Parkinson’s disease (PD) is a condition that affects movement and coordination. Atypical Parkinsonism, or atypical Parkinsonian syndromes, are a group of several conditions that have symptoms that present similarly to those of Parkinson’s disease.
They may also have different complications and may not respond to traditional PD treatments.
Symptoms of PD come on gradually as neurons (nerve cells) in a part of the brain called the substantia nigra begin to die.
Doctors aren’t sure exactly what causes the cells to die. They’ve linked it to a misfolding of certain proteins in the brain. This could set off a reaction among other proteins, which start to form clumps and damage the brain. Over time, this leads to the loss of muscle control and dementia that characterize Parkinson’s.
Symptoms of PD and atypical Parkinsonism are often similar. For this reason, Parkinsonism is sometimes initially misdiagnosed as PD. This is why testing and imaging are so important in making an accurate diagnosis.
Researchers are working on ways to take images inside the living human brain of proteins associated with PD and Parkinsonism. Live brain imaging of these proteins would greatly boost doctors’ ability to diagnose and monitor these disorders.
One main difference between the two conditions is that atypical Parkinsonism symptoms tend to come on earlier than they do in typical PD. Symptoms such as falling, dementia, and hallucinations occur earlier in atypical Parkinsonism disorders.
PD symptoms often appear first on one side of the body. With atypical Parkinsonism, signs are often present on both sides at the beginning.
Another key difference between PD and atypical Parkinsonism is what’s happening in the brain.
If you have PD, you lose neurons that make a brain chemical called dopamine (which helps control movement), but your brain still has dopamine receptors. If you have an atypical Parkinsonian syndrome, however, you may be losing your dopamine receptors.
Atypical Parkinsonism disorders each have their own causes.
Scientists still don’t know why people develop PD or atypical Parkinsonism. PD and some Parkinsonism conditions may have a genetic component. Research also suggests that exposure to some environmental toxins may be to blame.
Read on to learn about the various Parkinsonism conditions.
Drug-induced Parkinsonism isn’t really a condition in itself. It’s a collection of symptoms caused by certain drugs, especially anti-psychotic and certain anti-nausea medications. The symptoms are side effects of the drugs and not related to PD.
These medications cause the body to mimic the symptoms of Parkinson’s by blocking the dopamine receptors. The best treatment can be to wean off the drug that’s causing the symptoms, then replace it. Always consult your doctor if you want to change medications.
Some people can’t discontinue the problematic medication because it’s the best one to treat their co-existing condition. In that case, people may choose to tolerate some degree of the symptoms of Parkinsonism in order to optimally treat the underlying condition.
The most common symptoms related to drug-induced Parkinsonism are:
- a tremor while at rest
- walking and balance problems
Vascular Parkinsonism (VP)
Vascular Parkinsonism (VP) is also called multiple-infarct Parkinsonism. Having one or more small strokes are thought to cause the symptoms, rather than the slow die-off of nerve cells that causes symptoms in typical PD. Between
Most people may not be aware they had strokes that led to VP because they’re minor and affect only a small part of the brain. Symptoms may progress in an abrupt fashion that’s a little different than the gradual progression of typical PD. This sometimes leads to a misdiagnosis of PD.
VP symptoms are similar to those of typical PD and most often affect the lower part of the body. Gait problems are common, characterized by shuffling and freezing while walking. People with VP also have difficulty maintaining posture. Other symptoms include:
- motor problems from a stroke, like weakness of part of the body
- pseudobulbar palsy, or the inability to control facial muscles
VD doesn’t respond well to the medications used to control typical PD. VD treatment focuses on managing stroke risk factors such as quitting smoking, maintaining a healthy weight, and managing high blood pressure.
Dementia with Lewy bodies (DLB)
Dementia with Lewy bodies (DLB) causes hallucinations and dementia, as well as tremor, slowness, and stiffness. Doctors don’t know the root cause of LBD, but they think it’s probably a mix of genetics, environment, and aging.
DLB results from clumping of proteins in the brain called Lewy bodies. Lewy bodies also form in PD, though they aren’t used in the diagnosis of either condition.
Some of their symptoms are similar, however, which can make it hard to distinguish between PD and DLB. The main difference is that dementia and hallucinations begin early in DLB, whereas movement problems predominate in early PD.
The first sign of DLB is often REM sleep behavior disorder. It can begin years before other symptoms of DLB. People with this disorder act out their dreams. They move and talk while dreaming instead of lying still.
DLB and a related condition, Parkinson’s disease dementia, share similar symptoms and result from clumps of the same protein, alpha-synuclein, in brain cells. Because the symptoms of both diseases relate so closely to those of PD, the three conditions are often collectively called Lewy body disorders.
Lewy body dementia is the second most common form of dementia, after Alzheimer’s disease. Symptoms include:
- hallucinations and delusions
- mood changes and reduced inhibitions
- difficulty with problem solving
- balance and coordination problems
- fluctuating blood pressure
Multiple system atrophy (MSA)
Multiple system atrophy (MSA) affects the autonomic nervous system, which controls involuntary processes of the body such as blood pressure, digestion, and eye movement.
Doctors haven’t pinpointed the exact causes of MSA. The same clumps of the protein alpha-synuclein that are found in brain cells in DLB are found in MSA, but they’re found in the glial cells instead of the nerve cells. Glial cells are the non-neuron cells in the brain and spinal cord.
Symptoms of MSA can include:
- balance problems and stiffness
- breathing problems, commonly at night
- slurred speech
- low blood pressure
- constipation and bladder problems
- difficulty controlling emotions
Progressive supranuclear palsy (PSP)
Progressive supranuclear palsy (PSP) causes cognitive issues and movement problems. Memory and thinking are especially affected.
