scholarly journals Estimation of the prevalence and incidence of motor neuron diseases in two Spanish regions: Catalonia and Valencia

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Maria A. Barceló ◽  
Mònica Povedano ◽  
Juan F. Vázquez-Costa ◽  
Álvaro Franquet ◽  
Marta Solans ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Muscular Atrophy ◽  
Population Based ◽  
Motor Neuron Diseases ◽  
Spanish Regions ◽  
Confounding Variables ◽  
The Individual ◽  
Primary Lateral ◽  
Lateral Sclerosis ◽  
Part Model

AbstractAccording to the degree of upper and lower motor neuron degeneration, motor neuron diseases (MND) can be categorized into amyotrophic lateral sclerosis (ALS), primary lateral sclerosis (PLS) or progressive muscular atrophy (PMA). Although several studies have addressed the prevalence and incidence of ALS, there is a high heterogeneity in their results. Besides this, neither concept has been previously studied in PLS or PMA. Thus, the objective of this study was to estimate the prevalence and incidence of MND, (distinguishing ALS, PLS and PMA), in the Spanish regions of Catalonia and Valencia in the period 2011–2019. Two population-based Spanish cohorts were used, one from Catalonia and the other from Valencia. Given that the samples that comprised both cohorts were not random, i.e., leading to a selection bias, we used a two-part model in which both the individual and contextual observed and unobserved confounding variables are controlled for, along with the spatial and temporal dependence. The prevalence of MND was estimated to be between 3.990 and 6.334 per 100,000 inhabitants (ALS between 3.248 and 5.120; PMA between 0.065 and 0.634; and PLS between 0.046 and 1.896), and the incidence between 1.682 and 2.165 per 100,000 person-years for MND (ALS between 1.351 and 1.754; PMA between 0.225 and 0.628; and PLS between 0.409–0.544). Results were similar in the two regions and did not differ from those previously reported for ALS, suggesting that the proposed method is robust and that neither region presents differential risk or protective factors.

Motor neuron disease

2018 ◽  
Author(s):  
Martin R. Turner
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Motor Neurons ◽  
Corticospinal Tract ◽  
Muscular Atrophy ◽  
Muscle Denervation ◽  
Genetic Overlap ◽  
Upper Motor Neurons ◽  
Primary Lateral ◽  
Lateral Sclerosis

Motor neuron disease (MND) is characterized by progressive muscular weakness due to simultaneous degeneration of lower and upper motor neurons (L/UMNs). Involvement of LMNs, arising from the anterior horns of the spinal cord and brainstem, leads to secondary wasting as a result of muscle denervation. Involvement of the UMNs of the motor cortex and corticospinal tract results in spasticity. In ~85% of cases, there is clear clinical involvement of both, and the condition is termed ‘amyotrophic lateral sclerosis’ (ALS; a term often used synonymously with MND). In ~13% of cases, there may be only LMN signs apparent, in which case the condition is termed ‘progressive muscular atrophy’, although such cases have a natural history that is to largely identical to that of ALS. In a very small group of patients (~2%), there are only UMN signs for at least the first 4 years, in which case the condition is termed ‘primary lateral sclerosis’; such cases have a uniformly slower progression. There is clinical, neuropathological, and genetic overlap between MND and some forms of frontotemporal dementia.

scholarly journals Progressive brainstem pathology in motor neuron diseases: Imaging data from amyotrophic lateral sclerosis and primary lateral sclerosis

Data in Brief ◽  
2020 ◽  
Vol 29 ◽  
pp. 105229 ◽  
Author(s):  
Peter Bede ◽  
Rangariroyashe H. Chipika ◽  
Eoin Finegan ◽  
Stacey Li Hi Shing ◽  
Kai Ming Chang ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Primary Lateral Sclerosis ◽  
Imaging Data ◽  
Motor Neuron Diseases ◽  
Primary Lateral ◽  
Lateral Sclerosis

scholarly journals Motor Neuron Diseases in Sub-Saharan Africa: The Need for More Population-Based Studies

