The epidemiology of amyotrophic lateral sclerosis in Moscow (Russia)

Brylev, Lev; Ataulina, Anastasia; Fominykh, Vera; Vorobyeva, A. A.; Истомина, Е. И.; Shikhirimov, Rafiz; Salikov, A V; Zakharova, M. N.; Guekht, Alla; Beghi, Ettore

doi:10.1080/21678421.2020.1752252

Cited by 8 publications

(6 citation statements)

References 7 publications

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“…Crude average annual incidence rates in Italy ranged from 1.33 per 100,000 person-years in Sardinia 25 to 3.22 per 100,000 person-years in Liguria. 26 The lowest incidence rates in all the European studies were reported for Serbia, 23 Russia, 27 and Cyprus. 28 The highest incidence rates reported were for Scotland, 29 Austria, 30 and Denmark.…”

Section: Resultsmentioning

confidence: 93%

Global Prevalence and Incidence of Amyotrophic Lateral Sclerosis

et al. 2023

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Background and Objectives:Amyotrophic Lateral Sclerosis (ALS) is a rare neurodegenerative disorder affecting upper and lower motor neurons. Due to its rarity and rapidly progressive nature, studying the epidemiology of ALS is challenging and a comprehensive picture of the global burden of this disease is lacking. The objective of this systematic review was to describe the global incidence and prevalence of ALS.Methods:We searched Medline, Embase, Global Health, PsycInfo, Cochrane Library, and CINAHL to identify articles published between January 1st, 2010, and May 6th, 2021. Studies that were population-based and reported estimates of prevalence, incidence, and/or mortality of ALS, were eligible for inclusion. This paper focuses on incidence and prevalence. Quality assessment was performed using a tool developed to evaluate methodology relevant to prevalence and incidence studies. This review was registered with PROSPERO, CRD42021250559.Results:This search generated 6,238 articles, of which 140 were selected for data extraction and quality assessment. Of these, 85 articles reported on incidence and 61 on prevalence of ALS. Incidence ranged from 0.26 per 100,000 person-years in Ecuador to 23.46 per 100,000 person-years in Japan. Point prevalence ranged from 1.57 per 100,000 in Iran to 11.80 per 100,000 in the United States. Many articles identified ALS cases from multiple data sources.Discussion:There is variation in reported incidence and prevalence estimates of ALS across the world. While registries are an important and powerful tool to quantify disease burden, such resources are not available everywhere. This results in gaps in reporting of the global epidemiology of ALS, as highlighted by the degree of variation (and quality) in estimates of incidence and prevalence reported in this review.

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Section: Resultsmentioning

confidence: 93%

Global Prevalence and Incidence of Amyotrophic Lateral Sclerosis

et al. 2023

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“…It is known that male gender is a risk factor for ALS.6 Male / female (M/F) rate in ALS patients varies between 1-3 in many studies (6)(7)(8)(9)(10)(11). In studies conducted in Antalya province and Thrace region in Turkey, this rate was found as 1.78 and 2 (8,9).…”

Section: Discussionmentioning

confidence: 99%

“…When it is examined at the mean age of onset of ALS, different results are obtained in a wide range of age. The mean age of onset of ALS has been reported as 56 and 60, respectively, in Russia and Greece, Turkey's northern and western neighbors (10,11). The mean age of onset of ALS in Turkey was reported as 56 and 58 in two different studies conducted in the western regions of Turkey (8,9).…”

Section: Discussionmentioning

confidence: 99%

Clinical and epidemiological features of amyotrophic lateral sclerosis in eastern Turkey

