Is Multiple System Atrophy An Autoimmune Disease?

Is Multiple System Atrophy An Autoimmune Disease?

Multiple system atrophy (MSA) the exact cause and the pathogenesis is unknown. Some studies have shown that multiple system atrophy can be due to autoimmune disease; however, there is not enough evidence to prove it.1

Is Multiple System Atrophy An Autoimmune Disease?

Pathology Of Multiple System Atrophy

In multiple system atrophy patient’s gliosis occurs in the brain.1 Gliosis is the accumulation of astrocytes (star-shaped cells that support and maintains the brain cells) to the damaged area and it causes scarring in the damaged parts of the brain. Then, glial cytoplasmic inclusions (GCI) develop in the brain and these are characteristic of the disease.

GCIs are abnormal structures and it contains clumps of proteins within the brain. GCI attract a specific type of protein known as alpha-synuclein protein. The alpha-synuclein protein is found in large quantities in the normal human brain, but the function of this protein is not clear.

In all cases of multiple system atrophy clumps of abnormal alpha-synuclein protein is found in many parts of the brain and spinal cord. These abnormal cells (known as inclusions) damages the normal cells which control coordination, autonomic functions, and balance. In multiple system atrophy, there is a progressive loss of neurons in various parts of the brain due to the alpha-synuclein protein. Many researchers believe that this overexpression of the alpha-synuclein protein can be the cause of multiple system atrophy.

The accumulation of alpha-synuclein is also seen in other diseases such as Parkinson’s disease 2 and dementia with Lewy bodies. Multiple system atrophy patients have similar symptoms as seen in Parkinson’s patients and MSA-P is one type of multiple system atrophy which is characterized by more Parkinson symptoms. However, the research done on alpha-synuclein is not adequate to build a strong connection and explain the pathogenesis of multiple system atrophy. More research is needed in this area of study.

Does Immunity Play A Role?

There has not been any conclusive data to suggest that autoimmune can cause multiple system atrophy. There was a study done about “Autoimmune antibody decline in Parkinson’s disease and Multiple System Atrophy; a step towards immunotherapeutic strategies”. The study states anti-α-synuclein naturally occurring autoantibodies are reduced in Parkinson patients and nearly absent in multiple system atrophy patients. Naturally occurring autoantibodies (NAb) is the main component (two thirds) of the human antibody pool. Nabs have regulatory and protective functions in the body. The Nabs have access to the central nervous system, and they remove the cell debris and prevents inflammation in the brain by binding and neutralizing the cytokines. Anti-alpha-synuclein antibodies have a neuroprotective function and this has been demonstrated in many animal models with Parkinson disease.

The study also states that levels of α-synuclein/NAbs immunocomplexes are also reduced in both Parkinson disease and multiple system atrophy patients. The study concluded that in a healthy brain the autoantibodies clears off the abnormal alpha-synuclein, but because of the absent anti-alpha-synuclein NAbs multiple system atrophy patients and Parkinson patients cannot clear off the abnormal alpha-synuclein therefore, these accumulate in the brain and gives rise to the symptoms and signs.

This mechanism is not actually an autoimmune process as immune cells are not attacking the brain in any way but there is not much evidence in the literature regarding autoimmunity giving rise to multiple system atrophy.

Conclusion

The exact cause and pathology of multiple system atrophy are unknown, there are not many studies done about the possibility of autoimmunity giving rise to multiple system atrophy. There is not much evidence to prove this as well in the literature. There was a study done about “Autoimmune antibody decline in Parkinson’s disease and Multiple System Atrophy; a step towards immunotherapeutic strategies”. The study states anti-α-synuclein naturally occurring autoantibodies are reduced in Parkinson patients and nearly absent in multiple system atrophy patients. The Nabs remove the cell debris and prevents inflammation in the brain by binding and neutralizing the cytokines. Reduction in these autoantibodies leads to accumulation of abnormal alpha-synuclein which can give rise to multiple system atrophy.

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