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Amyotrophic lateral sclerosis in Sardinia, insular Italy, 1995–2009

Pugliatti, Maura and Parish, Leslie D. and Cossu, Paola and Leoni, Stefania and Ticca, Anna F. and Saddi, M. Valeria and Ortu, Enzo and Traccis, Sebastiano and Borghero, Giuseppe and Puddu, Roberta and Chiò, Adriano and Pirina, Pietro (2013) Amyotrophic lateral sclerosis in Sardinia, insular Italy, 1995–2009. Journal of neurology, Vol. 260 (2), p. 572-579. eISSN 1432-1459. Article.

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DOI: 10.1007/s00415-012-6681-5

Abstract

Recent genetic studies suggest a Sardinian type of amyotrophic lateral sclerosis (ALS). Thus, ALS incidence, prevalence and survival were investigated in a large population of Sardinians aimed to disclose populationspecific patterns and their temporal changes. This is a population-based incidence and prevalence study in northern and central Sardinia, insular Italy (over 700,000 population). Incidence rates were computed for the time interval 1995–2009 and by quinquennia. Prevalence was computed for prevalence days 31 December 2004 and 2009. Onset-based survival for 1995–2009 is also reported. All ALS patients (El Escorial Criteria) in the study area were retrospectively included. The ALS crude incidence from 2005–2009 was 2.5 (95 % CIs: 0.1, 4.9), 3.4 in men and 1.6 in women. Onset occurred most often between the age of 65–74 years in men and 55–64 years in women. The ALS incidence tended to increase over the period 1995–2009. The mean age at onset was 61.7 years with no difference based on gender, varying significantly from 59.9 years in 1995–1999 to 63.9 years in 2005–2009. On December 31, 2009, the ALS crude prevalence was 10.8 per 100,000 (95 % CIs: 8.6, 13.1), 13.8 in men and 8.0 in women, whereas it was 6.3 per 100,000 (95 % CIs: 4.1, 8.6) on December 31, 2004 (M:F ratio of 0.95). Mean survival from onset was 37.0 months, with no difference based on gender, and a tendency to decrease during the period 1995–2009, in relation to type and age of onset. The population-based incidence and prevalence data of ALS in Sardinians indicate an increase of the disease occurrence over the past 40 years, providing support for a populationspecific variant of ALS in Sardinia.

Item Type:Article
ID Code:8934
Status:Published
Refereed:Yes
Uncontrolled Keywords:Amyotrophic lateral sclerosis, motor neuron disease, incidence, prevalence, survival, Sardinia
Subjects:Area 06 - Scienze mediche > MED/10 Malattie dell'apparato respiratorio
Area 06 - Scienze mediche > MED/26 Neurologia
Divisions:002 Altri enti e centri di ricerca del Nord Sardegna > Azienda ASL3, Nuoro > Ospedale "San Francesco"
002 Altri enti e centri di ricerca del Nord Sardegna > Azienda ASL1, Sassari > Ospedale "A. Segni", Ozieri
001 Università di Sassari > 01-a Nuovi Dipartimenti dal 2012 > Medicina Clinica e Sperimentale
Publisher:Springer
eISSN:1432-1459
Deposited On:26 Apr 2013 09:29

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