Elevated serum creatine kinase in the early stage of sporadic amyotrophic lateral sclerosis

AbstractObjectiveTo assess the changes of muscle-related biomarkers at the early stage of amyotrophic lateral sclerosis, and to confirm these findings in an experimental animal model.MethodsThirty-nine subjects with sporadic amyotrophic lateral sclerosis and 20 healthy controls were enrolled and longitudinally evaluated. We evaluated serum creatine kinase and creatinine levels and appendicular lean soft-tissue mass using dual X-ray absorptiometry. The levels of biomarkers at early ALS stages were estimated using linear mixed models with unstructured correlation and random intercepts. We also analyzed the longitudinal changes of serum creatine kinase and creatinine, together with the mRNA levels of acetylcholine receptor subunit γ (Chrng) and muscle-associated receptor tyrosine kinase, markers of denervation, in the gastrocnemius muscle of superoxide dismutase 1 (SOD1)G93A transgenic mice, an animal model of amyotrophic lateral sclerosis.ResultsThe estimated levels of creatine kinase were higher in subjects with amyotrophic lateral sclerosis at the early stage than in healthy controls, although the estimated appendicular lean soft-tissue mass and creatinine levels were equivalent between both groups, suggesting that the elevation of creatine kinase precedes both muscular atrophy and subjective motor symptoms in sporadic amyotrophic lateral sclerosis. In SOD1G93A mice, the serum levels of creatine kinase were elevated at 9  weeks of age (peri-onset) whenChrng started...
Source: Journal of Neurology - Category: Neurology Source Type: research

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What are the current trends and hot topics in neurology? The increasing insights into the genetics and molecular bases of neurological disorders open new perspectives for specific and personalized treatments. This is evidenced most dramatically by the antisense therapy for spinal muscular atrophy. Promising neurological research currently targets neurodegenerative diseases such as amyotrophic lateral sclerosis, Parkinson’s disease, Duchenne muscular atrophy, or Huntington’s disease. Likewise, brain-machine interfaces and neuroprosthetics offer huge potential. Never before has it been more important and more exc...
Source: BioMed Central Blog - Category: General Medicine Authors: Tags: Uncategorized Source Type: blogs
Authors: Seo DY, Heo JW, Ko JR, Kwak HB Abstract Neuroinflammation is a central pathological feature of several acute and chronic brain diseases, including Alzheimer disease (AD), Parkinson disease (PD), amyotrophic lateral sclerosis (ALS), and multiple sclerosis (MS). It induces microglia activation, mitochondrial dysfunction, the production of nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB), pro-inflammatory cytokines, and reactive oxygen species. Exercise, which plays an important role in maintaining and improving brain health, might be a highly effective intervention for preventing ...
Source: International Neurourology Journal - Category: Urology & Nephrology Tags: Int Neurourol J Source Type: research
CONCLUSIONS: Preliminary findings suggest that the RS-tDCS protocol is safe and feasible in individuals with ALS. Our protocol serves as a model for future long-term studies to evaluate the clinical and neurophysiological effects of tDCS using a telerehabilitation protocol in ALS. PMID: 31796701 [PubMed - as supplied by publisher]
Source: NeuroRehabilitation - Category: Rehabilitation Tags: NeuroRehabilitation Source Type: research
Conclusions: Students' visits to PALS may contribute to the maintenance of the QOL of the patients. Additionally, visits, with psychological support for the students, seem safe and could contribute to the students' psychological maturation as health professionals. Additional psychological support may be necessary for some students in fragile subgroups/moments.RESUMO Esclerose lateral amiotr ófica (ELA) é doença neurodegenerativa sem cura, mas tratamento multidisciplinar pode manter qualidade de vida (QOL). Embora profissionais de saúde possam ser afetados pela atuação na ELA, pouco...
Source: Arquivos de Neuro-Psiquiatria - Category: Neurology Source Type: research
(Mary Ann Liebert, Inc./Genetic Engineering News) The role of adenosine in neurodegeneration and neuroregeneration has led to growing attention on adenosine receptors as potential drug targets in a range of brain disorders, including neuroregenerative therapy and treatment for amyotrophyic lateral sclerosis (ALS).
Source: EurekAlert! - Medicine and Health - Category: International Medicine & Public Health Source Type: news
Publication date: 4 December 2019Source: Neuron, Volume 104, Issue 5Author(s): Katherine M. Wilson, Bhavana Muralidharan, Adrian M. IsaacsThe (GGGGCC)n repeat expansion in C9orf72, which is the most common cause of frontotemporal dementia and amyotrophic lateral sclerosis, is translated through repeat-associated non-AUG (RAN) translation. In this issue of Neuron, Cheng et al. (2019) report that the helicase DDX3X, which unwinds (or relaxes) RNA, suppresses RAN translation and toxicity.
Source: Neuron - Category: Neuroscience Source Type: research
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Source: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration - Category: Neurology Authors: Source Type: research
Publication date: January 2020Source: Artificial Intelligence in Medicine, Volume 102Author(s): Wenping Tang, Aiqun Wang, S. Ramkumar, Radeep Krishna Radhakrishnan NairAbstractParalyzed patients were increasing day by day. Some of the neurodegenerative diseases like amyotrophic lateral sclerosis, Brainstem Leison, Stupor and Muscular dystrophy affect the muscle movements in the body. The affected persons were unable to migrate. To overcome from their problem they need some assistive technology with the help of bio signals. Electrooculogram (EOG) based Human Computer Interaction (HCI) is one of the technique used in recent ...
Source: Artificial Intelligence in Medicine - Category: Bioinformatics Source Type: research
Publication date: Available online 4 December 2019Source: Stem Cell ResearchAuthor(s): E.I. Ustyantseva, S.P. Medvedev, A.S. Vetchinova, S.N. Illarioshkin, S.V. Leonov, S.M. ZakianAbstractAmyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by death of motor neurons. To date, neither etiology nor pathogenesis of ALS are known, which leads to the absence of an effective treatment strategy. ALS patient-specific induced pluripotent stem cells (iPSCs) represent an excellent tool for the disease study. We obtained iPSCs line from peripheral blood mononuclear cells of the patient with homozygous Asp90...
Source: Stem Cell Research - Category: Stem Cells Source Type: research
Alternating motion rate (AMR) is a standard measure often included in neurological examinations to assess orofacial neuromuscular integrity. AMR is typically derived from recordings of patients producing repetitions of a single syllable as fast and clear as possible on one breath. Because the task places high demands on oromotor performance, particularly articulatory speed, AMRs are widely considered to be tests of maximum performance and, therefore, likely to reveal underlying neurologic deficits. Despite decades of widespread use, biomechanical studies have shown that speakers often circumvent the presumed speed challeng...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
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