scholarly journals EEG functional network topology is associated with disability in patients with amyotrophic lateral sclerosis

2016 ◽  
Author(s):  
Matteo Fraschini ◽  
Matteo Demuru ◽  
Arjan Hillebrand ◽  
Lorenza Cuccu ◽  
Silvia Porcu ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Network Topology ◽  
Resting State ◽  
Minimum Spanning Tree ◽  
Clinical Status ◽  
Functional Network ◽  
Phase Lag ◽  
Beta Band ◽  
Significant Difference ◽  
Lateral Sclerosis

ABSTRACTAmyotrophic Lateral Sclerosis (ALS) is one of the most severe neurodegenerative diseases, which is known to affect upper and lower motor neurons. In contrast to the classical tenet that ALS represents the outcome of extensive and progressive impairment of a fixed set of motor connections, recent neuroimaging findings suggest that the disease spreads along vast non-motor connections. Here, we hypothesised that functional network topology is perturbed in ALS, and that this reorganisation is associated with disability. We tested this hypothesis in 21 patients affected by ALS at several stages of impairment using resting-state electroencephalography (EEG) and compared the results to 16 age-matched healthy controls. We estimated functional connectivity using the Phase Lag Index (PLI), and characterized the network topology using the minimum spanning tree (MST). We found a significant difference between groups in terms of MST dissimilarity and MST leaf fraction in the beta band. Moreover, some MST parameters (leaf, hierarchy and kappa) significantly correlated with disability. These findings suggest that the topology of resting-state functional networks in ALS is affected by the disease in relation to disability. EEG network analysis may be of help in monitoring and evaluating the clinical status of ALS patients.

scholarly journals EEG functional network topology is associated with disability in patients with amyotrophic lateral sclerosis

2016 ◽  
Vol 6 (1) ◽  
Author(s):  
Matteo Fraschini ◽  
Matteo Demuru ◽  
Arjan Hillebrand ◽  
Lorenza Cuccu ◽  
Silvia Porcu ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Network Topology ◽  
Functional Network ◽  
Lateral Sclerosis

The cortisol awakening response in amyotrophic lateral sclerosis is blunted and correlates with clinical status and depressive mood

2012 ◽  
Vol 37 (1) ◽  
pp. 20-26 ◽  
Author(s):  
Benno Roozendaal ◽  
Sungchul Kim ◽  
Oliver T. Wolf ◽  
Min Soo Kim ◽  
Kang-Keyng Sung ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Clinical Status ◽  
Depressive Mood ◽  
Cortisol Awakening Response ◽  
Lateral Sclerosis

Frontotemporal degeneration in amyotrophic lateral sclerosis (ALS): a longitudinal MRI one-year study

CNS Spectrums ◽  
2020 ◽  
pp. 1-10 ◽  
Author(s):  
Francesca Trojsi ◽  
Federica Di Nardo ◽  
Mattia Siciliano ◽  
Giuseppina Caiazzo ◽  
Cinzia Femiano ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Functional Connectivity ◽  
Disease Progression ◽  
Resting State ◽  
Diffusion Tensor ◽  
Middle Temporal Gyrus ◽  
Multisystem Disease ◽  
Longitudinal Changes ◽  
One Year ◽  
Lateral Sclerosis

Abstract Objective. Advanced neuroimaging techniques may offer the potential to monitor disease progression in amyotrophic lateral sclerosis (ALS), a neurodegenerative, multisystem disease that still lacks therapeutic outcome measures. We aim to investigate longitudinal functional and structural magnetic resonance imaging (MRI) changes in a cohort of patients with ALS monitored for one year after diagnosis. Methods. Resting state functional MRI, diffusion tensor imaging (DTI), and voxel-based morphometry analyses were performed in 22 patients with ALS examined by six-monthly MRI scans over one year. Results. During the follow-up period, patients with ALS showed reduced functional connectivity only in some extramotor areas, such as the middle temporal gyrus in the left frontoparietal network after six months and in the left middle frontal gyrus in the default mode network after one year without showing longitudinal changes of cognitive functions. Moreover, after six months, we reported in the ALS group a decreased fractional anisotropy (P = .003, Bonferroni corrected) in the right uncinate fasciculus. Conversely, we did not reveal significant longitudinal changes of functional connectivity in the sensorimotor network, as well as of gray matter (GM) atrophy or of DTI metrics in motor areas, although clinical measures of motor disability showed significant decline throughout the three time points. Conclusion. Our findings highlighted that progressive impairment of extramotor frontotemporal networks may precede the appearance of executive and language dysfunctions and GM changes in ALS. Functional connectivity changes in cognitive resting state networks might represent candidate radiological markers of disease progression.

