How to Recognize Signs and Symptoms of Shy Drager Syndrome
Shy-Drager Syndrome was the name for the condition now known as Multiple System Atrophy (MSA), a neurological condition first recognized in the 1960s. It has traditionally been diagnosed when someone began to lose control and coordination over a number of body systems, including:
- movement
- blood pressure
- Urinate
- breathing
- Swallow
This article examines what MSA is, why the name was changed, and how to differentiate this condition from so many other neurological disorders.
MSA is a neurodegenerative disease that causes you to slowly lose control and coordination over a number of body systems.
It was first developed in the 1960s by Dr. Milton Shy of the National Institutes of Health and Dr. Glen Drager recognized by Baylor College of Medicine. At that time, orthostatic hypotension was believed to be the main problem.
Over time and with increasing knowledge of this syndrome, its description and name have been updated to better reflect the extent of the disease.
The name Shy-Drager Syndrome was changed to MSA in 1998 after a
The paper outlined specific diagnostic criteria for the condition and proposed a name change to better reflect how the disease developed.
In addition to the name change, the 1998 paper also categorized the condition into two subgroups.
- MSA-P: has more Parkinson’s symptoms such as tremors, stiff muscles and movement problems
- MSA-C: has more cerebellar features, such as coordination problems alongside speech or vision difficulties
As described in the revised name multiple system atrophy, atrophy is the hallmark symptom of MSA.
There is a wide range of effects when it comes to MSA, based on the severity of the condition and the areas of the body that are most affected. About half of people with this condition become wheelchair bound due to a progressive lack of motor skills within 5 to 6 years of diagnosis.
Some of the symptoms reported in MSA include:
- fatigue
- weakness
- blurred vision
- Problems with bowel or bladder control
- incontinence
- erectile dysfunction
- constipation
- dry skin
Eventually, most people with this condition will develop some degree of autonomic dysfunction. The diagnosis can be further categorized based on unique symptoms and the body systems affected.
As mentioned above, MSA-P has an appearance similar to Parkinson’s disease with symptoms such as:
- slowed voluntary movements
- muscle stiffness
- bad balance
- jerky movements in the hands or fingers
About 90% of people with MSA experience MSA-P symptoms. The only symptom present in Parkinson’s disease, which is rare in MSA-P, is “pill-rolling” resting tremor. This is seen in less than 10% of people with MSA-P.
Cerebellar symptoms, which affect about 20% of people with MSA, include things like:
- uncoordinated voluntary movements
- progressive loss of balance
- slurred speech
- jerky eye movements
Other symptoms that can occur with both types of MSA include:
- exaggerated reflexes
- increased muscle tone
- muscle cramps
- curvature of the spine
- loud or hoarse breathing (stridor)
- sleep disorders
- cognitive problems
Researchers aren’t sure what causes MSA, but there appear to be some links between environmental and genetic factors.
In particular, changes in the alpha-synuclein (SNCA) gene may play a role in the development of MSA, but more research is needed to fully understand the condition.
If your healthcare professional suspects you have a neurological condition like MSA, the diagnosis begins with a review of your personal and family medical history and your symptoms.
Some other possible test methods that could be used include:
Another test – which is more of a drug challenge – is a trial with levodopa. Levodopa is a drug commonly used to treat Parkinson’s disease. If your doctor prescribes it for you and you experience little or no improvement, it is often a sign that you have MSA.
However, the only true “gold standard” test that can be performed to confirm an MSA diagnosis is a post-mortem test. It does this by testing the accumulation of certain substances and structural changes in your brain tissue after you die.
There is currently no standard treatment for MSA. Most treatments and medications offered for MSA focus on treating symptoms and may include:
The overall outlook for people with MSA is poor, and the condition is often fatal within a decade.
How quickly the disease progresses and how severe your symptoms are depends in part on how old you were at the time of diagnosis.
People who develop symptoms later in life are female, fall frequently, or have severe autonomic symptoms, which usually have the highest mortality rates.
While MSA can be difficult to diagnose and even more difficult to treat, it’s a disease that doesn’t typically appear until your sixth decade of life or later. Many conditions share the same symptoms as MSA, and an official diagnosis may never be made.
Talk to your doctor if you have mobility or balance problems, or if you often get dizzy when standing.