Epidemiology of Amyotrophic Lateral Sclerosis: A Population-Based Study in Israel

Background: Globally, the annual incidence and prevalence of amyotrophic lateral sclerosis (ALS) are estimated at 1.9 and 4.5 per 100,000 population, respectively. This study is aimed at describing the epidemiology of ALS in Israel in a real-world setting. Methods: A retrospective study was performed using the databases of Maccabi Healthcare Services (MHS), a 2-million-member health maintenance organization in Israel. The study included all MHS adults diagnosed with ALS between 1997 and 2013. In 2013, characteristics of ALS patients were compared to those of age-sex-matched patients without ALS. Survival after ALS diagnosis was assessed until death and until tracheostomy or death (follow-up through 2014). Results: In 2013 (n = 158), the prevalence of ALS was 8.1 per 100,000 population in MHS. In 1997-2013, a total of 375 ALS patients were diagnosed, corresponding to an average annual incidence of 1.8 per 100,000 population in MHS. The median survival from diagnosis to death was 3.5 years (95% CI 2.9-4.1), with approximately 28% surviving at least 10 years. Median tracheostomy-free survival was 2.5 years (95% CI 2.1-2.9). Conclusions: Results suggest that there is a relatively high prevalence of ALS in Israel. Further research is needed to investigate factors that may contribute to the survival of patients with ALS in Israel.

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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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Multiple sclerosis and amyotrophic lateral sclerosis: is there a link?

Multiple Sclerosis Journal ◽

10.1177/1352458511427719 ◽

2011 ◽

Vol 18 (6) ◽

pp. 902-904 ◽

Cited By ~ 22

Author(s):

Masoud Etemadifar ◽

Seyed-Hossein Abtahi ◽

Mojtaba Akbari ◽

Amir-Hadi Maghzi

Keyword(s):

Multiple Sclerosis ◽

Amyotrophic Lateral Sclerosis ◽

Fold Increase ◽

Population Based ◽

Isfahan Province ◽

First Degree Relatives ◽

The Family ◽

Reverse Analysis ◽

Als Patients ◽

Lateral Sclerosis

To date, there are no reports studying the rate of amyotrophic lateral sclerosis (ALS) in relatives of multiple sclerosis (MS) patients and vice versa. This study was designed to look into this issue using two population-based databases of MS and ALS in Isfahan province of Iran. We have searched for any first, second or third degree familial kinship between the Isfahan MS Society database and Isfahan ALS population. We compared the rate of ALS among the population of first degree relatives of MS patients, with the crude prevalence of ALS in the general population of Isfahan. On the other hand, a reverse analysis was carried out to compare the prevalence of MS in Isfahan with its rate amongst the first degree relatives of ALS patients. We found 10 families among which five had first degree kinship. The rate of the diseases was significantly higher in both comparisons among the family members ( p < 0.00001) and an odds ratios of more than 67 in both calculations showed a several-fold increase of ALS occurrence in the first degree relatives of MS patients and vice versa. In our study relatives of MS patients were significantly more prone to ALS and vice versa. This could give clues about the common features that the two disease share. Both diseases have an environmental and genetic component and these results mostly point toward genetic similarities.

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Prognosis of amyotrophic lateral sclerosis with cognitive and behavioural changes based on a sixty-month longitudinal follow-up

PLoS ONE ◽

10.1371/journal.pone.0253279 ◽

2021 ◽

Vol 16 (8) ◽

pp. e0253279

Author(s):

