Research Into Spinal Deformities 8 1st Edition T Kotwicki T B Grivas

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Research Into Spinal Deformities 8 1st Edition T Kotwicki T B Grivas
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RESEARCH INTO SPINAL DEFORMITIES 8
Research into Spinal Deformities 8, IOS Press, Incorporated, 2012. ProQuest Ebook Central,
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Research into Spinal Deformities 8, IOS Press, Incorporated, 2012. ProQuest Ebook Central,
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Research into Spinal Deformities 8

Edited by
Tomasz Kotwicki
Spine Disorders Unit, Department of Pediatric Orthopedics
University of Medical Sciences, Poznan, Poland
and
Theodoros B. Grivas
Orthopaedic and Trauma Department
“Tzanio” General Hospital of Piraeus, Piraeus, Greece

Amsterdam • Berlin • Tokyo • Washington, DC
Research into Spinal Deformities 8, IOS Press, Incorporated, 2012. ProQuest Ebook Central,
Copyright © 2012. IOS Press, Incorporated. All rights reserved.

© 2012 The authors and IOS Press.
All rights reserved. No part of this book may be reproduced, stored in a retrieval system,
or transmitted, in any form or by any means, without prior written permission from the publisher.
ISBN 978-1-61499-066-6 (print)
ISBN 978-1-61499-067-3 (online)
Library of Congress Control Number: 2012940567
Cover picture: Poznan Old Market Square by Ewa Kotwicka
Publisher
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Research into Spinal Deformities 8, IOS Press, Incorporated, 2012. ProQuest Ebook Central,
Copyright © 2012. IOS Press, Incorporated. All rights reserved.

In July 2012 the International Research Society of Spinal Deformities (IRSSD) held its
ninth biennial meeting in Poznan, Poland.
IRSSD was founded in 1994, but its history starts in Vermont, USA, where a group
of researchers first met in 1980 to discuss moiré topography techniques for the assess-
ment of trunk deformity in scoliosis. The inaugural workshops were organized by Drs.
Morey Moreland, Malcolm Pope and Gordon Armstrong and focused on instrumenta-
tion, school screening, quantification & computerization. Multidisciplinary participa-
tion from both public and private sector, and participants from Japan, USA, Canada
and European countries, contributed to the debates and discussion. Following the suc-
cess of this meeting, a series of biennial meetings devoted to
Surface Topography and
Spinal Deformity were organized. After Vermont, chronologically and geographically,
the meetings were held: 1982 in Munster, Germany, hosted by Drs. Burkhard Drerup,
Wolfgang Frobin & Eberhard Hieholzer; 1984 in Oxford, UK, organized by Allan
Turner-Smith & J. Derek Harris; 1986 in Canada organized by Ian Stokes, James R.
Pekelsky & Morey Moreland; and 1990 in Lisbon, Portugal hosted by A. Alberti. This
latter meeting was the last that focused on surface topography. A meeting that drew
together the fields of Spinal Deformity & Surface Topography and 3-D Scoliotic De-
formities was then held in 1992, in Montreal, Canada, at which discussions were held
to decide whether a new Society embracing both Spinal Deformity & Surface Topogra-
phy and 3-D Scoliotic Deformities, should be formally established. Two years later in
1994, in Pescara, Italy, further discussions during the meeting on Three-Dimensional
Analysis of Spinal Deformities, hosted by Moreno D’Amico, Aricia Merolli and Gior-
gio C. Santambrogio led to The International Research Society of Spinal Deformities
(IRSSD) being officially founded.
The creation of the new society reflected a desire to consolidate the momentum of
the preceding meetings and also to broaden their scope. Subsequently, the first IRSSD
meeting was held in 1996 in Sweden, hosted by John Sevastik, and included aetiology
& pathomechanisms of scoliosis as main topics. The second meeting was held in 1998
in Vermont and was hosted by Ian Stokes, who introduced new topics: molecular biol-
ogy & regulation of spinal growth. In the years since, the society has continued to cov-
er a broad range of issues related to scoliosis. The third meeting was subsequently held
in 2000 in Clermont Ferrand, France, hosted by Alain Tanguy and Bernard Peuchot,
the fourth in 2002 in Athens, Greece, organized by Dr. Theodoros B. Grivas, the fifth

Preface

Research into Spinal Deformities 8
T. Kotwicki and T.B. Grivas (Eds.)
IOS Press, 2012
© 2012 The authors and IOS Press. All rights reserved.
v
Research into Spinal Deformities 8, IOS Press, Incorporated, 2012. ProQuest Ebook Central,
Copyright © 2012. IOS Press, Incorporated. All rights reserved.

meeting in 2004 in Vancouver, Canada, hosted by Bonita J. Sawatzky and S.J. Tred-
well, the sixth meeting in 2006 in Ghent, Belgium, organized by Dirk Uyttendaele, the
seventh meeting in 2008 in Liverpool, England, organized by Peter H. Dangerfield, the
eighth meeting in 2010 in Montréal, Canada, organized by Carl-Eric Aubin, Ian A.F.
Stokes, Hubert Labelle, and Alain Moreau and finally the ninth IRSSD meeting in 2012
in Poznan, Poland, organized by Tomasz Kotwicki.
The society promotes a multidisciplinary approach to scoliosis and spinal problems,
with a strong emphasis on research in the field of etiology as well as the clinical effec-
tiveness of a wide range of interventions. In one form or another, the IRSSD has been
active for three decades, during which time it has encouraged open discussion in all
areas related to spinal deformities. Contribution, involvement and interaction among
people from all over the world has been a highlight of its activities. As Keith Bagnall
said “IRSSD has always been a vehicle to carry the initial, small flicker of the flame. In
the past, some of these flames have grown to become forest fires while others have
simply died for whatever reason”.
This book contains the Proceedings of the 9th IRSSD 2012 meeting: peer-reviewed
short papers or abstracts summarizing the 129 papers and posters included in the pro-
gram. It covers all aspects of spinal deformity research, including etiology, genetics,
biology, growth, metabolism, biomechanics, imaging technologies, innovations in
treatment, and treatment outcomes. These scientific proceedings provide the opportuni-
ty for readers to learn more about the latest development in this field that will be pre-
sented and discussed during the meeting.
We would like to thank all the participants: the authors for sharing their work with
us, as well as the members of the scientific committee, chaired by Dr. Theodoros B.
Grivas, MD, PhD. The members of the scientific committee, who deserve a particular
acknowledgment, divided into groups to review the various topics of the meeting
program, namely for Biomechanics, Movement, Posture: Jim Raso, Carl-Éric Aubin,
Moreno D’Amico, Yvan Petit, Nachi Chockalingam, Rene M. Castelein, Konstantinos
Sultanis, Aleksander Kabsch; for Genetics and Etiology: Alain Moreau, Keith Bagnall,
Peter Dangerfield, Nelson Tang; for Growth and Metabolism: Florina Moldovan,
Isabelle Villemure, Ian Stokes, Nathalie Alos, Wlodzimierz Samborski; for Imaging
and Measurements: Winnie Chu, Tomasz Kotwicki, Delphine Perie, Farida Cheriet; for
Treatment: Theodoros B. Grivas, Hans-Rudolf Weiss, Jack Cheng, Hubert Labelle,
Jean-Marc Mac-Thiong, Francisco Javier Sanchez Perez-Grueso, Tiziana Greggi, Jacek
Durmala, Seung Woo Suh; and finally, for Quality of Life: Lorenzo Aulisa, Jose Maria
Climent, Juan Bago, Maciej Glowacki and Nobumasa Suzuki.
We do hope that this book, representing the state of the art in scoliosis research in
2012, will stimulate further studies which will lead us closer to an understanding of
idiopathic scoliosis, which remains a fascinating and enigmatic disease. We hope that
this meeting will be a unique opportunity to meet in Poznan, Poland, in order to discuss
the topics related to spinal deformities, and to advance the study and research into spin-
al deformities for the benefit of patients.
Tomasz Kotwicki and Theodoros B. Grivas
Poznan, April 30th 2012
vi
Research into Spinal Deformities 8, IOS Press, Incorporated, 2012. ProQuest Ebook Central,
Copyright © 2012. IOS Press, Incorporated. All rights reserved.

Acknowledgements
The honorary patronage of the 9th Biennial IRSSD Meeting was attributed by:
President of the City of Poznan
President of the University of Medical Sciences of Poznan
Polish Society of Spine Surgery
Polish Orthopedic and Traumatologic Society
Polish Rehabilitation Society
Polish Physiotherapy Society
The meeting and this publication received valuable support from several companies
involved in the management of spinal deformities. This support made it possible to
enhance the quality of the meeting with number of activities and programs.

Strategic partners:
Medtronic
Diers
Alteris Implants
Main partners:
Stryker
Rehasport
Exhibitors:
Vigo
CHM
Samodzielny Publiczny Zaklad Zaopatrzenia Ortopedycznego
Protoma
Media patronage:
Ortopedia Polska: www.ortopediapolska.pl
Rynek Zdrowia: www.rynekzdrowia.pl
Medycyna Praktyczna

The Organizing Committee members deserve thanks for their work. Special thanks go
to Piotr Janusz, Agnieszka Zygmunt and Michał Karlik.

Tomasz Kotwicki
Poznan, April 2012






vii
Research into Spinal Deformities 8, IOS Press, Incorporated, 2012. ProQuest Ebook Central,
Copyright © 2012. IOS Press, Incorporated. All rights reserved.

Presidents of the International Research Society of Spinal Deformities

Yrs. Location President
1996 Stockholm Morey Moreland
1998 Burlington Dirk Uyttendaele
2000 Clermont-Ferrand Stig Willner (*)/Dirk Uyttendaele
2002 Athens Nobumasa Suzuki
2004 Vancouver Hubert Labelle
2006 Ghent Ian Stokes
2008 Liverpool Keith Bagnall
2010 Montreal Theo Grivas
2012 Poznan Carl-Eric Aubin












viii
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Copyright © 2012. IOS Press, Incorporated. All rights reserved.

