Huntington’s disease : hypothalamic, endocrine and metabolic aspects
Aziz, N.A.
Citation
Aziz, N. A. (2010, March 31). Huntington’s disease : hypothalamic, endocrine and metabolic aspects. Retrieved from https://hdl.handle.net/1887/15183
Version: Corrected Publisher’s Version
License: Licence agreement concerning inclusion of doctoral thesis in the
Huntington’s Disease
Hypothalamic, endocrine and metabolic aspects
N. Ahmad Aziz
ISBN: 978-94-6108023-3
Design & layout: N. Ahmad Aziz Cover design: Jorien M.M. van der Burg Printed by: GildePrint, Enschede
© 2010 N.A. Aziz
Copyright of each chapter is with the publisher of the journal in which the work has appeared. No part of this book may be reproduced or distributed in any form, by print, photoprint, microfilm, digital file, or any other means without prior written permission from the copyright holder.
The studies described in this thesis were partly supported by the Netherlands Organization for Scientific Research (grant number 017.003.098 to N.A.Aziz).
Huntington’s Disease
Hypothalamic, endocrine and metabolic aspects
Proefschrift ter verkrijging van
de graad van Doctor aan de Universiteit Leiden
op gezag van de Rector Magnificus prof. mr. P.F. van der Heijden volgens besluit van het College der Promoties
te verdedigen op woensdag 31 maart 2010 klokke 16:15 uur
door
Nasir Ahmad Aziz
geboren te Kandahar (Afghanistan)
in 1981
Promotiecommissie
Promotores:
Prof. dr. R.A.C. Roos Prof. dr. H. Pijl
Overige leden:
Prof. dr. H.P.H. Kremer (Rijksuniversiteit Groningen) Prof. dr. J.A. Romijn
Prof. dr. D.F. Swaab (Universiteit van Amsterdam, Nederlands Instituut voor Neurowetenschappen)
,PvD× Ölµ D× íÊkßvC éÆ ÙìVß×
.ÙëoClÛ ÖCoC éÆ ÙìÛCpF ælÛq D×
(íwØz 1256-1317) ÍDGÂC lØe×
We are a wave, our calm is our naught,
We have no rest, lest we will be not.
Muhammad Iqbal (1877-1938 CE)
To my father,
who taught me reason and perseverance To my mother,
who bestowed me with love and inspiration To Jorien,
for sharing the joys and sorrows of life with me
C ontents
Chapter 1 GENERAL INTRODUCTION & AIMS OF THE THESIS 1
Rev Neurosci (2007); 18: 223-251
PART I SECONDARY SIGNS OF HUNTINGTON’S DISEASE 43
Chapter 2 Weight loss in Huntington’s disease increases with higher CAG repeat number 45 Neurology (2008); 71(19): 1506-1513
Chapter 3 Normal and mutant HTT interact to affect clinical severity and progression in
Huntington’s disease 57
Neurology (2009); 73(16):1280-1285
Chapter 4 Sleep and circadian rhythm alterations correlate with depression and cognitive
impairment in Huntington’s disease 73
Parkinsonism Rel Disorders (in press)
Chapter 5 Autonomic symptoms in patients and premanifest mutation carriers of Huntington’s
disease 87
Eur J Neurol (in press)
PART II HYPOTHALAMIC PATHOLOGY IN HUNTINGTON’S DISEASE 99 Chapter 6 Hypocretin and melanin-concentrating hormone in patients with Huntington’s disease 101
Brain Pathol (2008); 18(4): 474-483
PART III ENDOCRINE STUDIES IN HUNTINGTON’S DISEASE 119 Chapter 7 Increased hypothalamic-pituitary-adrenal axis activity in Huntington’s disease 121
J Clinical Endocrinol Metab (2009); 94(4):1223-8
Chapter 8 Growth hormone and ghrelin secretion are associated with clinical severity in
Huntington’s disease 135
C ontents
Eur J Neurol (in press)
Chapter 9 Altered thyrotropic and lactotropic axes regulation in Huntington’s disease 149
Clinical Endocrinol (Oxf.) (in revision) Chapter 10 Leptin secretion rate increases with higher CAG repeat number in Huntington’s disease patients 161
Clinical Endocrinol (Oxf.) (in press) Chapter 11 Delayed onset of the diurnal melatonin rise in patients with Huntington’s disease 173
J Neurol (2009) 256:1961–1965 PART IV METABOLIC STUDIES IN HUNTINGTON’S DISEASE 183
Chapter 12 Systemic energy homeostasis in Huntington’s disease patients 185
J Neurol Neurosurg Psychiatry (in press) Chapter 13 SYNOPSIS, CONCLUSIONS & FUTURE PERSPECTIVES 197
APPENDICES 205
A Summary and conclusions in Dutch 207
B Excerpt in Persian 215
C Acknowledgments 217
D Curriculum vitae 219
E List of publications 221