore
authors.forename: Ilaria
authors.initials: I
authors.lastname: Giordano
authors.forename: Sara
authors.initials: S
authors.lastname: De Matteis
authors.forename: Tecla
authors.initials: T
authors.lastname: Tucci
authors.forename: Valeria Russo
authors.initials: VR
authors.lastname: Cinzia
authors.forename: Fabiana
authors.initials: F
authors.lastname: Rossi
authors.forename: Imma
authors.initials: I
authors.lastname: Castaldo
authors.forename: Vincenzo Brescia
authors.initials: VB
authors.lastname: Morra
authors.forename: Luigi
authors.initials: L
authors.lastname: Di Maio
authors.forename: Alessandro
authors.initials: A
authors.lastname: Filla
authors.forename: Giuseppe
authors.initials: G
authors.lastname: De Michele
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics / Q000401:mortality*; D007558:Italy / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D012044:Regression Analysis; D012189:Retrospective Studies; D016019:Survival Analysis; D019680:Trinucleotide Repeat Expansion / Q000235:genetics; D055815:Young Adult