Changing epidemiology of motor neurone disease in Scotland

Abstract

ObjectivesScotland benefits from an integrated national healthcare team for motor neurone disease (MND) and a tradition of rich clinical data capture using the Scottish MND Register (launched in 1989; one of the first national registers). The Scottish register was relaunched in 2015 as Clinical Audit Research and Evaluation of MND (CARE-MND), an electronic platform for prospective, population-based research. We aimed to determine if incidence of MND is changing over time. Methods Capture-recapture methods determined incidence of MND in 2015-16. Incidence rates for 2015-16 and 1989-98 were direct age and sex standardised to allow time period comparison. Phenotypic characteristics and socioeconomic status of the cohort are Powered by Editorial Manager® and ProduXion Manager® from Aries Systems Corporation described. ResultsCoverage of the CARE-MND platform was 99%. Crude incidence in the 2015-17 period was 3.83/100,000 person-years (95% CI 3.53-4.14). Direct age standardised incidence in 2015 was 3.42/100,000 (95% CI 2.99-3.91); in 2016, 2.89/100,000 (95% CI 2.50-3.34). The 1989-98 direct standardised annual incidence estimate was 2.32/100,000 (95% CI 2.26-2.37). 2015-16 standardised incidence was 66.9% higher than Northern European estimates. Socioeconomic status was not associated with MND. ConclusionsOur data show a changing landscape of MND in Scotland, with a rise in incidence by 36.0% over a 25-year period. This is likely attributable to ascertainment in the context of improved neurological services in Scotland. Our data suggest that CARE-MND is a reliable national resource and findings can be extrapolated to other Northern European populations.

Motor Neuron Disease Register for England, Wales and Northern Ireland—an analysis of incidence in England

“…Standardizing incidence to the European standard population resulted in an estimate of 2.26/ 100,000 person-years, higher than the estimate of 1.4/100,000 reported in Northern Ireland (22). Our imputed incidence standardized to the US population is lower at 1.87/100,000 person-years, which comparable to the 1.89/100,000 person years reported for Northern Europe in 2017, but lower than 3.83/100,000 person-years reported in Scotland (2,42).…”

“…The MND Register as a federated database is relatively new, and the collection of data was initiated at different times by individual participating sites. Detailed reporting by population register in the Republic of Ireland and Scotland have shown that data quality and ascertainment improves over time (42,44,45). As more centres contribute data, we will be able to perform capture-recapture analysis of overlapping areas allowing more accurate incidence estimates.…”

Epidemiology of amyotrophic lateral sclerosis: an update of recent literature

“…Incidence studies are important, not only in terms of gaining knowledge of disease burden but also to compare if and how the disease burden differs across populations of different characteristics. Recent studies reported an incidence of ALS between 0.6 and 3.8 per 100 000 person-years [1 ▪ ,2,3,4 ▪ ,5,6,7 ▪▪ ,8]. In Europe, the incidence of ALS is higher, ranging from 2.1 to 3.8 per 100 000 person-years [1 ▪ ,2,3,7 ▪▪ ].…”

The Relationship between Selected Demographic Factors and Speech Organ Dysfunction in Sporadic ALS Patients

“…The men to women ratio in the study sample was 1.4, and the ratio described in the literature is 1-2 [19][20][21]. The mean age of ALS onset was 53.61 years (while the average is 51-66 years) [18,[21][22][23][24], and 68% of the patients had spinal disorders (literature average 58-82%) [25][26][27]. Such a distribution in a relatively large group of patients allows for drawing conclusions and transposing them to the general population of ALS patients.…”