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Life
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Motor Neuron Disease
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Motor Neuron Disease

A special issue of Life (ISSN 2075-1729). This special issue belongs to the section "Physiology and Pathology".

Deadline for manuscript submissions: closed (22 September 2023) | Viewed by 10550

Special Issue Editor

Dr. Jessica Mandrioli

E-Mail Website
Guest Editor
1. Department of Biomedical, Metabolic and Neural Sciences, University of Modena and Reggio Emilia, 41125 Modena, Italy
2. Neurology Unit, Department of Neuroscience, Azienda Ospedaliero Universitaria di Modena, 41126 Modena, Italy
Interests: amyotrophic lateral sclerosis; neurological diseases; neuroepidemiology; neurodegeneration; neuroinflammation; neurogenetics; microbiota
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

We are delighted to announce a call for submissions to a Special Issue of Life on the topic of “Motor Neuron Diseases”.

Motor neuron diseases are a heterogenous group of diseases characterized by the underlying degeneration of motor neurons which diversely contributes to several pathomechanisms at play in affected individuals.

This Special Issue looks to update and advance the current knowledge on motor neuron diseases’ epidemiology, clinical and biological signatures, mechanisms, and treatments by gathering a collection of high-quality review and primary research articles.

The suggested potential topics include but are not restricted to the following: (i) the epidemiological and clinical characteristics of motor neuron diseases; (ii) genetic, environmental and other risk factors; and (iii) evaluation of the impact of interventions, including pharmaceutical and nonpharmaceutical approaches.

Papers providing new insights into mechanisms of mitochondrial dysfunction, neuroinflammation, protein misfolding, cellular trafficking dysfunction, and oxidative stress in the context of motor neuron diseases are welcome.

Finally, we encourage contributions highlighting overlapping genetic and clinical features with other neurodegenerative diseases, such as dementia, parkinsonism, and psychiatric or immune-mediated diseases.

Dr. Jessica Mandrioli
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. Life 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 2600 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

  • motor neuron diseases
  • neurodegeneration
  • epidemiology
  • pathomechanisms
  • disease heterogeneity
  • prognosis
  • clinical features
  • treatment
  • interventions
  • overlapping diseases

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Published Papers (4 papers)

