Neuroimaging in amyotrophic lateral sclerosis: current and emerging uses.

Neuroimaging in amyotrophic lateral sclerosis: current and emerging uses. Expert Rev Neurother. 2018 Apr 09;: Authors: Agosta F, Spinelli EG, Filippi M Abstract INTRODUCTION: Several neuroimaging techniques have been used to define in vivo markers of pathological alterations underlying amyotrophic lateral sclerosis (ALS). Growing evidence supports the use of magnetic resonance imaging (MRI) and positron emission tomography (PET) for the non-invasive detection of central nervous system involvement in patients with ALS. Areas covered: A comprehensive overview of structural and functional neuroimaging applications in ALS is provided, focusing on motor and extra-motor involvement in the brain and the spinal cord. Implications for pathogenetic models, patient diagnosis, prognosis, monitoring and the design of clinical trials are discussed. Expert commentary: State-of-the-art neuroimaging techniques provide fundamental instruments for the detection and quantification of upper motor neuron and extra-motor brain involvement in ALS, with relevance for both pathophysiologic investigation and clinical practice. Network-based analysis of structural and functional connectivity alterations and multimodal approaches combining several neuroimaging measures are promising tools for the development of novel diagnostic and prognostic markers to be used at the individual patient level. PMID: 29630421 [PubMed - as supplied by publisher]
Source: Expert Review of Neurotherapeutics - Category: Neurology Tags: Expert Rev Neurother Source Type: research

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ConclusionIntegrated PET-MR imaging demonstrates associations between markers for neuronal integrity and neuroinflammation and may provide valuable insights into disease mechanisms in ALS.
Source: NeuroImage: Clinical - Category: Radiology Source Type: research
ina Dolei The human endogenous retrovirus (HERV)-K, human mouse mammary tumor virus like-2 (HML-2) subgroup of HERVs is activated in several tumors and has been related to prostate cancer progression and motor neuron diseases. The cellular splicing factor 2/alternative splicing factor (SF2/ASF) is a positive regulator of gene expression, coded by a potent proto-oncogene, amplified, and abnormally expressed in tumors. TAR DNA-binding protein-43 (TDP-43) is a DNA/RNA-binding protein, negative regulator of alternative splicing, known for causing neurodegeneration, and with complex roles in oncogenesis. We used the cluster...
Source: Viruses - Category: Virology Authors: Tags: Communication Source Type: research
ger V Abstract Following the involvement of CHCHD10 in FrontoTemporal-Dementia-Amyotrophic Lateral Sclerosis (FTD-ALS) clinical spectrum, a founder mutation (p.Gly66Val) in the same gene was identified in Finnish families with late-onset spinal motor neuronopathy (SMAJ). SMAJ is a slowly progressive form of spinal muscular atrophy with a life expectancy within normal range. In order to understand why the p.Ser59Leu mutation, responsible for severe FTD-ALS, and the p.Gly66Val mutation could lead to different levels of severity, we compared their effects in patient cells. Unlike affected individuals bearing the p.Se...
Source: Neurobiology of Disease - Category: Neurology Authors: Tags: Neurobiol Dis Source Type: research
The objective of this study was...
Source: SafetyLit - Category: International Medicine & Public Health Tags: Economics of Injury and Safety, PTSD, Injury Outcomes Source Type: news
Motor neuron diseases are the result of dysfunction of upper motor neurons in the precentral gyrus of the frontal lobe and/or lower motor neurons in the ventral horn of the spinal cord. In general, they cause weakness without notable sensory symptoms or pain. The most common motor neuron disease is amyotrophic lateral sclerosis, which is the primary focus of this review. Other motor neuron diseases include hereditary spastic paraparesis, spinobulbar muscular atrophy, and infectious motor neuron diseases including polio and West Nile virus.
Source: The American Journal of Medicine - Category: General Medicine Authors: Tags: Review Source Type: research
Conditions:   Motor Neuron Disease, Amyotrophic Lateral Sclerosis;   ALS (Amyotrophic Lateral Sclerosis);   Dysarthria, Flaccid;   Dysarthria, Spastic;   Dysarthria, Mixed;   Dysphagia;   Dyspnea;   Aspiration Pneumonia;   Hypernasality;   Breathlessness Intervention:   Procedure: Music therapy Sponsors:   Alisa Apreleva;   ALS Centre Moscow Active, not recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Publication date: Available online 30 June 2017Source: Neuroscience LettersAuthor(s): Emma F. Smith, Pamela J. Shaw, Kurt J. De VosAbstractMitochondria are unique organelles that are essential for a variety of cellular processes including energy metabolism, calcium homeostasis, lipid biosynthesis, and apoptosis. Mitochondrial dysfunction is a prevalent feature of many neurodegenerative diseases including motor neuron disorders such as amyotrophic lateral sclerosis (ALS). Disruption of mitochondrial structure, dynamics, bioenergetics and calcium buffering has been extensively reported in ALS patients and model systems and h...
Source: Neuroscience Letters - Category: Neuroscience Source Type: research
ConclusionIntrasciatic injection of RCA60 or RCA120 induces nerve injury and muscle atrophy, which can be properly evaluated by a comprehensive platform using FG-guided quantitative 3D topographic histological analysis, MRI and the CatWalk behavioral test.
Source: Journal of Neuroscience Methods - Category: Neuroscience Source Type: research
The Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) is the preferred measure of health outcome in clinical trials in motor neuron disease (MND). It, however, does not provide a preference-based health utility score required for estimating quality-adjusted life-years in economic evaluations for health technology assessments.
Source: Value in Health - Category: International Medicine & Public Health Authors: Source Type: research
Authors: Walker LC, Meadows MR, Du Y, March LK, Jones JK Abstract BACKGROUND: Amyotrophic lateral sclerosis (ALS) is devastating, leading to paralysis and death. Disease onset begins pre-symptomatically through spinal motor neuron (MN) axon die-back from musculature at ∼47 days of age in the mutant superoxide dismutase 1 (mSOD1G93A) transgenic ALS mouse model. This period may be optimal to assess potential therapies. We previously demonstrated that post-symptomatic adipose-derived stem cell conditioned medium (ASC-CM) treatment is neuroprotective in mSOD1G93A mice. We hypothesized that early disease onset treat...
Source: Restorative Neurology and Neuroscience - Category: Neurology Tags: Restor Neurol Neurosci Source Type: research
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