Epidemiology of Amyotrophic Lateral Sclerosis in the Republic of Cyprus: A 25-Year Retrospective Study

2017 ◽  
Vol 48 (1-2) ◽  
pp. 79-85 ◽  
Author(s):  
Christiana A. Demetriou ◽  
Petros M. Hadjivasiliou ◽  
Kleopas A. Kleopa ◽  
Yiolanda P. Christou ◽  
Eleni Leonidou ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Age Of Onset ◽  
Age At Onset ◽  
Positive Family History ◽  
Temporal Trends ◽  
Male Predominance ◽  
The Republic ◽  
Republic Of Cyprus ◽  
Als Patients ◽  
Lateral Sclerosis

Introduction: Amyotrophic lateral sclerosis (ALS) is a rare, rapidly progressive neurodegenerative disease. Despite wide variability in the incidence and prevalence of ALS, there is evidence of positive temporal trends and an increase in incidence with age. The aim of this study was to conduct a detailed epidemiological investigation of ALS in Cyprus. Methods: All registered Cypriot ALS patients in the Republic of Cyprus from January 1985 until December 2014 were included. Socio-demographic information was extracted from patient files. Results: The study identified 179 ALS patients, of whom 7 had a positive family history. The mean age at onset was 58.6 years and a slight male predominance was observed. Average annual crude incidence was 1.26 cases/100,000 person-years and at the beginning of 2015, prevalence of ALS was 7.9 cases/100,000 population. Both incidence and prevalence displayed an increasing trend, even after age-standardization of incidence rates. Conclusions: Incidence, prevalence and main socio-demographic characteristics of ALS in Cyprus were similar to those of other European countries, without any geographic clustering of the disease. Additionally, an increased incidence through the years was confirmed. However, observations such as a higher male prevalence and a younger mean age of onset compared to published literature require further investigation.

scholarly journals A Retrospective Study of the Clinical Phenotype and Predictors of Survival in non-Caucasian Hispanic Patients with Amyotrophic Lateral Sclerosis.

2019 ◽  
Author(s):  
Claudia Marisol Sánchez-Martínez ◽  
José Alberto Choreño-Parra ◽  
Lilia Nuñez-Orozco ◽  
Noel Isaías Placencia-Álvarez ◽  
Laura Marcela Alvis-Cataño ◽  
...  
Keyword(s):  
Overall Survival ◽  
Amyotrophic Lateral Sclerosis ◽  
Prognostic Factors ◽  
Retrospective Study ◽  
Survival Time ◽  
Clinical Characteristics ◽  
Clinical Phenotype ◽  
Age At Onset ◽  
Als Patients ◽  
Lateral Sclerosis

Abstract Background. Little is known about the clinical phenotype of amyotrophic lateral sclerosis (ALS) in non-Caucasian populations. Here, we aimed to describe the clinical characteristics, prognostic factors and survival of Mexican patients with ALS. Methods. We conducted a retrospective study by reviewing the medical records of patients with ALS that attended and were regularly followed at a third level hospital in Mexico City from 2000 to 2015. We calculated absolute and relative frequencies of the clinical characteristics from all the participants. We also estimated correlation coefficients between clinical features and overall survival. Additionally, survival rates were compared for all participants grouped according to different clinical features using the Kaplan-Meier method and the log-rank test. Results. We enrolled 45 ALS patients, 53.33% had spinal-onset ALS and 46.66% presented bulbar ALS. The male/female ratio was 0.8. The mean age at onset of symptoms was 58.11 years. Mean survival time from onset was 64.73 ± 34.83 months. Cumulative survival rate after 5 years of disease onset was 44.44%. Age at onset and age at diagnosis inversely correlated with overall survival time. Also, we found that bulbar-onset, short diagnostic delay, percutaneous endoscopic gastrostomy, mechanical ventilation, and lower total cholesterol serum levels were associated with short survival. Conclusions. The clinical characteristics of Mexican ALS patients differ from the disease phenotype observed in Caucasians. Nonetheless, the predictive value of certain well-recognized prognostic factors remains consistent in our population. The current study provides relevant information for a better understanding of prognostic factors in ALS patients from Mexico and other Latin American countries.