Inside minds, beneath diseases: social cognition in amyotrophic lateral sclerosis-frontotemporal spectrum disorder

2020 ◽  
Vol 91 (12) ◽  
pp. 1279-1282
Author(s):  
Patricia Lillo ◽  
Paulo Caramelli ◽  
Gada Musa ◽  
Teresa Parrao ◽  
Ricardo Hughes ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cognitive Impairment ◽  
Social Cognition ◽  
Digit Span ◽  
Short Version ◽  
The Social ◽  
Significant Difference ◽  
Similar Disease ◽  
Post Hoc ◽  
Lateral Sclerosis

ObjectiveTo compare social cognition performance between patients with amyotrophic lateral sclerosis (ALS) and those patients with behavioural variant frontotemporal dementia (bvFTD).MethodsWe included 21 participants with ALS, 20 with bvFTD and 21 healthy controls who underwent a comprehensive cognitive battery, including the short version of the Social Cognition and Emotional Assessment (Mini-SEA), which comprises the faux pas test and Facial Emotion Recognition Test (FERT); Mini-Mental State Examination; Frontal Assessment Battery; lexical fluency (F-A-S), category fluency (animals/minute), digit span (direct and backwards) tests and the Hayling test. A post hoc analysis was conducted with the patients with ALS divided into two subgroups: patients without cognitive impairment (ALScn; n=13) and patients with cognitive impairment (ALSci; n=8).ResultsNo significant difference was noted between participant groups in terms of the age, sex and education. ALS-total group and patients with bvFTD had similar disease durations. Patients with ALSci performed poorly when compared with controls with regard to the FERT (p<0.001), the faux pas (p<0.004) and the Mini-SEA (p<0.002) total scores. Moreover, patients with bvFTD performed poorly in comparison with controls in executive and social cognition tests. The performance of patients with ALSci was similar to that of patients with bvFTD, while the performance of patients with ALScn was similar to that of controls.DiscussionOur findings support a cognitive continuum between ALS and bvFTD and shed light on the cognitive heterogeneity of ALS, expanding its possible neuropsychological profiles.

Changes of Resting State Brain Networks in Amyotrophic Lateral Sclerosis

NeuroImage ◽  
2009 ◽  
Vol 47 ◽  
pp. S46
Author(s):  
B Mohammadi ◽  
K Kollewe ◽  
A Samii ◽  
K Krampfl ◽  
R Dengler ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Resting State ◽  
Brain Networks ◽  
Lateral Sclerosis

scholarly journals Factors associated with assisted ventilation use in amyotrophic lateral sclerosis: a nationwide population-based study in Korea

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Seo Yeon Yoon ◽  
Han-Kyoul Kim ◽  
Mi Ji Kim ◽  
Jee Hyun Suh ◽  
Ja-Ho Leigh
Keyword(s):  
Socioeconomic Status ◽  
Amyotrophic Lateral Sclerosis ◽  
Medical Condition ◽  
Management Strategies ◽  
Population Based ◽  
Assisted Ventilation ◽  
Invasive Ventilation ◽  
Factors Associated ◽  
Significant Difference ◽  
Lateral Sclerosis

AbstractFew studies have investigated the factors associated with assisted ventilation use in amyotrophic lateral sclerosis (ALS) in western countries with a relatively small number of participants. This study aimed to evaluate the factors associated with assisted ventilation use using a large nationwide cohort covering the entire Korean population. We selected patients with primary or secondary diagnoses of ALS (ICD-10 code: G12.21) and a registration code for ALS (V123) in the rare intractable disease registration program. Covariates included in the analyses were age, sex, socioeconomic status and medical condition. Factors associated with non-invasive ventilation (NIV) and tracheostomy invasive ventilation (TIV) were evaluated. Logistic regression analyses were performed using odds ratios and 95% confidence intervals. In total, 3057 patients with ALS were enrolled. During the 6-year follow-up period, 1228 (40%) patients started using assisted ventilation: 956 with NIV and 272 with TIV. There was no significant difference in the assisted ventilation use according to sex, whereas different patterns of discrepancies were noted between the sexes: Females living in non-metropolitan areas showed decreased use of assisted ventilation, whereas high income levels showed a positive relationship with assisted ventilation use only in males. Patients aged ≥ 70 years showed decreased use of NIV. NIV use was more affected by socioeconomic status than TIV, whereas TIV showed a significant relationship with medical conditions such as nasogastric tube insertion and gastrostomy. We found that various factors, including age, socioeconomic status, and medical condition, were related with assisted ventilation use. Understanding the pattern of assisted ventilation use would help set optimal management strategies in patients with ALS.

scholarly journals Prognosis of amyotrophic lateral sclerosis with cognitive and behavioural changes based on a sixty-month longitudinal follow-up

