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Multiple system atrophy, formerly known as Shy-Drager syndrome, is a degenerative rare brain disorder that causes a worsening of symptoms over time. It's very rare, only occurring on average 0.6 in 100,000 people,1 though this number varies between studies.2
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Disclaimer: Before You Read
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Patients with the disorder start showing symptoms generally in adulthood and will experience gradual loss of involuntary functions such as digestion, heart rate and blood pressure, along with voluntary functions such as movement. Over time, complications from MSA can be quite severe and life-threatening.
What Is Multiple System Atrophy?
MSA is a rare progressive brain disorder, meaning that over time the affected cells in the brain break down and stop functioning.
It is also a late-onset condition, where affected individuals do not start showing symptoms until adulthood. The average age of onset is 55-60 years old,3 but can vary as much as 30-90 years old.4
MSA is actually a combination of three conditions that used to be known individually. Over time researchers have realized the similarities between these conditions and joined them together under a new name, while still recognizing some of the differences present between each. The original conditions were 5:
- Shy-Drager syndrome
- Olivopontocerebellar atrophy
- Striatonigral degeneration
Each came with their own hallmark finding. Shy-Drager was most known for affecting the autonomic nervous system (that is, involuntary functions that the body does without us having to think about it). Olivopontocerebellar atrophy affected primarily cerebellar function, which in general would affect the ability to control intentional movements. Striatonigral degeneration mainly caused symptoms affecting balance and movement.6
Today we still break down MSA into subtypes based on some of these differences,7 but the naming system is a little clearer.
MSA-P – This designation refers to cases of MSA with Parkinson-like features. As the name suggests, this subtype has many similarities to Parkinson’s disease. This includes slow movements, tremors, stiffness, and balance and coordination issues. This also includes autonomic nervous system affects, or those involuntary functions, similar to Shy-Drager syndrome.
MSA-C – The “C” in this subtype refers to cerebellar, meaning that this designation focuses on symptoms around coordination and balance. Also included are abnormal eye movements, speech problems and difficulty swallowing.
Who Does Multiple System Atrophy Typically Affect?
There is not a clear pattern of any ethnicities or backgrounds being more affected than others. The most recent studies show that males and females are seen to be equally affected.8 Earlier studies indicated males were affected more frequently than females.3
Multiple System Atrophy Symptoms
As mentioned earlier, MSA is broken down into 2 subtypes that are based on the primary set of symptoms that people experience. There are some aspects of the condition common to both subtypes.
Overall, the basic findings of MSA are the same between subtypes. This includes the timing of adulthood onset and progressive worsening of symptoms. On average, an affected person will pass away from the condition or related complications within 10 years after first noticing signs.9
In about 20-75% of cases,10 people may begin experiencing early symptoms distinct from the characteristic motor function symptoms. This can happen months or years before those typical symptoms with movement and coordination start. These early signs can be sexual dysfunction, bladder control issues,10 and becoming lightheaded or fainting upon standing.11
Lightheadedness upon standing
Tendency to fall
Uncoordinated limb movements
Impaired speech or a hoarse voice
Wide walking gait
Forward neck position
What Causes Multiple System Atrophy?
The affects of MSA are believed to be due to a build up of a specific protein, alpha-synuclein, in glial cells. Glial cells are cells of the nervous system that provide support and protection for neurons.13 When alpha-synuclein builds up, it starts a cascade of events that ultimately leads to cell loss in the brain.14
Despite an understanding of this process, it’s not clear what causes this to start in the first place.
MSA is not primarily considered a genetic condition, but newer research is looking at this closer. Some studies have shown an increased risk for MSA related to alterations in the genes COQ215 and SCNA,16 but that doesn’t mean that everyone with a genetic change in these genes will develop MSA.
Can You Prevent Multiple System Atrophy?
There is no known way to prevent the onset of systems and gradual progression of MSA.
Is Multiple System Atrophy Passed Down Through Families?
Traditionally, most adult-onset neurodegenerative diseases have been considered sporadic, or happening by chance, rather than being genetic in origin.
More recently, studies have shown that MSA may be a multifactorial disease, where several different types of factors play into the development.17 Some of these factors could be genetic, but the environment can also play a role. Because of this complex pattern, it’s difficult to produce statistics around inheritance.
