Multiple System Atrophy
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Multiple system atrophy (MSA) is a rare, progressive degenerative neurological disorder affecting your body’s involuntary (autonomic) functions, including blood pressure, breathing, bladder function and motor control.
Formerly called Shy-Drager syndrome, olivopontocerebellar atrophy or striatonigral degeneration, MSA shares many Parkinson’s disease-like symptoms, such as slow movement, rigid muscles and poor balance.
As the name represents, MSA is a multi-system neurodegenerative disorder that is characterised by a combination of symptoms including parkinsonism, cerebellar and pyramidal tract signs, and autonomic dysfunction. These overlap of symptoms make its diagnosis confusing clinically.
MSA is a combination of parkinsonism with autonomic dysfunction.
It includes Parkinsonism symptoms characterised by tremor (usually resting), stiffness or rigidity, Bradykinesia (slowed movements), and postural and gait instability.
Cerebellar signs include tremor with activity, past pointing (when reaching for objects), incoordination, slurred speech, and an unstable “drunk-like” gait.
Autonomic symptoms such as non-motor features like postural changes in heart rate, blood pressure, bowel, bladder and sexual functions.
These include light headedness/dizziness (related to changes in position, such as standing, and referred to as orthostatic hypotension), fainting spells, urinary retention or urgency (even incontinence), erectile dysfunction in men, constipation, and abnormal heat/cold intolerance and problems with sweat production.
Other problems like sleep disorders, restless legs, change in voice, and breathing difficulties are frequently seen in MSA.
It includes Parkinsonism symptoms characterised by tremor (usually resting), stiffness or rigidity, Bradykinesia (slowed movements), and postural and gait instability.
Cerebellar signs include tremor with activity, past pointing (when reaching for objects), incoordination, slurred speech, and an unstable “drunk-like” gait.
Autonomic symptoms such as non-motor features like postural changes in heart rate, blood pressure, bowel, bladder and sexual functions.
These include light headedness/dizziness (related to changes in position, such as standing, and referred to as orthostatic hypotension), fainting spells, urinary retention or urgency (even incontinence), erectile dysfunction in men, constipation, and abnormal heat/cold intolerance and problems with sweat production.
Other problems like sleep disorders, restless legs, change in voice, and breathing difficulties are frequently seen in MSA.
So far there is no blood test to identify MSA from other parkinsonian syndromes. Biomarkers such as alpha-synuclein, a protein abundantly found in Lewy body disorders, are being explored but remain controversial
MSA causes shrinkage of portions of your brain (cerebellum, basal ganglia and brainstem) that regulate many body functions, digestion and motor control.
When MSA brain is seen under a microscope, the damaged brain tissue shows nerve cells (neurons) that contain an abnormal amount of a protein called alpha-synuclein. Some clinicians say that this protein may be overexpressed in pons, cerebellum and basal ganglia in multiple system atrophy.
MRI brain is done to rule out other potential causes of parkinsonism like multiple strokes, inflammatory conditions, or accumulation of fluid in the brain. In MSA a brain MRI shows atrophy or degeneration of brainstem (pons, olivary nuclei), cerebellum, and basal ganglia. If the patients presents with parkinsonism and multiple parts of brain atrophy, then It might be diagnosed as MSA features These classic signs are not always seen though, so repeat imaging may be needed as the disease progresses and if concern regarding diagnosis remains.
At KIMS Hospitals Dr. Manas, Dr. Dhanunjaya Rao and colleagues are frequently conducting advanced special sequences in MRI and contrast enhanced other imaging modalities to identify the nerve tracts in brain and exploring the diagnosis of MSA. These techniques follow well established imaging methods. The functional MRI has an ability to look at the brain activity based on changes in regional blood flow, whereas DTI is used to examine changes in brain connectivity by measuring water diffusion along nerve fiber tracts in the brain.
MSA causes shrinkage of portions of your brain (cerebellum, basal ganglia and brainstem) that regulate many body functions, digestion and motor control.
When MSA brain is seen under a microscope, the damaged brain tissue shows nerve cells (neurons) that contain an abnormal amount of a protein called alpha-synuclein. Some clinicians say that this protein may be overexpressed in pons, cerebellum and basal ganglia in multiple system atrophy.
MRI brain is done to rule out other potential causes of parkinsonism like multiple strokes, inflammatory conditions, or accumulation of fluid in the brain. In MSA a brain MRI shows atrophy or degeneration of brainstem (pons, olivary nuclei), cerebellum, and basal ganglia. If the patients presents with parkinsonism and multiple parts of brain atrophy, then It might be diagnosed as MSA features These classic signs are not always seen though, so repeat imaging may be needed as the disease progresses and if concern regarding diagnosis remains.
At KIMS Hospitals Dr. Manas, Dr. Dhanunjaya Rao and colleagues are frequently conducting advanced special sequences in MRI and contrast enhanced other imaging modalities to identify the nerve tracts in brain and exploring the diagnosis of MSA. These techniques follow well established imaging methods. The functional MRI has an ability to look at the brain activity based on changes in regional blood flow, whereas DTI is used to examine changes in brain connectivity by measuring water diffusion along nerve fiber tracts in the brain.
Treatment mainly includes supportive rehabilitation, medications and lifestyle changes to help manage symptoms, but there is no cure. Initially MSA responds to levodopa/carbidopa, but overtime in months, the effect of medication wanes off. The condition progresses gradually and eventually leads to death.
More crippling symptoms, such as severe tightness muscles or contractures can be treated with “muscle relaxants” and sometimes by injection of botulinum toxin (i.e., Botox). Prevention of falls and aspiration (pneumonia) are major goals as these frequently lead to worsening disability and even death. Neuro rehabilitation techniques often helps in walking and balance, prevention contractures of joints and stiffness of muscles and help with activities of daily living are critical, speech therapist will take care of progressive speech and swallowing difficulties. Parkinson’s group is an inter disciplinary team help in acquiring access to all these facilities and help in taking care of patients. Autonomic symptoms like changes in blood pressure initially begins with increased fluid intake, salt in water and wearing pressure stockings or binder. If these are not enough, drugs like droxidopa and “blood volume increasers” (fludrocortisone) or “pressor agents” (e.g., midodrine) are used to prevent changes in blood pressures. Urinary and bowel symptoms can be treated with selected agents depending on the issue.
Some clinicians have tried Deep brain stimulation (DBS), however, is generally not recommended as poor outcomes have been reported and there is no benefit from DBS.
More crippling symptoms, such as severe tightness muscles or contractures can be treated with “muscle relaxants” and sometimes by injection of botulinum toxin (i.e., Botox). Prevention of falls and aspiration (pneumonia) are major goals as these frequently lead to worsening disability and even death. Neuro rehabilitation techniques often helps in walking and balance, prevention contractures of joints and stiffness of muscles and help with activities of daily living are critical, speech therapist will take care of progressive speech and swallowing difficulties. Parkinson’s group is an inter disciplinary team help in acquiring access to all these facilities and help in taking care of patients. Autonomic symptoms like changes in blood pressure initially begins with increased fluid intake, salt in water and wearing pressure stockings or binder. If these are not enough, drugs like droxidopa and “blood volume increasers” (fludrocortisone) or “pressor agents” (e.g., midodrine) are used to prevent changes in blood pressures. Urinary and bowel symptoms can be treated with selected agents depending on the issue.
Some clinicians have tried Deep brain stimulation (DBS), however, is generally not recommended as poor outcomes have been reported and there is no benefit from DBS.
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