A comprehensive review of amyotrophic lateral sclerosis

Abstract

Amyotrophic lateral sclerosis (ALS) is a late-onset fatal neurodegenerative disease affecting motor neurons with an incidence of about 1/100,000. Most ALS cases are sporadic, but 5–10% of the cases are familial ALS. Both sporadic and familial ALS (FALS) are associated with degeneration of cortical and spinal motor neurons. The etiology of ALS remains unknown. However, mutations of superoxide dismutase 1 have been known as the most common cause of FALS. In this study, we provide a comprehensive review of ALS. We cover all aspects of the disease including epidemiology, comorbidities, environmental risk factor, molecular mechanism, genetic factors, symptoms, diagnostic, treatment, and even the available supplement and management of ALS. This will provide the reader with an advantage of receiving a broad range of information about the disease.

The African turquoise killifish: A research organism to study vertebrate aging and diapause

“…For example, vertebrates have a more specialized nervous system which is divided into a distinct central nervous system that consists of a segmented brain and spinal cord, and a peripheral nervous system that contains ganglia and nerves. These two systems are targeted by different age‐related neural degenerative diseases (e.g., Alzheimer's disease in the central nervous system versus amyotrophic lateral sclerosis in the peripheral nervous system) (Niccoli, Partridge & Isaacs, 2017; Wenk, 2003; Zarei et al., 2015). Vertebrates also have a hypothalamo‐pituitary axis, which is critical for neuro‐endocrine signaling during aging (Veldhuis, 2008).…”

The Why of Sporadic Motor Neuron Disease - Many Factors, still a Mystery?

“…Melanin biosynthesis can be disturbed not only by HERVs but also by nicotine 8 that might contribute, in particular when consuming had begun already early in life, although irrespective of stopping its use later 1,2 . Already physiological neuromelanin is accumulating metals (lead, mercury, iron) and, metal works (welding etc.)…”

“…Already physiological neuromelanin is accumulating metals (lead, mercury, iron) and, metal works (welding etc.) have been described as a risk factor 1,2 . Natural neuromelanin has also paramagnetic properties due to its content of iron 6 and might account for a vulnerability to electric shock and eventually even to extremely low-frequency electromagnetic fields 1,2 .…”

“…The etiology of these diseases is still largely obscure. Currently there is some progress in understanding of the more rare familial forms of the disease (for instance about 5-10% of the ALS cases) from associations with genetic mutations 1 . These mutations are localized in the genes for superoxide dismutase-1 1 (SOD-1), and in the C9orf72 and TARDBP genes, respectively.…”

“…Motor neuron diseases comprise amyotrophic lateral sclerosis (ALS), progressive muscular atrophy, progressive bulbar palsy and pseudobulbar palsy 1 . They are neurodegenerative in nature and cause increasing disability and eventually, death.…”