Multiple system atrophy is a rare progressive neurodegenerative disorder that leads to stiffening of the muscles of the body.1 It is a rare disorder that can affect body’s involuntary movements or functions such as breathing, blood pressure and control of muscles. Previously it was known as the Shy-Drager syndrome and it has many symptoms similar to Parkinson’s disease.
What Are The First Signs Of Multiple System Atrophy?
Multiple system atrophy is a progressive disorder and its signs differ from person to person depending on, which part of the brain is affected first and how much of it is degenerated. They mostly present as a group of symptoms. The signs of Parkinsonism mostly occur as a result of degeneration of basal cells of ganglia.2 This will lead to stiff or rigid muscles making the movements of the body slow, trembling and difficult to begin. While walking the normal swinging of the arms is not present, but instead the person walks with rigid shuffling gait. The person will appear to be unsteady and not in balance making them more prone to falling while walking or initiating activities. They have a stooped posture and limbs usually tremble with jerks when standing in one position even for a small amount of time. However, in multiple system atrophy the patient is less prone to having tremors while resting as compared to Parkinson’s disease. The person also might find it difficult to articulate words and the voice changes to a higher pitch and it might become shaky. 3
There is loss of coordination of activities due to degenerative changes occurring in the cerebellum. There is loss of balance and the person might find it difficult to control body movements, especially the arms and legs. They will not be able to walk properly and instead walk with wide irregular steps. They also find it difficult to sit in a particular spot and have difficulty in performing small minor activities such as holding pen and reaching for particular items. They are not able to focus their eyes on objects, due to which there is difficulty in performing small tasks that need precision and rapid movement.
The internal body processes also do not function properly as a result of the autonomic nervous system being affected. The blood pressure of a person might change drastically when alternating between movements and can lead to lightheadedness or dizziness when suddenly standing up (orthostatic hypotension) or it might abnormally increase when the person is lying on bed. There might be bladder abnormalities, retention of urine, constipation, erectile dysfunction and vision of a person might be severely affected.
Multiple system atrophy usually affects adults after the age of 50 years and men are twice more prone to it as compared to women. Multiple system atrophy is of two types on the basis of symptoms that will present first.
In MSA-C there is loss of coordination and a person might find difficulty in maintaining balance. While MSA-P mostly presents as Parkinson’s disease with the exception of absence of tremors and levodopa is mostly ineffective in relieving symptoms of multiple system atrophy.
In both multiple system atrophy types, there is involvement of the autonomic nervous system that is dysfunctional. It is caused due to degeneration of multiple parts of brain and spinal cord. These parts include the basal ganglia that are responsible for controlling voluntary movements of muscles and balancing their action in same or opposite directions. The cerebellum helps in coordination of the activities of muscles or complex movements and also helps in maintaining balance. Autonomic nervous system controls body processes that are involuntary in action such as change in blood pressure in response to change in posture of a person. The reason for these parts undergoing degeneration is not known, but it is thought to be a result of synuclein (protein found in the brain that helps in communication between nerve cells) shape change and its accumulation in the brain cells.
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