What are atypical Parkinson disorders?
Atypical Parkinson disorders are a group of brain conditions that can look like Parkinson’s disease, especially at first. They cause symptoms of parkinsonism, which are slowness, stiffness, and balance problems. But they are different diseases.
Compared with Parkinson’s disease, atypical Parkinson disorders usually change faster, cause other problems earlier, and have a less than optimal response (less than 30% improvement) or do not respond as well to medications that contain levodopa. You may also hear them called “atypical parkinsonism” or “Parkinson-plus” conditions.
There are four main types.
Progressive supranuclear palsy (PSP)
PSP often causes falls early on, and the falls are often backward. It can make it hard to move the eyes, especially to look down. Speech may become slow or slurred, and swallowing can get harder. Behavioral problems can occur, including apathy, depression, impulsivity, and agitation.
Multiple system atrophy (MSA)
MSA can cause early autonomic dysfunction. The autonomic nervous system is the part of the nervous system that runs things you do not think about, such as blood pressure, the bladder, and sweating. Some people with MSA mainly have slowness and stiffness. Others mainly have trouble with balance and coordination. Early problems can include lightheadedness when standing, bladder trouble, and, in men, erection problems.
Corticobasal syndrome (CBS)
CBS usually starts on one side of the body. One hand or arm may become stiff, clumsy, or jerky. Some people feel as if their hand does not belong to them or moves on its own (alien hand syndrome). Trouble finding words or doing familiar tasks, like using a tool, can also happen.
Lewy body dementia (LBD)
Lewy body dementia affects thinking, movement, sleep, and alertness. Thinking and attention may change a lot from day to day. Many people see things that are not there or act out their dreams during sleep. It can also cause slowness and stiffness. Learn more about Lewy body dementia.
Common symptoms
Symptoms vary with the type of atypical parkinsonism, and that is where going to a fellowship-trained movement disorder specialist matters. A key rule is that symptoms seen in mid or late Parkinson’s disease occur early in atypical Parkinson disorders. These are called red flags:
- Early falls or trouble with balance
- Early and increasingly severe difficulty with speech or swallowing
- Trouble moving the eyes up or down
- Early, progressive difficulty with thinking and memory, leading to early dementia
- Early loss of the body’s ability to regulate blood pressure (neurogenic orthostatic hypotension) in different positions, causing lightheadedness when standing and/or knee buckling, which can cause passing out or falls
- Early loss of bladder control, causing incontinence
- Early psychosis: visual hallucinations or false beliefs (delusions)
Why the timing of symptoms matters
In Parkinson’s disease, problems such as lightheadedness when standing (neurogenic orthostatic hypotension) and other symptoms that are not about movement usually show up only after many years. In some atypical Parkinson disorders, such as MSA and Lewy body dementia, these same problems can show up in the first 2 to 3 years. That is one reason an accurate diagnosis by a movement disorder specialist matters.
Non-motor symptoms
Non-motor symptoms can often be more disabling and decrease quality of life more than the motor symptoms (tremor, slowness of movement, rigidity, and shuffling).
Daytime drooling (sialorrhea)
Daytime drooling, when it is more than mild, is an early sign of swallowing dysfunction. It is treated with oral medications and/or botulinum toxin injections.
Depression and anxiety
Depression and anxiety can occur before or after the movement (motor) symptoms. They are treated with a holistic therapy program, antidepressants, and anti-anxiety medications.
Delayed gastric emptying (gastroparesis)
Gastroparesis is a form of autonomic nervous system failure and is usually an early sign in multiple system atrophy and a later sign in Lewy body dementia, in which the stomach takes much longer than normal to empty. Symptoms can include:
- Feeling completely full after eating only a small amount of food
- Reflux (heartburn)
- Abdominal gas and bloating
- Weight loss
- Nausea or vomiting
Many people have no symptoms but have a poor response to immediate-release medications.
Psychosis (hallucinations or delusions)
Psychosis usually occurs in Lewy body dementia in the first 2 to 3 years. Psychosis is a symptom of more advanced Parkinson’s disease and usually occurs with dementia. Symptoms include:
- Visual hallucinations
- Auditory hallucinations (hearing sounds, music, or voices)
- A false sense of presence: seeing a shadow or figure of a person or animal in your peripheral vision that goes away once you try to look at it
- False beliefs (delusions)
What can be mistaken for psychosis
Visual hallucinations can be confused with vivid dreams and nightmares.
They can also be confused with sleep hallucinations when waking up (hypnopompic hallucinations) or when falling asleep (hypnagogic hallucinations). These can cause a false perception of objects, sounds, smells, or touch. Sleep hallucinations seem real, but they are not. They are common and usually not a cause for concern. Up to 70% of people experience them at least once. Sleep hallucinations are not psychosis, because true psychosis occurs at any time of the day, not just at night.
REM sleep behavior disorder (RBD)
RBD can occur 20 years before or after motor symptoms begin. It can occur only once and disappear years before motor symptoms. About 60% to 80% of people who have RBD will develop Parkinson’s disease, Lewy body dementia (LBD), or multiple system atrophy (MSA).
