Clinical features and prognosis of amyotrophic lateral sclerosis in Africa: the TROPALS study

2018 ◽  
Vol 90 (1) ◽  
pp. 20-29 ◽  
Author(s):  
Jaime Luna ◽  
Mouhamadou Diagana ◽  
Leila Ait Aissa ◽  
Meriem Tazir ◽  
Lamia Ali Pacha ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Clinical Features ◽  
Median Survival ◽  
Age At Onset ◽  
Young Patients ◽  
Northern Africa ◽  
Cox Proportional Hazards Model ◽  
Female Ratio ◽  
Western Africa ◽  
Lateral Sclerosis

ObjectiveWe describe and compare the sociodemographic and clinical features, treatments, and prognoses and survival times of patients with amyotrophic lateral sclerosis (ALS) in Africa.MethodologyWe conducted a multicentre, hospital-based cohort study in Africa. Patients with ALS diagnosed in the neurology departments of participating hospitals from 2005 to 2017 were included. Subgroup analysis was performed by subcontinent. Survival analyses were conducted using the Cox proportional hazards model.ResultsNine centres from eight African countries participated. A total of 185 patients with ALS were included: 114 from Northern Africa, 41 from Western Africa and 30 from Southern Africa. A male predominance (male to female ratio 2.9) was evident. The median age at onset was 53.0 years (IQR 44.5–64.0 years). The onset was bulbar in 22.7%. Only 47 patients (26.3%) received riluzole, mainly in Northern and Western Africa. The median survival from the time of diagnosis was 14.0 months (95% CI 10.7 to 17.2 months). The median survival was longer in Northern Africa (19.0 months, 95% CI 10.8 to 27.2 months) than in Western (4.0 months, 95% CI 0.8 to 7.1 months) and Southern (11.0 months, 95% CI 5.6 to 16.4 months) Africa (Breslow test, p<0.0001). Both subcontinental location and riluzole treatment independently affected survival.ConclusionMore African patients with ALS were male and younger and exhibited a lower proportion of bulbar onset compared with patients with ALS from Western nations. Survival was consistent with that in Western registers but far shorter than what would be expected for young patients with ALS. The research improves our understanding of the disease in Africa.

Age at onset influences on wide-ranged clinical features of sporadic amyotrophic lateral sclerosis

2009 ◽  
Vol 276 (1-2) ◽  
pp. 163-169 ◽  
Author(s):  
Naoki Atsuta ◽  
Hirohisa Watanabe ◽  
Mizuki Ito ◽  
Fumiaki Tanaka ◽  
Akiko Tamakoshi ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Clinical Features ◽  
Age At Onset ◽  
Sporadic Amyotrophic Lateral Sclerosis ◽  
Lateral Sclerosis

scholarly journals Depression and anxiety in a case series of amyotrophic lateral sclerosis: frequency and association with clinical features

2017 ◽  
Vol 15 (1) ◽  
pp. 58-60 ◽  
Author(s):  
Laura de Godoy Rousseff Prado ◽  
Isabella Carolina Santos Bicalho ◽  
Mauro Vidigal-Lopes ◽  
Vitor de Godoy Rousseff Prado ◽  
Rodrigo Santiago Gomez ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Depressive Symptoms ◽  
Clinical Features ◽  
Disease Duration ◽  
Age At Onset ◽  
Anxiety And Depression ◽  
Functional Score ◽  
Significant Difference ◽  
Clinically Significant ◽  
Lateral Sclerosis

