If you have a condition that causes slowed movements, tremors, rigidity and stiffness, you might be experiencing parkinsonism—but you may or may not have Parkinson’s disease.
Parkinsonism is the broader term for these symptoms, which are often caused by Parkinson’s disease. The rest of the time, they may be caused by other movement disorders, medications, head injuries, tumors or dementia.
If you have one of these other movement disorders—namely, progressive supranuclear palsy, multiple system atrophy or corticobasal syndrome, sometimes referred to as atypical parkinsonism—you might struggle to get a correct diagnosis.
“Atypical parkinsonism refers to a group of disorders that looks a lot like Parkinson’s disease, especially in the first year or two, so they’re commonly misdiagnosed,” says UNC Health neurologist Miriam Sklerov, MD. “These have different disease trajectories, and once you look a little closer, you see that it’s not typical Parkinson’s disease.”
Sometimes, your doctor may discover that it’s not Parkinson’s disease when you don’t respond to medication meant to improve symptoms of Parkinson’s. Your symptoms may also provide clues, Dr. Sklerov says.
Here’s what you need to know.
Symptoms of Progressive Supranuclear Palsy (PSP)
Progressive supranuclear palsy (PSP) is the most common of the atypical parkinsonian disorders but is still quite rare, with at least 30,000 cases in the United States (about a million people in the U.S. have Parkinson’s disease). Symptoms typically begin in a person’s 60s or 70s and can mimic Parkinson’s at first.
“There’s the slowness, shuffling, changes in vocal quality and facial expression, though you typically don’t see a tremor,” Dr. Sklerov says. “There are some red flags that this is not Parkinson’s disease, though.”
One of the biggest red flags is falls as one of the early symptoms; in Parkinson’s disease, falls occurring in the first five years are uncommon.
PSP also causes eye movement abnormalities; first, you’ll have trouble looking up and down, followed by difficulties looking side to side. Eventually, people with PSP may not be able to move their eyes or eyelids at all, and they can suffer from extreme dry eye. They may have muscle contractions in the face, and in combination with reduced blinking, they can look concerned or angry.
Compared to Parkinson’s disease, people with PSP may have earlier problems with swallowing and speech, and they also tend to experience some cognitive symptoms earlier.
“With PSP, we see executive dysfunction, or trouble planning multistep processes,” Dr. Sklerov says. “There may be impaired judgment or impulsivity. That may look like someone getting up to grab something and starting to walk before getting their walker, or putting the next bite of food in their mouth before they’ve chewed their previous bite.”
In addition to struggling to plan multistep processes, a person with PSP typically has trouble performing multistep movements, a problem known as apraxia.
“They’ll lose control of complex types of movements like using a screwdriver, cutting a steak with a knife, or saluting as if you were a soldier,” Dr. Sklerov says, noting that this is not a loss of basic motor function but rather the ability to execute specific movements. “They can still do all the movements, but not in order.”
Symptoms of Multiple System Atrophy (MSA)
There are likely between 13,000 and 42,000 cases in the U.S. of multiple system atrophy (MSA), an atypical parkinsonism that tends to begin in a person’s 50s with slowness and shuffling. Some people have a tremor, but Dr. Sklerov says it tends to look a little different to a movement disorders specialist.
If a person with MSA is started on levodopa, the main medication for Parkinson’s disease, they tend to have little reduction in symptoms and experience side effects at very low doses of the medicine, which may often be the first sign that the condition is an atypical parkinsonism.
MSA affects multiple systems of the brain, including areas that control coordination, fluidity of movement and autonomic function.
“People with MSA can have cerebellar ataxia, resulting in a loss of balance and coordination.” Dr. Sklerov says. “They may have a wide gait and stagger. They may veer to one side or another while walking, and have slurred speech, almost like someone who has had too much alcohol.”
Sometimes, the symptoms of cerebellar ataxia are more prominent than the parkinsonian symptoms in MSA.
Symptoms related to the autonomic nervous system can indicate that a patient is dealing with MSA rather than Parkinson’s disease.
“The autonomic nervous system handles tasks that we don’t have to think about—our heart rate, blood pressure, pupil dilation, controlling and releasing urine, to name a few.” Dr. Sklerov says. “When this system is affected, a person might experience significant blood pressure shifts when they change position, like standing up from a reclined position, to the point they pass out. They might have to rely on a catheter because they can’t control their bladder.”
Cognitive changes or impairment are not as common in MSA as other parkinsonian disorders, Dr. Sklerov says.
Symptoms of Corticobasal Syndrome (CBS)
Corticobasal syndrome (CBS) is the rarest of these disorders, and there aren’t good numbers on how many people have it. Dr. Sklerov says it’s also difficult to diagnose.
A person with CBS may have the slowness or shuffling associated with Parkinson’s disease, but tremor is less common. However, the person may have other movement issues, such as twitching or jerking, twisting their body when in neutral positions, and involuntary muscle contractions and movements.
“It is very asymmetrical, so you may only see symptoms on one side to start,” Dr. Sklerov says.
As with PSP, there may be apraxia, so that movements with multiple steps can’t be performed properly, and earlier-than-usual falls.
People with CBS may experience what’s known as alien limb phenomenon, when your limb does something without your control. More than an involuntary muscle contraction or twist, this might look like holding your arm up in the air without realizing you’re doing so.
Speech is often affected, and there may be a variety of other cognitive symptoms, including memory loss, impulsivity and difficulties with organization.
Treatment Options for Atypical Parkinsonism
Treating Parkinson’s disease involves a variety of lifestyle modifications, supportive therapies, medications and surgeries. But for these atypical parkinsonian disorders, medications that work for Parkinson’s don’t help much and surgery is not recommended.
As of now, there are no medications or treatments that cure these conditions or target them specifically, but there is help available.
“While it’s true that there’s no cure, there are so many ways to address quality of life,” Dr. Sklerov says. “There is control over how the disease journey looks.”
People with atypical parkinsonism do benefit from the lifestyle modifications and supportive therapies that can help people with Parkinson’s disease; Dr. Sklerov says an interdisciplinary clinic specializing in atypical parkinsonism or Parkinson’s disease can help you identify the best options for your symptoms.
“Physical therapy, occupational therapy and speech therapy are all helpful,” Dr. Sklerov says. “We definitely encourage cardiovascular exercise, because that slows down disease progression in all neurodegenerative disorders.”
If you’re having non-motor symptoms, such as issues with sleep, mood or bowel function, medication can help with those.
Atypical parkinsonian disorders progress more quickly than Parkinson’s disease. While a person with Parkinson’s disease might expect to live 10 to 30 years with the disease, people with PSP tend to live eight years with the disease, and people with CBS tend to decline more quickly, in six to eight years.
If you or a loved one has been diagnosed with an atypical parkinsonism, it’s important to have early discussions with your family and care team about how you want to be cared for as your disease advances.
You’ll need a support system during this difficult process, and help is available.
“We work with PSP and MSA foundations, which have disease-specific support groups,” Dr. Sklerov says. “There are really specific groups, too; for example, groups for adult children of people with these disorders or for their care partners. It can be helpful to connect with people in your shoes.”
Dr. Sklerov encourages people who have been diagnosed with PSP, MSA or CBS to ask their doctor about clinical trials; because these conditions are so rare, participation can make a real difference.
“There may be no cure right now, but we are on our way,” Dr. Sklerov says. “Research in these three diseases has exponentially expanded in less than a decade because of the number of clinical studies. There is hope.”
If you’re concerned about symptoms you’re experiencing, talk to your doctor. If you need a doctor, find one near you.