A Review Article on "Amyotrophic Lateral Sclerosis"

The neurodegenerative disease known as amyotrophic lateral sclerosis (ALS) typically affects the motor system, while extra-motor symptoms are becoming more widely acknowledged. Progressive muscle weakness and wasting are caused by the death of upper and lower motor neurons in the motor cortex, brain stem nuclei, and the anterior horn of the spinal cord. ALS frequently begins in a specific area and then spreads to other parts of the body, where respiratory muscle failure usually limits survival to two to five years after the disease first manifests. Extra-motor symptoms, including behavioral abnormalities, executive dysfunction, and linguistic issues, can occur in as many as 50% of cases. These issues are severe enough to meet the clinical criteria for frontotemporal dementia (FTD) in 10% to 15% of people. An autosomal dominant inheritance pattern is suggested by the family history of 10% of ALS patients. The remaining 90% are categorized as having sporadic ALS and do not have any family members who are affected. Only a portion of the causes of ALS are known, and they seem to be diverse. More than 20 genes have been linked to ALS thus far. A hexanucleotide repeat expansion in the C9orf72 gene is the most frequent genetic cause of ALS, accounting for 30% to 50% of family cases and 7% of sporadic cases. These expansions highlight the molecular similarities between ALS and FTD and are also a common cause of frontotemporal dementia. There is currently no cure or effective treatment for ALS, and multidisciplinary care—which includes symptom management, respiratory and nutritional support, and more—remains the cornerstone of treatment. Numerous facets of ALS are covered in this study, including its epidemiology, aetiology, pathophysiology, clinical characteristics, differential diagnosis, investigations, treatment, and prospects for the future.