- Poster presentation
- Open Access
Progressive supranuclear palsy (Steele - Richardson - Olszewski syndrome) and dementia
© Psychogios et al.; licensee BioMed Central Ltd. 2008
- Published: 17 April 2008
- Supportive Care
- Differential Development
- Cortical Region
- Progressive Supranuclear Palsy
The progressive supranuclear palsy (PSP) constitutes the second most common parkinsonian disorder after the idiopathic form of the disease with an incidence of 5.3 newly diagnosed cases per 100,000 people annually and prevalence 1.39 per 100,000. Clinically, the disease presents itself with not only motor symptoms but also dementia. The aim of this study is to review and present all recent data, specifically those related to the neuropathology and biochemistry, of dementia in PSP.
Recent advances in molecular and genetic research of PSP are being reviewed. MRI and PET findings are analytically described, while the utility of other exams, like EEG, in the differential diagnosis between PSP and other dementias evaluated.
As depicted from the previous mentioned imaging methods and research, the reported significant learning deficits in PSP are associated with disease-related lesions located not only at subcortical (globus pallidus, mesencephalon, corpus striatum), but also at cortical regions (prefrontal and premotor cortex). A wide spectrum of symptoms is correlated to the differential development of these lesions from the neurofibrillary tangles within the cortex.
The progression of the condition to dementia is a characteristic element of PSP, but does not occur in the same manner in all patients. The type of the neurofibrillary tangles and the range of the regions that they affect, differentiate this condition from other neurodegenerative disorders. Further research is required in relation to the understanding of the neurotransmitter systems involved in the memory and cognitive impairment of PSP, which will be the cornerstone for the discovery and implementation of novel supportive care regimens.
- Dubois B., Slachvesky A., Pillon B., Beato R., Villalponda J.M., Litvan I.: “Applause sign” helps to discriminate PSP from FTD and PD. Neurology. 2005, 64: 2132-2133. 10.1212/01.WNL.0000165977.38272.15.View ArticlePubMedGoogle Scholar
- Millar D., Griffiths P., Zermansky A.J, Burn D.J.: Characterizing Behavioral and Cognitive Dysexecutive Changes in Progressive Supranuclear Palsy. Movement Disorders. 2006, 21: 199-207. 10.1002/mds.20707.View ArticlePubMedGoogle Scholar
- Mott R.T., Dickson D.W., Trojanowski J.Q., Zhukareva V., Lee V.M., Forman M., Van Deerlin V., Ervin J.F., Wang Deng-Shun B.A., Schmechel D.E., Hulette C.M.: Neuropathologic, Biochemical, and Molecular Characterization of the Frontotemporal Dementias. J Neuropathol Exp Neurol. 2005, 64: 420-428.PubMedGoogle Scholar
- Cotelli M., Borroni B., Manenti R., Alberici A., Calabria M., Agosti C., Ginex V., Ortelli P., Binetti G., Zanetti O.: Action and Object Naming in Frontotemporal Dementia, Progressive Supranuclear Palsy, and Corticobasal Degeneration. Neuropsychology. 2006, 20 (5): 558-565. 10.1037/0894-4184.108.40.2068.View ArticlePubMedGoogle Scholar
This article is published under license to BioMed Central Ltd.