Epidemiology Of Amyotrophic Lateral Sclerosis Research Articles

Prognosis and epidemiology of amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis is a disease with highly variable clinical features and prognosis. We analyzed the prognostic indicators of age, sex, bulbar or spinal onset, body mass index (BMI), and forced vital capacity (FVC) for 728 deceased patients from the Emory ALS Clinic. The median overall survival was 29.8 months from symptom onset, 15.8 months from diagnosis, and 14.3 months from the initial clinic visit. While univariate analyses revealed that each of the identified clinical features was strongly associated with patient survival, in multivariable analyses only age, BMI, and FVC measured at the first clinic visit were independent prognostic indicators; bulbar onset and sex were not significantly associated with survival prognosis after adjustment for the other clinical features.

Amyotrophic lateral sclerosis/motor neuron disease deaths in the United States, 1999–2009

Our objective was to examine trends and epidemiology of amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND) associated deaths in the United States. ALS/MND associated death rates and trends in the United States for 1999–2009 were examined using the multiple cause-of-death mortality data. Age-specific and age-adjusted death rates were calculated. For 1999–2009, the average annual age-adjusted death rate was 2.17/100,000 persons. The age-specific rate increased with age until 75–79 years. Males experienced a higher death rate than females. There was no definitive trend in the annual ALS/MND associated death rate, although analyses suggested a possible decrease (p = 0.05); however, the rate increased for persons 20–49 years of age and declined for persons ≥ 65 years of age. The annual rate for males decreased whereas the rate for females showed no change. In conclusion, the suggested decreasing annual ALS/MND associated death rate for 1999–2009 contrasts with earlier reports indicating that the incidence and death rate of ALS were increasing. While the ALS/MND associated death rate slightly increased among adults 20–49 years of age, rates declined among two subpopulations at higher risk for ALS/MND – males and persons ≥ 65 years of age. Continued monitoring of ALS/MND mortality data and additional epidemiological studies will be important to further elucidate these epidemiological trends.

Population based epidemiology of amyotrophic lateral sclerosis using capture–recapture methodology

BackgroundVariation in the incidence rate in epidemiological studies on amyotrophic lateral sclerosis (ALS) may be due to a small population size and under ascertainment of patients. The previously reported incidence...

Epidemiology of amyotrophic lateral sclerosis in Isfahan, Iran

Few studies are carried out on the epidemiology of amyotrophic lateral sclerosis (ALS) in Middle East with no reports from Iran. To determine the epidemiological and clinical features of ALS amongst the Iranian population living in Isfahan, Iran. Medical records of all hospitals with a neurology department and outpatient neurology clinics in Isfahan province from 2002 to 2006 were reviewed, and all patients with the diagnosis of ALS according to El Escorial diagnostic criteria were extracted and related demographic and clinical data were gathered and analyzed. We found 98 new patients (66 men and 32 women) with definite, probable, or possible ALS. The average annual incidence was 0.42/100,000, with the highest incidence rate amongst those aged 70-74. On 21 March 2006, the crude prevalence was 1.57/100,000. Median survival from onset was 48 months (95% confidence interval 34-61) and survival rates for 1, 3,and 5 years after the onset were 94%, 66%, and 32%, respectively. The incidence and prevalence of ALS in the Iranian population seems to be lower compared to other populations and the survival of patients was longer than previously reported.

The epidemiology of amyotrophic lateral sclerosis in Reggio Emilia, Italy

Incidence and mortality rates of amyotrophic lateral sclerosis (ALS) vary between countries, and in some studies appear to increase over time. We performed a study to assess ALS incidence in a northern Italy area over a 10-year period. We identified the new cases of probable or definite ALS diagnosed among residents in Reggio Emilia province between 1996 and 2005 using several sources of data, such as death certificates, clinical records, hospital discharge registers and drug prescriptions. A total of 94 newly-diagnosed patients were identified. The average standardized incidence in the period was 2.0 and 1.0 cases/100,000/year, using the Italian and the world population, respectively, as reference. There was no variation in rates over time. Incidence was 1.3 in males and 0.8 in females. No cases were observed in patients under 35 years of age. Incidence increased after the age of 55 years, reaching a peak in the group aged 70-74 years and declining thereafter. We concluded that ALS incidence in this population was similar to that observed in other Italian regions and European countries, and no variation was identified during the study period.

Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues

Amyotrophic lateral sclerosis (ALS) is a relatively rare disease with a reported population incidence of between 1.5 and 2.5 per 100 000 per year. Over the past 10 years, the...

