The incidence of amyotrophic lateral sclerosis in southwestern Ontario, Canada

The incidence, prevalence, and mortality rates of amyotrophic lateral sclerosis in southwestern Ontario, Canada, were determined for the years 1978 through 1982. The average annual incidence rate was 1.63 per 100,000 population. The prevalence rate was 4.9 per 100,000 on January 1, 1983. The average annual mortality rate was 1.52 per 100,000 population and increased with age, reaching a peak in the 70 to 79-year decade in both sexes. The male:female ratio was 1.2:1. The average age at diagnosis was 62.5 years, and the average duration from date of diagnosis was 2.5 years. The distribution of the disease was uneven in 12 counties, with the highest incidence in Perth (3.33) and Essex (2.43) counties. The city of Windsor, in an industrial region, had a high average annual incidence rate of 2.81.     

The trends in incidence of primary brain tumors in the population of rochester,minnesota

A number of reports have suggested an increasing incidence of primary brain tumors, especially malignant astrocytomas, in the elderly population. To investigate this issue, we analyzed the incidence and temporal trends of primary intracranial neoplasms diagnosed in the population of Rochester, Minnesota, over the 40 years between 1950 and 1990. The incidence of symptomatic primary brain tumors (excluding patients diagnosed incidentally at autopsy and by neuroimaging studies) increased from 9.5 per 100,000 population per year in 1950 to 1969 to 12.5 per 100,000 per year in 1970 to 1989; this change was not statistically significant (χ² trend, 1.89; p = 0.17). While the incidence of pituitary adenomas increased significantly between the two periods (χ² trend, 4.44;p = 0.04), the incidence trends of all gliomas, malignant astrocytomas, and meningiomas showed no change among persons younger than 65 years as well as those 65 years and older. The number of patients incidentally found to have neoplasms by neuroimaging studies increased in the recent 20-year period (χ² trend, 4.08;p = 0.04). The average age- and sex-adjusted incidence rates per 100,000 per year during the study period in the population of Roshester, Minnesota, for symptomatic tumors were 5.0 for all gliomas, 3.3 for malignant astrocytomas, 2.0 for meningiomas, and 2.4 for pituitary adenomas. In conclusion, our data indicate that the reported increase in the incidence of primary brain tumors is an artifact of improvement in diagnostic technology and practice.     

Trends in head injury incidence in New Zealand: a hospital-based study from 1997/1998 to 2003/2004

Traumatic brain injury (TBI) is a leading cause of disability and death in young adults. Globally, the incidence of TBI hospitalizations is estimated at 200-300 people per 100,000 annually. Using a national health database, we examined the incidence of TBI-related hospital discharges (including 1-day stays) to New Zealand Hospitals from 1997/1998 to 2003/2004. Crude annual hospital-based incidence rates for the total population ranged from 226.9 per 100,000 in 1998/1999 to a high rate of 349.2 in 2002/2003. There was a noticeable increase in rates with the change from ICD-9 to ICD-10 diagnostic codes and there was also disparity in incidence rates according to ethnicity, age and gender. Crude annual hospital-based incidence rates for males and females in Maori (689/100,000 and 302.8/100,000 person-years) and Pacific Island populations (582.6/100,000 and 217.6/100,000 person-years) were much higher than those for the remaining population (435.4/100,000 and 200.9/100,000 person-years), particularly for males. The overall age-standardized hospital- based incidence rate for 2003/2004 was 342 per 100,000 per year (95% CI = 337-349/100,000), and 458 per 100,000 per year for Maori (95% CI = 438-479/100,000) with Maori males experiencing a peak in incidence between 30 and 34 years of age that was not evidenced for the wider population. Standardized hospital-based incidence rates for the total population and for Maori by age, gender and ICD-10 diagnostic codes are also examined.     

