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.

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

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.

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 Informal Caregiving in Amyotrophic Lateral Sclerosis (ALS): A High Caregiver Burden and Drastic Consequences on Caregivers’ Lives

2021 ◽  
Vol 11 (6) ◽  
pp. 748
Author(s):  
Pavel Schischlevskij ◽  
Isabell Cordts ◽  
René Günther ◽  
Benjamin Stolte ◽  
Daniel Zeller ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Functional Status ◽  
Disease Severity ◽  
Caregiver Burden ◽  
Rating Scale ◽  
Depression Scale ◽  
Anxiety And Depression ◽  
Daily Routine ◽  
Work Lives ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that causes progressive autonomy loss and need for care. This does not only affect patients themselves, but also the patients’ informal caregivers (CGs) in their health, personal and professional lives. The big efforts of this multi-center study were not only to evaluate the caregivers’ burden and to identify its predictors, but it also should provide a specific understanding of the needs of ALS patients’ CGs and fill the gap of knowledge on their personal and work lives. Using standardized questionnaires, primary data from patients and their main informal CGs (n = 249) were collected. Patients’ functional status and disease severity were evaluated using the Barthel Index, the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) and the King’s Stages for ALS. The caregivers’ burden was recorded by the Zarit Burden Interview (ZBI). Comorbid anxiety and depression of caregivers were assessed by the Hospital Anxiety and Depression Scale. Additionally, the EuroQol Five Dimension Five Level Scale evaluated their health-related quality of life. The caregivers’ burden was high (mean ZBI = 26/88, 0 = no burden, ≥24 = highly burdened) and correlated with patients’ functional status (rp = −0.555, p < 0.001, n = 242). It was influenced by the CGs’ own mental health issues due to caregiving (+11.36, 95% CI [6.84; 15.87], p < 0.001), patients’ wheelchair dependency (+9.30, 95% CI [5.94; 12.66], p < 0.001) and was interrelated with the CGs’ depression (rp = 0.627, p < 0.001, n = 234), anxiety (rp = 0.550, p < 0.001, n = 234), and poorer physical condition (rp = −0.362, p < 0.001, n = 237). Moreover, female CGs showed symptoms of anxiety more often, which also correlated with the patients’ impairment in daily routine (rs = −0.280, p < 0.001, n = 169). As increasing disease severity, along with decreasing autonomy, was the main predictor of caregiver burden and showed to create relevant (negative) implications on CGs’ lives, patient care and supportive therapies should address this issue. Moreover, in order to preserve the mental and physical health of the CGs, new concepts of care have to focus on both, on not only patients but also their CGs and gender-associated specific issues. As caregiving in ALS also significantly influences the socioeconomic status by restrictions in CGs’ work lives and income, and the main reported needs being lack of psychological support and a high bureaucracy, the situation of CGs needs more attention. Apart from their own multi-disciplinary medical and psychological care, more support in care and patient management issues is required.

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

scholarly journals A Systematic Review of Genotype–Phenotype Correlation across Cohorts Having Causal Mutations of Different Genes in ALS

2020 ◽  
Vol 10 (3) ◽  
pp. 58 ◽  
Author(s):  
Owen Connolly ◽  
Laura Le Gall ◽  
Gavin McCluskey ◽  
Colette G Donaghy ◽  
William J Duddy ◽  
...  
Keyword(s):  
Systematic Review ◽  
Amyotrophic Lateral Sclerosis ◽  
Respiratory Failure ◽  
Motor Neuron ◽  
Motor Neurons ◽  
Disease Duration ◽  
Phenotype Correlation ◽  
Motor Neuron Diseases ◽  
Relationship Of ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis is a rare and fatal neurodegenerative disease characterised by progressive deterioration of upper and lower motor neurons that eventually culminates in severe muscle atrophy, respiratory failure and death. There is a concerning lack of understanding regarding the mechanisms that lead to the onset of ALS and as a result there are no reliable biomarkers that aid in the early detection of the disease nor is there an effective treatment. This review first considers the clinical phenotypes associated with ALS, and discusses the broad categorisation of ALS and ALS-mimic diseases into upper and lower motor neuron diseases, before focusing on the genetic aetiology of ALS and considering the potential relationship of mutations of different genes to variations in phenotype. For this purpose, a systematic review is conducted collating data from 107 original published clinical studies on monogenic forms of the disease, surveying the age and site of onset, disease duration and motor neuron involvement. The collected data highlight the complexity of the disease’s genotype–phenotype relationship, and thus the need for a nuanced approach to the development of clinical assays and therapeutics.

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