Contents
Preface v
Tomasz Kotwicki and Theodoros B. Grivas
Acknowledgements vii
Chapter 1. Invited Lectures
Whither the Etiopathogenesis (and Scoliogeny) of Adolescent Idiopathic
Scoliosis? 3
R. Geoffrey Burwell and Peter H. Dangerfield
Pre-Existent Rotation of the Normal Spine at Different Ages and Its
Consequences for the Scoliotic Mechanism 20
René M. Castelein
Chapter 2. Genetics and Aetiology
Podium Presentations
Role of High Central Leptin Activity in a Scoliosis Model Created in Bipedal
Amputated Mice 31
Tao Wu, Xu Sun, Zezhang Zhu, Xin Zheng, Bangping Qian, Feng Zhu,
Jack C.Y. Cheng and Yong Qiu
Maternal Age at Birth: Does It Dictate the Epigenotypic Expression of
the Trunkal Asymmetry of a Child? 36
Theodoros B. Grivas, Constantinos Mihas, Christina Mazioti, Nikolaos Zisis,
Samantha Sakellaropoulou, Antonios Akriotis and R. Geoffrey Burwell
Secondary Scoliosis After Thoracotomy in Patients with Aortic Coarctation and
Patent Ductus Arteriosus 43
Marek Roclawski, Rafal Pankowski, Andrzej Smoczynski, Marcin Ceynowa,
Wojciech Kloc, Wojciech Wasilewski, Piotr Jende, Wieslaw Liczbik,
Piotr Beldzinski, Witold Libionka, Olaf Pierzak, Stanislaw Adamski
and Miroslaw Niedbala
Association Study of IL-17RC, CHL1, DSCAM and CNTNAP2 Genes
Polymorphisms with Adolescent Idiopathic Scoliosis Susceptibility in
a Chinese Han Population 47
Song Zhou, Zezhang Zhu, Xusheng Qiu, Weifei Wu, Weijun Wang, Zhen Liu,
Feng Lv and Yong Qiu
Mutation Analysis of MESP2, HES7 and DUSP6 Gene Exons in Patients with
Congenital Scoliosis 52
Xu-Sheng Qiu, Song Zhou, Hua Jiang, Ming-Liang Ji, Qi Ding, Feng Lv,
Zhen Liu, Nelson Tang, Jack C.Y. Cheng and Yong Qiu




ix
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Copyright © 2012. IOS Press, Incorporated. All rights reserved.

Poster Presentations
Estrogen Receptor 2 Expression in Back Muscles of Girls with Idiopathic
Scoliosis – Relation to Radiological Parameters 59
Błażej Rusin, Tomasz Kotwicki, Aleksandra Głodek, Miroslaw Andrusiewicz,
Paulina Urbaniak and Małgorzata Kotwicka
Ever-Present Factors in Healthy Children that Can Deform Their Spines.
Opposition to Dickson’s Paradigm on Lordosis 63
P.J.M. van Loon
A Similar Approach in Bracing of Adolescent Scoliosis and Kyphosis with
the Use of Growth Itself in Thoracolumbar Lordotic Intervention (TLI) 68
P.J.M. van Loon, M. Roukens, F.B. Thunnissen and J. Munneke
Chapter 3. Biomechanics, Movement, Posture
Podium Presentations
The Structure of Postural Disorders and Spinal Deformities in Age and Gender
According to Computer Optical Topography 77
V.N. Sarnadskiy
Integrated Assessment of Back Muscles Bioelectrical Activity and H-Reflex
Research in AIS 83
I. Syngayevskaya, S. Bumakova, D. Pinchuk and M. Dudin
Peculiarities of Brain Functioning in Children with Adolescence Idiopathic
Scoliosis (AIS) According to EEG Studies 87
D. Pinchuk, M. Dudin, S. Bekshayev and O. Pinchuk
Patterns of Weight Bearing Impact Sagittal Spinal Balance 91
Hans-Rudolf Weiss
A Multibody-Based Approach to the Computation of Spine Intervertebral
Motions in Scoliotic Patients 95
Gabriel Abedrabbo, Paul Fisette, Pierre-Antoine Absil, Philippe Mahaudens,
Christine Detrembleur, Maxime Raison, Xavier Banse, Carl-Eric Aubin and
Maryline Mousny
Finite Element Model of Spinal Hemiepiphysiodesis: Effect of Contact
Conditions, Initial Conditions, and Growth 99
B. Kumar, D.I. Bylski-Austrow and Y. Liu
The Effect of Leg Length Discrepancy on Pelvis and Spine Kinematics During
Gait 104
R. Needham, N. Chockalingam, D. Dunning, A. Healy, E.B. Ahmed and
A. Ward
LBP and Lower Limb Discrepancy: 3D Evaluation of Postural Rebalancing via
Underfoot Wedge Correction 108
Moreno D’Amico, Piero Roncoletta, Francesca Di Felice, Daniele Porto,
Rosa Grazia Bellomo and Raoul Saggini




x
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Lombo-Sacral Joint Efforts During Gait: Comparison Between Healthy and
Scoliotic Subjects 113
Maxime Raison, Laurent Ballaz, Christine Detrembleur,
Philippe Mahaudens, Julien Lebleu, Paul Fisette and Maryline Mousny
The Effect of Frontpacks, Shoulder Bags and Handheld Bags on 3D Back Shape
and Posture in Young University Students: An ISIS2 Study 117
J. Bettany-Saltikov and L. Cole
Poster Presentations
Biomechanical Analysis of Spino-Pelvic Parameters in Adolescent Idiopathic
Scoliosis After Spinal Instrumentation and Fusion: A Case Study 125
Saba Pasha, Carl-Eric Aubin, Hubert Labelle, Stefan Parent and
Jean-Marc Mac-Thiong
Variations in Bioelectric Activity During Symmetric Loading and Asymmetric
Stretching of Paraspinal Extensors in Young Adult Women with Mild Single
Curve Scoliosis 129
Wiesław Chwała, Maciej Płaszewski and Paweł Kowalski
Present Day Explanation of the Clinical Signs in the Biomechanical Aetiology of
the So-Called Idiopathic Scoliosis (1995–2011). The Relationship Between
the “Model of Hips Movement” and the Character of Scoliosis; Three Groups
and Four Types. The Causative Role of “Gait” and “Standing ‘at Ease’ on
the Right Leg” 133
Tomasz Karski
Typical and Atypical AIS. Pathogenesis 139
M. Dudin and D. Pinchuk
Leg Length Discrepancy in Scoliotic Patients 146
Moreno D’Amico, Piero Roncoletta, Francesca Di Felice, Daniele Porto,
Rosa Grazia Bellomo and Raoul Saggini
Validation of a Multi-Segment Spinal Model for Kinematic Analysis and
a Comparison of Different Data Processing Techniques 151
R. Needham, N. Chockalingam, R. Naemi, T. Shannon and A. Healy
Inter-Examiner, Intra-Session and Inter-Session Reliability of Gait Measurement 155
Justyna Leszczewska, Dariusz Czaprowski, Paulina Pawłowska,
Karolina Białobrzewska, Anna Gębicka and Tomasz Kotwicki
Classification of Postural Disorders and Spinal Deformities in the Three
Dimensions According to Computer Optical Topography 159
V.N. Sarnadskiy
The Evolution of State of Vertebral Column in Three Planes 164
Victor Pecherskiy, Ludmila Lihacheva, Aleksei Chicherin, Michail Dudin
and Dmitrii Pinchuk
Neurohumoral Regulation in Children with Idiopathic Scoliosis 168
T. Khaymina, T. Avaliani, M. Dudin and D. Pinchuk
Photodynamic Impact on the Epiphyseal Plates 174
S. Kurchenko, A. Shashko, M. Dudin, V. Mikhailov, G. Netylko and
V. Ashmarov


