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 was traditionally diagnosed when someone began to lose control and coordination over a range of body systems, including:
- blood pressure
This article will explore what MSA is, why the name was changed, and how to tell this condition apart from so many other neurological diseases.
MSA is a neurodegenerative condition that causes you to slowly lose control and coordination over a range of body systems.
It was first recognized in the 1960s by Dr. Milton Shy of the National Institutes of Health and Dr. Glen Drager of the Baylor College of Medicine. Back then, orthostatic hypotension was believed to be the primary problem.
As time went on and knowledge about this syndrome increased, its description and name were updated to better reflect the scope of the disease.
Who Gets Shy Drager syndrome?
MSA is a rare neurological disease. It often appears in people around age 60, and men are twice as likely to develop the condition as women.
Overall, there are only about
The name Shy Drager Syndrome was changed to MSA in 1998 after a
The paper outlined specific diagnostic criteria for the condition and suggested the name change to better reflect the way the disease originates.
In addition to the name change, the 1998 paper also categorized the condition into two subgroups.
- MSA-P: features more parkinsonian symptoms like tremors, rigid muscles, and movement problems
- MSA-C: has more cerebellar features, like coordination problems alongside speech or vision difficulties
As described in the revised name, multiple system atrophy, atrophy is the hallmark symptom of MSA.
There’s a wide range of effects when it comes to MSA, based on the severity of the condition and what areas of the body are most impacted. About half of the 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 with MSA include:
- blurred vision
- problems with bowel or bladder control
- erectile dysfunction
- dry skin
Eventually, most people with this condition progress to some degree of autonomic dysfunction. The diagnosis may further be categorized based on unique symptoms, and what body systems are affected.
As mentioned above, MSA-P has a similar presentation as Parkinson’s disease, with symptoms like:
- slowed voluntary movements
- muscle stiffness
- poor balance
- jerking movements in the hands or fingers
About 90% of all people with MSA will experience MSA-P symptoms. The one symptom that’s common in Parkinson’s disease that’s rarely seen in MSA-P is the “pill-rolling” resting tremor. This is seen in less than 10% of people with MSA-P.
- uncoordinated voluntary movements
- progressive balance loss
- slurred speech
- jerkiness to eye movements
Other symptoms that can appear in either type of MSA include:
Researchers aren’t really sure what causes MSA, but there appear to be some links in both environmental and genetic factors.
Specifically, alterations 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 neurologic disorder like MSA, diagnosis will start with a review of your personal and family medical history, and what symptoms you’re experiencing.
Some other possible testing methods that might be used include:
- autonomic testing
- bladder function tests
- magnetic resonance imaging (MRI) of the brain
- positron emission tomography (PET) scan of the brain
Another test — which is more of a medication challenge — is a trial of levodopa. Levodopa is a medication often used to treat Parkinson’s disease. If your doctor prescribes it to you and you have little to no improvement, this is often a sign that you have MSA.
The only true “gold standard” testing that can be done to confirm an MSA diagnosis, though, is post-mortem testing. This is done by testing the buildup of certain substances and structural changes in your brain tissue after you die.
Currently, there’s no standard treatment for MSA. Most treatments and medications offered for MSA focus on symptoms management and can include things like:
- ropinirole, pramipexole, or amantadine to control movements or tremors
- midodrine to help increase blood pressure
- oxybutynin, desmopressin, or catheterization for urinary problems
- sildenafil, tadalafil, or vardenafil for impotence
- speech therapy
- feeding support or supplements
- respiratory support with CPAP or tracheostomy
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 depend in part on how old you were at the time of diagnosis.
People who develop symptoms later in life are female, fall a lot, or who have severe autonomic symptoms usually have the highest mortality rates.
While it can be difficult to diagnose MSA and even more difficult to treat it, it’s a disease that usually doesn’t 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 with your healthcare professional if you’re experiencing problems with movement or balance, or if you often become dizzy when you stand.