OBJECTIVE: To evaluate the survival of patients with amyotrophic lateral sclerosis (ALS) in an Italian population and to assess the effects of selected prognostic indicators on survival. BACKGROUND: Median survival of ALS patients has been reported to range between 12 and 23 months from diagnosis and between 23 and 36 months from onset of symptoms. Although several negative prognostic factors have been identified, the overall picture still needs clarification. METHODS: We included patients enrolled in an Italian ALS Regional Register (population 4,529,003) during the calendar year 1998. The diagnosis was confirmed by an ad hoc committee using the original El Escorial criteria. Each case was regularly followed up until death or December 31, 2002, whichever came first. Survival was assessed with the Kaplan-Meier method in the whole sample, by level of diagnostic certainty, and by selected prognostic indicators (age, sex, bulbar or spinal onset, and disease duration). Multivariate analysis was done with the Cox proportional hazard function. RESULTS: The sample comprised 79 patients (33 female; 46 male) aged 28-85 years (mean age 64.4 years). Onset of symptoms was bulbar in 30% of cases. Mean symptom duration at diagnosis was 13.3 months. ALS was definite in 43%, probable in 29%, possible in 6%, and suspected in 22%. By December 31, 2002, 56 cases (71%) had died. The cumulative probability of surviving after diagnosis was 78% at 12 months, 56% at 24 months, and 32% at 48 months. Median survival from onset was 39.2 months and from diagnosis 30.6 months. Multivariate analysis confirmed definite ALS at diagnosis and older age as adverse prognostic factors. CONCLUSIONS: Survival of ALS patients in the present sample was slightly longer than previously reported. Better palliative care and supportive treatment may explain the difference. Older age and the presence of definite ALS at diagnosis are poor prognostic predictors. Copyright 2005 S. Karger AG, Basel.
OBJECTIVE: To evaluate the survival of patients with amyotrophic lateral sclerosis (ALS) in an Italian population and to assess the effects of selected prognostic indicators on survival. BACKGROUND: Median survival of ALSpatients has been reported to range between 12 and 23 months from diagnosis and between 23 and 36 months from onset of symptoms. Although several negative prognostic factors have been identified, the overall picture still needs clarification. METHODS: We included patients enrolled in an Italian ALS Regional Register (population 4,529,003) during the calendar year 1998. The diagnosis was confirmed by an ad hoc committee using the original El Escorial criteria. Each case was regularly followed up until death or December 31, 2002, whichever came first. Survival was assessed with the Kaplan-Meier method in the whole sample, by level of diagnostic certainty, and by selected prognostic indicators (age, sex, bulbar or spinal onset, and disease duration). Multivariate analysis was done with the Cox proportional hazard function. RESULTS: The sample comprised 79 patients (33 female; 46 male) aged 28-85 years (mean age 64.4 years). Onset of symptoms was bulbar in 30% of cases. Mean symptom duration at diagnosis was 13.3 months. ALS was definite in 43%, probable in 29%, possible in 6%, and suspected in 22%. By December 31, 2002, 56 cases (71%) had died. The cumulative probability of surviving after diagnosis was 78% at 12 months, 56% at 24 months, and 32% at 48 months. Median survival from onset was 39.2 months and from diagnosis 30.6 months. Multivariate analysis confirmed definite ALS at diagnosis and older age as adverse prognostic factors. CONCLUSIONS: Survival of ALSpatients in the present sample was slightly longer than previously reported. Better palliative care and supportive treatment may explain the difference. Older age and the presence of definite ALS at diagnosis are poor prognostic predictors. Copyright 2005 S. Karger AG, Basel.
Authors: F Agosta; A Chiò; M Cosottini; N De Stefano; A Falini; M Mascalchi; M A Rocca; V Silani; G Tedeschi; M Filippi Journal: AJNR Am J Neuroradiol Date: 2010-04-01 Impact factor: 3.825
Authors: Terrell E Brotherton; Yingjie Li; Deborah Cooper; Marla Gearing; Jean-Pierre Julien; Jeffrey D Rothstein; Kevin Boylan; Jonathan D Glass Journal: Proc Natl Acad Sci U S A Date: 2012-03-19 Impact factor: 11.205
Authors: Bryan J Traynor; Michael Nalls; Shiao-Lin Lai; Raphael J Gibbs; Jennifer C Schymick; Sampath Arepalli; Dena Hernandez; Marcel P van der Brug; Janel O Johnson; Allissa Dillman; Mark Cookson; Cristina Moglia; Andrea Calvo; Gabriella Restagno; Gabriele Mora; Adriano Chiò Journal: Proc Natl Acad Sci U S A Date: 2010-06-21 Impact factor: 11.205