scholarly journals Evaluation of Peripheral Immune Activation in Amyotrophic Lateral Sclerosis

2020 ◽  
Author(s):  
Mengli Wang ◽  
Zhen Liu ◽  
Juan Du ◽  
Yanchun Yuan ◽  
Bin Jiao ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Neurodegenerative Diseases ◽  
Early Onset ◽  
Late Onset ◽  
Meta Analysis ◽  
Age At Onset ◽  
Serum Levels ◽  
Patient Groups ◽  
Als Patients ◽  
Lateral Sclerosis

Abstract Background: Accumulating evidence has revealed that immunity plays an important role in amyotrophic lateral sclerosis (ALS) progression. However, the results regarding the serum levels of immunoglobulin and complement are inconsistent in patients with ALS. Although immune dysfunctions have also been reported in patients with other neurodegenerative diseases, few studies have explored whether immune dysfunction in ALS is similar to that in other neurodegenerative diseases.Methods: Serum levels of immunoglobulin and complement were measured in 245 patients with ALS, 65 patients with multiple system atrophy (MSA), 60 patients with Parkinson’s disease (PD), and 82 healthy controls (HCs). A meta-analysis including data from this study was performed to evaluate the differences in the levels of immunoglobulin and complement between ALS patients and HCs. The serum levels of immunoglobulin and complement were compared between patient groups and HCs or between ALS patient groups established by age at onset, site at onset, disease duration, or disease severity. The correlations between the levels of immunoglobulin and complement and the clinical characteristics of ALS were analysed using Spearman correlation analysis.Results: The pooled results showed that patients with ALS had higher C4 levels than did HCs, and no significant differences between these two groups in IgG, IgA, IgM, or C3 levels were found. Multiple comparisons revealed that there were no significant differences between patients with ALS and other neurodegenerative diseases in IgG, IgA, IgM, C3, or C4 levels. In addition, the IgG levels were lower in early-onset ALS patients than in late-onset ALS patients and HCs. The correlations between age at onset of ALS and IgG and IgA levels were significantly positive. Moreover, spinal-onset ALS patients had lower serum IgG levels than did HCs, but no difference was found between bulbar-onset ALS patients and HCs.Conclusions: Peripheral immunity abnormalities existed in patients with ALS, and lower IgG levels were associated with early-onset ALS.

scholarly journals Evaluation of Peripheral Immune Activation in Amyotrophic Lateral Sclerosis

2021 ◽  
Vol 12 ◽  
Author(s):  
Mengli Wang ◽  
Zhen Liu ◽  
Juan Du ◽  
Yanchun Yuan ◽  
Bin Jiao ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Neurodegenerative Diseases ◽  
Late Onset ◽  
Meta Analysis ◽  
Age At Onset ◽  
Serum Levels ◽  
Als Patients ◽  
Immune Dysfunctions ◽  
Lateral Sclerosis

Accumulating evidence has revealed that immunity plays an important role in amyotrophic lateral sclerosis (ALS) progression. However, the results regarding the serum levels of immunoglobulin and complement are inconsistent in patients with ALS. Although immune dysfunctions have also been reported in patients with other neurodegenerative diseases, few studies have explored whether immune dysfunction in ALS is similar to that in other neurodegenerative diseases. Therefore, we performed this study to address these gaps. In the present study, serum levels of immunoglobulin and complement were measured in 245 patients with ALS, 65 patients with multiple system atrophy (MSA), 60 patients with Parkinson's disease (PD), and 82 healthy controls (HCs). Multiple comparisons revealed that no significant differences existed between patients with ALS and other neurodegenerative diseases in immunoglobulin and complement levels. Meta-analysis based on data from our cohort and eight published articles was performed to evaluate the serum immunoglobulin and complement between patients with ALS and HCs. The pooled results showed that patients with ALS had higher C4 levels than HCs. In addition, we found that the IgG levels were lower in early-onset ALS patients than in late-onset ALS patients and HCs, and the correlations between age at onset of ALS and IgG or IgA levels were significant positive. In conclusion, our data supplement existing literature on understanding the role of peripheral immunity in ALS.

scholarly journals What causes amyotrophic lateral sclerosis?