2015 ◽  
Vol 2015 ◽  
pp. 1-9 ◽  
Author(s):  
Emmanuel Quansah ◽  
Thomas K. Karikari
Keyword(s):  
Motor Neuron ◽  
Muscular Atrophy ◽  
Clinical Care ◽  
Treatment Options ◽  
Molecular Genetic ◽  
Population Based ◽  
Sub Saharan Africa ◽  
Motor Neuron Diseases ◽  
African Populations ◽  
Sub Saharan

Motor neuron diseases (MNDs) are devastating neurological diseases that are characterised by gradual degeneration and death of motor neurons. Major types of MNDs include amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA). These diseases are incurable, with limited disease-modifying treatment options. In order to improve MND-based biomedical research, drug development, and clinical care, population-based studies will be important. These studies, especially among less-studied populations, might identify novel factors controlling disease susceptibility and resistance. To evaluate progress in MND research in Africa, we examined the published literature on MNDs in Sub-Saharan Africa to identify disease prevalence, genetic factors, and other risk factors. Our findings indicate that the amount of research evidence on MNDs in Sub-Saharan Africa is scanty; molecular and genetics-based studies are particularly lacking. While only a few genetic studies were identified, these studies strongly suggest that there appear to be population-specific causes of MNDs among Africans. MND genetic underpinnings vary among different African populations and also between African and non-African populations. Further studies, especially molecular, genetic and genomic studies, will be required to advance our understanding of MND biology among African populations. Insights from these studies would help to improve the timeliness and accuracy of clinical diagnosis and treatment.

scholarly journals ALS2-Related Motor Neuron Diseases: From Symptoms to Molecules

Biology ◽  
2022 ◽  
Vol 11 (1) ◽  
pp. 77
Author(s):  
Marcello Miceli ◽  
Cécile Exertier ◽  
Marco Cavaglià ◽  
Elena Gugole ◽  
Marta Boccardo ◽  
...  
Keyword(s):  
Motor Neuron ◽  
System Level ◽  
Gene Encoding ◽  
Motor Neuron Diseases ◽  
Spastic Paralysis ◽  
Protein Partners ◽  
Juvenile Amyotrophic Lateral Sclerosis ◽  
The Brain ◽  
Primary Lateral ◽  
Lateral Sclerosis

Infantile-onset Ascending Hereditary Spastic Paralysis, Juvenile Primary Lateral Sclerosis and Juvenile Amyotrophic Lateral Sclerosis are all motor neuron diseases related to mutations on the ALS2 gene, encoding for a 1657 amino acids protein named Alsin. This ~185 kDa multi-domain protein is ubiquitously expressed in various human tissues, mostly in the brain and the spinal cord. Several investigations have indicated how mutations within Alsin’s structured domains may be responsible for the alteration of Alsin’s native oligomerization state or Alsin’s propensity to interact with protein partners. In this review paper, we propose a description of differences and similarities characterizing the above-mentioned ALS2-related rare neurodegenerative disorders, pointing attention to the effects of ALS2 mutation from molecule to organ and at the system level. Known cases were collected through a literature review and rationalized to deeply elucidate the neurodegenerative clinical outcomes as consequences of ALS2 mutations.

ParaCom An IoT based affordable solution enabling people with limited mobility to interact with machines

2022 ◽  
Vol 6 (1) ◽  
pp. 0-0
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Primary Lateral Sclerosis ◽  
Motor Neuron Diseases ◽  
Body Balance ◽  
Limited Mobility ◽  
Morse Code ◽  
Communication Controller ◽  
Primary Lateral ◽  
Lateral Sclerosis

There are many people in this world who don’t have the ability to communicate with others due to some unforeseen accident. User’s who are paralyzed and/or suffering from different Motor Neuron Diseases (MND) like Amyotrophic Lateral Sclerosis (ALS), Primary Lateral Sclerosis etc, by making them more independent. Patients suffering from these diseases are not able to move their arms and legs, lose their body balance and the ability to speak. Here we propose an IoT based communication controller using the concept of Morse Code Technology which controls the smartphone of the user. This paper proposes a solution to give the user ability to communicate to other people using machine as an intermediator. The device will require minimal inputs from the user.

