The epidemiology of amyotrophic lateral sclerosis (ALS/MND) in people aged 80 or over

Forbes, Raeburn B; Colville, Shuna; Swingler, Robert

doi:10.1093/ageing/afh013

Cited by 103 publications

(89 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…5). In a previous report, the proportion of ALS patients in Scotland in 1989-98 with an age at onset of 80 years or more was 11.1% (21). In our study, the percentage of patients whose age at disease onset was !…”

Section: Discussionsupporting

confidence: 46%

See 1 more Smart Citation

Changes in the Clinical Features of Amyotrophic Lateral Sclerosis in Rural Japan

et al. 2013

View full text Add to dashboard Cite

Objective To assess longitudinal changes in the clinical features of patients with amyotrophic lateral sclerosis (ALS), we performed a retrospective hospital-based study covering 35 years. Methods We investigated 287 patients (154 men and 133 women) with sporadic ALS hospitalized at the Department of Neurology at Gunma University Hospital (Japan) between 1978 and 2012. All patients fulfilled the diagnostic criteria for definitive, probable or laboratory-supported probable ALS according to the revised El Escorial criteria. Results Two hundred patients (69.7%) exhibited limb onset and 87 patients (30.3%) exhibited bulbar onset of the disease. The percentage of patients who showed bulbar onset of the disease increased steadily over the 35 years from 14.2% to 38.3% (2008-12) (p<0.01, r=0.470). The mean age at onset was 62.1 ± 11.7 years, and the age at onset increased significantly over time from 51.7 years (1978-82) to 64.9 years (2008-12) (p<0.001, r=0.294). In addition, the percentage of patients whose age at onset was 70 years or more increased from 0% (1978-82) to 38.2% . The percentage of ALS patients with dementia increased from 0% (1978-82) to 20.2% (2008-12). Conclusion Our findings demonstrate that, among patients with sporadic ALS, the age at disease onset, the proportion of patients with disease onset at 70 years of age or higher, the proportion of patients with dementia and the proportion of patients with bulbar onset ALS have increased significantly over the past 35 years. The longitudinal changes observed in the clinical features of ALS may reflect the increasing age at disease onset.

show abstract

Section: Discussionsupporting

confidence: 46%

“…The diagnosis of ALS is currently based on a combination has been reported (17)(18)(19)(20)(21)(22)(23)(24). These findings suggest that the epidemiology and clinical features of ALS may have changed over time.…”

Section: Introductionmentioning

confidence: 98%

Changes in the Clinical Features of Amyotrophic Lateral Sclerosis in Rural Japan

et al. 2013

View full text Add to dashboard Cite

show abstract

“…15 Older age at onset is associated with decreased likelihood of upper motor neuron involvement (20%), increased probability of bulbar onset (some studies report up to 50% with onset after 80 years of age), and poor prognosis. 9,22,23 Onset after 80 years is associated with a particularly short survival. 16 Rate of progression Although median survival in ALS is generally around 3 years from diagnosis, variability in survival is remarkable and reflects the variability in the rate of disease progression.…”

Section: Age At Onsetmentioning

confidence: 99%

The phenotypic variability of amyotrophic lateral sclerosis

2014

View full text Add to dashboard Cite

| Classic textbook neurology teaches that amyotrophic lateral sclerosis (ALS) is a degenerative disease that selectively affects upper and lower motor neurons and is fatal 3-5 years after onset-a description which suggests that the clinical presentation of ALS is very homogenous. However, clinical and postmortem observations, as well as genetic studies, demonstrate that there is considerable variability in the phenotypic expression of ALS. Here, we review the phenotypic variability of ALS and how it is reflected in familial and sporadic ALS, in the degree of upper and lower motor neuron involvement, in motor and extramotor involvement, and in the spectrum of ALS and frontotemporal dementia. Furthermore, we discuss some unusual clinical characteristics regarding presentation, age at onset and disease progression. Finally, we address the importance of this variability for understanding the pathogenesis of ALS and for the development of therapeutic strategies.

show abstract

“…Several reasons have been put forward, such as difficulty in diagnosing ALS among the elderly because of comorbidity, difficulty in access to specialised care or a more rapid and aggressive disease and shorter survival that cause elderly patients to die before the diagnosis of ALS is established 3,7,9,10 . Identification of subpopulations at risk is likely to help to our understanding of the disease pathogenesis.…”

Section: Introductionmentioning

confidence: 99%

Amyotrophic lateral sclerosis: A higher than expected incidence in people over 80 years of age

Aragonés

Altimiras

Roura-Poch

et al. 2016

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

View full text Add to dashboard Cite

Objective: To determine the age-specific incidence and clinical-epidemiological characteristics of an Amyotrophic lateral sclerosis (ALS) cohort patient in Catalonia (Spain). Methods and results: New cases diagnosed between January 1, 2004 and December 31, 2013 were 41 (20 men and 21 women), with an annual crude incidence rate of 2.7 per 100,000 person-years (95% CI 1.90 -3.59). The incidence rate increased with age reaching a peak in the age-group of 70 to 79 years. There was a non significant decrease in the incidence rate in the group of patients over 80 (P value = 0.75) 17.99 per 100 000 person years (95% CI 7.81 -28.17). The percentage of patients over age 80 was 29.3% and over age 85 was 9.8%. The prevalence rate at the end of the study period was 8.38/100.000 of the total population. Mean age at symptom onset was 76.0 years. Onset of symptoms was bulbar or generalized in 36.6% of cases.Conclusions: ALS incidence in Osona is within the range of other countries across Europe. Our results suggest that the age-specific incidence rate of ALS increases with age through the oldest age groups suggesting an age-risk effect to develop the disease.

show abstract

The epidemiology of amyotrophic lateral sclerosis (ALS/MND) in people aged 80 or over

Cited by 103 publications

References 18 publications

Changes in the Clinical Features of Amyotrophic Lateral Sclerosis in Rural Japan

Changes in the Clinical Features of Amyotrophic Lateral Sclerosis in Rural Japan

The phenotypic variability of amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis: A higher than expected incidence in people over 80 years of age

Contact Info

Product

Resources

About