Semiautomated Evaluation of the Primary Motor Cortex in Patients with Amyotrophic Lateral Sclerosis at 3T ADULT BRAIN

BACKGROUND AND PURPOSE: Amyotrophic lateral sclerosis is a neurodegenerative disease involving the upper and lower motor neurons. In amyotrophic lateral sclerosis, pathologic changes in the primary motor cortex include Betz cell depletion and the presence of reactive iron-loaded microglia, detectable on 7T MR images as atrophy and T2*-hypointensity. Our purposes were the following: 1) to investigate the signal hypointensity-to-thickness ratio of the primary motor cortex as a radiologic marker of upper motor neuron involvement in amyotrophic lateral sclerosis with a semiautomated method at 3T, 2) to compare 3T and 7T results, and 3) to evaluate whether semiautomated measurement outperforms visual image assessment. MATERIALS AND METHODS: We investigated 27 patients and 13 healthy subjects at 3T, and 19 patients and 18 healthy subjects at 7T, performing a high-resolution 3D multiecho T2*-weighted sequence targeting the primary motor cortex. The signal hypointensity-to-thickness ratio of the primary motor cortex was calculated with a semiautomated method depicting signal intensity profiles of the cortex. Images were also visually classified as "pathologic" or "nonpathologic" based on the primary motor cortex signal intensity and thickness. RESULTS: The signal hypointensity-to-thickness ratio of the primary motor cortex was greater in patients than in controls (P
Source: American Journal of Neuroradiology - Category: Radiology Authors: Tags: ADULT BRAIN Source Type: research

Related Links:

In conclusion, Axon-seq provides an improved method for RNA-seq of axons, increasing our understanding of peripheral axon biology and identifying therapeutic targets in motor neuron disease.Graphical Abstract
Source: Stem Cell Reports - Category: Stem Cells Source Type: research
Abstract Hepatocyte growth factor is an endogenous pleiotropic factor shown to act as a potent neuroprotectant against disease progression in animal models of amyotrophic lateral sclerosis, which is a devastating, adult-onset motor neuron disease. To evaluate the safety, tolerability, and pharmacokinetics of recombinant 5-residue-deleted human hepatocyte growth factor (KP-100) injected intrathecally through an implantable catheter connected to a subcutaneous port, we conducted a first-in-human phase I trial of intrathecal KP-100 in 15 Japanese patients with amyotrophic lateral sclerosis. The regimen was a single i...
Source: The Journal of Clinical Pharmacology - Category: Drugs & Pharmacology Authors: Tags: J Clin Pharmacol Source Type: research
CONCLUSIONS We identified a novel frameshift mutation associated with JALS. JALS and generally typical ALS, with the same FUS mutation, can appear in a family and present a phenomenon of anticipation. For diagnosis of central nervous system degeneration in adolescents with bulbar symptoms, great attention should be paid to JALS. PMID: 30507891 [PubMed - in process]
Source: Medical Science Monitor - Category: Research Tags: Med Sci Monit Source Type: research
This article is protected by copyright. All rights reserved.
Source: Journal of Neurochemistry - Category: Neuroscience Authors: Tags: Original Article Source Type: research
Vivek S Yedavalli, Abhijit Patil, Parinda ShahJournal of Clinical Imaging Science 2018 8(1):53-53 Motor neuron diseases (MNDs) are a debilitating subset of diseases, which result in progressive neuronal destruction and eventual loss of voluntary muscular function. These entities are often challenging to distinguish and accurately diagnose given overlapping clinical pictures and overall rarity. This group of diseases has a high morbidity and mortality rate overall and delineating each type of disease can help guide appropriate clinical management and improve quality of life for patients. Of all MNDs, amyotrophic lateral sc...
Source: Journal of Clinical Imaging Science - Category: Radiology Authors: Source Type: research
An extensive microglial-astrocyte-monocyte-neuronal cross talk seems to be crucial for normal brain function, development, and recovery. However, under certain conditions neuroinflammatory interactions between brain cells and neuroimmune cells influence disease outcome and brain pathology. Microglial cells express a range of functional states with dynamically pleomorphic profiles from a surveilling status of synaptic transmission to an active player in major events of development such as synaptic elimination, regeneration, and repair. Also, inflammation mediates a series of neurotoxic roles in neuropsychiatric conditions a...
Source: Neuroimmunomodulation - Category: Allergy & Immunology Source Type: research
Mutations in the gene encoding superoxide dismutase 1 (SOD1) lead to misfolding and aggregation of SOD1 and cause familial amyotrophic lateral sclerosis (FALS). However, the implications of wild-type SOD1 misfolding in sporadic forms of ALS (SALS) remain unclear. By screening human memory B cells from a large cohort of healthy elderly subjects, we generated a recombinant human monoclonal antibody (α-miSOD1) that selectively bound to misfolded SOD1, but not to physiological SOD1 dimers. On postmortem spinal cord sections from 121 patients with ALS, α-miSOD1 antibody identified misfolded SOD1 in a majority of cas...
Source: Science Translational Medicine - Category: Biomedical Science Authors: Tags: Research Articles Source Type: research
In this study, we examined SIRT1/AMPK activities and identified significant decreases in the ALS‐MSCs compared to normal healthy control origi nated BM‐MSCs. This decline was restored by pre‐treatment with resveratrol (RSV), measured using quantitative PCR, NAD/NADH assay, and immunoblot analysis. Neuro‐progenitor markers were increased in RSV treated ALS‐MSCs (RSV/ALS‐MSCs). The differentiated ALS‐MSCs exhibited a cell body an d dendritic shape similar to neurons. RSV/ALS‐MSCs showed significantly increased differentiation rate as compared to the untreated differentiated ALS‐MSCs (ALS‐dMSCs). The neuri...
Source: Journal of Tissue Engineering and Regenerative Medicine - Category: Biotechnology Authors: Tags: RESEARCH ARTICLE Source Type: research
ConclusionsThe sensory system in ALS patients was commonly impaired. Cervical muscles showed the highest electrodiagnostic sensitivity. The highest positive rate was generated from detecting the spinal segment onset and the special distal muscles onset ALS in our optimized test method. Through this improved examination based on the most affected individual muscles, physicians can greatly optimize the test duration and significantly reduce patient discomfort.
Source: Neurological Sciences - Category: Neurology Source Type: research
Over a decade ago, the Eurovent study [1] examined the practice of home mechanical ventilation (HMV) in Europe and showed that the prevalence was increasing and indications for use were growing, but that there were wide inequalities in provision. Services offered varied considerably between countries [2], with no obvious standardisation of care. Subsequent prevalence work worldwide has confirmed the expansion of this area, and randomised trials have established clearer indications for initiation of long-term noninvasive ventilation (NIV), e.g. in chronic obstructive pulmonary disease (COPD) [3–5] and motor neurone di...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Editorials Source Type: research
More News: ALS | Brain | Iron | Motor Neurone Disease | Neurology | Pathology