Atypical parkinsonism

What is atypical parkinsonism?

Atypical parkinsonism (also called atypical Parkinson’s) refers to conditions that share similar symptoms and features with Parkinson’s disease. These uncommon disorders may result from certain medications, illnesses, or other conditions of the nervous system. 

Parkinsonism vs Parkinson’s disease 

Parkinsonism is a general term describing Parkinson’s disease and other neurological conditions with similar symptoms. The most common disorder causing parkinsonism is Parkinson’s disease, while other conditions that cause atypical parkinsonism are more rare. 

The four most common atypical parkinsonism disorders are: 

  • Dementia with Lewy Bodies (DLB): This condition is the most common of the four atypical parkinsonism disorders. People with DBL may exhibit difficulty moving, thinking, sleeping, and focusing. They may also have hallucinations that cause them to see, smell, hear, or feel things that aren’t there. 
  • Progressive supranuclear palsy (PSP): PSP affects balance, walking, eye movements, and swallowing. People with PSP may also experience problems with thinking and focusing. 
  • Multiple system atrophy (MSA): MSA affects movement and control of the body’s involuntary (autonomic) processes. 
  • Corticobasal Syndrome (CBS): In this condition, areas of the brain shrink, and nerve cells die off over time. It is the rarest of the four atypical parkinsonism disorders. People with CBS have problems with moving, balancing, speaking, swallowing, and thinking.

Symptoms of atypical parkinsonism

The symptoms of atypical parkinsonism can vary depending on the specific type of disorder, but often include a combination of motor and non-motor symptoms. 

Atypical parkinsonism symptoms may include the following: 

  • Memory loss/confusion (dementia) 
  • Muscle stiffness or rigidity 
  • Postural instability and frequent falls 
  • Problems making facial expressions 
  • Slowed movements (bradykinesia) 
  • Soft or low voice/slurred speech 
  • Tremors 

While symptoms may appear similar, atypical parkinsonism often doesn’t respond well to the standard medication (levodopa) used to treat Parkinson’s disease.

Causes of atypical parkinson’s

Conditions that may cause atypical parkinsonism include: 

  • Brain infections such as encephalitis and meningitis  
  • Brain injuries such as stroke or repeated head injuries from contact sports or accidents 
  • Brain damage from anesthesia drugs used during surgery 
  • Infections such as HIV/AIDS  
  • Medications, especially drugs used to treat psychiatric conditions or nausea 
  • Poisoning with carbon monoxide, mercury, or other toxic chemicals 
  • Wilson disease, a rare condition that causes copper to build up in the liver, brain, and other organs 

Symptoms of atypical parkinsonism may improve once the underlying cause is identified and treated. For example, treating infections or poisoning can decrease symptoms. Other conditions causing atypical parkinsonism are progressive and cannot be reversed with treatment. 

Diagnosing atypical parkinsonism

Atypical parkinsonism disorders can be very difficult to diagnose. These disorders are frequently mistaken for Parkinson’s disease, dementia, or other neurological conditions. They may also be confused with one another.

There are no blood tests or imaging studies to detect and confirm atypical parkinsonism disorders. Movement disorders specialists make a diagnosis based on clinical observations, with expert knowledge of subtle differences between conditions.

Neurologists at Penn Medicine’s Movement Disorders Center have decades of experience seeing and diagnosing these rare and complex conditions. Our reputation and skill draw patients from around the region.

The Lewy Body Dementia Association (Lewy Body Dementia Specialist in Philadelphia, PA (lbda.org)) recognizes Penn Medicine as a Center of Excellence for the diagnosis and treatment of people with DBL.

Specialized treatment for atypical parkinsonism disorders

There are no cures for these atypical parkinsonism conditions. Treatments aim to manage symptoms and maximize quality of life. 

Medications 

Atypical parkinsonian disorders share symptoms with Parkinson’s disease. However, the medication that works for Parkinson’s disease (levodopa), doesn’t usually work for these conditions. Some people with atypical parkinsonism may respond briefly to treatment with levodopa, but the effects usually wear off over time. Other medications may help treat psychiatric and cognitive conditions that develop due to atypical parkinsonism. 

Rehabilitative therapies 

A comprehensive treatment approach may help people with atypical parkinsonism manage symptoms. The most helpful treatments include physical therapy, occupational therapy, and speech therapy. These treatments focus on: 

  • Maintaining independence with daily activities for as long as possible 
  • Preventing complications, such as falls that can cause injuries or inhalation of food (aspiration) while eating

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