Analysis of Extrinsic Factors Affecting Pallestesic Threshold (VT) Of Amyotrophic Lateral Sclerosis Patients

1987 ◽  
pp. 125-128
Author(s):  
M. L. Delodovici ◽  
E. Terazzi ◽  
C. Pasetti ◽  
P. Pinelli
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Extrinsic Factors ◽  
Factors Affecting ◽  
Lateral Sclerosis

Variation in noninvasive ventilation use in amyotrophic lateral sclerosis

Neurology ◽  
2019 ◽  
Vol 93 (3) ◽  
pp. e306-e316 ◽  
Author(s):  
Nimish J. Thakore ◽  
Brittany R. Lapin ◽  
Erik P. Pioro ◽  
Loutfi S. Aboussouan
Keyword(s):  
Clinical Trials ◽  
Amyotrophic Lateral Sclerosis ◽  
Noninvasive Ventilation ◽  
Rating Scale ◽  
Factors Affecting ◽  
Cox Proportional Hazard ◽  
Bulbar Onset ◽  
Cox Proportional Hazard Models ◽  
R Factors ◽  
Lateral Sclerosis

ObjectiveWe sought to examine prevalence and predictors of noninvasive ventilation (NIV) in a composite cohort of patients with amyotrophic lateral sclerosis (ALS) followed in a clinical trials setting (Pooled Resource Open-Access ALS Clinical Trials database).MethodsNIV initiation and status were ascertained from response to question 12 of the revised ALS Functional Rating Scale (ALSFRS-R). Factors affecting NIV use in patients with forced vital capacity (FVC) ≤50% of predicted were examined. Predictors of NIV were evaluated by Cox proportional hazard models and generalized linear mixed models.ResultsAmong 1,784 patients with 8,417 simultaneous ALSFRS-R and FVC% measures, NIV was used by 604 (33.9%). Of 918 encounters when FVC% ≤50%, NIV was reported in 482 (52.5%). Independent predictors of NIV initiation were lower FVC% (hazard ratio [HR] 1.27, 95% confidence interval [CI] 1.17–1.37 for 10% drop), dyspnea (HR 2.62, 95% CI 1.87–3.69), orthopnea (HR 4.09, 95% CI 3.02–5.55), lower bulbar and gross motor subscores of ALSFRS-R (HRs 1.09 [95% CI 1.03–1.14] and 1.13 [95% CI 1.07–1.20], respectively, per point), and male sex (HR 1.73, 95% CI 1.31–2.28). Adjusted for other variables, bulbar onset did not significantly influence time to NIV (HR 0.72, 95% CI 0.47–1.08). Considerable unexplained variability in NIV use was found.ConclusionNIV use was lower than expected in this ALS cohort that was likely to be optimally managed. Absence of respiratory symptoms and female sex may be barriers to NIV use. Prospective exploration of factors affecting adoption of NIV may help bridge this gap and improve care in ALS.

scholarly journals Low Frequency-Repetitive Nerve Stimulation and Factors Affecting Decremental Response in Amyotrophic Lateral Sclerosis: A Retrospective Study of 449 Cases

2021 ◽  
Author(s):  
Jinghong Zhang ◽  
Hongfen Wang ◽  
Fei Yang ◽  
Zhengqing He ◽  
Fang Cui ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Retrospective Study ◽  
Sample Size ◽  
Nerve Stimulation ◽  
Low Frequency ◽  
Small Sample ◽  
Accessory Nerve ◽  
Repetitive Nerve Stimulation ◽  
Factors Affecting ◽  
Lateral Sclerosis

Abstract A number of studies have demonstrated that decremental response to low frequency repetitive nerve stimulation (LF-RNS) is frequently observed in amyotrophic lateral sclerosis (ALS). However, due to the small sample size involved in previous studies, large discrepancies exist about the positivity rates of LF-RNS tests and factors affecting decremental response. This retrospective study of 449 cases, the largest sample size ever reported, shows that the overall positivity rate of LF-RNS is 49.9%. 3Hz RNS delivered to the accessory nerve has the best sensitivity and highest positivity rate. It obviously increases in response to upper limb onset, disease progression rate < 0.5 score/month, definite ALS and electromyography positive(EMG(+)) in sternocleidomastoid muscle. There is a linear increase in the decrement percentage of CMAP amplitude at 3Hz RNS delivered to the accessory nerve in response to longer disease duration, longer MUP duration and greater MUP duration increment percentage. These findings substantially advance the understanding of RNS results in ALS patients and effectively instruct clinical application.