Epidemiology of primary intracranial neoplasms in Manitoba, Canada

The incidence of primary intracranial tumors in Manitoba, Canada was reviewed. From 1980 through 1985, 657 tumors were diagnosed. The crude incidence rates were 10.2/100,000 for males and 10.8/100,000 for females. The three most common tumors were: astrocytoma 281 (43%), meningioma 145 (22%), and pituitary adenoma 111 (17%). Average annual incidence rates for all tumors showed a bimodal distribution with one peak in the 0-4 age group (4.2/100,000), and the other in the 60-69 age group (27.2/100,000). For malignant astrocytoma, the age-specific annual incidence rate increased to the seventh decade where it reached a peak of 14.3/100,000. The incidence of benign astrocytoma remained relatively constant with age at 1.1/100,000. The annual incidence of meningioma increased with age up to the eighth decade reaching 7.2/100,000. Of the 145 meningiomas, 56 (39%) were meningotheliomatous, 48 (33%) transitional, 10 (7%) malignant, 7 (5%) fibroblastic, 6 (4%) psammomatous, 3 (2%) angioblastic, and 15 (10%) lacked pathologic diagnosis. The annual incidence of pituitary adenoma showed two peaks, the first occurring in the third decade (2.6/100,000) and the second in the eighth decade (3.2/100,000). Although the incidence of meningioma was relatively high, the clinical features and pathologic patterns of these tumors were not unlike those previously reported in the literature.     

Distribution of epidermal growth factor receptor gene amplification in brain tumours and correlation to prognosis

In 75 gliomas and 31 meningiomas, mutations at the epidermal growth factor receptor (EGFR) gene locus were restricted to gliomas. The ligands of this receptor, epidermal growth factor and transforming growth factor alpha, lacked quantitative changes at their loci in gliomas and meningiomas. EGFR gene amplification occurred in astrocytomas, oligodendrogliomas, ependymomas and glioblastomas. The frequency of this mutation significantly increased with the malignancy grade and the patient's age. Especially in glioblastomas of individuals aged over 64 years, EGFR gene mutations were observed without chromosome-10-specific allele losses. This finding contradicts the hypothesis that deletion of one entire chromosome 10 regularly precedes EGFR gene amplification in primary glioblastomas of patients aged over 50 years. It was found that most individuals whose gliomas carry an EGFR gene mutation have a poor prognosis, comparable to that of glioblastoma patients even when the tumour is graded as benign.     

Prevalence of Parkinson's disease in Estonia

OBJECTIVES: To investigate the prevalence of idiopathic Parkinson's disease (PD) in Tartu district of South Estonia, with a population of 153,240 on prevalence day, 1 January 1996. METHODS: The community-based method of case ascertainment was used, followed by neurologic examination. RESULTS: The age-adjusted prevalence was 152 per 100,000 population, 159 for urban and 139 for the rural group, 154 for men and 153 for women. The age-specific prevalence increased from 22 per 100,000 population in the age group 40-49 years up to 1232 per 100,000 population in the age group 70-79 years. The mean age of PD patients was 71.4 years, the mean age at onset of the symptoms - 66.9 years. CONCLUSIONS: When comparing the prevalence rates with other studies of Caucasian populations in Europe, the results are similar except for slightly but not significantly higher prevalence rates in the urban population in Estonia.     

Carpal tunnel syndrome incidence in a general population.

OBJECTIVE: To determine the incidence of carpal tunnel syndrome (CTS) in a general population of a restricted area in the middle part of Italy. METHODS: Prospective study to identify cases of CTS, newly diagnosed on the basis of clinical symptoms and delay in distal conduction velocity of the median nerve. In the 8-year period from 1991 to 1998, cases were identified by electromyographic services in the Siena area of Local Health District No. 7 of Tuscany (Italy). This area has a population of 120,000. RESULTS: In the 8-year period, 3,142 cases (79.7% women and 20.3% men; mean age, 55 years; range, 13 to 97 years) were identified. The mean annual crude incidence was 329 cases per 100,000 person-years, and the standardized incidence was 276. The sex-specific incidences were 139 for men and 506 for women. The mean annual incidence for men increased moderately but significantly during the study period, whereas that for women remained constant. The age-specific incidence for women increased gradually with age, reaching a peak between 50 and 59 years, after which it declined. In men, there was a bimodal distribution with peaks between 50 and 59 years and between 70 and 79 years. Rural and industrial areas had higher crude and age- and sex-specific incidences than did urban areas. CONCLUSIONS: The incidence in the Siena area is about threefold that reported in the Rochester area (Minnesota) and is similar to that of Marshfield (Wisconsin). The different results with respect to US reports may depend on case inclusion criteria and occupational activities of the population at risk.     

