The main aim of this thesis was to assess clinical, neuropathological and neurochemical aspects within this spectrum, but focused mainly on PSP. Furthermore, we describe a mutation in a novel neurodegenerative disorder.

Chapter 1 provides a general overview of PSP, including clinical, genetic and pathological aspects. In chapter 2.1 a comparative study on survival in two large cohorts of PSP and FTD patients is described. The next chapters focus on the clinicoradiological (chapter 2.2) and neurochemical (chapter 3) aspects of the midcingulate cortex in PSP. The latter includes an analysis of twenty different receptors from seven neurotransmitter systems in 16 post-mortem PSP brains. Chapter 4 comprises studies of PSP-like disorders. In chapter 4.1 we describe the various steps to link a mutation to a novel neurodegenerative disorder clinically characterized by dementia and/or parkinsonism. Chapter 4.2 contains the description of neuropathological changes of a presymptomatic carrier of a frontotemporal dementia (FTD) tau mutation, a related hereditary tauopathy. Finally, in the general discussion (chapter 5) the main findings in light of the current knowledge about the disease are discussed and recommendations for future research are made.

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J.C. van Swieten (John) , A.J.W. Boon (Agnita)
Erasmus University Rotterdam
The studies in this thesis were financially supported by Parkinson Vereniging, 2020 European Union Framework Programme for Research and Innovation, Hersenstichting Nederland, Internationale Stichting Alzheimer Onderzoek, Alzheimer Nederland, Fonds Nuts-Ohra, Prinses Beatrix Fonds and Stichting Dioraphte
Department of Neurology

Chiu, W. Z. (2017, December 6). Novel Insights into Progressive Supranuclear Palsy and Related Disorders. Retrieved from