The Chemistry of Multiple System Atrophy

Introduction

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Multiple System Atrophy is a neurodegenerative disorder where a person’s body slowly deteriorates over time with the blockage of the person’s nerves inside the brain or brain stem.  This causes the eventual failure of almost if not all autonomic functions and eventually all bodily functions, leading to death.  I choose MSA as my Chemistry of project because I knew very little about this disease that wreaks havoc on many unsuspecting victims.  I first found out that it existed when my Grandmother was diagnosed with Parkinson’s disease 3 years ago.  After  about  a year and most medication was just mildly subduing the symptoms, more tests were ran and she was diagnosed with a disease that no one had ever heard of, Multiple System Atrophy.  In my personal experience with this disease it is a very slow, and painful process. Not only for the one diagnosed but for the entire family.  After being diagnosed my Grandmother did everything in her power to fight back, she was not going to let a diagnosis rule her life.  After many physical therapy sessions and just as much voice therapy she was doing it, living with MSA.  It was a slow progression, that unless you saw her with weeks in between every visit you did not notice.  It wasn’t until she took a trip to Florida to say a last goodbye that it hit us all.  When she came home she was a different person, she looked frail, she wasn’t the strong woman we had all known her to be.  That was a long summer.  And an even longer fall.  When the time finally came she was moved to the nursing home right after Thanksgiving.  When she was put in the nursing home she was able to walk and speak fragmented sentences.  Eleven days later she passed away.  That is why I choose to learn more about this awful disease.  It leaves no survivors, only those with empty places in their hearts.  

Composition of ...

MSA is the product of several failures in the nervous and cerebellar systems in the body.  Since MSA is considered a Parkinsonian condition, there is a common underlying condition of these types of diseases is when a protein called alpha-synuclein piles into deposits in the nervous system accumulating so that nerves are unable to function. MSA involves the dementia of the frontal lobes (where reasoning of higher levels occurs) and in the temporal lobes (where memory is processed). Both are likely to occur, with as much as a 3 year lag time between the initial onset of one to the full development.  These are also accompanied by a Parkinson-like movement disorder. One of the possible places for this disease to take initial place is in the synuclein gene, SCNA, which is the gene that provides instruction on the production of alpha-synuclein.



Main Chemicals, Compounds, Components


One of the most commonly found reasons for MSA is the deposits of the protein called alpha-synuclein.  These build-ups particularly occurring in oligodendroglia, a cell that makes a coating on nerve cells that lets them conduct electrical signals quickly, called myelin. Atrophy, or shrinkage and deterioration, of the brain specifically the cerebellum, basal ganglia, and brainstem, which regulate internal body function, digestion, and motor control. However, there are no known causes or cures to MSA.  All are purely just similarities in several cases but remain unproven.

Chemistry's Role

Multiple System Atrophy has a probable known cause in the SNCA gene, where the alpha-synuclein is encoded.  Alpha-synuclein is a protein found inside neural tissue with an unknown purpose at this point in time.  Alpha-synuclein is made up of 140 amino acids.  It also lacks a one single 3D structure that is stable.  The SNCA gene codes the alpha-synuclein to overproduce on nerve endings in the brain.  This then forms the blockage on the nerve endings causing failure in autonomic functions.  This is considered to be the main cause of the side effects and symptoms of Multiple System Atrophy.


Background Research

MSA is a progressive neurodegenerative disorder.  It consists of a combination of symptoms that affect both the autonomic nervous system and movement. (Autonomic: the part of the nervous system that controls involuntary action such as breathing or digestion.) The death of different types of nerve cells in the spinal cord and brain emulate the progressive loss of function and eventually death.  Symptoms come in many forms, some of the most  common are slowness of movement or stiffness, impaired, croaky, or quivering voice, and clumsiness or incoordination.  Multiple System Atrophy, or MSA, is usually classified into two different groups. This disease take course in adults 55 to 60 years of age.  A mandatory presence of autonomic failure is recommended for the diagnosis of MSA-P, or Multiple System Atrophy-Parkinsonian Type.  In this type most patients will display cerebellar signs, including but not limited to abnormal postures, sleep disturbances (REM-rapid eye movement and PLMS-periodic limb movements in sleep), depression, apathy, and anxiety.  MSA-C, the cerebellar form of MSA, is characterized by a progressive onset of symptoms in adults.  These include but are not limited to limb ataxia and cerebellar oculomotor dysfunction. Along with this will come autonomic failure and the start of a parkinsonian development.  It was once called Shy-Drager's Syndrome sharing many qualities with Parkinson's disease. Treatment often included lifestyle changes and medications. MSA can be labeled with possible, probable, or definite.  MSA affects three parts of the nervous system.  The motor portion, the balance and coordination part, and the autonomic division part. Although not much is known about MSA doctors have narrowed it down to few key areas and probable origins in the body and DNA.


Resources

About the Author

Ashton Albright attends Billings Senior High School as a junior.  She enjoys dancing and spending time with friends.  Ashton also enjoys skiing and ice skating, but ironically really dislikes the cold.  She is a part of the Senior High Key Club and Senior Advocates.  Her favorite thing to do after school is spend time with her Lab, Windsor, who has more energy than she could ever wish to have, but he is the best listener and cuddler.  









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