Population-based studies on incidence, survival rates, and genetic alterations in astrocytic and oligodendroglial gliomas

Published data on prognostic and predictive factors in patients with gliomas are largely based on clinical trials and hospital-based studies. This review summarizes data on incidence rates, survival, and genetic alterations from population-based studies of astrocytic and oligodendrogliomas that were carried out in the Canton of Zurich, Switzerland (approximately 1.16 million inhabitants). A total of 987 cases were diagnosed between 1980 and 1994 and patients were followed up at least until 1999. While survival rates for pilocytic astrocytomas were excellent (96% at 10 years), the prognosis of diffusely infiltrating gliomas was poorer, with median survival times (MST) of 5.6 years for low-grade astrocytoma WHO grade II, 1.6 years for anaplastic astrocytoma grade III, and 0.4 years for glioblastoma. For oligodendrogliomas the MSTwas 11.6 years for grade II and 3.5 years for grade III. TP53 mutations were most frequent in gemistocytic astrocytomas (88%), followed by fibrillary astrocytomas (53%) and oligoastrocytomas (44%), but infrequent (13%) in oligodendrogliomas. LOH 1p/19q typically occurred in tumors without TP53 mutations and were most frequent in oligodendrogliomas (69%), followed by oligoastrocytomas (45%), but were rare in fibrillary astrocytomas (7%) and absent in gemistocytic astrocytomas. Glioblastomas were most frequent (3.55 cases per 100,000 persons per year) adjusted to the European Standard Population, amounting to 69% of total incident cases. Observed survival rates were 42.4% at 6 months, 17.7% at one year, and 3.3% at 2 years. For all age groups, survival was inversely correlated with age, ranging from an MST of 8.8 months (<50 years) to 1.6 months (>80 years). In glioblastomas, LOH 10q was the most frequent genetic alteration (69%), followed by EGFR amplification (34%), TP53 mutations (31%), p16INK4a deletion (31%), and PTEN mutations (24%). LOH 10q occurred in association with any of the other genetic alterations, and was the only alteration associated with shorter survival of glioblastoma patients. Primary (de novo) glioblastomas prevailed (95%), while secondary glioblastomas that progressed from low-grade or anaplastic gliomas were rare (5%). Secondary glioblastomas were characterized by frequent LOH 10q (63%) and TP53 mutations (65%). Of the TP53 mutations in secondary glioblastomas, 57% were in hot-spot codons 248 and 273, while in primary glioblastomas, mutations were more evenly distributed. G:C-->A:T mutations at CpG sites were more frequent in secondary than primary glioblastomas, suggesting that the acquisition of TP53 mutations in these glioblastoma subtypes may occur through different mechanisms.     

An epidemiologic study of febrile seizures and epilepsy in children

Prevalence and annual incidence rates of febrile seizures and types of epilepsy were investigated in all children of the Altenburg district (German Democratic Republic) being 0-14 years old. The mean total population for the period 1982 through 1985 was 108,905. The proportion of children aged 0-14 years was 20,132. The average annual incidence of febrile seizures in children being 0-5 years old was 240.8/100,000. For children between 0 and 14 years the rate of epilepsy 51.1/100,000. Based on 87 epileptic patients, the exact point prevalence rate per 1000 on December 31, 1985, was 4.4.     

Epidemiology of dementia in a Finnish population

An epidemiological study of dementia was carried out in the city of Turku (population 164,568) in Finland. A total of 421 patients with moderate to severe dementia were found. Degenerative dementia, i.e. presenile and senile dementia, was present in 218 patients (51.8 %), and in 152 patients (36.1 %) the dementia was associated with arteriosclerosis, including multi-infarct and combined dementia. The prevalence rate of dementia, all types, was 256 per 100,000 population, and 1,961 per 100,000 population over 65 years old. The age-specific prevalence rates of dementia increased with advancing age from 0.1 % in the age group 55–64 years to 11 % in the age group over 85 years. The peak annual incidence rate for all types of dementia was 58 per 100,000 population, and 447 per 100,000 population over 65 years old. Both prevalence and incidence figures suggested a female preponderance in dementia of degenerative origin and in dementia with associated arteriosclerosis.     

Incidence of subarachnoid haemorrhage: an Australian national hospital morbidity database analysis

Incidences of subarachnoid haemorrhage (SAH) in Australia have been reported in regional studies with variable rates. We investigated the national SAH rate and evaluated the trend over the 10 years from 1998 to 2008. The crude SAH incidence, not related to trauma or arteriovenous malformation, was estimated at 10.3 cases per 100,000 person-years (95% confidence interval [CI]: 10.2-10.4). Females have a higher incidence of SAH (12.5 cases per 100,000; 95% CI: 12.3-12.8) compared to males (8.0 cases per 100,000; 95% CI: 7.8-8.3), with age-adjusted incidence increases with increasing age for both sexes. Less than 10% of SAH occurred in the first three decades of life. The peak age group for patients to experience SAH was between 45 years and 64 years, accounting for almost 45% of the overall annual SAH admissions. Aneurysms located in the anterior circulation were a more common source of rupture compared to those located in the posterior circulation (rate ratio 3.9; 95% CI: 3.6-4.2). Contrary to contemporary observations in the literature, we did not observe a decline in the incidence of SAH during this specified study period.     