Shan Ye ◽

Pingping Jin ◽

Lu Chen ◽

Nan Zhang ◽

Dongsheng Fan

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Survival Time ◽

Progression Rate ◽

Behavioural Changes ◽

Significant Difference ◽

Long Term Follow Up ◽

Behaviour Changes ◽

Als Patients ◽

Lateral Sclerosis

Objective Approximately 50% of amyotrophic lateral sclerosis (ALS) patients have cognitive and behavioural dysfunction in varying degrees and forms. Previous studies have shown that cognitive and behavioural changes may indicate a poor prognosis, and cognitive function gradually deteriorates over the course of disease, but the results of different studies have been inconsistent. In addition, there are relatively limited long-term follow-up studies tracking death as an endpoint. The purpose of this study was to investigate the clinical prognostic characteristics of ALS patients with cognitive behavioural changes through long-term follow-up in a cohort. Methods A total of 87 ALS patients from 2014 to 2015 in the Third Hospital of Peking University were selected and divided into a pure ALS group, an ALS with behavioural variant of frontotemporal dementia (ALS-bvFTD) group, and an ALS with cognitive and behaviour changes group. All patients were followed up for 60 months. The main end point was death and tracheotomy. Results There was no significant difference in survival curve between pure ALS and ALS with cognitive and behavioural change group, but the survival time of ALS-bvFTD group was significantly lower than the other two groups (P < 0.001). For those who was followed up to the endpoint, the survival time of the ALS-bvFTD group was significantly shorter than that of the pure ALS group (t = 5.33, p < 0.001) or the ALS with cognitive and behaviour changes group (t = 4.25, p < 0.001). The progression rate of ALS Functional Rating Scale–Revised (FRS-R) scores from recruitment to endpoint was significantly faster in the ALS-bvFTD group than in the pure ALS group (z = 2.68, p = 0.01) or the ALS with cognitive and behavioural changes group (z = 2.75, p = 0.01). There was no significant difference in survival time (t = 0.52, P = 0.60) or FRS-R score progression rate (z = 0.31, p = 0.76) between the pure ALS group and the ALS with cognitive and behavioural changes group. The total Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen (ECAS) score was positively correlated with survival time (r = 0.38, p = 0.01). Conclusion ALS-bvFTD patients have shorter survival time. The total ECAS score may be correlated with survival time.

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Early Childhood Shigellosis and Attention Deficit Hyperactivity Disorder: A Population-Based Cohort Study with a Prolonged Follow-up

Journal of Attention Disorders ◽

10.1177/1087054720940392 ◽

2020 ◽

pp. 108705472094039

Author(s):

Eugene Merzon ◽

Yuval Gutbir ◽

Shlomo Vinker ◽

Avivit Golan Cohen ◽

Dana Horwitz ◽

...

Keyword(s):

Early Childhood ◽

Health Maintenance Organization ◽

Neurological Complications ◽

Population Based ◽

Stool Culture ◽

Health Maintenance ◽

Hyperactivity Disorder ◽

Shigella Spp

Background: Although the short-term neurological complications of Shigella spp. are well described, potential neuropsychiatric outcomes have not been studied yet. We investigated the association between early childhood shigellosis and subsequent ADHD. Methods: This is a retrospective population-based cohort. Using a large Health Maintenance Organization database, the prevalence of ADHD was investigated among children aged 5–18 years who underwent stool culture prior to the age of 3 years. Results: Of 52,761 children with a stool culture examined, 5,269 (9.98%) had Shigella-positive results. The rate of ADHD was 10.6% and 8.6% among children with Shigella-positive and Shigella-negative stool cultures, respectively ( p < .001). Adjusted odds ratio for ADHD after controlling for gender and socioeconomic status was 1.21 (CI 1.13–1.29, p < .001). The younger the child was during Shigella gastroenteritis, the higher was the association with ADHD ( p < .001). Conclusion: Early childhood shigellosis is associated with an increased rate of long-term ADHD.

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The Incidence of Amyotrophic Lateral Sclerosis in Ohio 2016–2018: The Ohio Population-Based ALS Registry

Neuroepidemiology ◽

10.1159/000515103 ◽

2021 ◽

pp. 1-10

Author(s):

Angeline S. Andrew ◽

Erik P. Pioro ◽

Meifang Li ◽

Xun Shi ◽

Jiang Gui ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Rural Areas ◽

Clinical Information ◽

Population Based ◽

Incidence Rates ◽

The State ◽

Newly Diagnosed ◽

Disease Etiology ◽

Als Patients ◽

Lateral Sclerosis

Introduction: Amyotrophic lateral sclerosis (ALS) is a fatal, neuromuscular disease with no cure. ALS incidence rates have not been assessed specifically in Ohio, yet the state contains both metropolitan and rural areas with a variety of environmental factors that could contribute to disease etiology. We report the incidence of ALS in Ohio residents diagnosed from October 2016 through September 2018. Methods: We engaged practitioners from 9 Ohio sites to identify newly diagnosed ALS patients and to complete case report forms with demographic and clinical information. ALS was diagnosed according to the Awaji criteria and classified as either definite, probable, or possible. We developed a method to estimate missing cases using a Poisson regression model to impute cases in counties with evidence of undercounting. Results: We identified 333 newly diagnosed ALS patients residing in Ohio during the 2-year index period and found incidence rates varied in the 88 state counties. After incorporating the estimated 27% of missing cases, the corrected crude annual incidence was 1.96/100,000 person-years, and the age- and gender-standardized incidence was 1.71/100,000 person-years (standardized to the 2010 US census). Discussion/Conclusion: The estimated Ohio incidence of ALS is overall similar to that reported in other states in the USA. This study reveals a geospatial variation in incidence within the state, and areas with higher rates warrant future investigation.

