What Is Atypical Parkinson’s Disease?

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Atypical Parkinson’s disease, also referred to as atypical parkinsonism disorders, or Parkinson’s plus, includes a group of conditions that cause symptoms like those of Parkinson’s disease as well as additional effects.

Your doctor would diagnose atypical Parkinson’s disease based on your symptoms and on a physical examination. Often, these disorders have more severe symptoms, are more disabling, and progress more rapidly than Parkinson’s disease.

Additionally, the treatments that are effective for managing Parkinson’s disease are not as helpful for managing atypical Parkinson's disease, and there are no other treatments specifically known to help.

Atypical Parkinson's disease can make it difficult to walk

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Types and Symptoms 

Like Parkinson’s disease, atypical Parkinson’s disease can cause resting tremors, a masked face (expressionless face), impaired balance, difficulty walking, and slow, stiff muscle movements.

Atypical Parkinson's disease can also cause severe balance problems, dementia, action tremor (tremor occurring with the voluntary movement of a muscle), and emotional and behavioral changes. Each type of atypical parkinsonism has its own characteristic set of symptoms.

Types and symptoms include:

  • Dementia with Lewy bodies: This condition causes gradual progression of motor symptoms like those of Parkinson’s disease, along with rapidly worsening dementia, often with hallucinations.
  • Progressive supranuclear palsy (PSP): The most noticeable effects are severe balance problems, falling, limitations in eye movement, and a surprised-appearing facial expression.
  • Corticobasal ganglionic degeneration (CBS): Symptoms include abnormal muscle movements of one side of the body, difficulty problem solving, and trouble using objects.
  • Multisystem atrophy (MSA): This rare, fatal condition causes severe dizziness and impaired balance, impaired coordination, ataxia (lack of motor control with purposeful movements), difficulty speaking, blood pressure problems, incontinence, inappropriate emotional expressions, and erectile impairment. It can progress rapidly within a few years.

Causes 

Each type of atypical parkinsonism has its own cause. Generally, there is degeneration or damage in the basal ganglia, which is a small area in the brain that facilitates voluntary muscle movement. Most types of atypical parkinsonism also involve other areas of brain impairment, which cause the additional symptoms.

Molecular brain damage, oxidative damage, and inflammation are believed to play a possible role in Parkinsonism.

Causes of each type of atypical parkinsonism:

  • Dementia with Lewy bodies: This condition is associated with degeneration of brain cells and accumulation of Lewy bodies, a type of protein, in the cerebral cortex. It isn’t known why these changes occur.
  • PSP: This condition is associated with degeneration of the areas in the brainstem that control eye movements and facilitate voluntary muscle movement. Often, there is also an accumulation of tau protein in the areas of degeneration. At the current time, the cause of degeneration and protein accumulation is not known.
  • MSA: This is a rapidly degenerative condition in which several areas of the cerebellum and brainstem undergo atrophy (shrinking of the tissue), which is why the symptoms are so extensive and varied. It is associated with the accumulation of alpha-synuclein protein in the brain. The cause of these changes is not known.
  • CBS: This condition is associated with the accumulation of tau protein in the cerebral cortex and the basal ganglia, of unknown cause.

Diagnosis 

Atypical parkinsonism is diagnosed based on clinical features. The symptoms and how quickly they develop often point to the diagnosis. However, because the different conditions are similar to each other and Parkinson’s disease, the specific effects that distinguish each condition might not emerge until several years after the disease starts.

Additionally, several of the conditions also have characteristics that can help distinguish them during a physical examination:

  • Dementia with Lewy bodies: This condition can cause dementia, which would be noticeable during a mental status examination and during the discussion with the doctor.
  • MSA: This condition can cause severe ataxia, as well as substantial abnormalities in blood pressure or pulse.
  • PSP: This condition can cause decreased ability to move the eyes.
  • CBS: The physical signs of this condition would be more severe on one side of the body than the other side.

Sometimes diagnostic testing might be used to help verify the diagnosis or identify other problems that might need to be treated.

For example, if the symptoms are limited to one side of the body, brain magnetic resonance imaging (MRI) could be necessary to determine whether a stroke or traumatic injury is causing the symptoms. Additionally, some types of atypical parkinsonism may show atrophy that can be seen with brain imaging tests.

Treatment 

The treatment for atypical Parkinson's disease differs depending on the specific condition. Sometimes medications that are used to treat Parkinson’s disease can improve some of the symptoms of atypical parkinsonism, making the effects of the condition more tolerable.

Research on treatments for atypical parkinsonism that may prevent oxidative damage and degeneration of the nerves in the brain is ongoing.

Physical therapy can also help you maintain your physical balance, coordination, and control, and optimize your muscle strength. You might need to use a walker or wheelchair if your balance is severely impaired.

Coping

Living with atypical Parkinson’s disease is a challenge. Each of the conditions that cause it is less common than Parkinson’s disease, so you might not know other people going through the same experience as you.

This can feel very isolating for you and for the people who care about you. Consider finding a support group so you can talk with other people who are living with the same condition.

Prognosis

Several types of atypical parkinsonism worsen quickly and will make it hard for you to take care of yourself independently.

It can be difficult to be a caregiver of a loved one who has atypical Parkinson’s disease. Many families need professional assistance in the home or in a specialized caregiving facility. You and your family will have to decide what’s right for you.

Summary

Atypical Parkinson's disease includes several conditions that cause symptoms of Parkinson's disease, as well as additional symptoms.

Several types of atypical Parkinson's disorders—corticobasal ganglionic degeneration, dementia with Lewy bodies, multisystem atrophy, and progressive supranuclear palsy—are associated with gradual degeneration of certain areas of the brain and a buildup of protein deposits in the brain.

Usually, these conditions worsen, and within 10 years it can become difficult to remain independent. Parkinson's disease treatment can minimally improve the symptoms.

A Word From Verywell

Learning about your prognosis with some types of atypical Parkinson's disease can be emotionally overwhelming. Consider speaking with a therapist who can help you learn to cope with your emotions. And make sure you are attentive to your own self-care, both physically and emotionally.

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