F1000Research ◽  
2017 ◽  
Vol 6 ◽  
pp. 371 ◽  
Author(s):  
Sarah Martin ◽  
Ahmad Al Khleifat ◽  
Ammar Al-Chalabi
Keyword(s):  
Risk Factors ◽  
Amyotrophic Lateral Sclerosis ◽  
Respiratory Failure ◽  
Neurodegenerative Disease ◽  
Motor Neurons ◽  
Environmental Risk ◽  
Age Of Onset ◽  
Genetic Associations ◽  
Male Predominance ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis is a neurodegenerative disease predominantly affecting upper and lower motor neurons, resulting in progressive paralysis and death from respiratory failure within 2 to 3 years. The peak age of onset is 55 to 70 years, with a male predominance. The causes of amyotrophic lateral sclerosis are only partly known, but they include some environmental risk factors as well as several genes that have been identified as harbouring disease-associated variation. Here we review the nature, epidemiology, genetic associations, and environmental exposures associated with amyotrophic lateral sclerosis.

scholarly journals A retrospective study of the clinical phenotype and predictors of survival in non-Caucasian Hispanic patients with amyotrophic lateral sclerosis

BMC Neurology ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Claudia Marisol Sánchez-Martínez ◽  
José Alberto Choreño-Parra ◽  
Lilia Nuñez-Orozco ◽  
Noel Placencia-Álvarez ◽  
Laura Marcela Alvis-Castaño ◽  
...  
Keyword(s):  
Overall Survival ◽  
Amyotrophic Lateral Sclerosis ◽  
Prognostic Factors ◽  
Retrospective Study ◽  
Survival Time ◽  
Clinical Characteristics ◽  
Clinical Phenotype ◽  
Age At Onset ◽  
Als Patients ◽  
Lateral Sclerosis

Abstract Background Little is known about the clinical phenotype of amyotrophic lateral sclerosis (ALS) in non-Caucasian populations. Here, we aimed to describe the clinical characteristics, prognostic factors and survival of Mexican patients with ALS. Methods We conducted a retrospective study by reviewing the medical records of patients with ALS that attended and were regularly followed at a third level hospital in Mexico City from 2000 to 2015. We calculated absolute and relative frequencies of the clinical characteristics from all the participants. We also estimated correlation coefficients between clinical features and overall survival. Additionally, survival rates were compared for all participants grouped according to different clinical features using the Kaplan-Meier method and the log-rank test. Results We enrolled 45 ALS patients, 53.33% had spinal-onset ALS and 46.66% presented bulbar ALS. The male/female ratio was 0.8. The mean age at onset of symptoms was 58.11 years. Mean survival time from onset was 64.73 ± 34.83 months. Cumulative survival rate after 5 years of disease onset was 44.44%. Age at onset and age at diagnosis inversely correlated with overall survival time. Also, we found that bulbar-onset, short diagnostic delay, percutaneous endoscopic gastrostomy, mechanical ventilation, and lower total cholesterol serum levels were associated with short survival. Conclusions The clinical characteristics of Mexican ALS patients differ from the disease phenotype observed in Caucasians. Nonetheless, the predictive value of certain well-recognized prognostic factors remains consistent in our population. The current study provides relevant information for a better understanding of prognostic factors in ALS patients from Mexico and other Latin American countries.

scholarly journals A Retrospective Study of the Clinical Phenotype and Predictors of Survival in non-Caucasian Hispanic Patients with Amyotrophic Lateral Sclerosis.

2019 ◽  
Author(s):  
Claudia Marisol Sánchez-Martínez ◽  
José Alberto Choreño-Parra ◽  
Lilia Nuñez-Orozco ◽  
Noel Isaías Placencia-Álvarez ◽  
Laura Marcela Alvis-Cataño ◽  
...  
Keyword(s):  
Overall Survival ◽  
Amyotrophic Lateral Sclerosis ◽  
Prognostic Factors ◽  
Retrospective Study ◽  
Survival Time ◽  
Latin American ◽  
Clinical Characteristics ◽  
Age At Onset ◽  
Als Patients ◽  
Lateral Sclerosis