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Research

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13 pages, 485 KiB  
Article
Implications of Artificial Intelligence Algorithms in the Diagnosis and Treatment of Motor Neuron Diseases—A Review
by Diego Lopez-Bernal, David Balderas, Pedro Ponce, Mario Rojas and Arturo Molina
Life 2023, 13(4), 1031; https://doi.org/10.3390/life13041031 - 17 Apr 2023
Cited by 4 | Viewed by 2133
Abstract
Motor neuron diseases (MNDs) are a group of chronic neurological disorders characterized by the progressive failure of the motor system. Currently, these disorders do not have a definitive treatment; therefore, it is of huge importance to propose new and more advanced diagnoses and [...] Read more.
Motor neuron diseases (MNDs) are a group of chronic neurological disorders characterized by the progressive failure of the motor system. Currently, these disorders do not have a definitive treatment; therefore, it is of huge importance to propose new and more advanced diagnoses and treatment options for MNDs. Nowadays, artificial intelligence is being applied to solve several real-life problems in different areas, including healthcare. It has shown great potential to accelerate the understanding and management of many health disorders, including neurological ones. Therefore, the main objective of this work is to offer a review of the most important research that has been done on the application of artificial intelligence models for analyzing motor disorders. This review includes a general description of the most commonly used AI algorithms and their usage in MND diagnosis, prognosis, and treatment. Finally, we highlight the main issues that must be overcome to take full advantage of what AI can offer us when dealing with MNDs. Full article
(This article belongs to the Special Issue Motor Neuron Disease)
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16 pages, 1247 KiB  
Article
Insight into Elderly ALS Patients in the Emilia Romagna Region: Epidemiological and Clinical Features of Late-Onset ALS in a Prospective, Population-Based Study
by Giulia Gianferrari, Ilaria Martinelli, Cecilia Simonini, Elisabetta Zucchi, Nicola Fini, Maria Caputo, Andrea Ghezzi, Annalisa Gessani, Elena Canali, Mario Casmiro, Patrizia De Massis, Marco Curro’ Dossi, Silvia De Pasqua, Rocco Liguori, Marco Longoni, Doriana Medici, Simonetta Morresi, Alberto Patuelli, Maura Pugliatti, Mario Santangelo, Elisabetta Sette, Filippo Stragliati, Emilio Terlizzi, Veria Vacchiano, Lucia Zinno, Salvatore Ferro, Amedeo Amedei, Tommaso Filippini, Marco Vinceti, ERRALS GROUP and Jessica Mandrioliadd Show full author list remove Hide full author list
Life 2023, 13(4), 942; https://doi.org/10.3390/life13040942 - 3 Apr 2023
Cited by 3 | Viewed by 2830
Abstract
Few studies have focused on elderly (>80 years) amyotrophic lateral sclerosis (ALS) patients, who represent a fragile subgroup generally not included in clinical trials and often neglected because they are more difficult to diagnose and manage. We analyzed the clinical and genetic features [...] Read more.
Few studies have focused on elderly (>80 years) amyotrophic lateral sclerosis (ALS) patients, who represent a fragile subgroup generally not included in clinical trials and often neglected because they are more difficult to diagnose and manage. We analyzed the clinical and genetic features of very late-onset ALS patients through a prospective, population-based study in the Emilia Romagna Region of Italy. From 2009 to 2019, 222 (13.76%) out of 1613 patients in incident cases were over 80 years old at diagnosis, with a female predominance (F:M = 1.18). Elderly ALS patients represented 12.02% of patients before 2015 and 15.91% from 2015 onwards (p = 0.024). This group presented with bulbar onset in 38.29% of cases and had worse clinical conditions at diagnosis compared to younger patients, with a lower average BMI (23.12 vs. 24.57 Kg/m2), a higher progression rate (1.43 vs. 0.95 points/month), and a shorter length of survival (a median of 20.77 vs. 36 months). For this subgroup, genetic analyses have seldom been carried out (25% vs. 39.11%) and are generally negative. Finally, elderly patients underwent less frequent nutritional- and respiratory-supporting procedures, and multidisciplinary teams were less involved at follow-up, except for specialist palliative care. The genotypic and phenotypic features of elderly ALS patients could help identify the different environmental and genetic risk factors that determine the age at which disease onset occurs. Since multidisciplinary management can improve a patient’s prognosis, it should be more extensively applied to this fragile group of patients. Full article
(This article belongs to the Special Issue Motor Neuron Disease)
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12 pages, 922 KiB  
Article
MND Phenotypes Differentiation: The Role of Multimodal Characterization at the Time of Diagnosis
by Giuseppe Meo, Pilar M. Ferraro, Marta Cillerai, Chiara Gemelli, Corrado Cabona, Federico Zaottini, Luca Roccatagliata, Flavio Villani, Angelo Schenone and Claudia Caponnetto
Life 2022, 12(10), 1506; https://doi.org/10.3390/life12101506 - 27 Sep 2022
Cited by 3 | Viewed by 1896
Abstract
Pure/predominant upper motor neuron (pUMN) and lower motor neuron (pLMN) diseases have significantly better prognosis compared to amyotrophic lateral sclerosis (ALS), but their early differentiation is often challenging. We therefore tested whether a multimodal characterization approach embedding clinical, cognitive/behavioral, genetic, and neurophysiological data [...] Read more.
Pure/predominant upper motor neuron (pUMN) and lower motor neuron (pLMN) diseases have significantly better prognosis compared to amyotrophic lateral sclerosis (ALS), but their early differentiation is often challenging. We therefore tested whether a multimodal characterization approach embedding clinical, cognitive/behavioral, genetic, and neurophysiological data may improve the differentiation of pUMN and pLMN from ALS already by the time of diagnosis. Dunn’s and chi-squared tests were used to compare data from 41 ALS, 34 pLMN, and 19 pUMN cases with diagnoses confirmed throughout a 2-year observation period. Area under the curve (AUC) analyses were implemented to identify the finest tools for phenotypes discrimination. Relative to ALS, pLMN showed greater lower limbs weakness, lower UMN burden, and progression rate (p < 0.001–0.04). PUMN showed a greater frequency of lower limbs onset, higher UMN burden, lower ALSFRS-r and MRC progression rates (p < 0.001–0.03), and greater ulnar compound muscle action potential (CMAP) amplitude and tibial central motor conduction time (CMCT) (p = 0.05–0.03). The UMN progression rate was the finest measure to identify pLMN cases (AUC = 90%), while the MRC progression rate was the finest tool to identify pUMN (AUC = 82%). Detailed clinical and neurophysiological examinations may significantly improve MNDs differentiation, facilitating prognosis estimation and ameliorating stratification strategies for clinical trials enrollment. Full article
(This article belongs to the Special Issue Motor Neuron Disease)
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Other

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18 pages, 675 KiB  
Opinion
Miswired Proprioception in Amyotrophic Lateral Sclerosis in Relation to Pain Sensation (and in Delayed Onset Muscle Soreness)—Is Piezo2 Channelopathy a Principal Transcription Activator in Proprioceptive Terminals Besides Being the Potential Primary Damage?
by Balázs Sonkodi
Life 2023, 13(3), 657; https://doi.org/10.3390/life13030657 - 27 Feb 2023
Cited by 13 | Viewed by 2590
Abstract
Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative multisystem disease, with an unknown pathomechanism, resulting in progressive motoneuron loss. In 90–95% of cases, ALS is sporadic, but close to 10% of ALS is familial with inherited gene mutations from family members. Recently, a [...] Read more.
Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative multisystem disease, with an unknown pathomechanism, resulting in progressive motoneuron loss. In 90–95% of cases, ALS is sporadic, but close to 10% of ALS is familial with inherited gene mutations from family members. Recently, a non-contact dying-back injury mechanism theory of ALS postulated that irreversible intrafusal proprioceptive terminal degeneration induces the non-resolving progressive impairment of the proprioceptive circuitry, leading to motoneuron loss, progressive overloading and depletion of the central nervous system, and eventually to death. The current manuscript proposes that irreversible Piezo2 channelopathy of this proprioceptive terminal degeneration induces constantly activated and dysregulated transcription process in ALS, providing access to underlying pathogenic gene variants and letting the cell-type-specific noncoding DNA mutations become more apparent. This opinion piece proposes that ALS genes are associated with the Piezo2 channelopathy mechanism both downstream and upstream, and their mutations, along with the aging process, could explain the non-contact dying-back injury mechanism theory of ALS. Moreover, irreversible microinjury of the Piezo2 ion channel could be the primary damage or the root cause of death in ALS. Finally, the current manuscript also depicts the pathomechanism as to why ALS is considered a painless disease. Full article
(This article belongs to the Special Issue Motor Neuron Disease)
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