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Impact of Percutaneous Endoscopic Gastrostomy (PEG) on the Evolution of Disease in Patients with Amyotrophic Lateral Sclerosis (ALS)

Nutrients ◽

10.3390/nu13082765 ◽

2021 ◽

Vol 13 (8) ◽

pp. 2765

Author(s):

Juan J. López-Gómez ◽

María D. Ballesteros-Pomar ◽

Beatriz Torres-Torres ◽

Begoña Pintor-De la Maza ◽

María A. Penacho-Lázaro ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Percutaneous Endoscopic Gastrostomy ◽

Anthropometric Parameters ◽

Endoscopic Gastrostomy ◽

Frequent Event ◽

The Mean ◽

Als Patients ◽

Prevalent Symptom ◽

Lateral Sclerosis

Dysphagia is a highly prevalent symptom in Amyotrophic Lateral Sclerosis (ALS), and the implantation of a percutaneous endoscopic gastrostomy (PEG) is a very frequent event. The aim of this study was to evaluate the influence of PEG implantation on survival and complications in ALS. An interhospital registry of patients with ALS of six hospitals in the Castilla-León region (Spain) was created between January 2015 and December 2017. The data were compared for those in whom a PEG was implanted and those who it was not. A total of 93 patients were analyzed. The mean age of the patients was 64.63 (17.67) years. A total of 38 patients (38.8%) had a PEG implantation. An improvement in the anthropometric parameters was observed among patients who had a PEG from the beginning of nutritional follow-up compared to those who did not, both in BMI (kg/m2) (PEG: 0 months, 22.06; 6 months, 23.04; p < 0.01; NoPEG: 0 months, 24.59–23.87; p > 0.05). Among the deceased patients, 38 (40.4%) those who had an implanted PEG (20 patients (52.6%) had a longer survival time (PEG: 23 (15–35.5) months; NoPEG 11 (4.75–18.5) months; p = 0.01). A PEG showed a survival benefit among ALS patients. Early implantation of a PEG produced a reduction in admissions associated with complications derived from it.

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Telemedicine for management of patients with amyotrophic lateral sclerosis through COVID-19 tail

Neurological Sciences ◽

10.1007/s10072-020-04783-x ◽

2020 ◽

Author(s):

Alessandro Bombaci ◽

◽

Gianmarco Abbadessa ◽

Francesca Trojsi ◽

Letizia Leocani ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Neurological Diseases ◽

Healthcare Systems ◽

Digital Tools ◽

Social Restriction ◽

Containment Measures ◽

Life Threatening ◽

Als Patients ◽

Lateral Sclerosis

Abstract Over the last months, due to coronavirus disease (COVID-19) pandemic, containment measures have led to important social restriction. Healthcare systems have faced a complete rearrangement of resources and spaces, with the creation of wards devoted to COVID-19 patients. In this context, patients affected by chronic neurological diseases, such as amyotrophic lateral sclerosis (ALS), are at risk to be lost at follow-up, leading to a higher risk of morbidity and mortality. Telemedicine may allow meet the needs of these patients. In this commentary, we briefly discuss the digital tools to remotely monitor and manage ALS patients. Focusing on detecting disease progression and preventing life-threatening conditions, we propose a toolset able to improve ALS management during this unprecedented situation.

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Changes in Retinal OCT and Their Correlations with Neurological Disability in Early ALS Patients, a Follow-Up Study

Brain Sciences ◽

10.3390/brainsci9120337 ◽

2019 ◽

Vol 9 (12) ◽

pp. 337 ◽

Cited By ~ 5

Author(s):

Pilar Rojas ◽

Rosa de Hoz ◽

Ana I. Ramírez ◽

Antonio Ferreras ◽

Elena Salobrar-Garcia ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Rating Scale ◽

P Value ◽

Ophthalmological Examination ◽

Fiber Layer ◽

Neurological Disability ◽

Als Patients ◽

Lateral Sclerosis ◽

Visual Changes

Background: To compare early visual changes in amyotrophic lateral sclerosis (ALS) patients with healthy controls in a baseline exploration, to follow-up the patients after 6 months, and to correlate these visual changes with neurological disability. Methods: All patients underwent a comprehensive neurological and ophthalmological examination. A linear mixed analysis and Bonferroni p-value correction were performed, testing four comparisons as follows: Control baseline vs. control follow-up, control baseline vs. ALS baseline, control follow-up vs. ALS follow-up, and ALS baseline vs. ALS follow-up. Results: The mean time from the diagnosis was 10.80 ± 5.5 months. The analysis of the optical coherence tomography (OCT) showed: (1) In ALS baseline vs. control baseline, a macular significantly increased thickness of the inner macular ring temporal and inferior areas; (2) in ALS follow-up vs. ALS baseline, a significant macular thinning in the inner and outer macular ring inferior areas; (3) in ALS follow-up vs. ALS baseline, a significant peripapillary retinal nerve fiber layer (pRNFL) thinning in the superior and inferior quadrants; and (4) ALS patients showed a moderate correlation between some OCT pRNFL parameters and Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) score. Conclusion: The OCT showed retinal changes in patients with motoneuron disease and could serve as a complementary tool for studying ALS.

