Review on Motor Neuron Disease: Pathogenesis, Biomarkers, Diagnostics, and Current Therapeutic Approaches.

Abstract

Motor neuron disease (MND) is a severe adult-onset neurodegenerative disorder that most commonly presents as amyotrophic lateral sclerosis (ALS). It is the third most prevalent neurodegenerative disease after Alzheimer’s disease and Parkinson’s disease. Over the past ten years, there have been significant improvements in patient treatment as well as a rapid growth in scientific research. Consequently, it now seems possible to develop sensible medications based on significant pathogenic pathways. ALS shows significant variation in both its presentation and subsequent course of clinical development, with an average one-year delay between the beginning of symptoms and diagnosis. A significant percentage of patients survive into their second decade, even though 50% of them die within 3–4 years after the onset of symptoms, mainly from respiratory failure.

Although motor neuron disease is typically sporadic, 10% of cases have a genetic component. According to recent studies, new genes linked to motor neuron diseases have been found. It has been challenging to identify targets for neuroprotection, rehabilitation, and pathological motor neuron disease. The study of pathophysiological pathways through biomarker research increases our understanding of disease and identifies targets for new therapeutic approaches. Males are more likely than females to suffer from motor neuron disorders. The medication used to treat the disease was the riluzole, a disorder modification agent. These medications have the ability to enhance life's cycle. In the treatment of motor neuron disorders, multimodal intensive care is essential This review describes the most important findings and highlights the developments in the pathophysiology, diagnostics, and biomarkers of the disease, as well as the state of motor neuron disease treatment at the moment.

Abbreviations: MND: Motor Neuron Disease; ALS: Amyotrophic Lateral Sclerosis; UMNs: Upper Motor Neurons; LMNs: Lower Motor Neurons; FTD: Fronto-Temporal Dementia; EMG: Electromyography; Progressive muscular atrophy (PMA).

Conclusion:

 Several biomarkers and recently released medications with strong pharmacological effect are used in this investigation. Every neurological condition, including ALS, UMND, LMND, FTD and other, affects the neurons that regulate signal progression and provide the muscular system strength. One of the primary contributions to the development of neuronal abnormalities and the promotion of neurological illnesses is the ubiquitinated protein TDP-43. Previous studies have shown that mutation in the TARDBP and FUS genes also cause neuronal degeneration and muscular atrophy.