Psychological morbidity in amyotrophic lateral sclerosis: Depression, anxiety, hopelessness

2018 ◽  
Author(s):  
Dorothée Lulé ◽  
Albert C. Ludolph ◽  
Andrea Kübler
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Psychosocial Adjustment ◽  
Clinical Symptoms ◽  
Well Being ◽  
Psychological Morbidity ◽  
Extrinsic Factors ◽  
Psychological Well Being ◽  
Holistic View ◽  
Intrinsic Factors ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis is a devastating condition with progressive loss of movement, speech, and respiratory function, and no available cure. Following the development of clinical symptoms and after receiving a diagnosis, patients may develop psychological morbidity, such as depression, anxiety, and hopelessness. However, many patients adjust successfully in the course of the disease and maintain good psychological well-being, so that a decline in psychological well-being does not necessarily accompany loss of physical function. There are several major determinants of good psychological adjustment to chronic and terminal disease—intrinsic factors such as coping strategies and internal locus of control, and extrinsic factors such as high (perceived and actual) social support by families and multidisciplinary professional teams. Providing care with a holistic view of the patient is probably the most effective approach to supporting patients’ psychosocial adjustment to the disease and minimizing depression, anxiety, and hopelessness.

scholarly journals Life factors affecting depression and burden in amyotrophic lateral sclerosis caregivers

2014 ◽  
Vol 15 (3-4) ◽  
pp. 292-297 ◽  
Author(s):  
Kristen Qutub ◽  
David Lacomis ◽  
Stephen M. Albert ◽  
Eleanor Feingold
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Factors Affecting ◽  
Lateral Sclerosis

scholarly journals Milano–Torino Staging and Long-Term Survival in Chinese Patients with Amyotrophic Lateral Sclerosis

Cells ◽  
2021 ◽  
Vol 10 (5) ◽  
pp. 1220
Author(s):  
Ruojie He ◽  
Minying Zheng ◽  
Ling Lian ◽  
Xiaoli Yao
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Disease Progression ◽  
Rating Scale ◽  
Age At Onset ◽  
Chinese Patients ◽  
Term Survival ◽  
Long Term Survival ◽  
Factors Affecting ◽  
Lateral Sclerosis

(1) Background: The aim of this longitudinal study was to evaluate the association between disease progression according to the Milano–Torino staging (MITOS) system and long-term survival in Chinese patients with amyotrophic lateral sclerosis (ALS). We also examined factors affecting MITOS progression. (2) Methods: Patients were enrolled and underwent follow-up at 6, 12, 18, and 24 months, and their demographic and clinical data, including the Milano–Torino stage, Amyotrophic Lateral Sclerosis Functional Rating Scale—Revised (ALSFRS-R) score and neuropsychiatric data, were evaluated. The sensitivity and specificity of predicting survival outcomes based on MITOS progression and ALSFRS-R score decline from baseline to 6 months were compared. The associations between MITOS progression from baseline to 6 months and survival outcome at 12, 18 and 24 months were examined, and factors associated with disease progression were evaluated with subgroup analyses. (3) Results: Among the 100 patients included, 74% were in stage 0 at baseline, and approximately 95% progressed to a higher stage of the MITOS system at 24 months. MITOS progression from baseline to 6 months and ALSFRS-R decline showed comparable value for predicting survival at 12, 18, and 24 months. MITOS progression from baseline to 6 months is strongly associated with death outcomes. Older age at onset and increased depression and anxiety scores may be related to disease progression. (4) Conclusions: MITOS progression during the early disease course could serve as a prognostic marker of long-term survival and may have utility in clinical trials. Age at onset and diagnosis and neuropsychiatric factors might be associated with disease progression.

scholarly journals Factors Affecting Energy Metabolism and Prognosis in Patients with Amyotrophic Lateral Sclerosis

2021 ◽  
pp. 1-8
Author(s):  
Mika Kurihara ◽  
Shigeki Bamba ◽  
Shoko Yasuhara ◽  
Akihiko Itoh ◽  
Taishi Nagao ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Amyotrophic Lateral Sclerosis ◽  
Regression Analysis ◽  
Energy Metabolism ◽  
Energy Expenditure ◽  
Skeletal Muscle Index ◽  
Factors Affecting ◽  
Factors Associated ◽  
Respiratory Status ◽  
Lateral Sclerosis