Yevgi

Bilge

2021

Journal of Health Sciences and Medicine

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Aim:The number of studies on the epidemiological and clinical data of amyotrophic lateral sclerosis (ALS) patients in Turkey is quite low and the studies on this subject reflect the data of western regions of Turkey. In this study, we aimed to present the demographic, clinical and mortality features of ALS patients diagnosed in the last 10 years in a large reference hospital in the Eastern Anatolia region of Turkey. Material and Method: 42 ALS patients diagnosed between January 2011 and January 2021 in Atatürk University, Faculty of Medicine, Department of Neurology were included in the study. The data of the patients were obtained by retrospectively scanning the patient electronic files registered in the database of our hospital. The age, gender, examination findings, clinical course, treatments they received and the cause of death of the patients were recorded. Results: 26 of ALS patients were men and 16 were women. The mean age of onset was 53.4±12.3 and the mean diagnostic delay was 13.6±6.9 months. 64.2% of the patients had onset in the spinal region, and 35.7% in the bulbar region. Weakness/atrophy of the upper extremity muscles and swallowing difficulty were the most common onset symptoms. All ALS patients were using riluzole. 11 ALS patients died. The mean time between diagnosis and death in patients who died was 27.2±18.6 months. The most common causes of death in ALS patients were pneumonia and sepsis. Conclusion: Age of onset of ALS was low in our patients. The number of patients in the clinically definite ALS group was the highest. Weakness/atrophy of the upper extremity muscles and swallowing difficulty were the most common onset symptoms. Approximately 2/3 of the patients had spinal, and 1/3 bulbar region onset. There was no significant difference between spinal and bulbar onset patients in terms of gender, age of disease onset, mortality, and life expectancy of patients with death. The rate of using riluzole was high. The most common causes of death in ALS patients were pneumonia and sepsis.

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“…The study host, ALS Centre Moscow (Russia), is a collaboration between three Moscow hospitals that has provided home-based multidisciplinary care for pwALS and their families since 2012. The incidence of ALS in Russia is 1.25 per 100,000 person-years [74]. Approximately 110 ALS patients out of approximately 950 pwALS residing in the Moscow region receive treatment from the team.…”

Section: Facilitymentioning

confidence: 99%

Home-Based Music Therapy to Support Bulbar and Respiratory Functions of Persons with Early and Mid-Stage Amyotrophic Lateral Sclerosis—Protocol and Results from a Feasibility Study

Kolomeytseva¹,

Brylev

Eshghi

et al. 2022

Brain Sciences

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Respiratory failure, malnutrition, aspiration pneumonia, and dehydration are the precursors to mortality in ALS. Loss of natural communication is considered one of the worst aspects of ALS. This first study to test the feasibility of a music therapy protocol for bulbar and respiratory rehabilitation in ALS employs a mixed-methods case study series design with repeated measures. Newly diagnosed patients meeting the inclusion criteria were invited to participate, until the desired sample size (n = 8) was achieved. The protocol was delivered to participants in their homes twice weekly for six weeks. Individualised exercise sets for independent practice were provided. Feasibility data (recruitment, retention, adherence, tolerability, self-motivation and personal impressions) were collected. Bulbar and respiratory changes were objectively measured. Results. A high recruitment rate (100%), a high retention rate (87.5%) and high mean adherence to treatment (95.4%) provide evidence for the feasibility of the study protocol. The treatment was well tolerated. Mean adherence to the suggested independent exercise routine was 53%. The outcome measurements to evaluate the therapy-induced change in bulbar and respiratory functions were defined. Findings suggest that the protocol is safe to use in early- and mid-stage ALS and that music therapy was beneficial for the participants’ bulbar and respiratory functions. Mean trends suggesting that these functions were sustained or improved during the treatment period were observed for most outcome parameters: Maximal Inspiratory Pressure, Maximal Expiratory Pressure, Peak Expiratory Flow, the Center for Neurologic Study—Bulbar Function Scale speech and swallowing subscales, Maximum Phonation Time, Maximum Repetition Rate—Alternating, Maximum Repetition Rate—Sequential, Jitter, Shimmer, NHR, Speaking rate, Speech–pause ratio, Pause frequency, hypernasality level, Time-to-Laryngeal Vestibule Closure, Maximum Pharyngeal Constriction Area, Peak Position of the Hyoid Bone, Total Pharyngeal Residue C24area. Conclusion. The suggested design and protocol are feasible for a larger study, with some modifications, including aerodynamic measure of nasalance, abbreviated voice sampling and psychological screening.

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The epidemiology of amyotrophic lateral sclerosis in Moscow (Russia)

Cited by 8 publications

References 7 publications

Global Prevalence and Incidence of Amyotrophic Lateral Sclerosis

Global Prevalence and Incidence of Amyotrophic Lateral Sclerosis

Clinical and epidemiological features of amyotrophic lateral sclerosis in eastern Turkey

Home-Based Music Therapy to Support Bulbar and Respiratory Functions of Persons with Early and Mid-Stage Amyotrophic Lateral Sclerosis—Protocol and Results from a Feasibility Study

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