Multiple system atrophy is a multifactorial disease affecting various organs of the body. These organs are controlled by the autonomous nervous system. Various risk factors exist for the development of multiple system atrophy. The condition is progressive and sudden death is seen in complicated condition.
Who Is At Risk For Multiple System Atrophy?
Multiple system atrophy is the condition of shrinkage of the brain and causes autonomous system dysfunction.1 There is no known cause of the disease and it is believed that the disease automatically initiates and progresses without any particular and conclusive cause. However, it is found that what happens during the progression or initiation of disease. Although the exact cause remains unknown, research indicates that there are various risk factors that increase the incidence of the condition. These factors are-
Genetic Factors– Genetic factors have a minimum impact on the development of multiple system atrophy. The disease rarely runs into families and if it does, the pattern of inheritance is not clear. In most of the cases, the condition occurs sporadically.
Mutation– Mutation of various genes also plays an important role in the development of the disease. The major characteristics of the disease are the aggregation of alpha-synuclein.2 The production of synuclein is controlled by synuclein gene SCNA. Researchers believed that variation in this gene causes the condition to progress. TREM2 p.R47H substitution increases the activation of microglial cells leading to initiation of inflammatory processes in the body. Inflammation also increases the risk of multiple system atrophy.
Environmental Factors– Environmental factors such as low education level and toxins present in the environment also lead to a surge in MSA incidences.
Occupational Factors– Some studies conclude that machine operators and assembles at the plant are at higher risk of developing MSA. This risk increases with an increase in the number of years of occupation. Further, regular exposure in the toxic environment during occupation further increases the risk.
Lifestyle– Lifestyle plays a vital role in increasing the multiple system atrophy risk. Although no relation found between the risk of MSA and alcohol consumption, eating excessive meat increases the risk of multiple system atrophy.
Inflammation– It has been found that inflammatory mediators and the accompanying inflammation are also the risk factor for multiple system atrophy. Thus increase dose of aspirin reduces the risk of multiple system atrophy. Proinflammatory cytokines synthesized by the microglial cells and tumor necrosis factor alpha are also thought to be responsible for increasing the risk of multiple system atrophy.
Injury– Multiple concussions i.e. mild traumatic brain injury also causes brain dysfunction. The risk of multiple system atrophy increases if the injury causes damage to the autonomous nervous system. Further, the damage to the brain may be permanent or temporary.
Multiple System Atrophy Complications
As the condition affects various organs, the unmanaged condition causes various complications. Following are the complications that may occur in MSA patients-
Sudden Death– As the muscle of the body becomes inefficient, particularly in MSA-C type, the sudden death of the patient may occur due to spasm in vital muscles.3
Pressure Ulcers– The patients with multiple system atrophy are not able to move frequently due to weakness, fatigue, and stiff muscles; it may lead to pressure ulcers.
Cardiac Disorders– Cardiac arrest may also occur due to multiple system atrophy.
Airway Obstruction– Vocal cord abductors are paralyzed, and the adductor activates to cause active narrowing causing stridor and acute airway obstruction.
Aspiration Pneumonia And Acute Aspiration– Dysphagia is caused due to improper functioning of the laryngeal and esophageal sphincters. This leads to aspiration pneumonia and acute aspiration.
Urinary Tract Dysfunction– The severity of neurogenic urinary tract dysfunction increases.
The exact cause of the disease is not known however various studies are done to identify the risk factors for the development of multiple system atrophy. Gene mutation is thought to be the primary risk factor for this condition. Mutation in a gene controlling the synthesis of synuclein and inflammatory processes increases its risk. Other factors include environmental factors, occupational factors, and lifestyle.