<b><i>Background/Aims:</i></b> Nutritional status is a factor affecting prognosis in patients with amyotrophic lateral sclerosis (ALS). Here, we aimed to clarify the factors associated with hypermetabolism and the prognosticators of ALS. <b><i>Methods:</i></b> Forty-two inpatients (22 men, 20 women) diagnosed with ALS according to the revised El-Escorial criteria were investigated. The following data were retrospectively analyzed: anthropometric measurements, blood biochemistry, disease severity, basal energy expenditure (BEE), resting energy expenditure (REE) measured by indirect calorimetry, spirometry, and bioelectrical impedance analysis. Single and multiple regression analysis was performed to examine factors affecting REE and metabolic changes (defined as the ratio of REE to fat-free mass [FFM]). The Kaplan-Meier method was used to examine factors associated with the occurrence of cumulative events (death or tracheostomy). <b><i>Results:</i></b> Among the 42 inpatients, REE was significantly higher than BEE, indicating hypermetabolism in ALS. Multiple regression analysis revealed that REE/FFM is strongly associated with the skeletal muscle index (−3.746 to −1.532, <i>p</i> &#x3c; 0.0001) and percent forced vital capacity (%FVC) (−0.172 to −0.021, <i>p</i> = 0.013). Moreover, both the skeletal muscle index and %FVC were significant prognosticators associated with the occurrence of cumulative events. <b><i>Conclusions:</i></b> Energy metabolism was elevated in ALS, and respiratory status and muscle mass were associated with the hypermetabolism and poor prognosis. Adequate nutritional support may improve outcomes in ALS by preventing deterioration of respiratory status and reduction in muscle mass.

scholarly journals Factors Affecting Aggregate Formation in Cell Models of Huntington’s Disease and Amyotrophic Lateral Sclerosis

Acta Naturae ◽  
2013 ◽  
Vol 5 (2) ◽  
pp. 81-89 ◽  
Author(s):  
V. F. Lazarev ◽  
D. V. Sverchinskyi ◽  
M. V. Ippolitova ◽  
A. V. Kaznacheyeva ◽  
I. V. Guzhova ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cell Model ◽  
Tissue Transglutaminase ◽  
Mutant Huntingtin ◽  
Aggregate Formation ◽  
Protective Function ◽  
Mutant Proteins ◽  
Factors Affecting ◽  
Intracellular Content ◽  
Lateral Sclerosis

Most neurodegenerative pathologies stem from the formation of aggregates of mutant proteins, causing dysfunction and ultimately neuronal death. This study was aimed at elucidating the role of the protein factors that promote aggregate formation or prevent the process, respectively, glyceraldehyde-3-dehydrogenase (GAPDH) and tissue transglutaminase (tTG) and Hsp70 molecular chaperone. The siRNA technology was used to show that the inhibition of GAPDH expression leads to a 4550% reduction in the aggregation of mutant huntingtin, with a repeat of 103 glutamine residues in a model of Huntingtons disease (HD). Similarly, the blockage of GAPDH synthesis was found for the first time to reduce the degree of aggregation of mutant superoxide dismutase 1 (G93A) in a model of amyotrophic lateral sclerosis (ALS). The treatment of cells that imitate HD and ALS with a pharmacological GAPDH inhibitor, hydroxynonenal, was also shown to reduce the amount of the aggregating material in both disease models. Tissue transglutaminase is another factor that promotes the aggregation of mutant proteins; the inhibition of its activity with cystamine was found to prevent aggregate formation of mutant huntingtin and SOD1. In order to explore the protective function of Hsp70 in the control of the aggregation of mutant huntingtin, a cell model with inducible expression of the chaperone was used. The amount and size of polyglutamine aggregates were reduced by increasing the intracellular content of Hsp70. Thus, pharmacological regulation of the function of three proteins, GAPDH, tTG, and Hsp70, can affect the pathogenesis of two significant neurodegenerative diseases.

Factors affecting early diagnosis of amyotrophic lateral sclerosis

2018 ◽  
Vol 81/114 (3) ◽  
pp. 353-357
Author(s):  
Peter Matejička ◽  
Martina Gažiová ◽  
Michal Minár
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Early Diagnosis ◽  
Factors Affecting ◽  
Lateral Sclerosis
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