Abstract Background. Little is known about the clinical behaviour of amyotrophic lateral sclerosis (ALS) in non-Caucasian populations. Here, we aimed to describe the clinical characteristics, prognostic factors and survival of Mexican patients with ALS. Methods. We conducted a retrospective study by reviewing the medical records of patients with ALS that attended and were regularly followed at a third level hospital in Mexico City from 2000 to 2015. We calculated absolute and relative frequencies of the clinical characteristics from all the participants. We also estimated correlation coefficients between continuous variables and overall survival. Additionally, survival rates were compared for all participants grouped according to different clinical features using the Kaplan-Meier method and the log-rank test. Results. We enrolled 45 ALS patients, 53.33% had spinal-onset and 46.66% presented bulbar ALS. The male/female ratio was 0.8. The mean age at onset of symptoms was 58.11 years. Mean survival time from onset was 64.73 ± 34.83 months. Cumulative survival rate after 5 years of disease onset was 44.44%. Age at onset and age at diagnosis inversely correlated with overall survival time. Also, we found that bulbar-onset, short diagnostic delay, percutaneous endoscopic gastrostomy, mechanical ventilation, and lower total cholesterol serum levels were associated with short survival. Conclusions. The clinical characteristics of Mexican ALS patients differ from the disease phenotype observed in Caucasians. Nonetheless, the predictive value of certain well-recognized clinical prognostic factors remains consistent in our population. The current study may provide relevant information for the management of ALS patients from Mexico and other Latin American countries.

scholarly journals A Retrospective Study of the Clinical Phenotype and Predictors of Survival in non-Caucasian Hispanic Patients with Amyotrophic Lateral Sclerosis.

2019 ◽  
Author(s):  
Claudia Marisol Sánchez-Martínez ◽  
José Alberto Choreño-Parra ◽  
Lilia Nuñez-Orozco ◽  
Noel Isaías Placencia-Álvarez ◽  
Laura Marcela Alvis-Cataño ◽  
...  
Keyword(s):  
Overall Survival ◽  
Amyotrophic Lateral Sclerosis ◽  
Prognostic Factors ◽  
Retrospective Study ◽  
Survival Time ◽  
Clinical Characteristics ◽  
Clinical Phenotype ◽  
Age At Onset ◽  
Als Patients ◽  
Lateral Sclerosis

Abstract Background. Little is known about the clinical phenotype of amyotrophic lateral sclerosis (ALS) in non-Caucasian populations. Here, we aimed to describe the clinical characteristics, prognostic factors and survival of Mexican patients with ALS. Methods. We conducted a retrospective study by reviewing the medical records of patients with ALS that attended and were regularly followed at a third level hospital in Mexico City from 2000 to 2015. We calculated absolute and relative frequencies of the clinical characteristics from all the participants. We also estimated correlation coefficients between clinical features and overall survival. Additionally, survival rates were compared for all participants grouped according to different clinical features using the Kaplan-Meier method and the log-rank test. Results. We enrolled 45 ALS patients, 53.33% had spinal-onset ALS and 46.66% presented bulbar ALS. The male/female ratio was 0.8. The mean age at onset of symptoms was 58.11 years. Mean survival time from onset was 64.73 ± 34.83 months. Cumulative survival rate after 5 years of disease onset was 44.44%. Age at onset and age at diagnosis inversely correlated with overall survival time. Also, we found that bulbar-onset, short diagnostic delay, percutaneous endoscopic gastrostomy, mechanical ventilation, and lower total cholesterol serum levels were associated with short survival. Conclusions. The clinical characteristics of Mexican ALS patients differ from the disease phenotype observed in Caucasians. Nonetheless, the predictive value of certain well-recognized prognostic factors remains consistent in our population. The current study provides relevant information for a better understanding of prognostic factors in ALS patients from Mexico and other Latin American countries.

scholarly journals FUS mutations dominate TBK1 mutations in FUS/TBK1 double-mutant ALS/FTD pedigrees

Neurogenetics ◽  
2021 ◽  
Author(s):  
David Brenner ◽  
Kathrin Müller ◽  
Serena Lattante ◽  
Rüstem Yilmaz ◽  
Antje Knehr ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Correlation Analysis ◽  
Double Mutant ◽  
Disease Duration ◽  
Late Onset ◽  
Age At Onset ◽  
Phenotype Correlation ◽  
Whole Exome ◽  
Als Patients ◽  
Lateral Sclerosis

