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Causes
By Mayo Clinic staff
The cause of progressive supranuclear palsy isn't known, but research has revealed a few clues. The signs and symptoms of the disease result from deterioration of brain cells in your brainstem, cerebral cortex, cerebellum and basal ganglia. These areas of your brain help you control body movements. This explains why their deterioration leads to the coordination and movement problems of the disease.
Researchers have found that these deteriorating brain cells of people with progressive supranuclear palsy have abnormal quantities of a protein called tau. It's not yet clear if these "clumps" of tau are defective from the start or damaged later. Abnormal quantities of tau are also found in other neurodegenerative disorders, such as Alzheimer's disease.
Scientists have several theories about what might cause this process of brain cell deterioration, including:
- A virus. Some speculate that a unique virus enters your body, taking many years before it becomes active and starts causing signs and symptoms.
- Genetic mutations. It's possible that random changes (mutations), which may develop in everyone's genes, occur in specific genes to damage the cells involved in progressive supranuclear palsy.
- Environmental exposure. Some scientists think an unknown chemical in the environment could be what causes this disease. This chemical could be in something you eat, drink or breathe. For example, certain tropical fruits have been linked to progressive supranuclear palsy-like symptoms in people on some Caribbean islands.
- Damage from free radicals. As you process food for energy, your body produces substances called free radicals. Free radicals are believed to contribute to aging and certain diseases. They may damage the brain cells involved with the signs and symptoms of progressive supranuclear palsy.
- Progressive supranuclear palsy fact sheet. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/psp/detail_psp.htm. Accessed Dec. 14, 2010.
- Dickson DW, et al. Neuropathology of variants of progressive supranuclear palsy. Current Opinion in Neurology. 2010; 23:394.
- Ropper AH, et al. Degenerative diseases of the nervous system. In: Ropper AH, et al. Adams and Victor's Principles of Neurology. 9th ed. New York, N.Y.: The McGraw-Hill Companies; 2009. http://www.accessmedicine.com/content.aspx?aID=3639296&searchStr=progressive+supranuclear+palsy. Accessed Dec. 14, 2010.
- Hyun JH, et al. Behavioral changes as the earliest clinical manifestation of progressive supranuclear palsy. Journal of Clinical Neurology. 2010;6:148.
- Golbe LI. Progressive supranuclear palsy: Some answers. CurePSP. http://www.psp.org/about. Accessed Dec. 14, 2010.
- Stamelou M, et al. Short-term effects of coenzyme Q10 in progressive supranuclear palsy: A randomized, placebo-controlled trial. Movement Disorders. 2008;23:942.
- Josephs KA (expert opinion). Mayo Clinic, Rochester, Minn. Dec. 29, 2010.
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