Descriptive Epidemiology of Amyotrophic Lateral Sclerosis in Japan, 1995-2001

BACKGROUND: This study was conducted to describe the epidemiologic features of amyotrophic lateral sclerosis (ALS) in Japan by examining annual trends in mortality (1995-2001), and to discuss the background factors possibly responsible for the recent variations in the mortality rate.METHODS: Trends in both the age-adjusted and age-specific mortality rates of ALS were examined by using the data obtained from the vital statistics of Japan between 1995 and 2001.RESULTS: There were small increases in the number of ALS deaths (from 1249 to 1400 per year) and the crude mortality rates (from 1.00 to 1.10 per 100,000 population) between 1995 and 2001. The age-adjusted mortality rate of ALS (adjusted using the 1985 model population of Japan) has decreased (from 0.84 per 100,000 population in 1995 to 0.74 in 2001). Age-specific mortality rates have been increasing particularly in the population older than 70 years of age, with the peak in mortality in the 70- to 80-year old age group.CONCLUSIONS: ALS mortality rates increased proportionally more for elderly population during the study period. Further epidemiologic studies will be needed to clarify the possible background factors contributing to the increase in ALS mortality in the elderly.

Epidemiology of amyotrophic lateral sclerosis in Hordaland county, western Norway

The incidence, prevalence and prognosis of amyotrophic lateral sclerosis (ALS) in the county of Hordaland, western Norway were determined for the years 1978 through 1988. The average annual incidence rate was 1.60 per 100,000 population with a male to female ratio of 1.26 (95% confidence interval: 0.76-2.09). The maximal age-specific annual incidence was 8.12 per 100,000 and occurred in the age-group between 61 and 65 years. The prevalence of ALS was 3.67 per 100,000 on December 31, 1988. The average age at the onset of the disease was 60.9 years ranging from 34 to 82 years of age. Survival was studied with life table techniques. Median survival from the onset of symptoms was 28.0 months overall. In patients with bulbar onset the median survival was 24.0 months whereas it was 40 months in patients with spinal onset of disease (log rank test, P = 0.0004). The difference in survival between ALS with bulbar or spinal onset was not explained by age or sex differences in the two groups.

Epidemiology of amyotrophic lateral sclerosis in the province of Modena, Italy. Influence of environmental exposure to lead

A retrospective study of all admissions to the University of Modena Neurological Department from 1976-1986; 51 cases of amyotrophic lateral sclerosis (ALS) were found. ALS mean annual incidence was 0.78/100,000 inhabitants, while prevalence was 2.35/100,000. Mean age at onset was 61.43 years, mean and median duration of illness were, respectively, 28.83 and 24.5 months, and survival at 5 years post-onset was 24.4%. A tendency to higher incidence and prevalence of ALS in the ceramic district, when compared with those of the rest of the province was found. However, the small number of cases did not allow any conclusive statistical correlation between environmental exposure to lead and frequency of ALS.

Amyotrophic lateral sclerosis in Finland.

The epidemiology of amyotrophic lateral sclerosis (ALS) has been studied in Finland since 1963. The country is suitable for epidemiological investigations because of its stable population structure and accurate population registers.

Epidemiology of amyotrophic lateral sclerosis: 1. A case-control comparison based on ALS deaths.

Deaths coded to amyotrophic lateral sclerosis (ALS) for the years 1963 to 1967 among U.S. males were matched against military files to identify 504 World War II veterans. Military controls for these 504 men were matched for year of birth, date of entry into military service, and branch of service. Hospital records of 37 representative deaths coded as ALS among veterans were reviewed, and 36 were accepted as definite ALS. Military records were abstracted for information about preservice demographic and other factors, the physical examination for entry into service, and the medical history and factors characterizing the military career during World War II. There was a deficit of blacks with ALS, which did not attain statistical significance ( p = 0.08). Several differences in preservice occupations were found: Truck drivers and “operatives” were in excess. There was no significant difference between ALS cases and controls in aggregate scores on sports activity before service, although baseball was more often listed by the ALS cases than by controls. The ALS patients significantly more often gave histories of trauma or surgical operations before service, and during service had a significant excess of hospitalizations for trauma and in particular for fractures of the limbs.

The epidemiology of amyotrophic lateral sclerosis in Finland: A study based on the death certificates of 421 patients

The mortality and prevalence of ALS in the various counties of Finland was studied. The work was based on death certificates derived from a 10-year period from 1963 to 1972, and altogether 421 cases were found. The mean duration of the disease was 2.7 years, and the mean age at death was 61.2 years. The average annual mortality rate was 0.91 per 100,000. The male to female ratio was 0.87 to 1, males outnumbered females only in age groups under 65. Some clustering of the cases seems to be taking place in the south-eastern part of the country. The rural to urban distribution of the patients' places of birth and domiciles did not differ markedly from that of the whole population. After ALS itself pneumonia was the most common direct cause of death. Of other significant conditions coded in the death certificates schizophrenia and cancer did not occur more often than could be expected by chance. No evidence of inheritance of the disease was found.

The distribution of amyotrophic lateral sclerosis in Finland

The epidemiology of amyotrophic lateral sclerosis in Finland was investigated. The distribution of the disease was calculated according to the places of birth and the present domiciles of 82 living cases. The mean frequency was 10.3 cases per 10,000 births, and the mean prevalence was 1.74 cases per 100,000 inhabitants. Both the highest frequencies, from 23.7 to 11.0, and the highest prevalences, from 7.0 to 3.2, were found in the south-eastern parts of the country. Comparable figures for multiple sclerosis were low in the same areas.