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

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 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 <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.

scholarly journals A Dynamic Bayesian Network model for simulation of disease progression in Amyotrophic Lateral Sclerosis patients

2017 ◽  
Author(s):  
Alessandro Zandonà ◽  
Matilde Francescon ◽  
Maya Bronfeld ◽  
Andrea Calvo ◽  
Adriano Chiò ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Amyotrophic Lateral Sclerosis ◽  
Bayesian Network ◽  
Disease Progression ◽  
Motor Neurons ◽  
Clinical Status ◽  
Dynamic Bayesian Network ◽  
Practice Research ◽  
The Individual ◽  
Lateral Sclerosis

Background. Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease primarily affecting upper and lower motor neurons in the brain and spinal cord. The heterogeneity in the course of ALS clinical progression and ultimately survival, coupled with the rarity of this disease, make predicting disease outcome at the level of the individual patient very challenging. Besides, stratification of ALS patients has been known for years as a question of great importance to clinical practice, research and drug development. Methods. In this work, we present a Dynamic Bayesian Network (DBN) model of ALS progression to detect probabilistic relationships among variables included in the Pooled Resource Open-Access ALS Clinical Trials Database (PRO-ACT), which provides records of over 10,700 patients from different clinical trials, and with over 2,869,973 longitudinally collected data measurements. Results. Our model unravels new dependencies among clinical variables in relation to ALS progression, such as the influence of basophil count and creatine kinase on patients’ clinical status and the respiratory functional state, respectively. Furthermore, it provided an indication of ALS temporal evolution, in terms of the most probable disease trajectories across time at the level of both patient population and individual patient. Conclusions. The risk factors identified by out DBN model could allow patients' stratification based on velocity of disease progression and a sensitivity analysis on this latter in response to changes in input variables, i.e. variables measured at diagnosis.

scholarly journals A Dynamic Bayesian Network model for simulation of disease progression in Amyotrophic Lateral Sclerosis patients

2017 ◽  
Author(s):  
Alessandro Zandonà ◽  
Matilde Francescon ◽  
Maya Bronfeld ◽  
Andrea Calvo ◽  
Adriano Chiò ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Amyotrophic Lateral Sclerosis ◽  
Bayesian Network ◽  
Disease Progression ◽  
Motor Neurons ◽  
Clinical Status ◽  
Dynamic Bayesian Network ◽  
Practice Research ◽  
The Individual ◽  
Lateral Sclerosis

Background. Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease primarily affecting upper and lower motor neurons in the brain and spinal cord. The heterogeneity in the course of ALS clinical progression and ultimately survival, coupled with the rarity of this disease, make predicting disease outcome at the level of the individual patient very challenging. Besides, stratification of ALS patients has been known for years as a question of great importance to clinical practice, research and drug development. Methods. In this work, we present a Dynamic Bayesian Network (DBN) model of ALS progression to detect probabilistic relationships among variables included in the Pooled Resource Open-Access ALS Clinical Trials Database (PRO-ACT), which provides records of over 10,700 patients from different clinical trials, and with over 2,869,973 longitudinally collected data measurements. Results. Our model unravels new dependencies among clinical variables in relation to ALS progression, such as the influence of basophil count and creatine kinase on patients’ clinical status and the respiratory functional state, respectively. Furthermore, it provided an indication of ALS temporal evolution, in terms of the most probable disease trajectories across time at the level of both patient population and individual patient. Conclusions. The risk factors identified by out DBN model could allow patients' stratification based on velocity of disease progression and a sensitivity analysis on this latter in response to changes in input variables, i.e. variables measured at diagnosis.

scholarly journals Observational study of patients in Spain with amyotrophic lateral sclerosis: correlations between clinical status, quality of life, and dignity

2017 ◽  
Vol 16 (1) ◽  
Author(s):  
Yolanda Martínez-Campo ◽  
Christian Homedes ◽  
Ana Lazaro ◽  
Raquel Alarcón ◽  
David Campo ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Amyotrophic Lateral Sclerosis ◽  
Observational Study ◽  
Clinical Status ◽  
Lateral Sclerosis

Automated Acoustic Analysis of Oral Diadochokinesis to Assess Bulbar Motor Involvement in Amyotrophic Lateral Sclerosis

2020 ◽  
Vol 63 (1) ◽  
pp. 59-73 ◽  
Author(s):  
Panying Rong
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Acoustic Analysis ◽  
Speaking Rate ◽  
Disease Stage ◽  
Healthy Controls ◽  
Tongue Movement ◽  
Major Barrier ◽  
Syllable Repetition ◽  
Oral Diadochokinesis ◽  
Lateral Sclerosis

Purpose The purpose of this article was to validate a novel acoustic analysis of oral diadochokinesis (DDK) in assessing bulbar motor involvement in amyotrophic lateral sclerosis (ALS). Method An automated acoustic DDK analysis was developed, which filtered out the voice features and extracted the envelope of the acoustic waveform reflecting the temporal pattern of syllable repetitions during an oral DDK task (i.e., repetitions of /tɑ/ at the maximum rate on 1 breath). Cycle-to-cycle temporal variability (cTV) of envelope fluctuations and syllable repetition rate (sylRate) were derived from the envelope and validated against 2 kinematic measures, which are tongue movement jitter (movJitter) and alternating tongue movement rate (AMR) during the DDK task, in 16 individuals with bulbar ALS and 18 healthy controls. After the validation, cTV, sylRate, movJitter, and AMR, along with an established clinical speech measure, that is, speaking rate (SR), were compared in their ability to (a) differentiate individuals with ALS from healthy controls and (b) detect early-stage bulbar declines in ALS. Results cTV and sylRate were significantly correlated with movJitter and AMR, respectively, across individuals with ALS and healthy controls, confirming the validity of the acoustic DDK analysis in extracting the temporal DDK pattern. Among all the acoustic and kinematic DDK measures, cTV showed the highest diagnostic accuracy (i.e., 0.87) with 80% sensitivity and 94% specificity in differentiating individuals with ALS from healthy controls, which outperformed the SR measure. Moreover, cTV showed a large increase during the early disease stage, which preceded the decline of SR. Conclusions This study provided preliminary validation of a novel automated acoustic DDK analysis in extracting a useful measure, namely, cTV, for early detection of bulbar ALS. This analysis overcame a major barrier in the existing acoustic DDK analysis, which is continuous voicing between syllables that interferes with syllable structures. This approach has potential clinical applications as a novel bulbar assessment.

Sign in / Sign up

Export Citation Format

Share Document