AbstractMutations in FUS and TBK1 often cause aggressive early-onset amyotrophic lateral sclerosis (ALS) or a late-onset ALS and/or frontotemporal dementia (FTD) phenotype, respectively. Co-occurrence of mutations in two or more Mendelian ALS/FTD genes has been repeatedly reported. However, little is known how two pathogenic ALS/FTD mutations in the same patient interact to shape the final phenotype. We screened 28 ALS patients with a known FUS mutation by whole-exome sequencing and targeted evaluation for mutations in other known ALS genes followed by genotype–phenotype correlation analysis of FUS/TBK1 double-mutant patients. We report on new and summarize previously published FUS and TBK1 double-mutant ALS/FTD patients and their families. We found that, within a family, mutations in FUS cause ALS while TBK1 single mutations are observed in FTD patients. FUS/TBK1 double mutations manifested as ALS and without a manifest difference regarding age at onset and disease duration when compared to FUS single-mutant individuals. In conclusion, TBK1 and FUS variants do not seem to interact in a simple additive way. Rather, the phenotype of FUS/TBK1 double-mutant patients appears to be dominated by the FUS mutation.

scholarly journals Prognostic Role of CSF β-amyloid 1–42/1–40 Ratio in Patients Affected by Amyotrophic Lateral Sclerosis

2021 ◽  
Vol 11 (3) ◽  
pp. 302
Author(s):  
Tiziana Colletti ◽  
Luisa Agnello ◽  
Rossella Spataro ◽  
Lavinia Guccione ◽  
Antonietta Notaro ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Prognostic Biomarker ◽  
Age At Onset ◽  
Rate Of Progression ◽  
Amyloid Aβ ◽  
Β Amyloid ◽  
Als Patients ◽  
State Examination ◽  
Lateral Sclerosis

The involvement of β-amyloid (Aβ) in the pathogenesis of amyotrophic lateral sclerosis (ALS) has been widely discussed and its role in the disease is still a matter of debate. Aβ accumulates in the cortex and the anterior horn neurons of ALS patients and seems to affect their survival. To clarify the role of cerebrospinal fluid (CSF) Aβ 1–42 and Aβ 42/40 ratios as a potential prognostic biomarker for ALS, we performed a retrospective observational study on a cohort of ALS patients who underwent a lumbar puncture at the time of the diagnosis. CSF Aβ 1–40 and Aβ 1–42 ratios were detected by chemiluminescence immunoassay and their values were correlated with clinical features. We found a significant correlation of the Aβ 42/40 ratio with age at onset and Mini Mental State Examination (MMSE) scores. No significant correlation of Aβ 1–42 or Aβ 42/40 ratios to the rate of progression of the disease were found. Furthermore, when we stratified patients according to Aβ 1–42 concentration and the Aβ 42/40 ratio, we found that patients with a lower Aβ 42/40 ratio showed a shorter survival. Our results support the hypothesis that Aβ 1–42 could be involved in some pathogenic mechanism of ALS and we suggest the Aβ 42/40 ratio as a potential prognostic biomarker.

scholarly journals A single case study: myoelectrically controlled exoskeletal mobilizer for amyotrophic lateral sclerosis (ALS) patients

1989 ◽  
Vol 13 (3) ◽  
pp. 145-148 ◽  
Author(s):  
W. F. Sauter ◽  
G. Bush ◽  
J. Sommerville
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Age Of Onset ◽  
Muscular Atrophy ◽  
Single Case ◽  
Clinical Pathology ◽  
Single Case Study ◽  
Progressive Muscle Weakness ◽  
Als Patients ◽  
Lateral Sclerosis

Introduction and clinical pathology of Amyotrophic Lateral Sclerosis (ALS) Amyotrophic Lateral Sclerosis (ALS) also known as Lou Gehrig's disease is the generic name for progressive muscular atrophy and bulbar palsy. It refers to all disorders of the cortico spinal pathways which are characterized by progressive muscle weakness. After multiple sclerosis, ALS is the most common purely neurological disorder (Janiszewski et al. 1983). Information available from the ALS Society indicates that the most common age of onset is 55 years.

Sign in / Sign up

Export Citation Format

Share Document