xi
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Chapter 4. Growth and Metabolism
Podium Presentations
The Value of Different Risser Grading Systems in Determining Growth Maturity
of Girls with Adolescent Idiopathic Scoliosis 183
Weijun Wang, Xin Zhen, Xu Sun, Zezhang Zhu, Feng Zhu, T.P. Lam,
Jack C.Y. Cheng and Yong Qiu
Upper Arm Length Model Suggests Transient Bilateral Asymmetry Is
Associated with Right Thoracic Adolescent Idiopathic Scoliosis (RT-AIS) with
Implications for Pathogenesis and Estimation of Linear Skeletal Overgrowth 188
R. Geoffrey Burwell, Ranjit K. Aujla, Michael P. Grevitt,
Tabitha L. Randell, Peter H. Dangerfield, Ashley A. Cole, Alanah S. Kirby,
Frances J. Polak, Roland K. Pratt, John K. Webb and Alan Moulton
Analysis of Body Growth Parameters in Girls with Adolescent Idiopathic
Scoliosis: Single Thoracic Idiopathic Scoliosis Versus Single Lumbar
Idiopathic Scoliosis 195
Zhen Liu, Zezhang Zhu, Jing Guo, Saihu Mao, Weijun Wang,
Bangping Qian, Feng Zhu, Winnie Chu, Jack C.Y. Cheng
and Yong Qiu
Height Velocity Curves in Female Patients with Idiopathic Scoliosis 202
Masaaki Chazono, Sigeru Soshi, Yoshikuni Kida, Kurando Hashimoto,
Takeshi Inoue, Yousuke Nakamura, Akira Shinohara, Keishi Marumo,
Katsuki Kono and Nobumasa Suzuki
Poster Presentations
The In-Vivo Effect of Torque on Growth in Caudal Vertebrae 209
Robert Rizza, XueCheng Liu and John Thometz
Early Development of the Costovertebral Joints 213
Po-Jung Chen and Witold Wozniak
Severe Spinal Deformity and Multiple Vertebral Collapses in Juvenile Cushing
Syndrome: A Case Report 217
Enrico Pola, Barbara Rossi, Marilda Mormando, Luigi Aurelio Nasto,
Debora Colangelo, Silvia Della Casa, Laura De Marinis and
Lorenzo Aulisa
Chapter 5. Imaging and Measurement
Podium Presentations
An Improved Kinematic Model of the Spine for Three-Dimensional Motion
Analysis in the Vicon System 227
Mirosława M. Długosz, Daria Panek, Paweł Maciejasz, Wiesław Chwała
and Witold Alda
Discordance in Spinal and Thoracic Correction Using Powerful Full Screw
Constructs in Idiopathic Scoliotic 232
Konstantinos Soultanis, Theodoros B. Grivas, Konstantinos A. Starantzis,
Nikolaos A. Stavropoulos, Christos Markopoulos and
Panayotis Papagelopoulos
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The Rib Vertebra Angle Difference and Its Measurement in 3D for
the Evaluation of Early Onset Scoliosis 238
G. Foley, C.E. Aubin, H. Labelle, J. Sanders, J. d’Astous, C. Johnston
and S. Parent
Analysis of the Anterior Trunk Symmetry Index (ATSI). Preliminary Report 242
Lukasz Stolinski, Tomasz Kotwicki, Dariusz Czaprowski,
Joanna Chowanska and Nobumasa Suzuki
Goniometer Evaluation of Thoracic Kyphosis and Lumbar Lordosis in Subjects
During Growth Age: A Validity Study 247
Aristide R. Gravina, Claudio Ferraro, Antonio Frizziero, Marco Ferraro
and Stefano Masiero
Poster Presentations
Correlation of Idiopathic Scoliosis Assessments Between Newly Developed
Milwaukee Topographic Scanner and Quantec 255
C.H. Lim, C. Tassone, X.C. Liu, J.G. Thometz and R. Lyon
Trunk Asymmetry in one Thousand School Children Aged 7–10 Years 259
Lukasz Stolinski and Tomasz Kotwicki
How to Measure Kyphosis in Everyday Clinical Practice: A Reliability Study
on Different Methods 264
Fabio Zaina, Sabrina Donzelli, Monia Lusini and Stefano Negrini
Why X-Rays Are Not Reliable to Assess Sagittal Profile: A Cross Sectional
Study 268
Fabio Zaina, Paolo Pizzetti, Sabrina Donzelli, Francesco Negrini and
Stefano Negrini
The Method of Geometrical Comparison of 3-Dimensional Objects Created
from DICOM Images 273
Dominik Gaweł, Kamil Danielewicz and Michał Nowak
Assessment of Trunk Asymmetry in Transversal Plane by Geometric Outline of
Trunk Deformation (GOTD) 277
I.M. Kowalski, T. Giżewski, K. Zaborowska-Sapeta,
H. Protasiewicz-Fałdowska and P. Siwik
Investigation of a Low Cost Method to Quantify Cosmetic Defect 282
Thomas Shannon and Nachiappan Chockalingam
Is Curve Direction Correlated with the Side of Dominant Displacement of
Cerebellar Tonsil and Syrinx Deviation in Thoracic Scoliosis Secondary to
Chiari Malformation Type I and Syringomyelia? 286
Tao Wu, Zezhang Zhu, Xu Sun, Huang Yan, Xin Zheng, Bangping Qian,
Feng Zhu, Winnie Chu, Jack C.Y. Cheng and Yong Qiu
Telediagnostic 3D School Screening of Back Curvatures and Posture Using
Structured Light Method – Pilot Study 291
Wojciech Glinkowski, Jakub Michoński, Bożena Glinkowska,
Agnieszka Żukowska, Robert Sitnik and Andrzej Górecki




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Chapter 6. Treatment
Podium Presentations
Biomechanical Analysis of Proximal Junctional Kyphosis: Preliminary Results 299
M. Cammarata, X. Wang, J.-M. Mac-Thiong and C.E. Aubin
Biomechanical Analysis of Pedicle Screw Density in Spinal Instrumentation for
Scoliosis Treatment: First Results 303
Xiaoyu Wang, Carl-Eric Aubin, A. Noelle Larson, Hubert Labelle
and Stefan Parent
Biomechanical Analysis of Forces Sustained by Iliac Screws in Spinal
Instrumentation for Deformity Treatment: Preliminary Results 307
Frederique D. Perrault, Carl-Eric Aubin, Xiaoyu Wang and
Richard M. Schwend
Operative Treatment of Isthmic Spondylolisthesis with Posterior Stabilization
and ALIF. Cages Versus Autogenous Bone Grafts 311
Rafal Pankowski, Andrzej Smoczynski, Marek Roclawski, Marcin Ceynowa,
Wojciech Kloc, Wojciech Wasilewski, Piotr Jende, Wieslaw Liczbik,
Piotr Beldzinski, Witold Libionka, Olaf Pierzak, Stanislaw Adamski
and Miroslaw Niedbala
Surgical Treatment of Neuromuscular Scoliosis: Current Techniques 315
T. Greggi, F. Lolli, M. Di Silvestre, K. Martikos, F. Vommaro, E. Maredi,
S. Giacomini, A. Baioni and A. Cioni
Intraoperative Neurophysiologic Monitoring (INM) in Scoliosis Surgery 319
Rafal Pankowski, Krzysztof Dziegiel, Marek Roclawski,
Andrzej Smoczynski, Marcin Ceynowa, Wojciech Kloc,
Wojciech Wasilewski, Piotr Jende, Wieslaw Liczbik, Piotr Beldzinski,
Witold Libionka, Olaf Pierzak, Stanislaw Adamski and Miroslaw Niedbala
Toward the Next Generation of Simulator for Intraoperative Navigation of
Scoliotic Spine Surgeries 322
Olivier Cartiaux, Carl-Éric Aubin, Hubert Labelle and Farida Cheriet
Surgical Treatment for Scoliosis Associated with Rare Disease 326
T. Greggi, F. Lolli, E. Maredi, M. Di Silvestre, K. Martikos, F. Vommaro,
S. Giacomini, A. Baioni and A. Cioni
Surgical Treatment of Early Onset Scoliosis in Neurofibromatosis 330
Tiziana Greggi and Konstantinos Martikos
Complications Incidence in the Treatment of Early Onset Scoliosis with
Growing Spinal Implants 334
T. Greggi, F. Lolli, M. Di Silvestre, K. Martikos, F. Vommaro, E. Maredi,
S. Giacomini, A. Baioni and A. Cioni
Smart Brace Versus Standard Rigid Brace for the Treatment of Scoliosis:
A Pilot Study 338
Edmond Lou, Douglas Hill, Jim Raso, Andreas Donauer, Marc Moreau,
James Mahood and Douglas Hedden
Correlation Between in-Brace Radiographic Correction and Short Time Brace
Results 342
Fabio Zaina, Sabrina Donzelli, Monia Lusini and Stefano Negrini


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Brace Wear Characteristics During the First 6 Months for the Treatment of
Scoliosis 346
Edmond Lou, Douglas Hill, Jim Raso, Andreas Donauer, Marc Moreau,
James Mahood and Douglas Hedden
Inclusion Criteria for Physical Therapy Intervention Studies on Scoliosis –
A Review of the Literature 350
H.R. Weiss
Characteristics of Patients with More than 20° of Improvement or Worsening
During Conservative Treatment of Adolescent Idiopathic Scoliosis 354
Stefano Negrini, Sabrina Donzelli, Monia Lusini and Fabio Zaina
Poster Presentations
SpineCor, Exercise and SPoRT Rigid Brace: What Is the Best for Adolescent
Idiopathic Scoliosis? Short Term Results from 2 Retrospective Studies 361
Fabio Zaina, Sabrina Donzelli, Alessandra Negrini, Michele Romano
and Stefano Negrini
Corrective Exercises in Multimodality Therapy of Idiopathic Scoliosis in
Children – Analysis of Six Weeks Efficiency – Pilot Study 365
N. Pugacheva
The Effectiveness of Percutaneous Vertebroplasty in the Treatment of Different
Aetiology Vertebral Body Fractures 372
Wojciech Kloc, Witold Libionka, Olaf Pierzak, Wieslaw Liczbik,
Piotr Beldzinski, Beata Szopa, Marek Roclawski, Rafal Pankowski,
Andrzej Smoczynski and Marcin Ceynowa
The Effect of Pressure Pad Location of Spinal Orthosis on the Treatment of
Adolescent Idiopathic Scoliosis (AIS) 375
M.S. Wong, M. Li, B. Ng, T.P. Lam, M. Ying, A. Wong and J. Cheng
Results of SpineCor Dynamic Bracing for Idiopathic Scoliosis 379
Aleksand Szwed and Maciej Kołban
Brace Treatment in Infantile/Juvenile Patients with Progressive Scoliosis Is
Worthwhile 383
Hans-Rudolf Weiss
Short-Term Effect (ATR, Kasperczyk’s Scale, Chest’s Mobility) of Using of
Physiotherapy Method in the Treatment of AIS – Pilot Study 387
I. Blicharska, A. Brzek and J. Durmala
Bracing Can Reduce High Degree Curves and Improve Aesthetics Immediately
After the End of Growth. Final Results of a Retrospective Case Series 393
Stefano Negrini, Sabrina Donzelli, Monia Lusini and Fabio Zaina
Idiopathic Scoliosis, Growth Zones, Magnetic Therapy 397
A. Arsenev, M. Dudin, V. Lednev, N. Belova, V. Mikhailov and G. Sokolov
Short-Term Effects of Combination of Several Physiotherapy Methods on the
Respiratory Function – A Case Report of Adolescent Idiopathic Scoliosis 402
Bartosz Wnuk, Joanna Frackiewicz, Jacek Durmala, Krzysztof Czernicki
and Karol Wadolowski
Rate of Surgey in a Sample of Patients Fulfilling the SRS Inclusion Criteria
Treated with a Chêneau Brace of Actual Standard 407
Hans-Rudolf Weiss and Mario Werkmann
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A Comparison of the Effects of Deep Tissue Massage and Therapeutic Massage
on Chronic Low Back Pain 411
Mateusz Romanowski, Joanna Romanowska and Marcin Grześkowiak
Chapter 7. Quality of Life
Podium Presentations
Quality of Life and Stress Level in Adolescents with Idiopathic Scoliosis
Subjected to Conservative Treatment 419
Edyta Kinel, Tomasz Kotwicki, Anna Podolska, Marianna Białek and
Wanda Stryła
The Influence of the Degree of Spine Curvature on the Quality of Life
Assessment Among Patients Treated Surgically with the Cotrel-Dubousset
Method 423
Bożena Gorzkowicz and Maciej Kołban
Psychological Aspects of Scoliosis Surgery in Children 428
Ryszard Tomaszewski and Magdalena Janowska
Is the SRS-22 Able to Detect Quality of Life (QoL) Changes During
Conservative Treatments? 433
Stefano Negrini, Sabrina Donzelli, Monica Dulio and Fabio Zaina
Bracing Does Not Change the Sport Habits of Patients 437
Stefano Negrini, Sabrina Donzelli, Francesco Negrini, Michele Romano
and Fabio Zaina
Chapter 8. Abstracts
Genetics and Aetiology
Scoliosis in the 22q11 Deletion Syndrome 445
D. Colo, C. van Weelden, M. Kruyt and R. Castelein
Ethiopathogenesis of the Adolescent Idiopathic Scoliosis Basing on
Neuroimaging and Neurophysiological Examinations with the Special
Emphasizing of Motor Evoked Potentials (MEP) 446
J. Huber and P. Rogala
Abnormally Lower Expression of Melatonin Receptor in Osteoblasts of
Adolescent Idiopathic Scoliosis Girls 447
A.P.Y. Yim, G.Q. Sun, H.Y. Yeung, K.M. Lee, K.W. Ng, T.P. Lam, Y. Qiu
and J.C.Y. Cheng
Association of CHD7 Polymorphism with Susceptibility to and Severity of
Idiopathic Scoliosis in Central European Population: Preliminary Study 448
P. Janusz, T. Kotwicki, M. Kotwicka and M. Andrusiewicz
Identification of Key Disease-Modifying Factors in Adolescent Idiopathic
Scoliosis 449
M. Elbakry, M. Taheri, S. Bouhanik, M. Akoume and A. Moreau
The Identification of Unique Gene Expression Profiles Associated with Cellular
and Biochemical Endophenotypes of Idiopathic Scoliosis 450
K.F. Gorman, C. Julien, Q. Yuan, A. Franco, D.S. Wang, S. Bouhanik,
G. Larouche, G. Lacroix and A. Moreau

