The impact of cognitive and behavioral impairment in amyotrophic lateral sclerosis.

The impact of cognitive and behavioral impairment in amyotrophic lateral sclerosis. Expert Rev Neurother. 2020 Feb 07;: Authors: Huynh W, Ahmed R, Mahoney C, Nguyen C, Tu S, Caga J, Loh P, Lin CS, Kiernan MC Abstract Introduction: A spectrum of non-motor manifestations in amyotrophic lateral sclerosis (ALS) patients has been increasingly recognized, with cognitive and behavioural impairments the most prominent. Evidence suggests that ALS overlaps on a pathological, genetic, and clinical level with frontotemporal dementia (FTD), thereby suggesting a frontotemporal spectrum disorder (ALS-FTSD). Cognitive impairment has been reported in up to 75% of ALS patients, whilst the rate of behavioural dysfunction ranges up to 50%.Areas covered: The present review explores the current understanding of cognitive and behavioral changes in ALS with a particular emphasis on its implications on prognosis and survival.Expert opinion: Further longitudinal studies are needed to clarify the evolution of cognitive impairment in ALS and how this may ultimately influence survival. Improving understanding of cognitive changes has important implications towards capacity of patients in making critical medical decisions. There is a need to develop a universally accepted and validated cognitive assessment tool to be administered in a multidisciplinary clinic that is efficient and sensitive, as well as being integrated into the design and analysis of future ALS drug trials. In addition, revisi...
Source: Expert Review of Neurotherapeutics - Category: Neurology Tags: Expert Rev Neurother Source Type: research

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Authors: Khosravi S, Harner ME Abstract Mitochondria perform a plethora of functions in various cells of different tissues. Their architecture differs remarkably, for instance in neurons versus steroidogenic cells. Furthermore, aberrant mitochondrial architecture results in mitochondrial dysfunction. This indicates strongly that mitochondrial architecture and function are intimately linked. Therefore, a deep knowledge about the determinants of mitochondrial architecture and their function on a molecular level is of utmost importance. In the past decades, various proteins and protein complexes essential for formatio...
Source: Biological Chemistry - Category: Chemistry Tags: Biol Chem Source Type: research
In this study, we characterized the structural features of three different DPR encoded by the hexanucleotide repeat GGGGCC, namely poly-GA, poly-GP and poly-PA. We showed that DPR are natively unstructured proteins and that only poly-GA forms in vitro fibrillary aggregates. Poly-GA fibrils are of amyloid nature as revealed by their high content in beta sheets. They neither bind Thioflavin T nor Primuline, the commonly used amyloid fluorescent dyes. Remarkably, not all of the poly-GA primary structure was part of fibrils amyloid core. PMID: 32223927 [PubMed - as supplied by publisher]
Source: Biochemical and Biophysical Research communications - Category: Biochemistry Authors: Tags: Biochem Biophys Res Commun Source Type: research
Publication date: Available online 29 February 2020Source: Journal of Molecular BiologyAuthor(s): Rebecca L. Casterton, Rachel J. Hunt, Manolis Fanto
Source: Journal of Molecular Biology - Category: Molecular Biology Source Type: research
Primary lateral sclerosis (PLS) is a rare type of motor neuron disease (MND) characterized by the loss of upper motor neurons without lower motor neuron involvement [1]. In contrast, amyotrophic lateral sclerosis (ALS) is a common type of MND that is occasionally complicated by frontotemporal dementia (FTD) as the TDP-43 proteinopathy. However, there are few studies on the relationship of PLS with ALS, FTD and TDP-43 proteinopathy because of the rarity of autopsy cases. Here, we report a 68-year-old woman who exhibited typical clinical symptoms of PLS at onset, which then progressed to FTD, although her pathological diagno...
Source: Journal of the Neurological Sciences - Category: Neurology Authors: Tags: Letter to the Editor Source Type: research
Abstract The deposition of proteins of abnormal conformation is one of the major hallmarks of the common neurodegenerative diseases including Alzheimer's, Parkinson's, amyotrophic lateral sclerosis, frontotemporal dementia, and prion diseases. Protein quality control systems have evolved to protect cells and organisms against the harmful consequences of abnormally folded proteins that are constantly produced in small amounts. Mutations in rare inherited forms of neurodegenerative diseases have provided compelling evidence that failure of protein quality control systems can drive neurodegeneration. With extensive k...
Source: Current Opinion in Neurobiology - Category: Neurology Authors: Tags: Curr Opin Neurobiol Source Type: research
We report a new class of natural-product-inspired covalent inhibitors of telomerase that target the catalytic active site. Age-Related Epigenetic Changes that Suppress Mitochondrial Function Today's open access research reports on two specific epigenetic changes observed in old individuals that act to reduce mitochondrial function. This joins an existing list of genes for which expression changes are known to impact mitochondrial function with age. A herd of hundreds of mitochondria are found ...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
Abstract Amyotrophic lateral sclerosis (ALS) is a fatal late-onset neurodegenerative disease that specifically affects the function and survival of spinal and cortical motor neurons. ALS shares many genetic, clinical, and pathological characteristics with frontotemporal dementia (FTD), and these diseases are now recognized as presentations of a disease spectrum known as ALS/FTD. The molecular determinants of neuronal loss in ALS/FTD are still debated, but the recent discovery of nucleocytoplasmic transport defects as a common denominator of most if not all forms of ALS/FTD has dramatically changed our understandin...
Source: Neurobiology of Disease - Category: Neurology Authors: Tags: Neurobiol Dis Source Type: research
Abstract Exploring the neurochemical continuum between frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) with respect to monoamines and kynurenines in cerebrospinal fluid (CSF) and serum, may be useful to identify possible new research/therapeutic targets. Hence, we analysed monoamines and kynurenines in CSF and serum derived from patients with FTD (n = 39), ALS (n = 23), FTD-ALS (n = 4) and age-matched control subjects (n = 26), using reversed-phase ultra-high performance liquid chromatography (RP-UHPLC) with electrochemical detection (EC...
Source: Neurochemical Research - Category: Neuroscience Authors: Tags: Neurochem Res Source Type: research
Conditions:   Motor Neuron Disease, Familial;   Amyotrophic Lateral Sclerosis With Dementia Interventions:   Device: Anodal bilateral motor cortex and cathodal spinal tDCS;   Device: Sham bilateral motor cortex and sham spinal tDCS Sponsor:   Azienda Ospedaliera Spedali Civili di Brescia Recruiting
Source: - Category: Research Source Type: clinical trials
Publication date: Available online 29 February 2020Source: Journal of Molecular BiologyAuthor(s): Rebecca L. Casterton, Rachel J. Hunt, Manolis Fanto
Source: Journal of Molecular Biology - Category: Molecular Biology Source Type: research
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