Microscopic pathology image showing autopsy specimen in progressive supranuclear palsy immuno stain. 100x.
What are the other Names for this Condition? (Also known as/Synonyms)
- Nuchal Dystonia-Dementia Syndrome
- Progressive Supranuclear Ophthalmoplegia
- Steele-Richardson-Olszewski Syndrome
What is Progressive Supranuclear Palsy? (Definition/Background Information)
- Progressive Supranuclear Palsy (PSP) is a rare brain disorder that results in abnormalities in walking, balance, and eye movements. It usually affects adults who are 60 years of age. This neurodegenerative condition is caused by the deterioration of certain brain cells
- Signs and symptoms associated with Progressive Supranuclear Palsy are problems with balance and frequent falls, stiffness of the body, vision and swallowing difficulties, and mild memory loss (dementia). The diagnosis of PSP includes a complete neurological exam and the use of imaging studies such as MRI scan of the brain
- The treatment of the disorder may involve medications and supportive therapy, but it cannot be cured. Even though Progressive Supranuclear Palsy is a progressive disorder, the condition generally does not lead to death
Who gets Progressive Supranuclear Palsy? (Age and Sex Distribution)
- Progressive Supranuclear Palsy is a rare disorder with an approximate incidence of 1 in 1000,000
- PSP typically affects elderly adults around the age of 60 years
- It affects both men and women, though men are affected more by this brain condition
- There is no racial, ethnic, or geographical predilection
What are the Risk Factors for Progressive Supranuclear Palsy? (Predisposing Factors)
Currently, the only identified risk factor associated with Progressive Supranuclear Palsy is age. PSP is typically observed in individuals around the age of 60 years.
It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.
Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.
What are the Causes of Progressive Supranuclear Palsy? (Etiology)
Presently, the exact cause of Progressive Supranuclear Palsy development is unknown.
- The condition is caused by the deterioration and damage of cells in the brainstem, cerebral cortex, cerebellum, and basal ganglia
- These are the areas which control movements of the body; any damage to these areas will lead to associated problems in body movements
- It is believed that the damage to the cells in the brainstem may be caused by:
- A virus attack
- Genetic factors
- Exposure to unknown chemicals in the environment
- Damage from free radicals: When the food is processed in the body, it produces free radicals that may play a role in the degeneration of these cells
What are the Signs and Symptoms of Progressive Supranuclear Palsy?
The signs and symptoms of Progressive Supranuclear Palsy are progressive. These include:
- Losing balance while walking, frequent falls
- Vision difficulties such as blurring of vision and double vision
- Inability to maintain eye contact during a conversation
- Facial expression abnormalities
- Stiffness of the body with jerks and spasms, clumsy movements
- Speech problems that include low volume voice, unable to speak clearly
- Difficulty in swallowing
- Personality changes that is indicated by increased irritability and anger outbursts, depression
- Increasing forgetfulness
How is Progressive Supranuclear Palsy Diagnosed?
Progressive Supranuclear Palsy may be diagnosed by the following exams and tests:
- Complete evaluation of medical history along with a thorough physical exam including the neurological exam
- MRI scan of the brain: The scan may reveal a reduction in size of the brain, which is one of the signs of PSP
- PET scan of the brain may show some early changes not apparent on a MRI scan
Note: The signs and symptoms of Progressive Supranuclear Palsy can be similar to other conditions leading to its delayed diagnosis.
Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.
What are the possible Complications of Progressive Supranuclear Palsy?
The possible complications associated with Progressive Supranuclear Palsy include:
- Instability while walking may lead to frequent falls, which may cause serious injuries
- Tasks that need a hand-eye coordination may be difficult to perform
- Disturbed sleep
- Swallowing difficulties, which may lead to choking or inhaling food into the airways, resulting in aspiration pneumonia
- An abrupt and careless behavior is seen among the affected individuals. They may act without thinking that can even lead to fatal accidents
How is Progressive Supranuclear Palsy Treated?
