Spatial epidemiology of amyotrophic lateral sclerosis in Piedmont and Aosta Valley, Italy: a population-based cluster analysis

BackgroundAmyotrophic lateral sclerosis (ALS) is often associated with cognitive and/or behavioural impairment. Cognitive reserve (CR) may play a protective role in offsetting cognitive impairment. This study examined the relationship between CR and longitudinal change in cognition in an Irish ALS cohort.MethodsLongitudinal neuropsychological assessment was carried out on 189 patients over 16 months using the Edinburgh cognitive and behavioural ALS screen (ECAS) and an additional battery of neuropsychological tests. CR was measured by combining education, occupation and physical activity data. Joint longitudinal and time-to-event models were fitted to investigate the associations between CR, performance at baseline and decline over time while controlling for non-random drop-out.ResultsCR was a significant predictor of baseline neuropsychological performance, with high CR patients performing better than those with medium or low CR. Better cognitive performance in high CR individuals was maintained longitudinally for ECAS, social cognition, executive functioning and confrontational naming. Patients displayed little cognitive decline over the course of the study, despite controlling for non-random drop-out.ConclusionsThese findings suggest that CR plays a role in the presentation of cognitive impairment at diagnosis but is not protective against cognitive decline. However, further research is needed to examine the interaction between CR and other objective correlates of cognitive impairment in ALS.

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Spatial cluster analysis of population amyotrophic lateral sclerosis risk in Ireland

Neurology ◽

10.1212/wnl.0000000000001477 ◽

2015 ◽

Vol 84 (15) ◽

pp. 1537-1544 ◽

Cited By ~ 14

Author(s):

J. Rooney ◽

A. Vajda ◽

M. Heverin ◽

M. Elamin ◽

A. Crampsie ◽

...

Keyword(s):

Cluster Analysis ◽

Amyotrophic Lateral Sclerosis ◽

Spatial Cluster ◽

Spatial Cluster Analysis ◽

Lateral Sclerosis

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Amyotrophic lateral sclerosis: a comparison of two staging systems in a population-based study

European Journal of Neurology ◽

10.1111/ene.13053 ◽

2016 ◽

Vol 23 (9) ◽

pp. 1426-1432 ◽

Cited By ~ 10

Author(s):

D. Ferraro ◽

D. Consonni ◽

N. Fini ◽

A. Fasano ◽

C. Del Giovane ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Population Based ◽

Population Based Study ◽

Staging Systems ◽

Lateral Sclerosis

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Amyotrophic lateral sclerosis is not linked to multiple sclerosis in a population based study

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2012-304864 ◽

2013 ◽

Vol 84 (8) ◽

pp. 940-941 ◽

Cited By ~ 6

Author(s):

P. T. C. van Doormaal ◽

A. Gallo ◽

W. van Rheenen ◽

J. H. Veldink ◽

M. A. van Es ◽

...

Keyword(s):

Multiple Sclerosis ◽

Amyotrophic Lateral Sclerosis ◽

Population Based ◽

Population Based Study ◽

Lateral Sclerosis

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Prognostic value of weight loss in patients with amyotrophic lateral sclerosis: a population-based study

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2020-322909 ◽

2020 ◽

Vol 91 (8) ◽

pp. 867-875 ◽

Cited By ~ 1

Author(s):

Mark R Janse van Mantgem ◽

Ruben P A van Eijk ◽

Hannelore K van der Burgh ◽

Harold H G Tan ◽

Henk-Jan Westeneng ◽

...

Keyword(s):

Weight Loss ◽

Amyotrophic Lateral Sclerosis ◽

Body Weight ◽

Prognostic Value ◽

Population Based ◽

Data Set ◽

Population Based Study ◽

Risk Of Death ◽

Lateral Sclerosis

ObjectiveTo determine the prevalence and prognostic value of weight loss (WL) prior to diagnosis in patients with amyotrophic lateral sclerosis (ALS).MethodsWe enrolled patients diagnosed with ALS between 2010 and 2018 in a population-based setting. At diagnosis, detailed information was obtained regarding the patient’s disease characteristics, anthropological changes, ALS-related genotypes and cognitive functioning. Complete survival data were obtained. Cox proportional hazard models were used to assess the association between WL and the risk of death during follow-up.ResultsThe data set comprised 2420 patients of whom 67.5% reported WL at diagnosis. WL occurred in 71.8% of the bulbar-onset and in 64.2% of the spinal-onset patients; the mean loss of body weight was 6.9% (95% CI 6.8 to 6.9) and 5.5% (95% CI 5.5 to 5.6), respectively (p<0.001). WL occurred in 35.1% of the patients without any symptom of dysphagia. WL is a strong independent predictor of survival, with a dose response relationship between the amount of WL and the risk of death: the risk of death during follow-up increased by 23% for every 10% increase in WL relative to body weight (HR 1.23, 95% CI 1.13 to 1.51, p<0.001).ConclusionsThis population-based study shows that two-thirds of the patients with ALS have WL at diagnosis, which also occurs independent of dysphagia, and is related to survival. Our results suggest that WL is a multifactorial process that may differ from patient to patient. Gaining further insight in its underlying factors could prove essential for future therapeutic measures.