Diagnosis of Multiple System Atrophy
Diagnosing this condition is based primarily on a patient’s symptoms and medical history.
Many of the symptoms of MSA overlap with other conditions, and this can make a clear diagnosis difficult. If you or someone you know is possibly affected by MSA, it can be important to seek out a medical professional who is familiar with MSA and how it differs from Parkinson’s disease. A proper diagnosis gives the best chance for finding effective treatments.
Some of the symptoms can also mimic the effects of toxins. A lack of coordination from MSA-C can also look like coordination issues due to alcohol, certain medications, lead, or lithium. Even a Vitamin B12 deficiency can look similar to the balance and coordination issues seen in MSA-C.12
How Do You Diagnose Multiple System Atrophy?
Guidelines for diagnosing MSA focus on 3 categories: definite, probable and possible. These guidelines were developed in 2008 by experts in MSA in across a range of specialties.11
Definite – A diagnosis made after death by examining the brain for findings that indicate the specific type of damage to brain cells. Since this requires a direct view of brain cells, this cannot be done when a patient is still living.
Probable – An adult over the age of 30 with sporadic and progressive symptoms, poor response to the medication levodopa, and severe autonomic failure.
Possible – A sporadic and progressive disorder in adults, showing parkinsonism or a cerebellar syndrome, with at least 1 suggestive feature of an autonomic dysfunction and 1 additional red flag feature.
How Do Doctors Test for Multiple System Atrophy?
There are a number of tests a doctor can perform that look at specific components of symptoms related to MSA, however there isn’t a single test that can diagnose the condition.6
Are there DNA Tests for Multiple System Atrophy?
There are no DNA tests that attempt to look for MSA. There is not a strong enough genetic component that would make DNA testing possible at this time.
Multiple System Atrophy Treatment Options
Can Multiple System Atrophy Be Treated?
Treatments for MSA focus on managing the symptoms and providing relief as the condition progresses.
One area of treatment focuses on managing the fainting or lightheadedness that occurs upon standing. Some medications may help control this, and one undergoing review is called Levodopa.18 Sometimes a physician may prescribe blood pressure medications. Compression garments, dietary choices, mild exercise, and sleeping with the head elevated are other non-medication options that may help with this symptom. 1
Other medications are being evaluated for managing symptoms related to hand tremors,6 however there aren’t medications targeted specifically at the balance and coordination aspects of MSA as a whole.
Neurorehabilitation efforts focused on occupational, physical and speech therapy are also options for patients that may help adapting to reduced abilities as the symptoms worsen.19 Treatments are also available for people who experience sleep apnea.20
A variety of other medications for some symptoms show favorable responses in certain patients, but not all.6 Working closely with a physician will allow the best selection of medications based on the specific symptoms and subtype of MSA present.
What is the Prognosis for Multiple System Atrophy?
MSA is a fatal disease, and it can be expected that affected individuals will not survive due either to the direct effects of the condition, or associated complications and infections. How quickly the condition progresses varies between people, and can depend on which subtype is present.6
Symptoms related to both motor functions and non-motor functions continue to worsen with time. Three years after diagnosis, 50% of patients need assistance walking,21 and 5 years after diagnosis 60% of patients typically need a wheelchair.22 On average, in about 6-8 years the patient generally remains bedridden.21
Does Multiple System Atrophy Go Away?
No, MSA is a lifelong condition and will not go away. Symptoms worsen over time and patients will require extra help.
Is There a Cure for Multiple System Atrophy?
No, there is no cure for the condition, but there are ongoing clinical studies and research efforts to identify new therapies that may act as neuroprotective agents aiming to slow down the rate of symptom progression.6
Where can I get support if my family is affected by Multiple System Atrophy?
MSA can be a devastating condition, and support from families who truly know what you are going through can help ease the journey. Support groups can meet in person or virtually. Some groups may have structured meetings, and some may communicate through an online message board or social media platform. These resources can help answer important questions and give a perspective on what to expect as the disease progresses. Also, just having an empathetic listening ear can be invaluable for many families. Below are some links to patient support organizations for MSA.
The MSA Coalition
Brain Support Network
International Parkinson and Movement Disorder Society
Defeat MSA Alliance