ABSTRACT Objective To investigate the frequency of anxiety and depression and their association with clinical features of amyotrophic lateral sclerosis. Methods This is a cross-sectional and descriptive study including a consecutive series of patients with sporadic amyotrophic lateral sclerosis according to Awaji’s criteria. Patients underwent clinical and psychiatric assessment (anxiety and depression symptoms). Results We included 76 patients. The men/women ratio was 1.6:1. Participants’ mean age at disease onset was 55 years (SD±12.1). Sixty-six patients (86.8%) were able to complete psychiatric evaluation. Clinically significant anxiety was found in 23 patients (34.8%) while clinically significant depression was found in 24 patients (36.4%). When we compared patients with and without depression a significant difference was seen only in the frequency of anxiety symptoms (p<0.001). We did further analysis comparing subgroups of patients classified according to the presence or not of anxiety and or depression, without any significant difference regarding sex, age at onset, initial form, disease duration or functional measures. A positive correlation between anxiety and depressive symptoms was found (p<0.001). Conclusion Anxiety and depressive symptoms were highly correlated and frequent in patients with amyotrophic lateral sclerosis. In addition, anxiety and depression were not associated with disease duration and presentation, sex, age at onset, and functional score.

The hypometabolic state: a good predictor of a better prognosis in amyotrophic lateral sclerosis

2021 ◽  
pp. jnnp-2021-326184
Author(s):  
Marina Cattaneo ◽  
Pierre Jesus ◽  
Andrea Lizio ◽  
Philippe Fayemendy ◽  
Nicoletta Guanziroli ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Indirect Calorimetry ◽  
Age At Onset ◽  
Predictive Values ◽  
Female Ratio ◽  
Non Invasive ◽  
Non Invasive Ventilation ◽  
Male Female Ratio ◽  
Hypometabolic State ◽  
Lateral Sclerosis

BackgroundMalnutrition and weight loss are negative prognostic factors for survival in patients with amyotrophic lateral sclerosis (ALS). However, energy expenditure at rest (REE) is still not included in clinical practice, and no data are available concerning hypometabolic state in ALS.ObjectiveTo evaluate in a referral cohort of patients with ALS the prevalence of hypometabolic state as compared with normometabolic and hypermetabolic states, and to correlate it with clinical phenotype, rate of progression and survival.DesignWe conducted a retrospective study examining REE measured by indirect calorimetry in patients with ALS referred to Milan, Limoges and Tours referral centres between January 2011 and December 2017. Hypometabolism and hypermetabolism states were defined when REE difference between measured and predictive values was ≤−10% and ≥10%, respectively. We evaluated the relationship between these metabolic alterations and measures of body composition, clinical characteristics and survival.ResultsEight hundred forty-seven patients with ALS were recruited. The median age at onset was 63.79 years (IQR 55.00–71.17). The male/female ratio was 1.26 (M/F: 472/375). Ten per cent of patients with ALS were hypometabolic whereas 40% were hypermetabolic. Hypometabolism was significantly associated with later need for gastrostomy, non-invasive ventilation and tracheostomy placement. Furthermore, hypometabolic patients with ALS significantly outlived normometabolic (HR=1.901 (95% CI 1.080 to 3.345), p=0.0259) and hypermetabolic (HR=2.138 (95% CI 1.154 to 3.958), p=0.0157) patients.ConclusionHypometabolism in ALS is not uncommon and is associated with slower disease progression and better survival than normometabolic and hypermetabolic subjects. Indirect calorimetry should be performed at least at time of diagnosis because alterations in metabolism are correlated with prognosis.

scholarly journals Correlation of Creatine Kinase Levels with Clinical Features and Survival in Amyotrophic Lateral Sclerosis

2017 ◽  
Vol 8 ◽  
Author(s):  
Hongfei Tai ◽  
Liying Cui ◽  
Yuzhou Guan ◽  
Mingsheng Liu ◽  
Xiaoguang Li ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Creatine Kinase ◽  
Clinical Features ◽  
Lateral Sclerosis

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.

Clinical features of amyotrophic lateral sclerosis in south-west China

2015 ◽  
Vol 16 (7-8) ◽  
pp. 512-519 ◽  
Author(s):  
Qianqian Wei ◽  
Xueping Chen ◽  
Zhenzhen Zheng ◽  
Rui Huang ◽  
Xiaoyan Guo ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Clinical Features ◽  
West China ◽  
South West ◽  
Lateral Sclerosis

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

2019 ◽  
Vol 90 (6) ◽  
pp. 666-673 ◽  
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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