What is Progressive Supranuclear Palsy?
Updated November 03, 2014.
Written or reviewed by a board-certified physician. See About.com's Medical Review Board.
Progressive supranuclear palsy (PSP) is the name of a syndrome (a collection of symptoms) and also a specific pattern of changes that can be seen in the brain under a microscope. The two are very tightly connected, though occasionally the microscopic changes of PSP cause a different pattern of symptoms than usual.
Progressive supranuclear palsy syndrome is a form of parkinsonism, meaning a disorder that resembles Parkinson’s disease but typically doesn’t respond to the same kinds of medication used to treat Parkinson’s disease.
There are a few other things that make PSP syndrome a bit different than Parkinson’s disease:
Under the microscope, progressive supranuclear palsy has a very distinct histopathological appearance, with protein aggregates forming that look unlike any other disease.
An example are so called “tufted astrocytes,” which look a bit like microscopic koosh balls deposited on the brain’s cortex. PSP is associated with deposits of tau protein, which is also found in other diseases like Alzheimer's and frontotemporal dementia.
On an MRI, it is usually possible to see shrinking at the top of the brainstem in an area called the midbrain. This area contains regions of the brain responsible for eye movement, which likely results in the characteristic difficulty moving the eyes among those with this disorder. The midbrain also contains regions that help us to walk smoothly.
It is important to distinguish between PSP syndrome and PSP histopathology. PSP histopathology is sometimes found to be the cause of entirely different syndromes, such as corticobasal syndrome or primary progressive aphasia (especially the nonfluent subtype). PSP histopathology occasionally is the cause of a frontotemporal dementia syndrome. Often, more typical and classic symptoms of PSP appear as the disease progresses over time.
Unlike other forms of neurodegenerative illnesses such as some forms of frontotemporal dementia or even Alzheimer’s, there hasn’t been a very tight genetic connection found for PSP. Instead, the disease seems to strike sporadically. Fortunately, it is much less common that Parkinson's disease, impacting about 1/100,000 people.
No two patients with PSP are exactly the same. Everyone progresses at a slightly different rate and in a slightly different fashion. That said, parkinsonism is expected to worsen over time. In late stages, someone with PSP is likely to need a wheelchair for safety, and may need help in order to swallow safely and communicate effectively.
There's no cure for PSP, though some clinical trials are ongoing. The main focus is on keeping people safe and as comfortable as possible, despite their illness.
Sources:
Ropper AH, Samuels MA. Adams and Victor's Principles of Neurology, 9th ed: The McGraw-Hill Companies, Inc., 2009.
Written or reviewed by a board-certified physician. See About.com's Medical Review Board.
Progressive supranuclear palsy (PSP) is the name of a syndrome (a collection of symptoms) and also a specific pattern of changes that can be seen in the brain under a microscope. The two are very tightly connected, though occasionally the microscopic changes of PSP cause a different pattern of symptoms than usual.
Progressive supranuclear palsy syndrome is a form of parkinsonism, meaning a disorder that resembles Parkinson’s disease but typically doesn’t respond to the same kinds of medication used to treat Parkinson’s disease.
There are a few other things that make PSP syndrome a bit different than Parkinson’s disease:
- PSP has more rigidity of the neck and torso (axial rigidity) than most cases of Parkinson’s disease.
- PSP leads to remarkable difficulty moving the eyes, especially up and down. While this may start in a subtle fashion, it can become so severe that moving the eyes may be almost impossible in late stages of the disease.
- While both Parkinson’s and PSP can lead to falls, patients with PSP are especially prone to falling. In fact, the most widely accepted diagnostic criteria for PSP require that the patient fall within the first year of symptoms.
- PSP is associated with different kinds of cognitive changes than Parkinson’s disease. For example, while both diseases can cause cognitive slowing, PSP has been connected with mild impulsivity and occasional with language changes such as primary progressive aphasia.
- Tremors are less common in PSP, though they may sometimes be present.
Under the microscope, progressive supranuclear palsy has a very distinct histopathological appearance, with protein aggregates forming that look unlike any other disease.
An example are so called “tufted astrocytes,” which look a bit like microscopic koosh balls deposited on the brain’s cortex. PSP is associated with deposits of tau protein, which is also found in other diseases like Alzheimer's and frontotemporal dementia.
On an MRI, it is usually possible to see shrinking at the top of the brainstem in an area called the midbrain. This area contains regions of the brain responsible for eye movement, which likely results in the characteristic difficulty moving the eyes among those with this disorder. The midbrain also contains regions that help us to walk smoothly.
It is important to distinguish between PSP syndrome and PSP histopathology. PSP histopathology is sometimes found to be the cause of entirely different syndromes, such as corticobasal syndrome or primary progressive aphasia (especially the nonfluent subtype). PSP histopathology occasionally is the cause of a frontotemporal dementia syndrome. Often, more typical and classic symptoms of PSP appear as the disease progresses over time.
Unlike other forms of neurodegenerative illnesses such as some forms of frontotemporal dementia or even Alzheimer’s, there hasn’t been a very tight genetic connection found for PSP. Instead, the disease seems to strike sporadically. Fortunately, it is much less common that Parkinson's disease, impacting about 1/100,000 people.
No two patients with PSP are exactly the same. Everyone progresses at a slightly different rate and in a slightly different fashion. That said, parkinsonism is expected to worsen over time. In late stages, someone with PSP is likely to need a wheelchair for safety, and may need help in order to swallow safely and communicate effectively.
There's no cure for PSP, though some clinical trials are ongoing. The main focus is on keeping people safe and as comfortable as possible, despite their illness.
Sources:
Ropper AH, Samuels MA. Adams and Victor's Principles of Neurology, 9th ed: The McGraw-Hill Companies, Inc., 2009.
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