scholarly journals A Cross-sectional population-based investigation into behavioral change in amyotrophic lateral sclerosis: subphenotypes, staging, cognitive predictors, and survival

2017 ◽  
Vol 4 (5) ◽  
pp. 305-317 ◽  
Author(s):  
Tom Burke ◽  
Marta Pinto-Grau ◽  
Katie Lonergan ◽  
Peter Bede ◽  
Meabhdh O'Sullivan ◽  
...  
2015 ◽  
Vol 44 (2) ◽  
pp. 91-98 ◽  
Author(s):  
Federica Edith Pisa ◽  
Giancarlo Logroscino ◽  
Anica Casetta ◽  
Laura Cecotti ◽  
Lorenzo Verriello ◽  
...  

Background: The prevalent use of antidepressants (ATDs) in patients with Amyotrophic Lateral Sclerosis (ALS) varies across cross-sectional and clinic-based published studies. This population-based cohort study assesses the real-world prevalence of the use of ATDs, its trajectory and the association of incident use with clinical characteristics. Methods: All patients with incident ALS in the Friuli Venezia Giulia region, Italy, from 2002 to 2009, were identified through multiple sources including health databases. Diagnosis was validated through clinical documentation review. ATDs prescriptions from 2000 to 2011 were obtained from regional database. The trajectory was estimated through generalized estimating equations for repeated measures logistic regression and the Hazard ratio (HR) of initiating ATDs through multivariate proportional hazard Cox regression. Results: In this cohort of 261 ALS cases, age-, sex-adjusted prevalence of the use of ATDs was 37.3%, higher than in general population. The trajectory increased by 16% in 1-year period across diagnosis. Age ≤67 years at diagnosis (HR 1.28, 95% CI 0.84-1.95) and bulbar onset (1.43, 95% CI 0.90-2.26) were positively associated with initiating ATDs after diagnosis. Conclusions: More than one-third of patients used ATDs. Depression may occur more frequently than previously reported. Depression may precede motor alterations and be related to both ALS diagnosis and progression.


2018 ◽  
Vol 4 (3) ◽  
pp. e239 ◽  
Author(s):  
Marie Ryan ◽  
Mark Heverin ◽  
Mark A. Doherty ◽  
Nicola Davis ◽  
Emma M. Corr ◽  
...  

ObjectiveTo assess temporal trends in familial amyotrophic lateral sclerosis (FALS) incidence rates in an Irish population and to determine factors influencing FALS ascertainment.MethodsPopulation-based data collected over 23 years, using the Irish amyotrophic lateral sclerosis (ALS) register and DNA biobank, were analyzed and age-standardized rates of FALS and associated familial neuropsychiatric endophenotypes were identified.ResultsBetween 1994 and 2016, 269 patients with a family history of ALS from 197 unique families were included on the register. Using stringent diagnostic criteria for FALS, the mean age-standardized FALS incidence rate for the study period was 11.1% (95% confidence interval [CI], 8.8–13.4). The FALS incidence rate increased steadily from 5.2% in 1994 to 19.1% in 2016, an annual increase of 0.7% (95% CI, 0.5–0.9, p < 0.0001). Inclusion of the presence of neuropsychiatric endophenotypes within kindreds increased the FALS incidence rate to 30%. The incidence of FALS in newly diagnosed individuals from known families increased significantly with time, accounting for 50% of all FALS diagnoses by 2016. The mean annual rate of recategorization from “sporadic ALS” to “FALS” was 3% (95% CI, 2.6–3.8).ConclusionsThe true population-based rate of FALS is at least 20%. Inclusion of extended endophenotypes within kindreds increases the rate of FALS to 30%. Cross-sectional analysis of clinic-based cohorts and stringent definitions of FALS underestimate the true rate of familial disease. This has implications for genetic counseling and in the recognition of presymptomatic stages of ALS.


2015 ◽  
Vol 16 (5-6) ◽  
pp. 324-330 ◽  
Author(s):  
Elisabetta Pupillo ◽  
Elisa Bianchi ◽  
Paolo Messina ◽  
Luca Chiveri ◽  
Christian Lunetta ◽  
...  

