What is atypical Parkinsonism?
People with atypical Parkinsonism do not tend to respond to traditional Parkinson's disease treatments.
In this article, learn about the symptoms and types of atypical Parkinsonism and the treatments available.
What is atypical Parkinsonism?
Symptoms of atypical Parkinsonism may include dementia, vocal changes, and difficulty walking.
Some people who have Parkinson's disease experience symptoms that are typical of the disease as well as other symptoms that are not typical of the disease. When this occurs, doctors call the condition atypical Parkinsonism or Parkinsonism plus syndrome.
Parkinson's disease is a progressive disorder that affects the brain, chiefly causing changes in movement.
Some of the most common symptoms include tremors, muscle stiffness, and changes in a person's gait when walking.
Atypical Parkinsonism has several known syndromes that affect a person's overall health. Examples include Lewy body dementia, a type of early-onset dementia.
Atypical Parkinsonism may not respond to traditional Parkinson's disease treatments, so getting a correct diagnosis is important to ensure a person receives the treatments most likely to be effective.
People with atypical Parkinsonism experience symptoms in addition to those typical of Parkinson's disease. The symptoms of Parkinson's disease include:
- tremors, or shaking movements that are usually in the hands
- slowed movements
- stiff muscles and lack of "swing" in the arms and legs while walking
People with atypical Parkinsonism do not tend to have a tremor at rest. They may also develop symptoms of late-stage Parkinson's at a faster rate. These symptoms include:
- vocal changes
- a sudden drop in blood pressure when standing up (orthostatic hypotension)
- difficulty walking
- early gaze palsy or paralysis, where a person may not be able to look up or down
- problems swallowing
- difficulty sleeping
A doctor will consider these and other symptoms when making a diagnosis.
Types of atypical Parkinsonism
Atypical Parkinsonism has several forms or types that are known to co-occur with Parkinson's disease symptoms. These include:
- Corticobasal degeneration (CBD): People with this condition experience stiffness in the extremities, sudden muscle jerking, and problems carrying out purposeful movements, such as reaching or grasping an object (apraxia).
- Dementia with Lewy bodies (DLB): Men older than age 50 with a family history of Parkinson's are most likely to have DLB. This condition causes early dementia as well as visual hallucinations.
- Progressive supranuclear palsy (PSP): PSP is the most common form of atypical Parkinsonism. The disease can affect a person's ability to look up and down and can cause postural instability that leads to frequent falls. This condition is associated more with women and people older than 60.
- Multiple system atrophy (MSA): MSA is the second most common form of atypical Parkinsonism. The condition causes unstable blood pressure and reddish skin discoloration, as well as bladder, bowel, and sexual dysfunction.
- Normal pressure hydrocephalus (NPH): This condition results in an excess of cerebrospinal fluid (CSF) that causes a person to experience symptoms such as difficulty walking, incontinence, and dementia.
- Vascular Parkinsonism: This condition causes a person to have many lower body symptoms, such as problems with walking and balance. A person will also have problems with blood flow, which can lead to stroke and mini-strokes.
Diagram showing the substantia nigra.
Image credit: Blausen.com staff, "Medical gallery of Blausen Medical 2014." WikiJournal of Medicine 1 (2), 2014.
While doctors do not know the exact cause of Parkinson's disease, they do know the disease destroys cells in an area of the brain called the substantia nigra.
This area is responsible for making a neurotransmitter called dopamine, which is associated with movement.
People with atypical Parkinson's have damage in the substantia nigra as well as to the cells outside this area of the brain that usually receive dopamine transmissions.
As a result, individuals may experience additional symptoms not associated with Parkinson's disease.
Currently, doctors do not consider atypical Parkinsonism to be hereditary, which means it does not appear to be passed down from parents to children.
Doctors do not have a definitive test to diagnose Parkinson's disease or atypical Parkinsonism. Instead, they must consider a person's overall symptoms and rule out other diseases.
A doctor will start by taking a person's medical history and asking about any symptoms they have observed. Diagnostic testing may include:
- Magnetic resonance imaging (MRI) scan: This test can help doctors identify abnormalities in the brain, such as fluid accumulation.
- Positron emission tomography: This imaging type can detect the movements of dopamine in the brain to determine if the dopamine is traveling to receptors or if the receptors are potentially damaged.
- Blood testing: A doctor may order tests to check the thyroid, liver function, kidney function, and more.
- Medications: A doctor may prescribe a drug designed to increase the amount of dopamine in the brain. If a person's symptoms improve, doctors may diagnose them with Parkinson's disease.
However, a person with atypical Parkinsonism may not improve with this medication because medicines designed for Parkinson's disease do not always have any effect on atypical Parkinsonism.
Sometimes making a definitive diagnosis of atypical Parkinson's can take time because symptoms do not fit into the most commonplace categories associated with the disease.
No cure exists for atypical Parkinsonism, but some medications can help reduce the severity of the symptoms. A doctor will also take into account the type of atypical Parkinsonism when prescribing treatments.
For example, doctors may treat dementia with Lewy bodies with levodopa, a Parkinson's medication. They may also prescribe medicines for dementia and psychosis, such as donepezil and quetiapine (Seroquel).
A doctor may also treat PSP with levodopa as well as medicines to promote sleep, such as zolpidem (Ambien). Sometimes, doctors will use Botulinum toxin (BOTOX) injections to reduce eye muscle twitching.
In addition to medications, a person can also benefit from physical and occupational therapy to enhance balance, improve strength, and encourage independence.
Atypical Parkinsonism may develop if a person has frequent concussions.
Parkinson's disease and atypical Parkinsonism cannot always be prevented, but there are certain risk factors a person can try to avoid.
Factors that influence the development of atypical Parkinsonism include:
- brain injury
- frequent concussions
- exposure to toxins, such as carbon monoxide, heavy metals, and pesticides
A person can take protective steps to avoid some of these risks by limiting chemical exposures and wearing a helmet when riding a bicycle or motorcycle.
Atypical Parkinson's represents a variety of conditions and symptoms in addition to typical Parkinson's symptoms.
A person should speak to their doctor as soon as possible if they are experiencing Parkinson's symptoms, as an early diagnosis may identify atypical Parkinsonism and associated conditions.