THE PATHOPHYSIOLOGY OF CHIARI MALFORMATION TYPE I WITH RESPECT TO STATIC AND PULSATILE INTRACRANIAL PRESSURE RADEK FRIČ A doctoral thesis Faculty of Medicine, University of Oslo Department of Neurosurgery, Oslo University Hospital - Rikshospitalet Oslo, Norway 2017 © Radek Frič, 2017 Series of dissertations submitted to the Faculty of Medicine, University of Oslo ISBN 978-82-8377-141-1 All rights reserved. No part of this publication may be reproduced or transmitted, in any form or by any means, without permission. Cover: Hanne Baadsgaard Utigard. Print production: Reprosentralen, University of Oslo. CONTENTS Abbreviations…………………………………………………………………………………..7 Summary .................................................................................................................................... 8 List of original publications ....................................................................................................... 9 1. Introduction ........................................................................................................................ 10 1.1 Background ..................................................................................................................... 10 1.2 History ............................................................................................................................ 13 1.3 Etiology of CMI .............................................................................................................. 16 1.4 Epidemiology of CMI ..................................................................................................... 17 1.5 Natural history of CMI ................................................................................................... 18 1.6 Clinical symptoms of CMI ............................................................................................. 19 1.7 Radiological findings in CMI ......................................................................................... 20 1.7.1 Syringomyelia and its pathophysiology ................................................................... 21 1.7.2 Skeletal anomalies at the CCJ .................................................................................. 24 1.7.3 CMI and hydrocephalus ........................................................................................... 25 1.7.4 Scoliosis in CMI ...................................................................................................... 26 1.8 Indication for treatment of CMI ..................................................................................... 26 1.9 State-of-the-art in the treatment of CMI ......................................................................... 27 1.9.1 Conservative treatment ............................................................................................ 27 1.9.2 Surgical treatment .................................................................................................... 28 1.10 Outcome after treatment for CMI ................................................................................. 32 1.11 Intracranial compliance in CMI .................................................................................... 32 1.12 Static and pulsatile ICP in CMI .................................................................................... 35 1.13 Idiopathic intracranial hypertension (IIH) .................................................................... 38 2. Aims of the thesis ........................................................................................................................... 40 3. Patients material and methods ................................................................................................... 41 3.1 Ethical considerations ..................................................................................................... 41 3.2 Simultaneous measurements of static and pulsatile pressure within intracranial and intraspinal compartments (Paper I) ....................................................................................... 41 3.2.1 Patients ..................................................................................................................... 41 3.2.2 Data acquisition ....................................................................................................... 42 3.2.3 Radiological assessment .......................................................................................... 42 3.2.4 Definition of tentative abnormal pulsatile and static pressure ................................. 43 3.2.5 Statistical analysis .................................................................................................... 43 2 3.3 CSF pressure gradient across the CCJ derived from phase-contrast MRI (Paper II) ..... 43 3.3.1 Patients ..................................................................................................................... 43 3.3.2 Data acquisition ....................................................................................................... 44 3.3.3 Radiological assessment .......................................................................................... 44 3.3.4 Definition of tentative abnormal pulsatile and static pressure ................................. 44 3.3.5 Statistical analysis .................................................................................................... 44 3.4 Perioperative changes in ICP following FMD (Paper III) .............................................. 44 3.4.1 Patients ..................................................................................................................... 44 3.4.2 Data acquisition ....................................................................................................... 45 3.4.3 Definition of tentative abnormal pulsatile and static pressure ................................. 45 3.4.4 Statistical analysis .................................................................................................... 45 3.5 A comparison of clinical, radiological, and ICP findings in CMI and IIH (Paper IV) .. 46 3.5.1 Patients ..................................................................................................................... 46 3.5.2 Data acquisition ....................................................................................................... 46 3.5.3 Radiological assessment .......................................................................................... 46 3.5.4 Definition of tentative abnormal pulsatile and static pressure ................................. 47 3.5.5 Statistical analysis .................................................................................................... 47 3.6 Cardiovascular comorbidity in CMI and IIH (Paper V) ................................................. 47 3.6.1 Patients ..................................................................................................................... 47 3.6.2 Data acquisition ....................................................................................................... 47 3.6.3 Prevalence of cardiovascular disease and diabetes versus pulsatile ICP ................. 48 3.6.4 Statistical analysis .................................................................................................... 48 4. Results .............................................................................................................................................. 49 4.1 Simultaneous measurements of static and pulsatile pressure within intracranial and intraspinal compartments (Paper I) ....................................................................................... 49 4.1.1 Patients ..................................................................................................................... 49 4.1.2 Radiological findings ............................................................................................... 49 4.1.3 Clinical symptoms versus radiological findings ...................................................... 49 4.1.4 Pressure recordings .................................................................................................. 49 4.1.5 Clinical symptoms versus pressure parameters ....................................................... 50 4.1.6 Radiological findings versus pressure parameters ................................................... 50 4.2 CSF pressure gradient across the CCJ derived from phase-contrast MRI (Paper II) ..... 50 4.2.1 Patients ..................................................................................................................... 50 4.2.2 Comparison of MRI-dP and CSF flow velocities in patients with CMI and healthy subjects .............................................................................................................................. 50 3 4.2.3 Invasive pulsatile ICP measurements and correlation with MRI-dP ....................... 51 4.2.4 Comparison of radiological findings ....................................................................... 51 4.3 Perioperative changes of ICP following FMD (Paper III) .............................................. 51 4.3.1 Patients ..................................................................................................................... 51 4.3.2. Pulsatile and static ICP after FMD ......................................................................... 51 4.4 A comparison of clinical, radiological, and ICP findings in CMI and IIH