Doctors don’t know what causes PSP. They do know that tau protein tends to clump in certain brain cells, which then die. Researchers are looking at environmental factors as a possible cause, but nothing definite is known.
- walking problems, with a tendency to fall backward
- slurred speech
- swallowing difficulty
- eyelid movement issues and vision problems
- lack of motivation and other behavioral changes
Corticobasal degeneration (CBD)
Corticobasal syndrome causes involuntary jerking and inability to posture limbs normally. These symptoms usually occur only on one side of the body. Doctors don’t yet understand why this happens, but researchers are trying to understand it and find ways to target the process.
One unusual and rare symptom of CBD is called alien limb phenomenon. This is when an arm or leg seems to move and perform actions on its own, and you don’t feel you have any control over it. This can cause people great distress and alarm.
People with CBD can also develop cognitive problems, such as difficulty thinking and organizing. One common problem is not being able to remember what common objects are used for.
PD symptoms vary from person to person. Some people have a tremor, usually on one side of the body. Others with PD have muscle freezing or balance difficulties. You might have PD symptoms that are mild for years. Someone else may have symptoms that worsen quickly.
Atypical Parkinsonian syndromes each have their own set of symptoms:
- VP: Gait and balance problems are common symptoms. Dementia, incontinence, and palsy also develop.
- LBD: Thinking and memory decline. Hallucinations, delusions, and difficulty staying alert are also symptoms.
- MSA: Walking and balance problems are common with this condition. You may also have symptoms related to autonomic dysfunction. This is when the autonomic nervous system (ANS) doesn’t function as it should. The ANS controls automatic bodily functions like breathing, digestion, and circulation. These symptoms might include:
- a sudden drop in blood pressure when you stand up (orthostatic hypotension)
- PSP: People with PSP have problems with walking and balance, eye movement, speech, and thinking skills. Falling backward and an inability to move eyes upward and downward are specific problems. Mood and behavioral issues also develop.
- CBD: The main symptoms of this condition are sudden jerking (myoclonus) and abnormal posturing of the limbs (dystonia). These often occur only on one side of the body. Speech issues, changes in behavior, and attention problems can also develop.
Diagnosing atypical Parkinsonism starts with your doctor reviewing all of your symptoms and your medical history. A neurological exam will also be part of the evaluation.
Your doctor might observe you walking across the room, sitting down, standing up, and performing other basic movements. They’ll look for problems with balance and coordination. Your doctor may also do some simple tests of your arm and leg strength.
You may take some tests of your mental ability, such as repeating back lists of numbers or answering questions about current events.
Your doctor may order imaging tests of the brain. Most of these will appear normal in PD, and may show shrinking in certain parts of the brain with MSA. Some commonly used tests include:
- Positron emission tomography (PET) scan: A radioactive dye called a tracer reveals signs of disease or injury to the brain.
- MRI scan: A magnetic field and radio waves create images of the inside of your body.
- DAT-SPECT: A type of CT scan checks the movement of dopamine in the brain.
No cures currently exist for atypical Parkinsonism. The goal of treatment is to manage symptoms for as long as possible. The appropriate medication for each disorder depends on your symptoms and how you respond to treatment.
For LBD, some people find relief from symptoms with cholinesterase inhibitors. These drugs increase the activity of neurotransmitters that affect memory and judgment.
For PSP, levodopa and similar drugs that act like dopamine, are helpful for some people.
Participating in physical or occupational therapy can also help with most of these conditions. Keeping physically active may help relieve symptoms. Check with your doctor if any specific exercises might be good for you.
Certain risk factors are known for PD, but little has been established for atypical Parkinsonism. The known risk factors for PD include:
- Advancing age. This is the most common risk factor for PD.
- Biological sex. Those assigned male at birth tend to develop PD more often than those assigned female at birth.
- Genetics. Many studies are exploring the genetic link to PD.
- Environmental causes. A variety of toxins have been linked to PD.
- Head trauma. Injuries to the brain are thought to contribute to PD onset.
Much research is ongoing to establish risk factors for atypical Parkinsonism disorders, especially in genetics.
Some atypical Parkinsonism disorders have obvious risk factors. For example, drug-induced Parkinsonism is related to certain drugs, and vascular Parkinsonism stems from previous strokes.
But the risk factors for the other Parkinsonisms is the subject of a lot of current research. Scientists are looking into why each of these conditions occur and how to slow or stop their progression.
Perhaps the most serious complication from any of these conditions is dementia.
You may first develop mild cognitive impairment (MCI), which may not interfere too much with your daily activities. If your thinking skills and memory gradually decline, you may need the assistance of family, a home health aide, or an assisted living facility.
Because these conditions affect balance and coordination, fall risk becomes an important concern. Having PD or atypical Parkinsonism means avoiding falls and fractures. Make your home safer by getting rid of throw rugs, lighting hallways at night, and installing grab bars in the bathroom.
Atypical Parkinsonian syndromes are progressive diseases. This means that their symptoms will continue to worsen over time. While no cures exist for these disorders yet, there are treatments that can help to slow their progression.
It’s important that you take your medications exactly as prescribed by your doctor. If you’re ever unsure about your treatment, call your doctor’s office.
PD and atypical Parkinsonism affect each person differently. Those differences include the type and severity of symptoms, as well as life expectancy.
One study found that assuming an average age of about 72 years at diagnosis, people with atypical Parkinsonism lived on average 6 more years.
Life expectancy estimates can vary greatly depending on your overall health. The healthier you are when you’re diagnosed, the better your chances of living longer with atypical Parkinsonism.