Epidemiology of primary intracranial tumours in NW Italy,a population based study: stable incidence in the last two decades

Data about the epidemiology of primary intracranial tumours (PIT) are still heterogeneous depending on different methodological approach in collecting data. In Valle d'Aosta, north west side of Italy, we have carried out a prospective consecutive population based study to calculate the incidence of PIT in the last decade (1992–1999) and to compare these rates with the previous period (1986–1991), data reported in a previous paper. The mean annual PIT incidence rate (ri) per 100,000 inhabitants was 25.48. The mean annual incidence rates in the two period of comparison were adjusted to the 1991 Italian population by the direct method. The standardised ratio was 26.43 in the previous period and 23.24 in the second decade. There is no statistically significant difference. The mean annual PIT incidence rates by tumour types were meningiomas 13.27/100,000 (men 9.77; women 16.7), neuroepithelial group 9,3 (men 10.62; women 8,1), adenomas 1.26, neurinomas 0,7. Mean annual incidence rates by tumour class were also stable. The stable incidence rate in the two periods and the similar incidence in England (21.04 /100,000 person year), strengthen the evidence for a stable incidence rate of PIT in the last decade. These three papers used similar methodology. The homogeneous methodology allows comparison and further evaluation. Received: 23 March 2001, Received in revised form: 2 July 2001, Accepted: 10 July 2001     

Incidence of Parkinson's disease in estonia

The incidence of Parkinson's disease (PD) was studied over a 9-year period (1990-1998) in Tartu county, Estonia, with a mean population of 156,417. Based on 264 patients, the average crude incidence rate of PD was 18.8 per 100,000 person-years. The age-adjusted incidence was 16.8/100,000; 18.3 for the urban group and 14.0 for the rural group; 16.6 for men and 17.1 for women. The age-specific incidence was 1.0/100,000 in the age range 30-39 years, reached a maximum of 117.2/100,000 in the age range 70-79 years and declined in the elderly. The incidence of PD was comparable with that found in developed countries, except for a slightly higher (not significant) incidence rate in the urban compared with the rural population in Estonia.     

Epidemiology of brain tumors: the national survey of intracranial neoplasms

A national survey, based on a probability sample of patients admitted to short-term hospitals in the United States during 1973 to 1974 with a discharge diagnosis of an intracranial neoplasm, was conducted in 157 hospitals. The annual incidence was estimated at 17,000 for primary intracranial neoplasms and 17,400 for secondary intracranial neoplasms--8.2 and 8.3 per 100,000 US population, respectively. Rates of primary intracranial neoplasms increased steadily with advancing age. The age-adjusted rates were higher among men than among women (8.5 versus 7.9 per 100,000). However, although men were more susceptible to gliomas and neuronomas, incidence rates for meningiomas and pituitary adenomas were higher among women.     

Reproductive factors have low impact on the risk of different primary brain tumours in offspring

OBJECTIVES: The aim of our study was to investigate whether reproductive factors influence the risk of primary brain tumours (PBT) in offspring. METHODS: Data on all deliveries in two Swedish counties from 1955 to 1990 were extracted from two birth registries. The follow-up period closed at the end of 1994, with subjects followed up to early middle age. Incidence rates of malignancy for 1958-1994 were obtained from the Swedish Cancer Registry. Standardised incidence ratios (SIR) and relative risks were calculated for astrocytomas, primitive neuroectodermal tumour, ependymoma and meningiomas in offspring. RESULTS: Few associations were detected. High birth weight indicated an increased risk for astrocytomas grade I and II for all primary brain tumours, and the risk was close to significance for astrocytomas grade I-II (SIR = 3.64; CI = 0.98-9.31). For children under 15 years of age the risk for astrocytomas grade I and II was further increased (SIR = 4.44; CI = 1.19-11.38). CONCLUSIONS: A consistent pattern of non-association indicated a low impact of intrauterine environment on the future development of primary brain tumours in offspring up to early middle age.     