Types of Motor Neuron Diseases

2017 ◽  
Author(s):  
Nimish Thakore ◽  
Erik P Pioro
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Spinal Muscular Atrophy ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Motor Neurons ◽  
Muscular Atrophy ◽  
Motor Neuron Diseases ◽  
The Subject ◽  
Upper Motor Neurons ◽  
Lateral Sclerosis

Disorders of lower motor neurons (LMNs, or anterior horn cells) and upper motor neurons (UMNs), jointly termed motor neuron disorders (MNDs), are diverse and numerous. The prototypical MND, namely amyotrophic lateral sclerosis (ALS), a relentlessly progressive lethal disorder of adults, is the subject of another section and will not be discussed further here. Other MNDs include spinal muscular atrophy (SMA), of which there are four types: Kennedy’s disease, Brown-Violetto-Van Laere, and Fazio-Londe syndromes, lower motor neuron disorders as part of neurodegenerations and secondary motor neuron disease as part of malignancy, radiation and infection.

scholarly journals The history behind ALS type 8: from the first phenotype description to the discovery of VAPB mutation

2021 ◽  
Author(s):  
Luiz Eduardo NOVIS ◽  
Mariana SPITZ ◽  
Hélio A. G. TEIVE
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Autosomal Dominant ◽  
Late Onset ◽  
Muscular Atrophy ◽  
Phenotype Correlation ◽  
Motor Neuron Diseases ◽  
The Past ◽  
Phenotype Description ◽  
Lateral Sclerosis

ABSTRACT Over the past 68 years, the Finkel type late-onset adult autosomal dominant spinal muscular atrophy (SMA) that is allelic with amyotrophic lateral sclerosis-8 (ALS8) gained a genotype-phenotype correlation among the motor neuron diseases through the work of groups led by Zatz and Marques Jr.

scholarly journals Motor Neuron Diseases and Neuroprotective Peptides: A Closer Look to Neurons

2021 ◽  
Vol 13 ◽  
Author(s):  
Emanuela Zuccaro ◽  
Diana Piol ◽  
Manuela Basso ◽  
Maria Pennuto
Keyword(s):  
Motor Neuron ◽  
Motor Neurons ◽  
Muscular Atrophy ◽  
Selective Vulnerability ◽  
Therapeutic Interventions ◽  
Motor Neuron Diseases ◽  
Somatomedin C ◽  
Beneficial Effects ◽  
Pituitary Adenylate Cyclase ◽  
Lateral Sclerosis

Motor neurons (MNs) are specialized neurons responsible for muscle contraction that specifically degenerate in motor neuron diseases (MNDs), such as amyotrophic lateral sclerosis (ALS), spinal and bulbar muscular atrophy (SBMA), and spinal muscular atrophy (SMA). Distinct classes of MNs degenerate at different rates in disease, with a particular class named fast-fatigable MNs (FF-MNs) degenerating first. The etiology behind the selective vulnerability of FF-MNs is still largely under investigation. Among the different strategies to target MNs, the administration of protective neuropeptides is one of the potential therapeutic interventions. Pituitary adenylate cyclase-activating polypeptide (PACAP) is a neuropeptide with beneficial effects in many neurodegenerative diseases, including Alzheimer’s disease, Parkinson’s disease, and more recently SBMA. Another neuropeptide that has a neurotrophic effect on MNs is insulin-like growth factor 1 (IGF-1), also known as somatomedin C. These two peptides are implicated in the activation of neuroprotective pathways exploitable in the amelioration of pathological outcomes related to MNDs.

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