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Genetic Association Studies in Idiopathic Scoliosis – Who Was Really Tested? 451
P. Harasymczuk, P. Janusz and T. Kotwicki
Adolescent Idiopathic Scoliosis (AIS): Metabolic Factors, Biomechanical Stress
and Estrogen Impact 452
F. Moldovan, I. Villemure, K. Fendri, G. Grimard, C. Zaouter, S. Parent,
A. Moreau, H. Labelle and S.A. Patten
Exome Sequencing Identifies Novel Candidate Mutations in Idiopathic 453
F. Moldovan, S.A. Patten, K. Fendri, S. Girard, C. Zaouter and P. Edery
A Line of Zebrafish with Increased Incidence of Spinal Deformities 454
H.G. Tomasiewicz, X.C. Liu, C. Tassone, P. North and J. Thometz
Biomechanics, Movement, Posture
High Osteopontin Plasma Level Associated with Abnormal Cortical Bone
Mineral Density in Girls with Adolescent Idiopathic Scoliosis 457
G.Q. Sun, T.P. Lam, N.G. Bobby, P.Y. Yim, K.M. Lee, A. Moreau, Y. Qiu
and J.C.Y. Cheng
Abnormal Bone Matrix Mineralization in Patients with Adolescent Idiopathic
Scoliosis 458
G.Q. Sun, T.P. Lam, N.G. Bobby, P.Y. Yim, K.M. Lee, Y. Qiu and
J.C.Y. Cheng
Porcine Spine Finite Element Model as an Alternative Tool for the Validation
of a Novel Fusionless Device 459
B. Hachem, C.E. Aubin, M. Driscoll and S. Parent
Static and Dynamic Postural Control in Girls with Idiopathic Scoliosis 460
Marzena Wiernicka, Dawid Łochyński, Tomasz Kotwicki,
Łukasz Michałowski, Ewa Kamińska, Jacek Lewandowski and
Elżbieta Hurnik
Vertebral Long Fusion is a Tubular Bone. A 3-D Analysis of Bone Fusion
Remodelling in Scoliosis Surgery Without, with and After Removal
the Instrumentation 461
A. Valassina, A.G. Aulisa and L. Aulisa
The Pelvic Lordosis: An Essential Adaptation in Human Evolution, and
a Determinant of Spinopelvic Alignment 462
M.M.A. Janssen, T. Vrtovec, F. Pernuš, F.C. Öner, E.E. Vereecke,
A.J. Channon, M.A. Viergever, K.L. Vincken and R.M. Castelein
Sagittal Spinal Profile and Spinopelvic Balance in Parents of Scoliotic Children,
as Compared to Normal Controls 463
M.M.A. Janssen, K.L. Vincken, S.M. van Raak, T. Vrtovec, B. Kemp,
M.A. Viergever, L.W. Bartels and R.M. Castelein
Influence on Mastication of Adolescent Idiopatic Scoliosis: Prevalence of
Reverse Chewing Cycles with and Without Brace 464
M.G. Piancino, T. Vallelonga, G. Frongia, M. Brayda, D. Braddebin,
C. Debernardi and P. Bracco
Analysis of Changes in Selected Body Characteristics in Many Years of
Observation of Children and Adolescents with Faulty Body Posture 465
M. Kluszczynski and J. Czernicki

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Growth and Metabolism
Influence of Dynamic Loading Parameters on Bone Growth Modulation 469
A.-L. Menard, B. Valteau, I. Londono, F. Moldovan, G. Grimard and
I. Villemure
Association of Volumetric Bone Mineral Density with Leptin and Soluble
Leptin Receptor in Girls with Adolescent Idiopathic Scoliosis 470
E.M.S. Tam, F.W.P. Yu, Z. Liu, T.P. Lam, B.K.W. Ng, K.M. Lee, Q. Yong
and J.C.Y. Cheng
Bone Quality in Adolescent Idiopathic Scoliosis Girls – A Case-Control Study 471
W.S. Yu, K.Y. Chan, F.W.P. Yu, H.Y. Yeung, K.M. Lee, K.W. Ng, Y. Qiu,
T.P. Lam and J.C.Y. Cheng
Elevated Soluble Leptin Receptor Level and Association with Body
Composition in Girls with Adolescent Idiopathic Scoliosis 472
E.M.S. Tam, H.Y. Yeung, Z. Liu, T.P. Lam, T. Ting, J.Y.F. Lok, B.K.W. Ng,
K.M. Lee, Q. Yong and J.C.Y. Cheng
Is AIS Under 20–30° a Chaotic Dynamical System? 473
J.C. de Mauroy and J.M. Ginoux
Abnormal Skeletal Growth Patterns in Adolescent Idiopathic Scoliosis –
A Longitudinal Study Till Skeletal Maturity 474
A.P.Y. Yim, H.Y. Yeung, W.J. Wang, V.W.Y. Hung, L. Qin, K.M. Lee,
T.P. Lam, Y. Qiu and J.C.Y. Cheng
Lower Handgrip Strength in Girls with Adolescent Idiopathic Scoliosis (AIS) –
A Case-Control Study 475
W.S. Yu, K.Y. Chan, F.W.P. Yu, H.Y. Yeung, K.M. Lee, K.W. Ng, Y. Qiu,
T.P. Lam and J.C.Y. Cheng
Nutritional Status of Children and Adolescents with Idiopathic Scoliosis –
Preliminary Results 476
E. Matusik, J. Durmala, P. Matusik and E. Malecka-Tendera
Imaging and Measurement
Neuronavigation-Guided Spine Surgery 479
P. Bełdziński, W. Kloc, O. Pierzak, W. Libionka, W. Liczbik and B. Szopa
Computational Morphometry of Semicircular Canals in Adolescent Idiopathic
Scoliosis (AIS) Based on High-Resolution MR Images 480
W.C.W. Chu, L. Shi, D. Wang and J.C.Y. Cheng
Statistical Difference in Cerebral Cortical Thickness in Patients with Adolescent
Idiopathic Scoliosis and Normal Controls Using Computational Techniques –
The Updated Data 481
W.C.W. Chu, L. Shi, D. Wang and J.C.Y. Cheng
Systematic Analysis of the Neurocentral Junction (NCJ) in the Normal Infantile,
Juvenile and Adolescent Spine 482
T. Schlösser, M. Janssen, H. Attrach, M. Viergever, K. Vincken and
R. Castelein
Are Bending Films Useful for Lenke Classification of Idiopathic Scoliosis? 483
M. Tyrakowski, T. Kotwicki, A. Koch, M. Drwięga, K. Lisiecka,
D. Ławniczak, S. Pietrzak and J. Czubak
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Sensitivity-to-Change of Full Torso Surface Topography Measurements in
Adolescents with Idiopathic Scoliosis and a Main Thoracic Curve 484
E.C. Parent, P.Q. Zhang, D. Hill, M. Moreau, D. Hedden and E. Lou
A New 3D Topographic Device for Monitoring Scoliosis Progression in
Children 485
X.C. Liu, J.C. Tassone, J.G. Thometz, R.M. Lyon, S. Tarima, C.H. Lim and
L.C. Paulsen
Treatment
Late Results of Surgical Treated Scoliosis with the Use of Selective and Extend
Instrumentation 489
S. Snela, G. Sokół and P. Jasiński
Radiological Correction of Severe Thoracic Idiopathic Scoliosis: Comparison of
Three Surgical Approaches Applied to Similar Curves 490
A. Koch, P. Janusz, P. Harasymczuk, D. Ławniczak and T. Kotwicki
A Survey Data Base Use by SRS Members 491
V.J. Raso, J. Sanders, D. Kumar, R. Knight, D. Burton, R. Bowen and
K. Spratt
The Influence of the Development of Scoliosis Posterior Surgery on 3-Planar
Deformity Correction 492
R. Pankowski, M. Rocławski, A. Smoczyński, M. Ceynowa, W. Kloc,
W. Wasilewski, P. Jende, W. Liczbik, P. Bełdziński, W. Libionka,
O. Pierzak, S. Adamski and M. Niedbała
An Alternative to a Randomized Control Design for Assessing the Efficacy and
Effectiveness of Bracing in AIS 493
D. Fong, K. Cheung, Y. Wong, W. Cheung, I. Fu, E. Kuong, K. Mak, M. To
and K. Luk
Standard Technique vs. CAD/CAM and Biomechanical Simulations for the
Design of Braces in Adolescent Idiopathic Scoliosis: First Results 494
F. Desbiens-Blais, J. Clin, S. Parent, H. Labelle and C.E. Aubin
Congenital Scoliosis, Riyadh Experience 495
M. Alfawareh, W. Attia, K. Almusrea and M. Halwani
Osteopathic Method for Treatment of Discal Hernias 496
Olga Kalinovskaya
Treatment’s Results of the Idiopathic Scoliosis, Depending on the Used
Instrumentation 497
S. Snela, P. Jasiński and G. Sokół
Osteoid Osteoma of the Lumbar Spine: Resection Without Fusion. Case Report
of Two Children 498
A. Koch, T. Kotwicki and A. Szulc
Comparison of Influence of Three Physiotherapeutic Techniques: Postisometric
Relaxation, Kinetic Control and Stabilization Exercises on the Flexibility of
Pelvic Girdle Muscles: Prospective, Randomized, Single Blinded Study 499
D. Czaprowski, J. Leszczewska, A. Kolwicz, P. Pawłowska,
A. Afeltowicz-Mich, D. Sitarski, A. Gębicka and T. Kotwicki