Descriptive epidemiology of malignant brain tumors in the Swiss Canton of Vaud

Incidence registration and survival data from brain neoplasms have been analyzed from the population-based system of the Cancer Registry of the Swiss Canton of Vaud, which has been operating in a particularly favorable environment for surveillance and using an integrated and homogeneous system of cancer registration. Between 1974 and 1987, a total of 401 cases was registered, corresponding to an age-adjusted rate for all malignant histotypes of 5.3/100,000 (world standard) for the period 1975-1980 and 5.0 for 1981-1987 in males, and of 3.9 and 3.7, respectively, in females. Thus, no increase in brain neoplasm incidence has taken place in this population, and indeed some decline has been observed in males, particularly for gliomas (from 2.7 to 2.0). During the most recent calendar period, incidence rates were 2.0 in males and 1.5 in females for gliomas, 0.9 in both sexes for astrocytomas, 0.4 in males and 0.1 in females for oligodendrogliomas, and 1.7 in males and 1.2 in females for other or unspecified histotypes. For both gliomas, astrocytomas and total brain neoplasms, the peak rate was observed in the age group 55-74, and some flattening of rates was registered above age 75. Overall 5-year survival was 16%. While there was no difference between sexes, survival rates were substantially lower at older (greater than or equal to 60 years, 3%) than at younger (less than 60 years, 26%) age, and for gliomas (7% at 5 years) than for other histological types. However, the histotype distribution could not explain the major difference in survival by age, since similar differences were observed for various histotypes.(ABSTRACT TRUNCATED AT 250 WORDS)     

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.     

Transient global amnesia: incidence in an urban population

We prospectively identified patients with transient global amnesia in an urban population of 163,000. In 1982, 16 patients experienced their first transient global amnesia. The annual incidence rate was 10 per 100,000 population, and 32 per 100,000 among those aged 50 years or older. There was a substantial female predominance in the incidence of transient global amnesia.     

The incidence of intracranial gliomas in southern Finland

146 intracranial gliomas were found in an epidemiological study of adults in southern Finland 1978-1980. The mean adult population was 896,000 in the study area. The age- and sex-adjusted incidence was 5.6/100,000/year for all gliomas and 4.7 for the histologically verified ones. The incidence was 6.4 for men and 4.9 for women. Of the histologically verified 126 (86%) neoplasms 41 (32%) were glioblastomas, 29 (23%) anaplastic astrocytomas, 30 (24%) benign astrocytomas and 26 (21%) oligodendrogliomas, mixed oligo-astrocytomas, ependymomas, medulloblastomas or gangliogliomas; 20 (14%) were without histological verification.     

MULTIPLE SCLEROSIS and HODGKIN''S DISEASE IN DENMARK

There has been a hypothesis that Hodgkin's disease in young adults and multiple sclerosis may have related causes because the age of clinical onset and the geographic distribution of both are similar. This hypothesis was tested for data in Denmark. Detweeen 1943-62, the average annual incidence rate for Hoadgkin's disease in Denmark was 2.25 per 100,000 population (2.68 male and 1.83 female). Between 1951-69, the average annual death rate for Hodgkin's disease was 2.15 per 100,000 (2.66 male and 1.64 female). The average annual incidence rate for multiple sclerosis in Denmark was calculated from age at onset for 2,481 prevalent cases of 1949, the 1940 population, and an average annual incidence of 128.86 cases for 1939-45: the average annual incidence rate per 100,000 was then 3.35 (3.00 male and 3.69 female). Age specific incidence and death rate for Hodgkin's disease in Denmark each showed a bimodal curve, with one peak at age 25-29 and the other at age 70-74; this was found for each sex, with male rates consistently higher than female. The age specific incidence rates for multiple sclerosis were clearly unimodal with a peak at age 25-29 more definite in females than males. Rates for MS were notably higher for young females than males but about equal by sex for those over the age of 30. The geographic distribution of multiple sclerosis within the counties (amter) of Denmark was markedly non-random, with the major concentration of high prevalence areas middle Jutland and on to Fyn. Geographic distribution of Hodgkin's disease, whether for the young or the old, and whether from incident or death cases, showed no significant variation from a homogeneous distribution. In formal testing there was no correlation of any Hodgkin's distribution with that of MS. A review of the Hodgkin's data for distribution in the United States, on which the original hypothesis was based, suggests the variation there may be little more than reporting artifact. Accordingly, we conclude that there is no relation between distributions of these two disorders, and the factors they do appear to have in common are either quite non-specific or of questionable validity. Thus there is no reason to believe that multiple sclerosis and Hodgkin's disease, even in the young, share a common etiology.