scholarly journals Comparative Assessment of Blood Metal/metalloid levels, Clinical Heterogeneity, and Disease Severity in Amyotrophic Lateral Sclerosis Patients

2022 ◽  
Author(s):  
Xing Qin ◽  
Peng Wu ◽  
Ting Wen ◽  
Rui Jia ◽  
Ronghua Zhang ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Disease Severity ◽  
Comparative Assessment ◽  
Clinical Heterogeneity ◽  
Lateral Sclerosis

scholarly journals Increased cerebrospinal fluid adenosine 5'-triphosphate in patients with amyotrophic lateral sclerosis

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Takamasa Nukui ◽  
Atsushi Matsui ◽  
Hideki Niimi ◽  
Tomoyuki Sugimoto ◽  
Tomohiro Hayashi ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cerebrospinal Fluid ◽  
Serum Creatinine ◽  
Disease Severity ◽  
Assay System ◽  
Atp Levels ◽  
Sum Score ◽  
Highly Sensitive ◽  
Severe Group ◽  
Lateral Sclerosis

Abstract Background Extracellular adenosine 5'-triphosphate (ATP) has been suggested to cause neuroinflammation and motor neuron degeneration by activating microglia and astrocytes in amyotrophic lateral sclerosis (ALS). Since we have developed a highly sensitive ATP assay system, we examined cerebrospinal fluid (CSF) ATP levels in patients with ALS whether it can be a useful biomarker in ALS. Methods Forty-eight CSF samples from 44 patients with ALS were assayed for ATP with a newly established, highly sensitive assay system using luciferase luminous reaction. CSF samples from patients with idiopathic normal pressure hydrocephalus (iNPH) were assayed as a control. Patients were divided into two groups depending on their disease severity, as evaluated using the Medical Research Council (MRC) sum score. Correlations between the CSF ATP levels and other factors, including clinical data and serum creatinine levels, were evaluated. Results CSF ATP levels were significantly higher in patients with ALS than in the iNPH (716 ± 411 vs. 3635 ± 5465 pmol/L, p < 0.01). CSF ATP levels were significantly higher in the more severe group than in the iNPH group (6860 ± 8312 vs. 716 ± 411 pmol/L, p < 0.05) and mild group (6860 ± 8312 vs. 2676 ± 3959 pmol/L, p < 0.05) respectively. ALS functional rating scale-revised (ALSFRS-R) (37.9 ± 5.7 vs. 42.4 ± 2.8, p < 0.01) and serum creatinine levels (0.51 ± 0.13 vs. 0.68 ± 0.23 mg/dL, p < 0.05) were significantly lower in the severe group than in the mild group respectively. A negative correlation of CSF ATP levels with MRC sum score was demonstrated in the correlation analysis adjusted for age and sex (r = -0.3, p = 0.08). Conclusions Extracellular ATP is particularly increased in the CSF of patients with advanced ALS. CSF ATP levels may be a useful biomarker for evaluating disease severity in patients with ALS.

scholarly journals Informal Caregiving in Amyotrophic Lateral Sclerosis (ALS): A High Caregiver Burden and Drastic Consequences on Caregivers’ Lives