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Validation of the Transpedicular Screws Implantation in Patients with Idiopathic
Scoliosis Using Computed Tomography 500
S. Kasongo, W. Jurasz and Ł. Bartochowski
The Effectiveness of Surgical Treatment of Spondylolisthesis with
Transpedicular Instrumentation 501
Ł. Bartochowski, W. Jurasz and A. Nowakowski
Quality of Life
Polish Validation of Brace Questionnaire (BrQ) 505
E. Kinel, T. Kotwicki, A. Podolska, M. Białek and W. Stryła
Subject Index 507
Author Index 511
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Morey Sargent Moreland, M.D.
1939–2011
Morey Moreland was born in March 1939 and died on the 2nd October 2011 in Pitts-
burgh. Without him, there would probably be no IRSSD. In 1980, he hosted the first
International Symposium on Surface Topography and Spinal Deformity. While the
focus of this meeting was narrow, he nurtured a steady evolution of further biennial
meetings – to include surface topography, and then to incorporate all three dimensional
aspects of spinal and trunk deformity. Then the need to take the broadest possible view
of the causes and treatment of spinal deformity became evident. The 3D meetings in
1992 in Montreal and 1994 in Pescara led to the formation of the International Re-
search Society of Spinal Deformity, and Morey served as our President at the first
meeting in 1996 in Stockholm. He purchased a gavel for future Presidents to use at our
meetings.
Morey never limited the scope of his work and his enthusiasm. As a paediatric or-
thopaedic surgeon he concentrated his clinical and surgical expertise on spinal deformi-
ty and hip dysplasia. After completing his Surgical Residency at the University of
Vermont, he researched mechanically modulated growth at the Nuffield Orthopaedic
Centre in Oxford, focusing on the effects of torsion on the growth plate, and the impli-
cations for development and treatment of these deformities. However, his work was
primarily about the people – the children and their parents, the researchers in the lab,
and his colleagues in the clinic and the operating room. His special gift and contribu-
tion was to bring people together to collaborate effectively, and to develop the careers
of young researchers and surgical residents. He did this selflessly. Morey and his wife
Marilyn have travelled several times to San Pedro Sula, Honduras to provide pediatric
orthopedic care and to help train local physicians as part of the medical mission by
CURE International (Honduras).

Morey and Marilyn sailing their boat on Lake Champlain, 1979.


