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Objective: To assess survival of ALS patients in general and in selected demographic and clinical subgroups comparing two periods (1998–2000 vs. 2008–2010). Methods: Newly diagnosed adults resident of Lombardy, Northern Italy from a population-based registry were included. Data were collected on age at diagnosis, sex, site of onset, diagnostic delay, and El-Escorial diagnostic category. Patients were followed until death or last observation. Survival was evaluated using Kaplan–Meier curves and Cox's proportional hazards models. Results: In 2008–2010 (267 patients), median survival was 2.4 years and 1-year, 2-year, 3-year and 5-year survival rates were 79%, 56%, 41% and 24%. Longer survival was associated with male sex, younger age, spinal onset, and longer diagnostic delay. Multivariable analysis confirmed higher death in 65–69yr (HR 2.8; 95% CI 1.4-5.6), 70–74yr (HR 3.2; 95% CI 1.6-6.3) and 75 + yr (HR 6.9; 95% CI 3.5-13.8) categories, compared to ≤49yr, in females (HR 1.4; 95% CI 1.02-1.8), compared to males, and in patients diagnosed after 6–12 months (HR 1.9; 95% CI 1.4-2.7), compared with longer diagnostic delay. In 1998–2000 (235 patients), median survival was 2.2 years. The 1-year, 2-year, 3-year and 5-year survival rates were 77%, 53%, 38% and 20%. When adjusting for demographic and clinical variables, the HR for death in 2008–2010 versus 1998–2000 was 0.80 (95% CI 0.66–0.98). A significant increase of survival in 2008–2010 was found only in patients aged 50–59yr and 70–74yr at diagnosis. Conclusions: Survival of ALS has increased over time in the last decades, especially in middle aged and elderly patients. The benefits of comprehensive care in selected age groups might explain our findings. [ABSTRACT FROM AUTHOR]

Objective: Data on and increase of the incidence of amyotrophic lateral sclerosis (ALS) are conflicting and reflect the use of differing populations and designs. We investigated the incidence of ALS using data from a large population-based registry (SLALOM) in two three-year periods using the same diagnostic criteria and equal methods of case ascertainment. Methods: The registry is based in Lombardy, a 10 million population area of Northern Italy. Using different sources of cases (hospital discharge and ambulatory records, regional claims and prescription records) from nine Lombardy provinces (population, 5,485,163), all patients with newly diagnosed ALS during the periods 1998–2000 and 2008–2010 were included. Results: A total of 235 and 440 newly diagnosed patients were traced during the two study periods. The corresponding incidence rates were, respectively, 1.61 and 2.72 per 100,000 person-years. Data varied with age, sex and province by a significant increase was found only in men aged 65 through 74 years. Conclusions: Based on our findings, the increased incidence of ALS with time is real and can be largely interpreted as a reflection of the advancing age of the general population. [ABSTRACT FROM AUTHOR]

The purpose of the study was to assess frequency and predictors of disability measures in ALS. One hundred and fourteen newly diagnosed patients resident in eight administrative districts of Lombardy, Italy (population 4,947,554), included in a population-based registry, were followed for 2570 person-months (mean 22.5 months). The cumulative time-dependent risk of wheelchair, percutaneous endoscopic gastrostomy, and assisted ventilation was estimated according to the Kaplan-Meier method. Predictors of disability (age, sex, disease duration at diagnosis, type of onset, El-Escorial diagnosis) were assessed with the Cox proportional hazard function. During follow-up, 29 patients (25.4%) became wheelchair bound, 51 (44.7%) received gastrostomy, and 47 (41.2%) received assisted ventilation. The median time to loss of ambulation was 46.7 months (95% CI 36.5-56.8). The median time to gastrostomy and assisted ventilation was 31.1 months (95% CI 26.8-35.4) and 34.6 months (95% CI 29.6-39.6), respectively. Spinal onset ALS was the only predictor of loss of ambulation. Predictors of gastrostomy were older age, definite ALS, and shorter disease duration. Shorter disease duration was the only predictor of assisted ventilation. In conclusion, patients with ALS differ in terms of measures and predictors of disability. These factors are sources of bias and confounding in randomized clinical trials. [ABSTRACT FROM AUTHOR]

