Advanced neuroimaging approaches in amyotrophic lateral sclerosis: refining the clinical diagnosis.

Advanced neuroimaging approaches in amyotrophic lateral sclerosis: refining the clinical diagnosis. Expert Rev Neurother. 2020 Jan 15;: Authors: Kassubek J, Müller HP Abstract Introduction: In the last decade, multiparametric magnetic resonance imaging (MRI) has achieved tremendous advances in applications to amyotrophic lateral sclerosis (ALS) to increase the understanding of the associated pathophysiology. The aim of this review is to summarize recent progress in the development of MRI-based techniques aiming to support the clinical diagnosis in ALS.Areas covered: The review of structural and functional MRI applications to ALS and its variants (restricted phenotypes) is focused on the potential of MRI techniques which contribute to the diagnostic work-up of patients with the clinical presentation of a motor neuron disease. The potential of specific MRI methods for patient diagnosis and monitoring is discussed, and the future design of clinical MRI applications to ALS is conceptualized.Expert opinion: Current multiparametric MRI allows for the use as a clinical biological marker and a technical instrument in the clinical diagnosis of patients with ALS and also of patients with ALS variants. Composite neuroimaging indices of specific anatomical areas derived from different MRI techniques might guide in the diagnostic applications to ALS. Such a development of ALS-specific MRI-based composite scores with sufficient discriminative power versus ALS mimics at ind...
Source: Expert Review of Neurotherapeutics - Category: Neurology Tags: Expert Rev Neurother Source Type: research

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The primary role of magnetic resonance imaging (MRI) in routine diagnostic work-up of motor neuron disease patients is currently still largely limited to exclusion of relevant non-degenerative pathologies. We here present an illustrative case of a 63-year-old woman with early stage Frontotemporal-Dementia-Amyotrophic-Lateral-Sclerosis (FTD-ALS) spectrum disorder showing a striking hypointense signal of the cortical band along the precentral gyrus, termed “Motor Band Sign” (MBS). Based on this finding, we analysed the frequency of the MBS in clinical routine MRIs in a large consecutive series of ALS patients (MR...
Source: Journal of the Neurological Sciences - Category: Neurology Authors: Source Type: research
Publication date: Available online 7 August 2019Source: NeuroscienceAuthor(s): Stefania Marcuzzo, Benedetta Terragni, Silvia Bonanno, Davide Isaia, Paola Cavalcante, Cristina Cappelletti, Emilio Ciusani, Ambra Rizzo, Giulia Regalia, Natsue Yoshimura, Giovanni Stefano Ugolini, Marco Rasponi, Giulia Bechi, Massimo Mantegazza, Renato Mantegazza, Pia Bernasconi, Ludovico MinatiAbstractAmyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting the corticospinal tract and leading to motor neuron death. According to a recent study, magnetic resonance imaging-visible changes suggestive of neurodegeneration seem a...
Source: Neuroscience - Category: Neuroscience Source Type: research
Conclusion The key problem with the ND field is the lack of understanding in the events preceding the development of protein-based markers – such as Tau – currently used to diagnose NDs. By this stage, the diseases become more difficult to treat. SncRNAs play an important regulatory role in the maintenance of the homeostatic brain. Therefore, changes in their concentration levels can be indicative of mechanistic changes that could precede protein-based markers. One single sncRNA biomarker is unlikely to differentiate between diseases. However, a combination of sncRNA biomarkers could be illustrative of the me...
Source: Frontiers in Genetics - Category: Genetics & Stem Cells Source Type: research
In amyotrophic lateral sclerosis (ALS), the “zebra sign” in the precentral gyrus on phase difference enhanced magnetic resonance imaging (PADRE) recently has been reported as a possible imaging biomarker for upper motor neuron (UMN) involvement. A previous study has shown that the “zebra sign” allowed us to differentiate patients with ALS from healthy subjects with excellent accuracy. We validated the usefulness of the sign for differentiating patients with ALS from healthy subjects and investigated whether the “zebra sign” can be observed other neurodegenerative disorders with UMN involvement.
Source: Journal of the Neurological Sciences - Category: Neurology Authors: Source Type: research
Conclusions The momentous advances in spinal imaging in ALS suggest the spinal metrics may soon be used as validated diagnostic, monitoring, and prognostic markers, contributing both to individualized patient care and pharmacological trials. Author Contributions ME, GQ, PB, and P-FP contributed equally to the conceptualization, drafting, and revision of the manuscript. Funding Peter Bede is supported by the Health Research Board (HRB—Ireland; HRB EIA-2017-019), the Iris O'Brien Foundation, the Irish Institute of Clinical Neuroscience IICN—Novartis Ireland Research Grant, and the Research Motor Neur...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
AbstractThe fourteen cases of Hirayama disease (HD) are presented in this article. HD is seldom disease characterized by juvenile muscular atrophy of upper extremities and benign course. All cases were diagnosed in the Research Center of Neurology (Moscow, Russia) during the year 2015 –2017. Such methods as MRI (magnetic resonance imaging), EMG (electromyography), and NCS (nerve conduction studies) have been used to confirm diagnosis of HD. Transcranial magnetic stimulation was used to exclude upper motor neuron involvement in two cases. The original scale of neurological distu rbances in HD has been proposed by auth...
Source: Neurological Sciences - Category: Neurology Source Type: research
This report illustrates that MRI plexus abnormalities can be seen in amyotrophic lateral sclerosis, possibly before symptoms, and may have clinical value in the diagnostic process.
Source: Journal of Clinical Neuromuscular Disease - Category: Neurology Tags: Case Review 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
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...
Source: Expert Review of Neurotherapeutics - Category: Neurology Tags: Expert Rev Neurother Source Type: research
AbstractPurposeThis paper aims to analyze the contribution of mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) in the detection of microstructural abnormalities in amyotrophic lateral sclerosis (ALS) and to evaluate the degree of agreement between structural and functional changes through concomitant diffusion tensor imaging (DTI), transcranial magnetic stimulation (TMS), and clinical assessment.MethodsFourteen patients with ALS and 11 healthy, age- and gender-matched controls were included. All participants underwent magnetic resonance imaging including DTI. TMS was additionally performed in ALS ...
Source: Neuroradiology - Category: Radiology Source Type: research
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