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Personally, I knew Morey as a colleague, friend and mentor. We worked together
from 1980 to 1989, and he and Marilyn would invite me to their house, and to share
their passion for skiing at Mad River Glen, and sailing their boat on Lake Champlain.
We always made a point to spend time together at IRSSD meetings. Morey’s legacy
lives on, but we shall miss him.
Ian Stokes
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Chapter 1
Invited Lectures
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Whither the etiopathogenesis
(and scoliogeny) of adolescent
idiopathic scoliosis?
R Geoffrey BURWELL,
a
* and Peter H DANGERFIELD,
b,c,d
a
Centre for Spinal Studies and Surgery, Nottingham University Hospitals Trust,
Queen’s Medical Centre Campus, Derby Road, Nottingham, NG7 2UH, UK.
b
University of Liverpool, Ashton Street, L69 3GE, UK,
c
Staffordshire University, Leek
Road, Stoke-on-Trent, ST4 2DF. UK,
d
Royal Liverpool Children’s Hospital, Eaton
Road, Liverpool, L12 2AP, UK.
Abstract
Although considerable progress had been made in the past two decades in
understanding the etiopathogenesis of adolescent idiopathic scoliosis (AIS), it still
lacks an agreed theory of etiopathogenesis. One problem may be that AIS results
not from one cause, but several that interact with various genetic predisposing
factors. There is a view there are two other pathogenic processes for idiopathic
scoliosis namely, initiating (or inducing), and those that cause curve progression.
Twin studies and observations of family aggregation have revealed significant
genetic contributions to idiopathic scoliosis, that place AIS among other common
disease or complex traits with a high heritability interpreted by the genetic variant
hypothesis of disease. We summarize etiopathogenetic knowledge of AIS as
theories of pathogenesis including recent multiple concepts, and blood tests for
AIS based on predictive biomarkers and genetic variants that signify disease risk.
There is increasing evidence for the possibility of an underlying neurological
disorder for AIS, research which holds promise. Like brain research, most AIS
workers focus on their own corner and there is a need for greater integration of
research effort. Epigenetics, a relatively recent field, evaluates factors concerned
with gene expression in relation to environment, disease, normal development and
aging, with a complex regulation across the genome during the first decade of life.
Research on the role of environmental factors, epigenetics and chronic non-
communicable diseases (NCDs) including adiposity, after a slow start, has
exploded in the last decade. Not so for AIS research and the environment where,
except for monozygotic twin studies, there are only sporadic reports to suggest that
environmental factors are at work in etiology. Here, we examine epigenetic
concepts as they may relate to human development, normal life history phases and
AIS pathogenesis. Although AIS is not regarded as an NCD, like them, it is
associated with whole organism metabolic phenomena, including lower body mass
index, lower circulating leptin levels and other systemic disorders. Some
epigenetic research applied to Silver-Russell syndrome and adiposity is examined,
from which suggestions are made for consideration of AIS epigenetic research,
cross-sectional and longitudinal. The word scoliogeny is suggested to include
etiology, pathogenesis and pathomechanism.
Keywords: Scoliosis, etiology, pathogenesis, scoliogeny, epigenetics
1
Corresponding Author: [email protected]
Research into Spinal Deformities 8
T. Kotwicki and T.B. Grivas (Eds.)
IOS Press, 2012
© 2012 The authors and IOS Press
. All rights reserved.
doi:10.3233/978-1-61499-067-3-3
3
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1. Terminology
The word etiology strictly means the factor(s) causing the AIS, pathogenesis mode of
origin of the morbid process, and pathomechanism sequence of events in the evolution
of its structural and functional changes that result from the pathological process [1].
The word
etiopathogenesis is used to embrace etiology and pathogenesis. We suggest
the word scoliogeny as the collective noun to include etiology, pathogenesis and
pathomechanism.
2. Introduction
Research into the causation of adolescent idiopathic scoliosis (AIS) draws heavily from
mechanical and biological disciples, but still lacks an agreed theory of etiopathogenesis
[2-7]. Genetic factors are believed to play an important role in the etiology of AIS with
considerable heterogeneity [4,5]. The research problem is complicated by the suspicion
that AIS may result not from one cause, but several that interact [2,8]. Genetic, and
now genomic, research on AIS have not yet provided the therapeutically-required
etiologic understanding. In other diseases and particularly diseases of developmental
origin [9] and late-onset chronic non-communicable diseases (NCDs) [10], research on
the role of environmental factors and epigenetics after a slow start has exploded in the
last decade [10-15]. Not so for AIS research and the environment where, except for
monozygotic twin studies, there are only sporadic reports suggesting that
environmental factors are at work in etiology [16]. Epigenetics, a relatively recent field
evaluates factors concerned with gene expression in relation to environment, disease,
normal development and aging, with a complex regulation across the genome during
the first decade of life. Elsewhere we commented on etiopathogenetic concepts as they
may relate to normal spine development and AIS pathogenesis [16]. Here we consider:
(1) some theories of pathogenesis including recent multiple concepts;
(2) blood tests for AIS based on predictive biomarkers and genetic variants that
signify disease risk;
(3) epigenetic concepts as they may relate to human development and life history
phases;
(4) AIS linked to the aging process as a non-communicable disease (NCD);
(5) findings from epigenetic methods applied to the Silver-Russell syndrome and
adiposity; and
(6) suggestions for applying epigenetic methods to AIS etiopathogenesis in cross-
sectional and longitudinal studies.
3. Etiopathogenesis 2011
In a review of Top Theories of AIS Wang et al [4] concluded:
• considerable progress had been made in the past two decades in understanding
the etiopathogenesis of AIS;
• current knowledge is still fragmented;
• we are still far from understanding fully the different etiopathogenetic
pathways and mechanisms for example –
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• the general skeletal and relative anterior spinal overgrowth (RASO) of AIS
girls have not been related securely to endocrinology, and
• the abnormal extra spinal skeletal length asymmetries of AIS girls are of
unknown pathogenetic significance;
• current treatment at best is treating the morphologic and functional sequelae of
AIS and not the cause of the disease;
• whatever hypothesis or theory, several fundamental questions and facts of AIS
need to be properly addressed and explained with 11 items listed.
4. Pathogenesis – theories, hypotheses and concepts 2012
Several theories, hypotheses and concepts for AIS pathogenesis more recently
implicating AIS as a systemic and/or multifactorial disorder, are providing hypotheses
to test. [2-4, 6]. These include:
• Relative anterior spinal overgrowth (RASO) [17].
• Asynchronous spinal neuro-osseous growth [18, 19].
• Thoracospinal concept [20].
• Dorsal shear forces and axial rotation instability [21].
• Flexural-torsional buckling from flexibility anisotropy [22].
• Biomechanical spinal growth modulation [23].
• Biomechanical theory [24].
• Intervertebral disc disorder [25-31].
• Deforming three joint complex hypothesis [32].
• Motor control disorder [33-35].
• Sensorimotor integration disorder & dystonia [35,36].
• Sensory integration disorder [37].
• Vestibular disorder [38].
• Body spatial orientation disorder [39].
• Neurodevelopmental disorder [40].
• Systemic and metabolic disorders involving –
oPlatelet calmodulin [41,42].
oMelatonin [43-46].
oMelatonin-signalling defect (MSD) [47,48].
oOsteopontin (OPN) and soluble CD44 (sCD44). Azeddine et al [49]
reported mean plasma OPN levels to be increased in:
• patients with idiopathic scoliosis, correlating significantly with
curve severity, and
• “an asymptomatic at-risk group” (offspring born from at least
one scoliotic parent); this finding, if confirmed, suggests
predictive biomarkers and possibly a prodromal stage with the
prospect of intervention early in deformity evolution.
In contrast, mean plasma levels of sCD44 were significantly lower in
patients with Cobb angles of 45 degrees or more. Drawing on evidence
from mouse models, it was concluded that OPN is essential to induce
scoliosis formation and curve progression through interactions with CD44
receptors,
“thus offering a first molecular concept to explain the
pathomechanism leading to the asymmetrical growth of the spine in
idiopathic scoliosis.” [49].
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Moreau and colleagues report that blood tests could be useful markers for
the diagnosis of idiopathic scoliosis and the prognosis of curve
progression: a functional scoliosis test [47-50], further refined recently
using a more accurate technology called cellular dielectric spectroscopy
[51]; and a biochemical scoliosis test
using raised plasma OPN and lower
sCD44 values. Moreau [52] states that OPN and sCD44 are not disease-
specific but when observations of both are combined they become highly
specific for idiopathic scoliosis. By binding free OPN, sCD44 can
prevent OPN from triggering scoliosis or curve progression. Moreau
considers that environmental factors could potentially affect the
circulating levels of OPN in humans. With colleagues he is conducting
tests to identify potentially useful therapeutic agents [52].
oEstrogens [53,54].
oLeptin [6,54-56] (see 9.1.3)
oOsteopenia [57-59].
• Developmental instability & symmetry control dysfunction [60,61].
• Intrinsic growth plate asymmetry hypothesis [62,63].
• Multiple pathogenetic processes.
oDouble neuro-osseous theory [6,64].
oThree metabolic processes [65].
oFour pathomechanisms [66].
5. Some comments on theories, hypotheses and concepts 2012
5.1. Attempts at integration of theories
None of the above theories entirely explain the pathogenesis of AIS [2-4,66]. In recent
surveys of these theories for AIS [2-4,6,65,66] integration is attempted involving
interacting pathomechanisms. In addition to predisposing factors [2,5], there is a view
that there are two other pathogenic processes for idiopathic scoliosis namely, initiating
(or inducing), and those that cause curve progression [2,67]. There is evidence that
curve progression, which increases during the curve acceleration phase [68], increases
through disc wedging during the rapid growth spurt with progressive vertebral wedging
occurring later [29,30]; and that in scoliosis the simultaneous occurrence of vertebral
displacement in 3-D, rather than a specific single disturbance of any one of the three
planes, triggers development of the deformity [69].
5.2. RASO phenomenon and asynchronous spinal neuro-osseous growth concept
Relative anterior spinal overgrowth (RASO) is established as a sagittal plane
phenomenon associated with scoliosis. Its etiologic mechanisms are unknown but curve
progression is thought to involve the Hueter-Volkmann effect [23]. Some workers
adduce evidence that RASO results from a spinal neuro-osseous growth disorder –
variously termed
vertebra-neural (Roth, van Loon), uncoupled (Porter), and
asynchronous [18] – of unknown etiology. Without riders these sagittal plane theories,
like other biomechanical theories, do not accommodate –
• normal trunk bilateral asymmetry [64,70,71],
• normal thoracic spinal axial vertebral rotation [72],
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• bilateral skeletal asymmetries of AIS [6,73],
• some bilateral neural [34,35] and vestibular [38] asymmetries, and
• the systemic and metabolic disorders associated with AIS.
The riders are secondary effects (epiphenomena) [2] and/or multiple factors possibly of
different weight in individuals with similar deformities.
5.3. Sensory and sensorimotor integration disorder and dystonia
There is increasing evidence for the possibility of an underlying neurological disorder
for AIS; this research needs to ne extended. The findings relate to sensory integration
[37], sensorimotor integration and dystonia [34-36], spinal cord [18,19], brain stem [8],
motor control [33], motor cortex [34],
supplementary motor area [35], brain white
matter [74] as part of the Human Connectome [75], cerebral cortex as a whole [76] and
vestibular system [38]. The two papers by Domenech et al focus attention on bilateral
asymmetric activity respectively of motor cortex [34] and supplementary motor area
[SMA, 35] as pathomechanisms for idiopathic scoliosis (IS). Reviewing the SMA paper
[35] Benoist [77] writes:
“These findings support the hypothesis that a sensorimotor integration underlies the
pathogenesis of IS. In addition, as suggested by the authors, these abnormal MRI
findings may represent a biomar
ker of IS disease and open the way to novel therapeutic
targets. This paper, winner of the EuroSpine Full Paper Award for 2010, has
limitations, which have been summarized in a Reviewer’s Comment by Freeman [78].”
5.4. Lessons from brain research – brain in a box
The wide gap between the above theories reveals current ignorance about the causation
of AIS indicating more integrated knowledge is needed. Like brain research [79,80],
most AIS workers are focused on their own corner. In brain research, which generates
about 60,000 papers per year, one team in Europe is planning to integrate these
discoveries and achieve a comprehensive understanding of the brain; this would be
done by building unifying computer models (‘brain in a box’) for which one billion
euros is being sought in 2012 from the European Union’s new decade-long Flagship
initiatives [79].
5.5. Grand unifying theory for AIS scoliogeny – brain and scoliosis in a box?
In AIS, the possible creation of a network approach to the pathogenesis by constructing
AIS diseasomes [81] was suggested [16]. The attainment of a grand unifying theory for
adolescent idiopathic scoliogeny seems unlikely at present. Finite element analysis of
spinal models of AIS pathogenesis (‘scoliosis in a box’) has provided for the testing of
specific questions, recently in connection with how accelerated growth profiles may
increase scoliosis progression and pose a progressive risk factor [82]. Building
a unifying computer model for AIS pathogenesis might be considered using
supercomputers with ever increasing memory. (‘brain and scoliosis in a box’)
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5.6. The biological Higgs – aging, AIS and scoliogeny
Biologists have recently pondered what fundamental discoveries might match the
excitement of the Higgs boson, the so-called ‘God Particle’ [83]. Some scientists
consider that the ability to slow aging would address Higgs-like fundamental questions
about human life. AIS, we suggest, may be viewed as a disorder of aging processes
during development. So questions about what pathways control aging – highly unlikely
to result from a unitary cause [83,84] – and some age-related diseases, may be relevant
to the scoliogeny of AIS (see 9.1.1).
5.7. Genetics, epigenetics and AIS scoliogeny
After mentioning genetics we focus on epigenetics and how it might relate to AIS
scoliogeny.
6. Genetics and the genetic variant hypothesis for complex disease 2012
6.1. Etiology of AIS is poorly understood
Twin studies and observations of family aggregation have revealed significant genetic
contributions to idiopathic scoliosis that position AIS among other common disease or
complex traits with high heritability [85]. However, despite many investigations, the
underlying etiology of idiopathic scoliosis is poorly understood [5,86].
6.2. Position for AIS
Based on gene-linkage studies, candidate gene approach and genome-wide association
studies, Wang et al [4] summarized the then position as follows:
“Recent evidence not only suggests that genetic factors play an important role in the
etiology of AIS but also revealed the considerable heterogeneity. Although the
continuation of the search for the exact
genetic factors is inevitable, it is also very
important to look at the functional and biological aspects to allow better understanding
of the etiopathogenesis if AIS.”
6.3. AIS Prognostic Test (Scoliscore)
Genetic profiling using 53 single nucleotide polymorphisms (SNPs), gene-to-gene
interactions, and the patient’s initial Cobb angle measurement, produced a score
ranging from 1-200 predicting scoliosis curve progression [87-90] and provided
a scientific basis for the
AIS Prognostic Test (Scoliscore, Axial Biotech, Inc.).
6.4. Genetic variant hypothesis and non-genetic factors for complex disease
Butcher and Beck [91] write:
“A spate of high-powered genome-wide association studies (GWAS) have recently
identified numerous single-nucleotide polymorphisms (SNPs) robustly linked with
complex disease. Despite interrogating the
majority of common human variation, these
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SNPs only account for a small proportion of the phenotypic variance, which suggests
genetic factors are acting in concert with non-genetic factors. Although environmental
measures are logical covariants for genotype-phenotype investigations, another non-
genetic intermediary exists, epigenetics.”
7. Epigenetics – perspective for AIS
7.1. Definition and epigenetic modifications
Epigenetics is generally defined as information heritable during cell division but not
contained within the DNA sequence itself, termed epigenetic modifications [16,92,93].
There are the three major ways organisms alter their DNAs inherited messages [93,94]:
• enzymes methylate DNA to modulate transcription;
• histone modifications and nucleosome positioning to induce or repress target
sequences; and
• non-coding small RNAs (including microRNAs and short interfering RNAs)
which attach themselves to messenger RNA to modify the expression of
specific genes.
7.2. DNA methylation
According to Talens et al [95]:
“DNA methylation may be the most suitable epigenetic mark for large-scale
epidemiological studies, since methyl groups are covalently bound to CpG
dinucleotides and are not lost during routine DNA extraction, unlike histone
modifications. This opens the possibility of exploiting existing DNA biobanks for
research purposes, to discover epigenetic risk factors for complex disease.”
7.3. Epigenetics at the epicentre of modern medicine
Epigenetics evaluates factors concerned with gene expression in relation to
environment, disease, normal development and aging, with a complex regulation across
the genome during the first decade of life [14-16].
Feinberg [11] writes:
“Epigenetics, the study of non-DNA sequence-related heredity, is at the epicenter of
modern medicine because it can help to explain the relationship between an
individual's genetic background, the environment, aging, and disease…”
7.4. Environmental factors and AIS
In the last 20 years, sporadic reports have suggested environmental factors are involved
in the etiopathogenesis and phenotypic expression in some subjects with AIS. We
review the evidence elsewhere [16].
7.5. Hypothesis of developmental instability for scoliosis
Speculation that genetic and environmental factors are involved the etiopathogenesis of
idiopathic scoliosis [96,97] was further developed by Goldberg and colleagues [60,61]
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who suggested that scoliosis is caused by environmental stress causing developmental
instability:
7.6. Heated indoor swimming pools, infants and AIS as a delayed epigenetic effect
McMaster et al [98,99] reported a statistically significant correlation between the
introduction of infants to heated indoor swimming pools and the development of AIS.
A neurogenic hypothesis was formulated to explain how neurotoxins produced by
chlorine may act on the infant’s immature central nervous system with an implication
of the brain's barrier and cerebral spinal fluid being involved. The delayed epigenetic
effects with the bony trunk deformity of AIS do not become evident until adolescence
[100]. There may be many such environmental factors acting in the first year of life to
initiate AIS and differing around the world (see Figure 1). Whatever the effects the
neurotoxic products may have on the immature brain, the process of puberty with its
increased growth velocity is suggested to play a role in the delayed phenotypic
expression of AIS [68,101].
Elsewhere we outlined and commented on etiopathogenetic concepts as they may relate
to normal spine development and AIS pathogenesis [16].
8. Human development – normal life history phases and epigenetics
8.1. Developmental plasticity and epigenetic programming
Reviewing child health, developmental plasticity and epigenetic programming,
Hochberg et al [9] describe how transition between life-history phases defines pre-adult
phases of predictive adaptive responses (plasticity) (Figure 1).
• Infancy-to-childhood (I/C) transition is associated with GH-IGFI axis
activation that affects adult height;
• Childhood-to-juvenility (C/J) transition affects body composition, adiposity
rebound and circulating leptin levels.
8.2. Life history phases, epigenetics and AIS
We applied this concept speculatively to AIS pathogenesis in susceptible girls [102].
9. Environment, non-communicable diseases
9.1. Idiopathic chronic non-communicable diseases (NCDs)
9.1.1. Types of NCDs, risk factors and prevention
Common chronic NCDs include obesity, diabetes, cardiovascular disease, respiratory
disease, cancer and schizophrenia attributed to genetic and environmental factors [10]
with a pathogenetic link being defined between obesity and cancer [103]. Risk factors
for NCDs include age, unhealthy diet, smoking, alcohol abuse, chemicals, and lack of
physical activity [83,100,104,105], the last possibly by impairing each of autophagy,
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Figure 1. Pre-adult periods of adaptive plasticity in the transition between life history phases (from Burwell
et al [102] after modification from Hochberg et al [9].
mitochondrial upgrade and energy production [106]. In studying the prevention of
NCDs, there is a move away from adults to mother, father, pregnancy and child [107],
with recent research revealing a link between gene promoter methylation in umbilical
cord tissue and later adiposity (Figure 2) [108].
9.1.2. AIS as an NCD?
Although AIS is not regarded as an NCD, like them and particularly like obesity, it is
often associated with whole organism but opposite metabolic phenomena, namely
lower body mass index (BMI) [109,110], lower circulating leptin levels [54-56], and
other systemic disorders [41-52].

