Supranuclear palsy, progressive
Progressive Supranuclear Palsy Progressive Supranuclear Palsy (PSP) is a rare neurodegenerative disorder characterized by a variety of motor and cognitive symptoms. It is classified as a type of atypical parkinsonism and is often misdiagnosed due to its similarity to other neurological conditions.
Overview[edit | edit source]
PSP is primarily marked by difficulties with balance, movement, vision, speech, and swallowing. The disease is progressive, meaning symptoms worsen over time, and it is caused by the deterioration of specific areas of the brain.
Symptoms[edit | edit source]
The symptoms of PSP can vary but typically include:
- Balance and gait difficulties: Patients often experience frequent falls, especially backward.
- Eye movement abnormalities: Difficulty moving the eyes, particularly in the vertical direction, is a hallmark of PSP.
- Stiffness and awkward movements: Similar to Parkinson's disease, but with more pronounced axial rigidity.
- Speech and swallowing problems: Dysarthria and dysphagia are common as the disease progresses.
- Cognitive changes: Some patients may experience changes in mood, behavior, and cognitive function.
Causes[edit | edit source]
The exact cause of PSP is unknown, but it is associated with the accumulation of tau protein in the brain. This accumulation leads to the degeneration of neurons in areas such as the brainstem and basal ganglia.
Diagnosis[edit | edit source]
Diagnosing PSP can be challenging due to its overlap with other neurodegenerative diseases. Diagnosis is primarily clinical, based on the characteristic symptoms and progression. Imaging studies, such as MRI, can help rule out other conditions.
Treatment[edit | edit source]
There is currently no cure for PSP, and treatment focuses on managing symptoms. Options include:
- Medications: Some medications used for Parkinson's disease may provide limited benefit.
- Physical therapy: Helps maintain mobility and balance.
- Speech therapy: Assists with communication and swallowing difficulties.
Prognosis[edit | edit source]
The progression of PSP varies among individuals, but it generally leads to severe disability within a few years. The average life expectancy after diagnosis is approximately 5 to 10 years.
Research[edit | edit source]
Ongoing research is focused on understanding the pathophysiology of PSP and developing potential treatments. Studies on tau-targeting therapies are of particular interest.
See Also[edit | edit source]
External Links[edit | edit source]
- [National Institute of Neurological Disorders and Stroke - PSP](https://www.ninds.nih.gov/Disorders/All-Disorders/Progressive-Supranuclear-Palsy-Information-Page)
- [CurePSP Foundation](https://www.psp.org/)
NIH genetic and rare disease info[edit source]
Supranuclear palsy, progressive is a rare disease.
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Contributors: Prab R. Tumpati, MD
