Predictors of long survival in amyotrophic lateral sclerosis: a population-based study.

BACKGROUND: Although amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder, some ALS cases can survive beyond 10 years. However, the predictors of long survival in ALS patients remain uncertain. OBJECTIVE: To define clinical predictors of long survival in a cohort of ALS incident cases. METHODS: One hundred-thirty incidents cases, diagnosed in 1998--1999 and classified according to the El Escorial criteria (EEC), were enrolled from a prospective population-based registry established in Puglia, Italy. All but two cases were followed-up until death or November 30, 2006. RESULTS: Thirteen patients (high 10% of the survivors) were classified as long survivors (LS), 13 as short survivors (SS) (low 10%), and 102 as average survivors (AS). LS presented a lower frequency of bulbar onset (8% versus 29% of AS and 39% of SS; p=0.1) and a significantly longer time between symptom onset to diagnosis [(ODI): 13 months versus 10 and 6; p=0.0005]. In multivariate analysis, predictors of long survival were younger age at diagnosis (>65 compared to < or =45 years: odds ratio (OR):18.9; 95%CI: 1.8-194.7; p=0.04), longer interval onset-diagnosis (< or =9 months compared to >9 months, OR: 7.9; 95%CI: 1.3-47; p=0.02) and clinical features with predominant upper motor neuron signs (OR: 8.5; 95%CI: 1.1-64.2; p=0.04). CONCLUSIONS: In this population-based study, younger age, longer interval onset to diagnosis, and clinical features with predominance of upper motor signs predicted long survival, while EEC category at diagnosis did not.

ALS multidisciplinary clinic and survival. Results from a population-based study in Southern Italy.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting motorneurons, for which there is no effective cure. Because of the multifactorial nature of impairment and disablity in ALS, multidisciplinary clinics (MDC) have been recently introduced in the management of ALS patients; their effects on survival remain, however, largely debated. OBJECTIVE: To compare survival of ALS patients who received their care at MDC with that of patients followed by general neurology clinics. METHODS: Source of the study was a prospective population-based registry of ALS established in Puglia, Southern Italy, in 1997. We examined survival of 126 out of 130 incident ALS cases that were diagnosed during the period 1998-99. RESULTS: 84 patients (67%) were enrolled and followed by MDC and the remaining 42 (33%) by general neurological clinics. No difference in median survival time from the diagnosis was observed between patients followed by ALS multidisciplinary (17.6 months) and general clinics (18 months). No beneficial effect was present among bulbar onset ALS (11.7 versus 23 months). In multivariate analysis management by ALS MDC was associated with only a 10% increase in survival probability at 12 months (HR: 0.91; 95%CI: 0.44-1.89; p = 0.9). CONCLUSIONS: In this population-based series, we found that in Southern Italy management of ALS by multidisciplinary clinics does not improve survival, regardless of site of symptoms onset.

Predictors of delay in the diagnosis and clinical trial entry of amyotrophic lateral sclerosis patients: a population-based study.

BACKGROUND: The El Escorial and the revised Airlie House diagnostic criteria for amyotrophic lateral sclerosis (ALS) were introduced to select patients for clinical trials. Heterogeneity of clinical presentation at onset and delay in diagnosis may decrease the likelihood for trial entry. OBJECTIVE: Identify risk factors for delay in the diagnosis and trial exclusion. METHODS: ALS incident cases were identified with El Escorial (EEC) and Airlie House criteria (AHC) through a population-based registry established in Puglia, Southern Italy, in the years 1998-99. RESULTS: 130 ALS incident cases were diagnosed with a median interval between onset of symptoms and diagnosis of 9.3 months and not different across both EEC and AHC categories. Twenty percent of cases were not eligible for clinical trials according to the AHC. About 5% of subjects in this series died with only lower motor neuron signs. Predictors for delay in the diagnosis were age between 65 and 75 years and spinal onset while fasciculations and cramps as first symptoms were predictors of exclusion from trials. CONCLUSIONS: In this population-based series, diagnostic delay was longer in subjects with spinal onset and age between 65 and 75 and fasciculation as first symptoms. About 80% of incident cases were trial eligible with AHC criteria. However, a significant number of subjects with ALS, characterized by a limited spread of signs, were not trial eligible while alive.