9.1.3. AIS, obesity, hypothalamic sensitivity and resistance to leptin
In the leptin-hypothalamic-sympathetic nervous system (LHS) concept for AIS
pathogenesis of girls, scoliosis initiation is attributed to increased hypothalamic
sensitivity to leptin [for central leptin activity see 111-113] with sympathoactivation
becoming asymmetric as an adverse hormetic effect [6,114,115]; this places AIS with
lower BMI at the opposite end of the spectrum from the central leptin resistance
concept for obesity [6]. The putative asymmetric sympathoactivation is viewed as
initiating the costo-vertebral asymmetry of right thoracic AIS in girls [6,20] that
produces the major phenotypic abnormality namely the scoliosis of a whole organism
(systemic) disorder.
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Figure 2. Diagram summarizing the study of Godfrey KM et al [108] who state that genome-wide
association studies showed fixed genetic variation makes little contribution to the risk of obesity, heart
disease, or diabetes. Godfrey et al conclude that their findings “…raise the possibility that the developmental
environment component may be equally or more important. We suggest that after consideration of the
selection of appropriate candidate genes, umbilical cords may be stored and the children later evaluated for
trunk deformity of both AIS and normal trunk asymmetry [64, 70, 71]
10. Methodological application of epigenetics to epidemiological studies
Some methods in DNA methylation profiling are reported by Zuo et al [116]. The
application of epigenetic methods to epidemiological studies is discussed here in
relation to some other diseases, and then how these methods might be applied to AIS.
11. Applying epigenetic methods to some other diseases
Mazzio and Solkiman [15] write:
“One of the greatest challenges in the study of epigenetics as it relates to disease is the
enormous diversity of proteins, histone modifications and DNA methylation patterns
associated with each unique maladaptive phenotype. This is further complicated by a
limitless combination of environmental cues that could alter the epigenome of specific
cell types, tissues, organs and systems.”
We examine here what has been found epigenetically for -
• Silver-Russell syndrome,
• Idiopathic chronic non-communicable diseases (NCDs) some of which afflict
children,
11.1. Silver-Russell syndrome (SRS)
Silver-Russell syndrome is a clinically and genetically heterogeneous congenital
disorder characterized by severe growth retardation [117] with bilateral skeletal
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asymmetry and genital anomalies. Bruce et al [118] found a dose-response relationship
between the degree of H19 hypomethylation and phenotype severity in SRS. The
association between severe H19 hypomethylation and specific anomalies of the spine,
elbows, hands and feet, and genital defects, was shown for the first time.
11.2. Idiopathic non-communicable diseases (NCDs)
11.2.1. Fixed genomic variation
According to Godfrey et al [108]:
“Fixed genomic variation explains only a small proportion of the risk of adiposity in
animal models; maternal diet alters offspring body composition, accompanied by
epigenetic changes in metabolic control genes. Little was known about whether such
processes operate in humans.”
11.2.2. Gene promoter methylation in umbilical cord tissue at birth and later adiposity
(Figure 2).
Using Sequenom MassARRAY, Godfrey et al [108] measured the methylation status of
68 CpGs 5’ from five candidate genes in umbilical cord tissue DNA of healthy
neonates. Methylation varied greatly at particular CpGs. They related methylation
status to maternal pregnancy diet and to child’s adiposity at age 9 years (by dual energy
X-ray absorptiometry) with replication established in a second independent cohort.
They concluded that: (1) a substantial component of metabolic disease risk has
a prenatal developmental basis, and (2) perinatal epigenetic analysis may have utility in
identifying individual vulnerability to later obesity and metabolic disease.
12. Suggestions for applying epigenetic methods to AIS etiopathogenesis
12.1. Methylation of candidate genes – cross-sectional study
As for Silver-Russell syndrome [117,118], we suggest that the possible selection of
appropriate candidate genes and evaluating their methylation status in relation to
physical and other characteristics of AIS subjects be considered. Familial and sporadic
AIS might be evaluated.
12.2. Methylation of DNA in umbilical cord tissue at birth and later AIS – longitudinal
study
As for adiposity (Figure 2) [108], the stored umbilical cords of children who later show
trunk distortion [64,70,71] and AIS deformity may be evaluated for their DNA
methylation status, similar to 11.2.2, and with familial and sporadic AIS again
evaluated.
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12.3. Methylation in buccal smear DNA and later AIS – longitudinal study
An evaluation similar to 12.2 might be considered using buccal smear DNA starting,
say at 5 years, with familial and sporadic AIS being evaluated.
13. The future – memorandum of understanding for AIS scoliogenic research?
We suggest consideration be given to forming a group to work out details of a possible
international collaborative effort for epigenetic scoliogenic research on AIS, perhaps
initially preparing a Memorandum of Understanding.
Acknowledgements
The research in Nottingham quoted in this paper has been undertaken in collaboration with:
Susan I Anderson, School of Graduate Entry Medicine and Health, University of Nottingham Medical
School, Derby, UK.
Ranjit K Aujla
a
,Ashley A Cole
a
, Michael P Grevitt
a
.
Theodoros B Grivas, Department of Trauma and Orthopedics, "Tzanio" General Hospital, Tzani and
Afendouli 1 st, Piraeus 18536, Greece.
Alanah S Kirby
a
.
Alan Moulton, Department of Orthopaedic Surgery, King’s Mill Hospital, Sutton Road, Mansfield NG17
4JL, UK.
Frances J Polak
a



Tabitha L Randell, Department of Child Health, Nottingham University Hospitals Trust, Queen’s Medical
Centre Campus, Nottingham,UK.
John K Webb
a
.
We thank Mr Lyndon Cochrane for the art work.
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Pre-existent Rotation of the Normal Spine
at Different Ages and Its Consequences
for the Scoliotic Mechanism
René M. CASTELEIN
a, 1
a
Department of Orthopaedics, University Medical Center Utrecht, The Netherlands
Abstract. The normal spine is not a symmetrical structure, as has been appreciated
for a long time. The reason why the main thoracic vertebrae in idiopathic scoliosis
predominantly rotate to the left at the infantile age and to the right at the
adolescent age is unclear. We provided an in-depth analysis of the rotational
patterns of the normal growing and adult spine in relation to closure of the
neurocentral junctions, organ anatomy and the convexity of the curve in idiopathic
scoliosis. We believe that the delicate upright human spino-pelvic balance can be
disturbed by a multitude of offenses and consequently an idiopathic scoliosis will
develop due to the rotational instability. From our results it can be concluded that
once this rotation starts to occur, it will naturally follow the pre-existent rotational
patterns that we have demonstrated to exist already in the normal spine.
Keywords. Pre-existent vertebral rotation, idiopathic scoliosis, closure of the
neurocentral junctions, organ anatomy
Introduction
Idiopathic scoliosis is a classic and intriguing orthopedic problem in which the spine
collapses into a three-dimensional deformity without any known cause. Idiopathic
scoliosis develops during the infantile, or in its most common form, during the
adolescent growth spurt. Prior to the growth spurts and after cessation of growth no
manifest persistent disorder, other than the spinal deformity, can consistently be
identified in these patients [1]. Therefore the etiology is termed multi-factorial. An
important component of the three-dimensional deformity is axial vertebral rotation. The
reason why the main thoracic vertebrae in idiopathic scoliosis predominantly rotate to
the left at the infantile age and to the right at the adolescent age is unclear. A number of
factors have been held responsible for the predominance of right sided thoracic curves
in idiopathic scoliosis, in particular asymmetry of the thoracic and abdominal organs
and the predominance of right handedness in the general population. As has been
appreciated for a long time, the normal spine is also not a symmetrical structure [2, 3].
In our line of research we investigated the rotational patterns of the normal paediatric
and adult spine in relation to closure of the neurocentral junctions and organ anatomy.