2021 ◽  
Vol 11 (6) ◽  
pp. 748
Author(s):  
Pavel Schischlevskij ◽  
Isabell Cordts ◽  
René Günther ◽  
Benjamin Stolte ◽  
Daniel Zeller ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Functional Status ◽  
Disease Severity ◽  
Caregiver Burden ◽  
Rating Scale ◽  
Depression Scale ◽  
Anxiety And Depression ◽  
Daily Routine ◽  
Work Lives ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that causes progressive autonomy loss and need for care. This does not only affect patients themselves, but also the patients’ informal caregivers (CGs) in their health, personal and professional lives. The big efforts of this multi-center study were not only to evaluate the caregivers’ burden and to identify its predictors, but it also should provide a specific understanding of the needs of ALS patients’ CGs and fill the gap of knowledge on their personal and work lives. Using standardized questionnaires, primary data from patients and their main informal CGs (n = 249) were collected. Patients’ functional status and disease severity were evaluated using the Barthel Index, the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) and the King’s Stages for ALS. The caregivers’ burden was recorded by the Zarit Burden Interview (ZBI). Comorbid anxiety and depression of caregivers were assessed by the Hospital Anxiety and Depression Scale. Additionally, the EuroQol Five Dimension Five Level Scale evaluated their health-related quality of life. The caregivers’ burden was high (mean ZBI = 26/88, 0 = no burden, ≥24 = highly burdened) and correlated with patients’ functional status (rp = −0.555, p < 0.001, n = 242). It was influenced by the CGs’ own mental health issues due to caregiving (+11.36, 95% CI [6.84; 15.87], p < 0.001), patients’ wheelchair dependency (+9.30, 95% CI [5.94; 12.66], p < 0.001) and was interrelated with the CGs’ depression (rp = 0.627, p < 0.001, n = 234), anxiety (rp = 0.550, p < 0.001, n = 234), and poorer physical condition (rp = −0.362, p < 0.001, n = 237). Moreover, female CGs showed symptoms of anxiety more often, which also correlated with the patients’ impairment in daily routine (rs = −0.280, p < 0.001, n = 169). As increasing disease severity, along with decreasing autonomy, was the main predictor of caregiver burden and showed to create relevant (negative) implications on CGs’ lives, patient care and supportive therapies should address this issue. Moreover, in order to preserve the mental and physical health of the CGs, new concepts of care have to focus on both, on not only patients but also their CGs and gender-associated specific issues. As caregiving in ALS also significantly influences the socioeconomic status by restrictions in CGs’ work lives and income, and the main reported needs being lack of psychological support and a high bureaucracy, the situation of CGs needs more attention. Apart from their own multi-disciplinary medical and psychological care, more support in care and patient management issues is required.

Spinal cord atrophy correlates with disease severity in amyotrophic lateral sclerosis

2013 ◽  
Vol 333 ◽  
pp. e447-e448
Author(s):  
H.M.T. Andrade ◽  
L.M.T. Branco ◽  
M. Albuquerque ◽  
F.P.G. Bergo ◽  
A. Nucci ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Disease Severity ◽  
Spinal Cord Atrophy ◽  
Lateral Sclerosis

Clinical heterogeneity in Italian patients with amyotrophic lateral sclerosis

2011 ◽  
Vol 82 (1) ◽  
pp. 83-87 ◽  
Author(s):  
I Piaceri ◽  
M Del Mastio ◽  
A Tedde ◽  
S Bagnoli ◽  
S Latorraca ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Clinical Heterogeneity ◽  
Lateral Sclerosis

scholarly journals Errata: Measuring Disease Severity in Duchenne and Becker Muscular Dystrophy

2011 ◽  
Vol 1 (2) ◽  
Author(s):  
Melinda F. Davis ◽  
Katalin H. Scherer ◽  
Timothy M. Miller ◽  
F. John Meaney
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Muscular Dystrophy ◽  
Disease Severity ◽  
Muscle Weakness ◽  
Respiratory Function ◽  
Rating Scale ◽  
Becker Muscular Dystrophy ◽  
Progressive Muscle Weakness ◽  
Lateral Sclerosis ◽  
Disease Specific

Reports an error in Davis et al. (2010).  The functional motor scale used in Davis et al. (2010) was the EK (Egen Klassifikation) Scale, rather than the Amyotrophic Lateral Sclerosis Functional Rating Scale (Steffensen et al., 2002; Cedarbaum & Stambler, 1997).  Both scales are 10-item, disease-specific measures that assess mobility and respiratory function in individuals with progressive muscle weakness.  This error does not change the conclusions. DOI:10.2458/azu_jmmss_v1i2_davis

Increased Cerebrospinal Fluid Adenosine 5'-triphosphate in Patients with Amyotrophic Lateral Sclerosis

