Accuracy of death certificates for amyotrophic lateral sclerosis varies significantly from north to south of Italy: Implications for mortality studies
ABSTRACT To evaluate the accuracy of death certificates (DCs) for amyotrophic lateral sclerosis (ALS) in different parts of Italy. Studies based on DC diagnosis for ALS have shown a reduced mortality comparing northern with southern Italy. These data are in contrast with results from other surveys on the incidence of ALS performed in Italy and other countries.
Archives of neurological clinics from northern (Milano, Monza, Pavia, and Bologna) and southern Italy including islands (Napoli, Sassari, Palermo, and Messina) were searched for patients discharged with a diagnosis of ALS in the period 1970-1995. Subjects affected by definite/probable ALS according to the Scottish Motor Neuron Disease Research Group diagnostic criteria were included. DCs were obtained from the vital statistic bureau. True positive rates (TPRs) and 95% confidence intervals (CIs) for proportions were calculated for northern and southern Italy separately. Multiple logistic regression analysis was performed according to gender, age at onset, age and year of death, and interval between onset and death.
We found 651 patients affected by definite/probable ALS; 573 of them had died by December 31, 1996. DCs were available for 566 subjects (411 from northern Italy and 155 from southern Italy). TPR was 66.7% (95% CI 61.9-71.2) for northern Italy and 51.6% (95% CI 43.5-59.7) for southern Italy (chi(2) = 10.9, p = 0.001). Logistic regression analysis showed an association between a lower accuracy of DCs and the interval between onset of symptoms and death. TPR calculations considering different death periods (1970-1982 and 1983-1996) showed comparable rates of accuracy over time.
Mortality statistics based on official death records do not accurately reflect interregional mortality for ALS in Italy.
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ABSTRACT: Background and purposeThe main objective of establishing the French register of amyotrophic lateral sclerosis (ALS) in the Limousin region (FRALim), was to assess the incidence of ALS, in this ageing region of Europe, over a 12-year period (2000-2011). Methods Patients were included if they lived in Limousin at the time of diagnosis of ALS according to El Escorial revised criteria and were identified by at least one of the following sources: (i) the French national body coordinating ALS referral centres; (ii) public and private hospitals in the region; (iii) health insurance data related to long-term diseases. ResultsThe FRALim register identified 279 incident cases (2000-2011). The crude and European population standardized incidences of ALS were as high as 3.19/100000 person-years of follow-up (95% CI 2.81-3.56) and 2.58/100000 person-years of follow-up (95% CI 2.27-2.89) respectively. Median age at onset was 70.8years (interquartile range 63.1-77.1). The standardized sex incidence ratio (male/female) was 1.3 overall, but 1.1 under the age of 65years, 1.7 between 65 and 75years and 1.9 above 75years. The exhaustiveness of the register has been estimated at 98.4% (95% CI 95.6-99.4) by capture-recapture analysis. Conclusion It was possible for the first time in France to monitor accurately the incidence of ALS over a long time period. It appears to be in the upper range of data reported in western countries. Patterns displayed here might anticipate the epidemiology of ALS in ageing western countries.European Journal of Neurology 06/2014; 21(10). DOI:10.1111/ene.12474 · 3.85 Impact Factor
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ABSTRACT: Encephalitis produces considerable morbidity in the United States, but morbidity rates among American Indian/Alaska Native (AI/AN) people have not been described. Hospitalization records listing an encephalitis diagnosis were analyzed by using Indian Health Service direct/contract inpatient data. For 1998-2010, there were 436 encephalitis-associated hospitalizations among AI/AN people, an average annual age-adjusted hospitalization rate of 3.1/100,000 population. The rate for infants (11.9) was more than double that for any other age group. Death occurred for 4.1% of persons hospitalized. Consistent with reports for the general U.S. population, the rate was high among infants and most (53.9%) hospitalizations were of unexplained etiology. The average annual rate during the study period appeared lower than for the general U.S. population, due particularly to lower rates in the elderly. Future community-based surveillance and mortality studies are needed to confirm these findings and examine reasons underlying the low rates of encephalitis in AI/AN people.The American journal of tropical medicine and hygiene 02/2014; 90(4). DOI:10.4269/ajtmh.13-0420 · 2.74 Impact Factor
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ABSTRACT: Amyotrophic lateral sclerosis (ALS), first described by Jean-Martin Charcot in the 1870s, is an age-related disorder that leads to degeneration of motor neurons. The disease begins focally in the central nervous system and then spreads relentlessly. The clinical diagnosis, defined by progressive signs and symptoms of upper and lower motor neuron dysfunction, is confirmed by electromyography. Additional testing excludes other conditions. The disease is heterogeneous, but most patients die of respiratory muscle weakness less than 3 years from symptom-onset. Like other age-related neurodegenerative diseases, ALS has genetic and environmental triggers. Of the five to 10% of cases that are inherited, mutations have been discovered for a high proportion. In addition to genetic factors, age, tobacco use, and athleticism may contribute to sporadic ALS, but important etiologies are unidentified for most patients. Complex pathophysiological processes, including mitochondrial dysfunction, aggregation of misfolded protein, oxidative stress, excitotoxicity, inflammation and apoptosis, involve both motor neurons and surrounding glial cells. There is clinical and pathological overlap with other neurodegenerative diseases, particularly frontotemporal dementia. The mechanisms leading to disease propagation in the brain are a current focus of research. To date, one medication, riluzole, licensed in 1996, has been proved to prolong survival in ALS. Numerous clinical trials have so far been unable to identify another neuroprotective agent. Researchers now aim to slow disease progression by targeting known pathophysiological pathways or genetic defects. Current approaches are directed at muscle proteins such as Nogo, energetic balance, cell replacement, and abnormal gene products resulting from mutations. Until better understanding of the causes and mechanisms underlying progression lead to more robust neuroprotective agents, symptomatic therapies can extend life and improve quality of life. Palliative care programs such as hospice give emotional and physical support to patients and families throughout much of the disease course.01/2013; 4(5):295-310. DOI:10.14336/AD.2013.0400295