RBD is frequently mistaken for visual hallucinations or nightmares, because people can have conversations or make purposeful movements in their sleep as if they were awake, such as hitting, kicking, or even rolling out of bed. People can injure themselves or their bed partners and are almost always completely unaware of moving in their sleep. RBD can usually be treated very effectively by your movement disorder specialist.
Neurogenic orthostatic hypotension (NOH)
Neurogenic orthostatic hypotension is a form of autonomic nervous system failure. It is an early sign of multiple system atrophy, but it can occur a few years after the onset of Lewy body dementia. Many people have no symptoms at all for up to 1 to 2 years, while others have symptoms when it starts. The most common symptoms, which occur minutes after standing, include:
- Lightheadedness when you stand up
- Knee buckling or sudden leg weakness
- Blurry vision
- Passing out, with or without involuntary movements that can be mistaken for seizures
We screen for neurogenic orthostatic hypotension. It is one of the symptoms of autonomic nervous system dysfunction, in which the body loses its ability to regulate blood pressure. It is important to recognize because neurogenic orthostatic hypotension does not follow the same blood pressure parameters that usually signal the need to treat.
Screening for NOH is important not only because of the risk of falls and passing out, which can cause injury. Many people take medication for hypertension to lower their blood pressure, and their doctors are unaware they are developing NOH. Since there can be large drops, sometimes of 40 to 80 points or more, this can cause dangerously low blood pressure when standing, leading to frequent passing out, falls, and emergency room visits. Use the BPMedTracker app to screen for NOH and print a detailed report for your doctor.
Sleep apnea
Sleep apnea occurs in about 60% to 80% of people with Parkinson disorders, and they are usually unaware of it. People will say they sleep just fine because they are unconscious all night or do not snore. But this does not mean they are getting into deep sleep (restorative sleep, when neurological repair happens) or that they do not have sleep apnea. A board-certified sleep specialist can diagnose and treat sleep apnea, which can in turn improve symptoms of Parkinson disorders.
Consequences of sleep apnea
- It can affect insulin levels, leading to high blood sugar (hyperglycemia) and possibly diabetes.
- It increases the risk of stroke and heart attack by increasing the formation of atherosclerotic plaque.
- It increases weight gain by affecting the release of hormones that affect fat breakdown.
- Chronic headaches
- Short-term memory loss
How we make the diagnosis
There is no single test for these conditions. The diagnosis is made mainly through a careful exam by a movement disorder specialist, and it often becomes clearer over time.
At your visit, we will:
- Ask about your symptoms, when they started, and how quickly they have changed
- Look closely at your eye movements, speech, balance, and walking
- Screen for neurogenic orthostatic hypotension and other autonomic problems
- Review your medicines and how well carbidopa/levodopa has worked for you
Some tests can help. A brain MRI can show patterns of change seen in some of these conditions. A DaTscan shows how the brain’s dopamine system is working. It can help tell these conditions apart from look-alikes such as essential tremor, but it cannot, by itself, tell Parkinson’s disease apart from atypical Parkinson disorders. You might also need an evaluation of your memory if you are having short-term memory loss.
Because these conditions can look alike at first, regular follow-up visits are an important part of getting the diagnosis right.
Treatment options
There is no cure or way to stop the progression of any Parkinson disorder (typical or atypical). Although medications seldom improve movement (motor symptoms) enough, the symptoms that are not involved with movement (non-motor symptoms), which are often more disabling, can be managed effectively with medications.
Medications
We often try carbidopa/levodopa. Some people improve, though usually less than people with Parkinson’s disease do. Other medicines can help with:
- Lightheadedness when standing (neurogenic orthostatic hypotension)
- Bladder problems and constipation
- Sleep problems and acting out dreams
- Mood changes, such as depression or anxiety
- Hallucinations and short-term memory loss
Some medicines can make hallucinations or confusion worse, especially in Lewy body dementia. Please check with us before starting any new medicine.
Make sure you bring a typed list of medications used to treat atypical Parkinson disorders that failed or caused side effects. Use BPMedTracker to track your medications and print a list.
Therapy and safety
- Physical therapy, for balance, walking, and fall prevention
- Occupational therapy, for daily tasks and home safety
- Speech therapy, for a soft voice or trouble swallowing
Planning and support
These conditions affect the whole family. We help you plan ahead, connect with support, and learn about research studies when they fit.
When to see a specialist
Consider seeing a movement disorder specialist if you or a loved one has:
- A Parkinson’s disease diagnosis, but medicines are not helping much
- Falls in the first few years of symptoms
- Lightheadedness when standing, or bladder trouble, early on
- Trouble moving the eyes, speaking, or swallowing
- Hallucinations or short-term memory loss early on
Call 940-828-MOVE (6683) to schedule a visit.
Support and resources
Living with a rare condition can feel lonely. These national groups offer education and support for people living with multiple system atrophy and the people who care for them.