There is no definitive treatment for Progressive Supranuclear Palsy (PSP) currently. However, the following treatment measures are generally taken:
- Carbidopalevodopa, dopamine agonists, and amantadine are recommended to increase the level of dopamine, a substance that sends signals to the brain to control movements
- Antidepressants, such as fluoxetine, imipramine, and amitriptyline, are used to help the symptoms of the disorder
- Botulinum toxin (or botox) is injected into the muscles surrounding the eyes. This is performed to help cure eyelid spasms
- Medications, such as the dietary supplement coenzyme Q-10, are being experimented to assess if they can aid in symptom improvement
- Drugs, such as levodopa,benztropine, and trihexyphenidyl, may help in reducing the symptoms that include stiffness of the limbs or slowness of movements. However, these medications have been more effective for Parkinson’s disease than for Progressive Supranuclear Palsy
- Physical therapy and occupational therapy help to improve the problems related to balance and movements and to proceed with the daily activities of life, without facing too much difficulties (such as falls)
- Eyeglasses with bifocal lens or prism lenses are recommended to correct some vision defects
- Therapies that are safer and less painful can be taught to help overcome difficulties associated with speech and swallowing
- Gastrostomy (tube placement into the stomach) may be needed in individuals who have severe swallowing difficulties
How can Progressive Supranuclear Palsy be Prevented?
Currently, there are no known measures to prevent Progressive Supranuclear Palsy, but research is underway to find out more about this degenerative disorder and ensure its better management.
What is the Prognosis of Progressive Supranuclear Palsy? (Outcomes/Resolutions)
- Prognosis of Progressive Supranuclear Palsy is indicated by the progressiveness of its signs and symptoms; but it is important to note that the disorder in itself is not fatal
- Treatment measures are available that can help reduce the severity and effects of the symptoms
Additional and Relevant Useful Information for Progressive Supranuclear Palsy:
- Progressive Supra Nuclear Palsy is also known as Steele-Richardson-Olszewski Syndrome.
- Even though the signs and symptoms of PSP may be similar to Parkinson disease or Alzheimer disease, the two are not the same and are not to be confused
What are some Useful Resources for Additional Information?
National Institute of Neurological Disorders and Stroke
P.O. Box 5801 Bethesda, MD 20824
Phone: (301) 496-5751
Toll-Free: (800) 352-9424
TDD: (301) 468-5981
Fax: (301) 402-2186
References and Information Sources used for the Article:
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001772/ (accessed on 5/6/2015)
http://www.ninds.nih.gov/disorders/psp/detail_psp.htm (accessed on 5/6/2015)
http://www.pspassociation.org.uk/ (accessed on 5/6/2015)
Helpful Peer-Reviewed Medical Articles:
Barsottini, O. G., Felicio, A. C., Aquino, C. C., & Pedroso, J. L. (2010). Progressive supranuclear palsy: new concepts. Arq Neuropsiquiatr, 68(6), 938-946.
Boeve, B. F. (2012). Progressive supranuclear palsy. Parkinsonism Relat Disord, 18 Suppl 1, S192-194. doi: 10.1016/S1353-8020(11)70060-8
Borroni, B., Agosti, C., Magnani, E., Di Luca, M., & Padovani, A. (2011). Genetic bases of Progressive Supranuclear Palsy: the MAPT tau disease. Curr Med Chem, 18(17), 2655-2660.
Levy, R. (2011). [Progressive supranuclear palsy: what's new?]. Geriatr Psychol Neuropsychiatr Vieil, 9(2), 191-201. doi: 10.1684/pnv.2011.0271
Louis, E. D., Babij, R., Ma, K., Cortes, E., & Vonsattel, J. P. (2013). Essential tremor followed by progressive supranuclear palsy: postmortem reports of 11 patients. J Neuropathol Exp Neurol, 72(1), 8-17. doi: 10.1097/NEN.0b013e31827ae56e
Litvan, I., Agid, Y., Calne, D., Campbell, G., Dubois, B., Duvoisin, R. C., ... & Hallett, M. (1996). Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) report of the NINDS-SPSP international workshop. Neurology, 47(1), 1-9.
STEELE, J. C., Richardson, J. C., & OLSZEWSKI, J. (1964). Progressive supranuclear palsy: a heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Archives of neurology, 10(4), 333-359.
Albert, M. L., Feldman, R. G., & Willis, A. L. (1974). Thesubcortical dementia'of progressive supranuclear palsy. Journal of Neurology, Neurosurgery & Psychiatry, 37(2), 121-130.