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

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Genetic and clinical features of Chinese sporadic amyotrophic lateral sclerosis patients with TARDBP mutations

Brain and Behavior ◽

10.1002/brb3.2312 ◽

2021 ◽

Author(s):

Feng Feng ◽

Hongfen Wang ◽

Jiajin Liu ◽

Zhanjun Wang ◽

Baixuan Xu ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Clinical Features ◽

Sporadic Amyotrophic Lateral Sclerosis ◽

Lateral Sclerosis

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

Einstein (São Paulo) ◽

10.1590/s1679-45082017ao3870 ◽

2017 ◽

Vol 15 (1) ◽

pp. 58-60 ◽

Cited By ~ 8

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.

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Excessive daytime sleepiness in Chinese patients with sporadic amyotrophic lateral sclerosis and its association with cognitive and behavioural impairments

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2018-318810 ◽

2018 ◽

Vol 89 (10) ◽

pp. 1038-1043 ◽

Cited By ~ 4

Author(s):

Shuangwu Liu ◽

Yan Huang ◽

Hongfei Tai ◽

Kang Zhang ◽

Zhili Wang ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Clinical Features ◽

Excessive Daytime Sleepiness ◽

Daytime Sleepiness ◽

Rating Scale ◽

Chinese Patients ◽

Sporadic Amyotrophic Lateral Sclerosis ◽

Cross Sectional ◽

Wake Patterns ◽

Lateral Sclerosis

ObjectiveTo examine the frequency and clinical features of excessive daytime sleepiness (EDS) and its association with cognitive and behavioural impairments in patients with amyotrophic lateral sclerosis (ALS).MethodsWe conducted a cross-sectional investigation to explore the frequency and clinical features of EDS in a group of 121 Chinese patients with ALS compared with 121 age-matched and sex-matched healthy subjects. EDS was diagnosed using the Epworth Sleepiness Scale (ESS). Other characteristics of patients with ALS including sleep quality, REM sleep behaviour disorder (RBD), restless legs syndrome (RLS), cognition, behaviour, depression and anxiety were also evaluated.ResultsEDS was significantly more frequent in patients with ALS than in controls (26.4% vs 8.3%; p<0.05). Patients with ALS with EDS scored lower scores on the revised ALS Functional Rating Scale (ALSFRS-R), Mini-Mental State Examination (MMSE), Montreal Cognitive Assessment (MoCA) and MMSE and MoCA delayed memory subitems and higher on the Frontal Behavioural Inventory (FBI) than patients with ALS without EDS. ESS scores correlated with global ALSFRS-R, FBI, MMSE and MoCA scores and MMSE and MoCA delayed memory scores. RLS and global ALSFRS-R scores were independently associated with EDS in patients with ALS.ConclusionsWe identified a high frequency of EDS symptoms in Chinese patients with ALS, and these patients might have more serious physical, cognitive and frontal behaviour impairment. Patients with ALS might improve quality of life from the timely recognition and optimised management of EDS symptoms. Our results further suggest that ALS is a heterogeneous disease that might exhibit abnormal sleep-wake patterns.

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Faculty Opinions recommendation of Novel optineurin mutations in patients with familial and sporadic amyotrophic lateral sclerosis.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.12796962.14073060 ◽

2011 ◽

Author(s):

Guy Rouleau

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Sporadic Amyotrophic Lateral Sclerosis ◽

Lateral Sclerosis

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Glutathione S-transferase Omega 2 DD genotype is associated with an increased risk of sporadic amyotrophic lateral sclerosis in Chinese men

Journal of International Medical Research ◽

10.1177/03000605211033219 ◽

2021 ◽

Vol 49 (7) ◽

pp. 030006052110332

Author(s):

Zhiliang Fan ◽

Hong Jiang ◽

Xueqin Song ◽

Yansu Guo ◽

Xinying Tian

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Healthy Subjects ◽

Chinese Population ◽

Sporadic Amyotrophic Lateral Sclerosis ◽

Glutathione S Transferase ◽

Genotype Frequencies ◽

Sporadic Als ◽

Increased Risk ◽

Chi Squared ◽

Lateral Sclerosis

Objective To investigate whether GSTA1, GSTO2, and GSTZ1 are relevant to an increased risk of amyotrophic lateral sclerosis (ALS) in a Chinese population. Methods In this study, 143 sporadic ALS (sALS) patients (83 men, 60 women) and 210 age- and sex-matched healthy subjects were enrolled. Blood samples were collected by venipuncture. Genomic DNA was isolated by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) according to the manufacturer’s instructions. The potential associations between ALS and GSTA1, GSTO2, and GSTZ1 polymorphisms were estimated using chi-squared analysis and unconditional logistic regression. Results The D allele and genotype frequencies of GSTO2 were increased in sALS patients compared with healthy subjects, indicating that the GSTO2 DD genotype was associated with an increased risk of sALS (odds ratio [OR] = 3.294, 95% confidence interval [CI] = 1.039–10.448). However, a significant association between the DD genotype and the risk of sALS was evident in men only (OR = 7.167, 95% CI = 1.381–37.202). Conclusion This study revealed that the D allele and genotype frequencies of GSTO2 were increased in sALS patients. The GSTO2 DD genotype was associated with an increased risk of sALS in men in a Chinese population.

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