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Longitudinal Timed Up and Go Assessment in Amyotrophic Lateral Sclerosis: A Pilot Study

European Neurology ◽

10.1159/000516772 ◽

2021 ◽

pp. 1-5

Author(s):

Eglė Sukockienė ◽

Ruxandra Iancu Ferfoglia ◽

Antoine Poncet ◽

Jean-Paul Janssens ◽

Gilles Allali

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Pilot Study ◽

Walking Ability ◽

University Hospitals ◽

Timed Up And Go ◽

Progressive Loss ◽

Bulbar Onset ◽

Als Patients ◽

Lateral Sclerosis

Progressive loss of walking ability in amyotrophic lateral sclerosis (ALS) has been scarcely studied as a potential predictive factor for survival in motor neuron disease. We aimed to assess the progression of gait decline and its association with mortality in ALS using the Timed Up and Go test (TUG). Patients were followed up prospectively at the Centre for ALS and Related Disorders in Geneva University Hospitals between 2012 and 2016. The TUG was performed at baseline and subsequent evaluations occurred every 3 months. At inclusion, patients were classified as unable to perform the TUG, “slow TUG” (>10.6 s), and “fast TUG” (≤10.6 s). In total, 68 patients with ALS (mean ± SD age: 68.6 ± 11.9 years; 50% female) were included. Baseline TUG was negatively correlated with the total ALSFRS-R score (r = −0.63, p < 0.001). At baseline, ALS patients with bulbar onset performed the TUG faster (9.9 ± 3.7 s) than the non-bulbar ones (17.3 ± 14.9 s, p = 0.008). Thirty of 68 (44%) patients died by the end of the follow-up period. The TUG performance at the first visit did not predict mortality. While we did not find any association with mortality in ALS and gait quantification, the TUG was feasible in a majority of ALS patients, was correlated with functional status, and could be of interest in the follow-up of non-bulbar ALS patients.

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The 6-min walk test as a new outcome measure in Amyotrophic lateral sclerosis

Scientific Reports ◽

10.1038/s41598-020-72578-3 ◽

2020 ◽

Vol 10 (1) ◽

Author(s):

Massimo Russo ◽

Christian Lunetta ◽

Riccardo Zuccarino ◽

Gian L. Vita ◽

Maria Sframeli ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Small Sample Size ◽

Small Sample ◽

Walk Test ◽

Multicentric Study ◽

History Of ◽

Als Patients ◽

Different Content ◽

Lateral Sclerosis

Abstract One of the issues highlighted in amyotrophic lateral sclerosis (ALS) clinical trials is the lack of appropriate outcome measures. The aim of this multicentric study was to evaluate the 6-min walk test (6MWT) as tool to monitor the natural history of a cohort of ALS patients followed up over a 6-month interval. Forty-four ambulant patients were assessed at baseline and after 1, 3 and 6 months. Eight out of forty-four lost the ability to walk before the end of the study. The 6MWT and the objective measures linked to motor function, such as 10 m walking test (10MWT) and Time-up and go (TUG), the ALSFRS-R and the ALSFRS-R items 7–9 showed a good responsiveness to change over the 6-month interval. There was a strong correlation between 6 and 10MWT, TUG, ALSFRS-R, ALSFRS-R items 7–9 and FVC% at baseline. There was no correlation with Edinburgh Cognitive and Behavioural ALS Screen (ECAS) and Modified Borg Scale (MBS). The Δ of 6MWT from T0 to T6 significantly correlated with the Δs of 10MWT and TUG. There was no correlation with the Δs of ALSFRS-R, ALSFRS-R items 7 9, ECAS, MBS and FVC%. The discordance between changes of the 6MWT and ALSFRS-R at 6 month highlights the different content validity among these instruments. The concordance among 6MWT, 10MWT and TUG indicates that the 6MWT is an objective, sensitive and robust tool to measure motor performances in a longitudinal setting. The main limitations of our study were the small sample size and the high percentage of patients (18%) lost at follow-up. Therefore, further studies on larger cohorts, and exploring the relation between 6MWT and need of ventilator support or survival could strengthen our results.

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