Author(s):  
Thais Pousada ◽  
Jessica Garabal-Barbeira ◽  
Cristina Martínez ◽  
Betania Groba ◽  
Laura Nieto-Riveiro ◽  
...  

(1) Background: The study is focused on the implementation of outcome measurement tools to assess the impact of an assistive device from a loan bank in the lives of people with Amyotrophic Lateral Sclerosis and Neuromuscular Diseases. The secondary purpose is to analyse the correct matching between the person and technology, derived from the counselling of an occupational therapist. (2) Methods: Cross-sectional study. The sample was formed by 28 people with rare neurodegenerative disorders. A specific questionnaire, the Psychosocial Impact of Assistive Device Scale (PIADS), and the Matching Person and Technology (MPT) tool were applied to collect the data. (3) Results: The dimension of the PIADS with the best score was competence, and the variations according to gender were not remarkable. The three dimensions of the PIADS (competence, adaptability, and self-esteem) were correlated positively between them and with the mean score of the MPT tool (p < 0.01). The type of assistive technology (AT), diagnosis, and correct match between person–technology are the main factors that condition a positive impact. (4) Conclusions: The results noted the importance of assessing the needs, demands, and contexts of people with rare neurodegenerative diseases to prescribe the best AT. Loan banks of AT have to be considered a valid service that complements their lack in public health services.


2021 ◽  
pp. jnnp-2020-324992
Author(s):  
Emmet Costello ◽  
James Rooney ◽  
Marta Pinto-Grau ◽  
Tom Burke ◽  
Marwa Elamin ◽  
...  

BackgroundAmyotrophic lateral sclerosis (ALS) is often associated with cognitive and/or behavioural impairment. Cognitive reserve (CR) may play a protective role in offsetting cognitive impairment. This study examined the relationship between CR and longitudinal change in cognition in an Irish ALS cohort.MethodsLongitudinal neuropsychological assessment was carried out on 189 patients over 16 months using the Edinburgh cognitive and behavioural ALS screen (ECAS) and an additional battery of neuropsychological tests. CR was measured by combining education, occupation and physical activity data. Joint longitudinal and time-to-event models were fitted to investigate the associations between CR, performance at baseline and decline over time while controlling for non-random drop-out.ResultsCR was a significant predictor of baseline neuropsychological performance, with high CR patients performing better than those with medium or low CR. Better cognitive performance in high CR individuals was maintained longitudinally for ECAS, social cognition, executive functioning and confrontational naming. Patients displayed little cognitive decline over the course of the study, despite controlling for non-random drop-out.ConclusionsThese findings suggest that CR plays a role in the presentation of cognitive impairment at diagnosis but is not protective against cognitive decline. However, further research is needed to examine the interaction between CR and other objective correlates of cognitive impairment in ALS.


2016 ◽  
Vol 23 (9) ◽  
pp. 1426-1432 ◽  
Author(s):  
D. Ferraro ◽  
D. Consonni ◽  
N. Fini ◽  
A. Fasano ◽  
C. Del Giovane ◽  
...  

2013 ◽  
Vol 84 (8) ◽  
pp. 940-941 ◽  
Author(s):  
P. T. C. van Doormaal ◽  
A. Gallo ◽  
W. van Rheenen ◽  
J. H. Veldink ◽  
M. A. van Es ◽  
...  

Author(s):  
Muhammad Shahid Iqbal ◽  
Salah-Ud-Din Khan ◽  
Eldowaik Mohamed Salah Saad ◽  
Muhammad Zahid Iqbal

Objective: The objective of the study was to evaluate the knowledge of ALS among students in a university in Malaysia. Methods: A cross-sectional and observational study was performed among the students of three different healthcare provider faculties (Medical, Pharmacy and Dental) in a university with the help of pre-validated research questionnaire. The Statistical Package for Social Science (SPSS) Version 24.0 was used to analyze and present the data. Results: A total of 268 university students from three faculties participated in the current study. The medical faculty students and final year students had more appropriate knowledge towards the ALS. Conclusion: Overall appropriate knowledge was observed among the studied faculty students. The present study concluded that medical students had adequate knowledge of ALS than the other two faculty students.


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