2020 ◽  
Author(s):  
Takamasa Nukui ◽  
Atsushi Matsui ◽  
Hideki Niimi ◽  
Tomohiro Hayashi ◽  
Nobuhiro Dougu ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Serum Creatinine ◽  
Disease Severity ◽  
Normal Pressure ◽  
Assay System ◽  
Atp Levels ◽  
Sum Score ◽  
Highly Sensitive ◽  
Severe Group ◽  
Lateral Sclerosis

Abstract Background: Extracellular adenosine 5'-triphosphate (ATP) has been suggested to cause neuroinflammation and motor neuron degeneration by activating microglia and astrocytes in amyotrophic lateral sclerosis (ALS). Since we have developed a highly sensitive ATP assay system, we examined CSF ATP levels in patients with ALS whether it can be a useful biomarker in ALS. Methods: Forty-eight CSF samples from 45 patients with ALS were assayed for ATP with a newly established, highly sensitive assay system using luciferase luminous reaction. CSF samples from patients with idiopathic normal pressure hydrocephalus (iNPH) were assayed as a control. Patients were divided into two groups depending on their disease severity, as evaluated using the Medical Research Council (MRC) sum score. Correlations between the CSF ATP levels and other factors, including clinical data and serum creatinine levels, were evaluated. Results: ALSFRS-R (37.9 ± 5.7 vs. 42.4 ± 2.8, p<0.01) and serum creatinine levels (0.51 ± 0.13 vs. 0.68 ± 0.23 mg/dL, p < 0.05) were significantly lower in the severe group than in the mild group respectively. CSF ATP levels were significantly higher in the more severe group than in the iNPH group (6860 ± 8312 vs. 716 ± 411 pmol/L, p < 0.05) and mild group (6860 ± 8312 vs. 2676 ± 3959 pmol/L, p < 0.05) respectively. A negative correlation of CSF ATP levels with MRC sum score was demonstrated in the correlation analysis (r = -0.3862, p < 0.01). Conclusions: Extracellular ATP is particularly increased in the CSF of patients with advanced ALS. CSF ATP levels may be a useful biomarker for evaluating disease severity in patients with ALS.

scholarly journals Elevated acute phase proteins reflect peripheral inflammation and disease severity in patients with amyotrophic lateral sclerosis

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
David R. Beers ◽  
Weihua Zhao ◽  
Daniel W. Neal ◽  
Jason R. Thonhoff ◽  
Aaron D. Thome ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Disease Progression ◽  
Disease Severity ◽  
Acute Phase ◽  
Disease Burden ◽  
Acute Phase Proteins ◽  
Disease Onset ◽  
Peripheral Inflammation ◽  
Operating Characteristics ◽  
Lateral Sclerosis

Abstract Amyotrophic lateral sclerosis (ALS) is a multifactorial, multisystem pro-inflammatory neuromuscular disorder compromising muscle function resulting in death. Neuroinflammation is known to accelerate disease progression and accentuate disease severity, but peripheral inflammatory processes are not well documented. Acute phase proteins (APPs), plasma proteins synthesized in the liver, are increased in response to inflammation. The objective of this study was to provide evidence for peripheral inflammation by examining levels of APPs, and their contribution to disease burden and progression rates. Levels of APPs, including soluble CD14 (sCD14), lipopolysaccharide binding protein (LBP), and C-reactive protein (CRP), were elevated in sera, and correlated positively with increased disease burden and faster progression. sCD14 was also elevated in patients’ CSF and urine. After a 3 year follow-up, 72% of the patients with sCD14 levels above the receiver operating characteristics cutoff were deceased whereas only 28% below the cutoff were deceased. Furthermore, disease onset sites were associated with disease progression rates and APP levels. These APPs were not elevated in sera of patients with Alzheimer’s Disease, frontotemporal dementia, or Parkinson’s Disease. These collective APPs accurately reflect disease burden, progression rates, and survival times, reinforcing the concept of ALS as a disorder with extensive systemic pro-inflammatory responses.

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