How Long Do You Live After Being Diagnosed With Multiple System Atrophy?

Multiple system atrophy (MSA) is a rare disorder, the prevalence of MSA ranges from 1-9 per 100,000 population and in the United States, MSA affects about 15,000 to 50,000 Americans. Both males and females are affected equally. MSA-P is seen more in the Western hemisphere and MSA-C is seen more in the Eastern Hemispheres. The onset is after 30 years usually, many patients are seen at the ages of 55-60 years.

Even though it is a rare disease the prognosis is quite poor as multiple system atrophy does not have any cure or treatment to stop or reduce the progression of the disease. Many research studies have done to find the life expectancy or the median survival age after the diagnosis of multiple system atrophy in patients. So, according to the studies many patients die either because of the disease or due to its complication within 6-10 years after the onset of symptoms. The progressive loss of motor functions eventually makes the patient bedridden and eventually they die of a cardio-pulmonary problem.⁽¹⁾

A Japanese study was done on 230 multiple system atrophy patients regarding the disease progression and survival, there were 131 men and 99 women were included in the study. 155 patients had MSA-C and 75 patients had MSA-P. The median interval requiring a walking aid, confiding to a wheelchair, bedridden and death was respectively 3,5,8,9 years. The median age for both motor and autonomic symptoms to occur together was 2 years and patients who had both these symptoms together within 3 years had a shorter life expectancy. MSA-C patients had a slower functional deterioration compared to the MSA-P, however, there was no difference in the survival rates. Patients who got the disease at older ages were bedridden quickly, they had reduced life expectancy.⁽²⁾

According to this study the factors that affect the survival rate

Age of onset – older patients had a poor prognosis
Clinical phenotype – MSA-P patients have rapid functional deterioration but the survival rates of MSA-P and MSA-C are the same. If both motor symptoms and autonomic symptoms occur together within 3 years after initial symptoms the survival rates were less.
Gender does not have any impact on the life expectancy of multiple system atrophy patients.

Causes Of Death In MSA Patients

Bronchopneumonia (48%)
Cardiopulmonary arrest – 33.3%
Sudden death (21%) – most sudden death is due to cardiopulmonary arrest, few cases due to aspiration and laryngeal stridor and some patient’s cause of sudden death are unknown.⁽³⁾
Bronchopneumonia, cardiopulmonary arrest, and sudden death are the three common causes of death in multiple system atrophy patients.

The other causes are:

Urinary tract infections – urinary dysfunction is the most common problem the multiple system atrophy patients face and 50% of them develop recurrent lower urinary tract infections and die from complications because of that. Most patients are on urinary catheters due to urinary incontinence.
Aspiration pneumonia 9.5%
Acute aspiration 4.8% – many patients have swallowing difficulties and are on percutaneous gastrostomy (PEG) feeding tubes to minimize the swallowing difficulties and aspirations, however, the aspirations tend to occur even while the patients are on the PEG tube.
Wasting syndrome – 14.3%

Conclusion

Multiple system atrophy is a rare neurodegenerative disorder. Even though it’s a rare disease the prognosis is quite poor as multiple system atrophy does not have any cure or treatment to stop or reduce the progression of the disease. according to the studies many patients die either because of the disease or due to its complication within 6-10 years after the onset of symptoms. The median interval requiring a walking aid, confiding to a wheelchair, bedridden and death was respectively 3,5,8,9 years. Factors affecting the survival are age at onset (older age reduce survival rates) and clinical phenotype if both motor symptoms and autonomic symptoms occur together within 3 years after initial symptoms the life expectancy is less. The common causes of death are bronchopneumonia (48%), cardiopulmonary arrest (33.3%), sudden death (21% including the cardiopulmonary arrest), urinary tract infections, acute aspiration, and aspiration pneumonia.

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How Long Do You Live After Being Diagnosed With Multiple System Atrophy?