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Brain Sciences
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Neuropathology and Novel Therapies for Motor Neuron Disease
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Neuropathology and Novel Therapies for Motor Neuron Disease

A special issue of Brain Sciences (ISSN 2076-3425). This special issue belongs to the section "Molecular and Cellular Neuroscience".

Deadline for manuscript submissions: closed (15 January 2021) | Viewed by 8989

Special Issue Editor

Dr. Chandler L. Walker

E-Mail Website
Guest Editor
Department of Biomedical and Applied Sciences, Indiana University School of Dentistry, 1121 W. Michigan Street, Room 260A, Indianapolis, Indiana 46202, USA
Interests: neurodegeneration; ALS; neurotrauma; neural regeneration
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Motor neuron diseases (MND) collectively cause progressive loss of motor control to muscles of the body, instigating a range of symptoms, including paralysis of the limbs, speech difficulties (dysphonia), swallowing dysfunction (dysphagia), and respiratory failure. Several forms of MND have been classified, and the most common and well known is amyotrophic lateral sclerosis (ALS). Some MND have a genetic component and are hereditary, though many cases are sporadic and their cause is unknown. Various cell types and structures in both the central nervous system and periphery contribute to the course of disease progression, and once diagnosed the disease has often reached advanced stages. Though the need is critical, there are no cures for MND, and available treatments are limited. As such, understanding disease progression before and beyond symptom onset, and identifying and developing novel therapies to delay or halt progression and extend lifespan, are of great importance. This Special Issue is dedicated to highlighting exciting research into key aspects of disease progression, as well as novel therapeutics that may have positive effects on MND at different stages of disease with the ultimate goal of identifying truly effective treatments for translation to clinical application.

Dr. Chandler L. Walker
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Brain Sciences is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2200 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • Neurodegeneration
  • ALS
  • Neuromuscular disease
  • Neural regeneration
  • Motor neuron disease

Published Papers (2 papers)

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12 pages, 2890 KiB  
Article
Esophageal Peristalsis Disorders in ALS Patients with Dysphagia
by Jerzy Tomik, Klaudia Sowula, Mateusz Dworak, Kamila Stolcman, Małgorzata Maraj and Piotr Ceranowicz
Brain Sci. 2020, 10(11), 820; https://0-doi-org.brum.beds.ac.uk/10.3390/brainsci10110820 - 06 Nov 2020
Cited by 1 | Viewed by 2258
Abstract
To detect the variations of esophageal peristalsis in amyotrophic lateral sclerosis (ALS) patients with predominantly bulbar or predominantly pseudobulbar clinical presentation by using esophageal manometry (EM). Fifteen ALS patients with pseudobulbar clinical presentation (PBP) and 13 patients with bulbar presentation (BP), fulfilling WFN [...] Read more.
To detect the variations of esophageal peristalsis in amyotrophic lateral sclerosis (ALS) patients with predominantly bulbar or predominantly pseudobulbar clinical presentation by using esophageal manometry (EM). Fifteen ALS patients with pseudobulbar clinical presentation (PBP) and 13 patients with bulbar presentation (BP), fulfilling WFN Criteria, were studied. EM was performed in all subjects using a flexible catheter with solid-state transducers. Swallowing was initiated with 5 to 10 mL of water (wet swallows) and saliva (dry swallows) and repeated at 30 s intervals. The manometric parameters were measured automatically and visualized by the computer system. The tracings were analyzed using Synectics software. In PBP patients, an increase of resting pressure value in the upper esophageal sphincter (UES) >45 mmHg, a wave-like course of resting pressure, and toothed peristaltic waves were observed. In BP patients, a low amplitude of peristaltic waves <30 mmHg (mean: 17 ± 5) was recorded, without signs of esophageal motility disturbance at onset or during progression. EM procedure allows objectively distinguishing dysphagia in ALS patients due to bulbar syndrome from the dysphagia due to pseudobulbar syndrome. It is important to identify PBP patients because of their high risk of aspiration. Full article
(This article belongs to the Special Issue Neuropathology and Novel Therapies for Motor Neuron Disease)
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19 pages, 387 KiB  
Opinion
Delayed Onset Muscle Soreness (DOMS): The Repeated Bout Effect and Chemotherapy-Induced Axonopathy May Help Explain the Dying-Back Mechanism in Amyotrophic Lateral Sclerosis and Other Neurodegenerative Diseases
by Balázs Sonkodi
Brain Sci. 2021, 11(1), 108; https://0-doi-org.brum.beds.ac.uk/10.3390/brainsci11010108 - 15 Jan 2021
Cited by 24 | Viewed by 6200
Abstract
Delayed onset muscle soreness (DOMS) is hypothesized to be caused by glutamate excitotoxicity-induced acute compression axonopathy of the sensory afferents in the muscle spindle. Degeneration of the same sensory afferents is implicated in the disease onset and progression of amyotrophic lateral sclerosis (ALS). [...] Read more.
Delayed onset muscle soreness (DOMS) is hypothesized to be caused by glutamate excitotoxicity-induced acute compression axonopathy of the sensory afferents in the muscle spindle. Degeneration of the same sensory afferents is implicated in the disease onset and progression of amyotrophic lateral sclerosis (ALS). A series of “silent” acute compression proprioceptive axonopathies with underlying genetic/environmental factors, damaging eccentric contractions and the non-resolving neuroinflammatory process of aging could lead to ALS disease progression. Since the sensory terminals in the muscle spindle could not regenerate from the micro-damage in ALS, unlike in DOMS, the induced protective microcircuits and their long-term functional plasticity (the equivalent of the repeated bout effect in DOMS) will be dysfunctional. The acute stress invoking osteocalcin, bradykinin, COX1, COX2, GDNF, PGE2, NGF, glutamate and N-methyl-D-aspartate (NMDA) receptors are suggested to be the critical signalers of this theory. The repeated bout effect of DOMS and the dysfunctional microcircuits in ALS are suggested to involve several dimensions of memory and learning, like pain memory, inflammation, working and episodic memory. The spatial encoding of these memory dimensions is compromised in ALS due to blunt position sense from the degenerating proprioceptive axon terminals of the affected muscle spindles. Dysfunctional microcircuits progressively and irreversibly interfere with postural control, with motor command and locomotor circuits, deplete the neuroenergetic system, and ultimately interfere with life-sustaining central pattern generators in ALS. The activated NMDA receptor is suggested to serve the “gate control” function in DOMS and ALS in line with the gate control theory of pain. Circumvention of muscle spindle-loading could be a choice of exercise therapy in muscle spindle-affected neurodegenerative diseases. Full article
(This article belongs to the Special Issue Neuropathology and Novel Therapies for Motor Neuron Disease)
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