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Population-based study of amyotrophic lateral sclerosis and occupational lead exposure in Denmark

Occupational and Environmental Medicine ◽

10.1136/oemed-2018-105469 ◽

2019 ◽

pp. oemed-2018-105469 ◽

Cited By ~ 4

Author(s):

Aisha S Dickerson ◽

Johnni Hansen ◽

Aaron J Specht ◽

Ole Gredal ◽

Marc G Weisskopf

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Conditional Logistic Regression ◽

Population Based ◽

Population Based Study ◽

Employment History ◽

National Patient Registry ◽

Increased Risk ◽

National Patient ◽

Pb Exposure ◽

Lateral Sclerosis

ObjectivesPrevious research has indicated links between lead (Pb) exposure and increased risk of neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS). In this study, we evaluated the association between occupational Pb exposures and ALS.MethodsALS cases were ascertained through the Danish National Patient Registry from 1982 to 2013 and age and sex-matched to 100 controls. Using complete employment history since 1964 from the Danish Pension Fund, cumulative Pb exposure was estimated for each subject via a Danish job exposure matrix. Associations were evaluated using conditional logistic regression analyses and stratified by sex.ResultsFor men with >50% probability of exposure, there was an increase in odds of ALS for exposures in the 60th percentile or higher during any time 5 years prior to diagnosis (aOR: 1.35; 95% CI 1.04 to 1.76) and 10 years prior to diagnosis (aOR: 1.33; 95% CI 1.03 to 1.72). No significant associations were observed in women, and there were no linear trends seen for Pb exposures for either sex.ConclusionsOur study indicates an association between consistently higher occupational Pb exposures and ALS. These findings support those of previously reported associations between ALS and specific occupations that commonly experience Pb exposure.

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Early weight loss in amyotrophic lateral sclerosis: outcome relevance and clinical correlates in a population-based cohort

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2018-319611 ◽

2019 ◽

Vol 90 (6) ◽

pp. 666-673 ◽

Cited By ~ 15

Author(s):

Cristina Moglia ◽

Andrea Calvo ◽

Maurizio Grassano ◽

Antonio Canosa ◽

Umberto Manera ◽

...

Keyword(s):

Weight Loss ◽

Amyotrophic Lateral Sclerosis ◽

Median Survival ◽

Population Based ◽

Study Cohort ◽

Respiratory Impairment ◽

Clinical Correlates ◽

Severe Weight Loss ◽

Bulbar Onset ◽

Lateral Sclerosis

ObjectivesTo assess the role of body mass index (BMI) and of the rate of weight loss as prognostic factors in amyotrophic lateral sclerosis (ALS) and to explore the clinical correlates of weight loss in the early phases of the disease.MethodsThe study cohort included all ALS patients in Piemonte/Valle d’Aosta in the 2007–2011 period. Overall survival and the probability of death/tracheostomy at 18 months (logistic regression model) were calculated.ResultsOf the 712 patients, 620 (87.1%) were included in the study. Patients ’ survival was related to the mean monthly percentage of weight loss at diagnosis (p<0.0001), but not to pre-morbid BMI or BMI at diagnosis. Spinal onset patients with dysphagia at diagnosis had a median survival similar to bulbar onset patients. About 20% of spinal onset patients without dysphagia at diagnosis had severe weight loss and initial respiratory impairment, and had a median survival time similar to bulbar onset patients.ConclusionsThe rate of weight loss from onset to diagnosis was found to be a strong and independent prognostic factor in ALS. Weight loss was mainly due to the reduction of nutritional intake related to dysphagia, but a subgroup of spinal onset patients without dysphagia at diagnosis had a severe weight loss and an outcome similar to bulbar patients. According to our findings, we recommend that in clinical trials patients should be stratified according to the presence of dysphagia at the time of enrolment and not by site of onset of symptoms.

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