INTRODUCTION: As amyotrophic lateral sclerosis (ALS) is a rare neurological disorder, many sources must be investigated for complete case ascertainment. Hospital discharge diagnoses (HDDs) are a source of administrative data for patients hospitalized for a given disease. The accuracy of HDDs was tested, to assess whether they could be used to trace patients with amyotrophic lateral sclerosis (ALS). METHODS: All the HDDs referring to patients with motor neuron disease (MND) (ICD-9 code 335.2) were identified from primary hospital discharge diagnoses made during the years 1994-95 in six target provinces of the Lombardy region in Northern Italy (total population 2 922 920). The medical records of the patients were reviewed and each patient was then accepted or rejected according to whether he/she did or did not satisfy the El Escorial criteria for the diagnosis of ALS. The ALS discharge diagnoses filed in the study period in the neurology departments of all the regional hospitals were the 'gold standard'. Newly diagnosed patients registered during the year 1998 in the same provinces served for the calculation of the incidence of the disease. Sensitivity, specificity and positive predictive value were calculated. Data were presented separately for all the patients and those who were indicated as first hospital admissions. RESULTS: During the study period a total of 1 012 122 hospital discharge records were coded in the six index provinces. The ICD-9 code 335.2 was reported in 408 of these (0.04%). These discharge diagnoses were made in 267 patients, 245 of whom were resident in Lombardy at the time of hospital admission. The hospital records of 237 cases were examined. In 95 of these the medical records showed a diagnosis other than ALS. Other motor neuron diseases were listed for 50 patients. Thirteen additional cases were traced through neurology department files. The sensitivity of the HDDs was 91.6%, the specificity 99.9%, the positive predictive value 65.4%. The corresponding values for first-hospitalized patients were 97.7, 99.9, and 37.3. The overall ALS incidence rate obtained from the HDDs was 2.1 per 100 000 (95% CI 1.3-3.3). The corresponding rate calculated after exclusion of the false positives and inclusion of the false negatives was 0.8 (95% CI 0.1-2.1) and that obtained using the 1998 cases registered in the same areas was 1.6 (95% CI 0.7-2.7). CONCLUSIONS: The incidence of ALS is overestimated when HDDs are used for the estimate. Nevertheless, with improved quality, administrative data could be useful for the estimate of prevalence and incidence. [ABSTRACT FROM AUTHOR]

Objectives: To assess the association between amyotrophic lateral sclerosis (ALS) and previous traumatic events, age of trauma, and site of injury., Methods: A population-based case-control study was performed in five European countries (Italy, Ireland, France, United Kingdom, Serbia). Newly diagnosed ALS patients and matched controls were interviewed to collect relevant demographic factors and exposures. Key clinical features at diagnosis were collected in ALS patients. Trauma was any accidental event causing an injury. Injuries were dated and classified according to cause, severity, type, site, and complications. All exposures were censored five years before symptoms onset. Risks were computed as odds ratios (OR) with 95% confidence intervals (CI) using univariate and multivariate conditional logistic regression models., Results: Five hundred and seventy-five ALS patients and 1150 controls were interviewed. Disabling traumatic events predominated in the cases (OR 1.54 (95% CI 1.24-1.92)) and maintained significance after adjustment, with a significant gradient. A history of 2 + head injuries was associated with an almost three-fold increased risk of ALS. The risk was almost two-fold when trauma occurred at age 35-54 years. Site of injury was uneventful., Conclusions: Traumatic events leading to functional disability or confined to the head are risk factors for ALS. Traumatic events experienced at age 35-54 years carry the highest risk.