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Cover Page

The following handle holds various files of this Leiden University dissertation:

http://hdl.handle.net/1887/74007

Author: Baake, V.

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530433-L-sub01-bw-Baake 530433-L-sub01-bw-Baake 530433-L-sub01-bw-Baake 530433-L-sub01-bw-Baake Processed on: 11-4-2019 Processed on: 11-4-2019 Processed on: 11-4-2019

Processed on: 11-4-2019 PDF page: 1PDF page: 1PDF page: 1PDF page: 1

Huntington’s Disease

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Financial support for the printing of this thesis was kindly provided by the Dutch Huntington Association.

PhD Thesis, Leiden University Medical Center, Leiden 2019 Design & lay-out: Ton, Wouter en Verena Baake

ISBN: 978-94-028-1471-2 Printed by: Ipskamp Printing © 2019 Verena Baake, Haarlem

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530433-L-sub01-bw-Baake 530433-L-sub01-bw-Baake 530433-L-sub01-bw-Baake 530433-L-sub01-bw-Baake Processed on: 11-4-2019 Processed on: 11-4-2019 Processed on: 11-4-2019

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Huntington’s Disease

Cognition and Apathy

Proefschrift

ter verkrijging van

de graad van Doctor aan de Universiteit Leiden, RSJH]DJYDQ5HFWRU0DJQL¿FXVSURIPU&--06WRONHU

volgens besluit van het College voor Promoties te verdedigen op woensdag 29 mei 2019

klokke 13:45 uur door Verena Baake

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Promotor:

Prof. Dr. R.A.C. Roos

Promotiecommissie:

Dr. D. Craufurd, the University of Manchester Prof. Dr. H.P.H. Kremer, Rijksuniversiteit Groningen Prof. Dr. R.C. van der Mast

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Table of contents

Chapter 1: Introduction ... 7

Chapter 2: Participants at the Leiden site of the REGISTRY study:a demographic approach ...15

Chapter 3: Cognitive decline in Huntington’s disease expansion gene carriers ...31

Chapter 4: ,QÀXHQFHRIPHGLFDWLRQXVHRQFRJQLWLYH performance in Huntington’s disease ...59

Chapter 5: Apathy and atrophy of subcortical brain structures in Huntington’s disease:a two-year follow-up study ...81

Chapter 6: Huntington’s disease gene expansion carriers are aware of their degree of apathy ...97

Chapter 7: Discussion and concluding remarks ... 109

Nederlandse Samenvatting ... 116

List of publications ... 120

Dankwoord ... 121

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