PLoS ONE ◽  
2021 ◽  
Vol 16 (8) ◽  
pp. e0253279
Author(s):  
Shan Ye ◽  
Pingping Jin ◽  
Lu Chen ◽  
Nan Zhang ◽  
Dongsheng Fan
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Survival Time ◽  
Progression Rate ◽  
Behavioural Changes ◽  
Significant Difference ◽  
Long Term Follow Up ◽  
Behaviour Changes ◽  
Als Patients ◽  
Lateral Sclerosis

Objective Approximately 50% of amyotrophic lateral sclerosis (ALS) patients have cognitive and behavioural dysfunction in varying degrees and forms. Previous studies have shown that cognitive and behavioural changes may indicate a poor prognosis, and cognitive function gradually deteriorates over the course of disease, but the results of different studies have been inconsistent. In addition, there are relatively limited long-term follow-up studies tracking death as an endpoint. The purpose of this study was to investigate the clinical prognostic characteristics of ALS patients with cognitive behavioural changes through long-term follow-up in a cohort. Methods A total of 87 ALS patients from 2014 to 2015 in the Third Hospital of Peking University were selected and divided into a pure ALS group, an ALS with behavioural variant of frontotemporal dementia (ALS-bvFTD) group, and an ALS with cognitive and behaviour changes group. All patients were followed up for 60 months. The main end point was death and tracheotomy. Results There was no significant difference in survival curve between pure ALS and ALS with cognitive and behavioural change group, but the survival time of ALS-bvFTD group was significantly lower than the other two groups (P < 0.001). For those who was followed up to the endpoint, the survival time of the ALS-bvFTD group was significantly shorter than that of the pure ALS group (t = 5.33, p < 0.001) or the ALS with cognitive and behaviour changes group (t = 4.25, p < 0.001). The progression rate of ALS Functional Rating Scale–Revised (FRS-R) scores from recruitment to endpoint was significantly faster in the ALS-bvFTD group than in the pure ALS group (z = 2.68, p = 0.01) or the ALS with cognitive and behavioural changes group (z = 2.75, p = 0.01). There was no significant difference in survival time (t = 0.52, P = 0.60) or FRS-R score progression rate (z = 0.31, p = 0.76) between the pure ALS group and the ALS with cognitive and behavioural changes group. The total Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen (ECAS) score was positively correlated with survival time (r = 0.38, p = 0.01). Conclusion ALS-bvFTD patients have shorter survival time. The total ECAS score may be correlated with survival time.

scholarly journals Poor Bone Quality in Patients With Amyotrophic Lateral Sclerosis

2020 ◽  
Vol 11 ◽  
Author(s):  
Jordi Caplliure-Llopis ◽  
Dolores Escrivá ◽  
María Benlloch ◽  
José Enrique de la Rubia Ortí ◽  
José María Estrela ◽  
...  
Keyword(s):  
Vitamin D ◽  
Amyotrophic Lateral Sclerosis ◽  
Bone Health ◽  
Clinical Status ◽  
Healthy Controls ◽  
Healthy Individuals ◽  
Bone Parameters ◽  
Als Patients ◽  
Lateral Sclerosis ◽  
Tsh Levels

Objective: Musculoskeletal functional deterioration in Amyotrophic lateral sclerosis (ALS) is associated with an increase in bone fractures. The purpose of this study was to evaluate the influence of sex, ALS type, on bone quality in patients with ALS compared to healthy controls. The impact on bone health of the clinical status and some metabolic parameters was also analyzed in ALS patients.Methods: A series of 33 voluntary patients with ALS, and 66 healthy individuals matched in sex and age underwent assessment of bone mass quality using quantitative ultrasound (QUS) of the calcaneus. Ultrasonic broadband attenuation (BUA), the speed of sound (SOS), stiffness index and T-score were measured. Bone mineral density (BMD) was estimated using standard equations. Apart from fat and muscle mass percentage determinations, clinical baseline measures in ALS patients included ALSFRS-R score, Barthel index for activities of daily living, pulmonary function measured using FVC, and muscular strength assessed by a modified MRC grading scale. Laboratory tests included serum calcium, 25-HO-cholecalciferol (Vitamin D), alkaline phosphatase (ALP), T4 and TSH.Results: All bone parameters evaluated were statistically significant lower in ALS patients than in healthy controls. ALS females showed significantly lower bone parameters than healthy females. According to the estimated BMD, there were 25 ALS patients (75.8%) and 36 (54.5%) healthy individuals showing an osteoporotic profile (BMD &lt;0.700 g/cm2). Only 16.7% of the ALS females had T-scores indicative of healthy bones. There was no correlation between any of the clinical parameters analyzed and the bone QUS measurements. Vitamin D and TSH levels positively correlated with all the bone parameters.Conclusions: This study confirms that ALS patients, particularly females, exhibited deteriorated bone health as compared to healthy individuals. These structural bone changes were independent of ALS subtype and clinical status. Bone health in ALS patients seems to be related to certain metabolic parameters such as Vitamin D and TSH levels.

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