1
Corresponding Author: René M. Castelein, MD, PhD, Department of Orthopaedics, G05.228,
University Medical Center Utrecht, 3508 GA Utrecht, The Netherlands; E-mail:
[email protected]
Research into Spinal Deformities 8
T. Kotwicki and T.B. Grivas (Eds.)
IOS Press, 2012
© 2012 The authors and IOS Press. All rights reserved.
doi:10.3233/978-1-61499-067-3-20
20
Research into Spinal Deformities 8, IOS Press, Incorporated, 2012. ProQuest Ebook Central,
Copyright © 2012. IOS Press, Incorporated. All rights reserved.

1. Pre-existent rotation in the normal adult spine
We hypothesized that there is a pre-existent axial rotational pattern of the normal,
nonscoliotic adult spine that corresponds to the direction of rotation seen
predominantly in adolescent idiopathic scoliosis . For this research line we developed a
new semi-automatic analysis method to calculate the axial vertebral rotation of each
vertebral level on transverse CT images in a systematic and reproducible way (Figure
1) [4, 5, 6].
We observed that in the normal nonscoliotic spine there is a pre-existent rotation to
the left at high thoracic levels and to the right at mid and low-thoracic levels (Figure 2)
[4]. In a study on the in vivo effect of three different body positions on the rotation of
the normal spine, we observed that the vertebral rotation of the thoracic spine was less
pronounced in quadrupedal position in comparison with the supine and upright-
positions [7]. The fact that the rotation of the normal adult spine is not randomly
distributed or symmetrical might explain the most prevalent patterns of rotation in
adolescent idiopathic scoliosis. Apparently, once the spine starts to decompensate into
a scoliosis, it will follow this built-in rotational pattern, rather than revert to the
opposite direction.
Figure 1. CT slice at level T5 in the transverse plane. Vertebral rotation was defined as the angle between the
mid-sagittal axis (line between the center of the sternum [S] and the center of the spinal canal [C]) and the
longitudinal axis of the vertebra (line through the anterior half of the vertebral body [V] and spinal canal
[C]). (Data compiled from Kouwenhoven et al. and Janssen et al. [4, 5, 6])
R.M.Castelein / Pre-Existent Rotation of the Normal Spine at Different Ages 21
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Figure 2. Vertebral rotation in the horizontal plane (mean and 95% confidence interval) at level T2-L5 in 50
persons with a normal nonscoliotic spine (black), and 37 persons with situs inversus totalis (grey). All
measurements are in degrees. (Data compiled from Kouwenhoven et al.[4, 6])
2. Pre-existent rotation in the normal growing spine
In the paediatric population we observed different rotational patterns in different age
cohorts. In infantiles (0-3 years old), the whole thoracolumbar spine was rotated to the
left, in juveniles (4-9 years old) the high thoracic vertebra were rotated to the left and
the mid and low thoracic vertebra predominantly to the right. At the adolescent age
(10-16 years old) an even more pronounced rotational pattern was seen as compared to
the juvenile age (Figure 3). These results match the convexity of the predominant
curves in infantile, juvenile and adolescent idiopathic scoliosis and support the
hypothesis that the direction of deformity in most types of scoliosis is determined by
the rotational pattern present in the normal spine [5, 8].
Figure 3. Mean vertebral rotation angles on each spinal level are shown for the infantile (0-3 years old),
juvenile (4-9 years old), and adolescent (10-16 years old) cohorts. Rotation to the left was defined as negative
and to the right as positive. Error bars indicate the standard error. (Preliminary data compiled from Janssen et
al. and Schlösser et al. [5, 8])
R.M.Castelein / Pre-Existent Rotation of the Normal Spine at Different Ages22
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3. Pre-existent rotation and closure of the neurocentral junctions
Asymmetrical growth and closure of the neurocentral junctions (NCJ) occur in the
development of pathological rotation of the spine, as is known in naturally occurring
scoliosis as well as in experimental scoliosis. The neurocentral junctions (NCJs) of the
vertebrae connect the pedicles and laminae to
the vertebral body bilaterally, and allow
for growth in radial direction of the spinal canal. The cause of the pre-existent rotation
of the normal spine is not clear. Asymmetrical growth of the NCJs may play an
essential role in the development of pre-existent axial rotation of the spine, without
necessarily being related to the etiology of scoliosis.
Preliminary results of a recently performed study show that in the normal spine the
NCJs close asymmetrically and that this closure is consistent with the pre-existent
rotation of the normal growing spine. In the left-sided rotated spine of infantiles, the
right NCJs were predominantly larger; whereas in the mid- and low thoracic spine of
juveniles, the left NCJ was predominantly larger and the spine was rotated to the right
(Figure 4). At the adolescent age the NCJs were already closed. In addition to the
closure patterns of the NCJs and rotation it was demonstrated that in the normal spine
the NCJs close first at lumbar levels and last in the mid- and low-thoracic spine. The
apex of the curvature in idiopathic scoliosis is mostly localized in this spinal region [8].
These preliminary results suggest that the asymmetry and regional pattern of
closure of the NCJs match the convexity of the curve in idiopathic scoliosis and the
region of onset of the deformity.
Figure 4. The graph shows the mean differences in NCJ surface area for each spinal level in the infantile and
juvenile cohorts. Difference in NCJ area was defined as the mean surface area of the right NCJ minus the
surface area of the left NCJ. Error bars indicate the standard error. (Preliminary data compiled from Schlösser
et al. [8])
R.M.Castelein / Pre-Existent Rotation of the Normal Spine at Different Ages 23
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4. Pre-existent rotation and organ anatomy
In a group of patients with situs inversus, we demonstrated a rotational pattern exactly
opposite to what was found in the normal adult population (Figure 2) [6]. In this group
handedness was distributed as in the general population, the vast majority was right-
handed. Therefore, we concluded that handedness does not influence the pre-existent
rotational patterns of the normal spine, but organ anatomy does.
5. Discussion and Conclusion
Our research has shown that: (1) in infantiles the mid and low-thoracic spine is
predominantly rotated to the left, while in juveniles, adolescents and adults the mid-
and low thoracic spine is predominantly rotated to the right; (2) the pre-existent
rotational vertebral patter
n increases if the human spine is brought from quadrupedal to
an upright position; (3) these pre-existent rotational patterns match the asymmetrical
closure of the NCJs; (4) the pre-existent rotation is related to organ anatomy, and not to
handedness; (5) these built-in rotational patterns match the convexity of the curve in
the most prevalent types of thoracic idiopathic scoliosis; and (6) the NCJs close last at
the levels at which idiopathic scoliosis predominantly occurs.
Naturally, the existence of pre-existent axial rotation of the thoracic spine does not
automatically result in the onset of scoliosis. However, we observed that the rotational
patterns of the normal spine are identical in direction –although less in magnitude- to
the rotation that occurs in idiopathic scoliosis in different regions of the spine and at
different ages. The human spine is the only spine in nature on which posteriorly
directed shear forces consistently act in certain (ill defined) areas. These forces have
been shown to decrease rotational stiffness substantially [7, 9, 10]. The occurrence of
scoliosis depends on whether the delicate
human spino-pelvic balance is disturbed,
which can happen due to a multitude of factors [1]. Apparently, once the spine starts to
decompensate into scoliosis, it follows these pre-existent rotational patterns.
In conclusion, the organ asymmetry and NCJ closure match the rotational patterns
of the normal growing and adult spine. It seems likely that once rotation occurs in
a developing scoliosis, it will follow the built-in underlying rotational pattern that we
have demonstrated to exist in the normal spine.
References
[1] Kouwenhoven JW, Castelein RM. The pathogenesis of adolescent idiopathic scoliosis: review of the
literature. Spine (Phila Pa 1976) 2008; Dec 15;33(26):2898-908.
[2] Nicoladoni C. anatomie und mechanismus der skoliose. in: Kocher, könig, von mikulicz, eds.
Bibliotheca medica. stuttgart: Verlag von erwin nagele; 1904. .
[3] Tubby AH. Symmetry and asymmetry, and their effect in the production of lateral curvature of the spine.
Proc R Soc Med. 1909;2(Sect Study Dis Child):247-57.
[4] Kouwenhoven JW, Vincken KL, Bartels LW, Castelein RM. Analysis of pre-existent vertebral rotation
in the normal spine. Spine (Phila Pa 1976) 2006; Jun 1;31(13):1467-72.
[5] Janssen MM, Kouwenhoven JW, Schlosser TP, Viergever MA, Bartels LW, Castelein RM, et al.
Analysis of pre-existent vertebral rotation in the normal infantile, juvenile, and adolescent spine. Spine
(Phila Pa 1976) 2011; Apr 1;36(7):E486-91.
R.M.Castelein / Pre-Existent Rotation of the Normal Spine at Different Ages24
Research into Spinal Deformities 8, IOS Press, Incorporated, 2012. ProQuest Ebook Central,
Copyright © 2012. IOS Press, Incorporated. All rights reserved.

[6] Kouwenhoven JW, Bartels LW, Vincken KL, Viergever MA, Verbout AJ, Delhaas T, et al. The relation
between organ anatomy and pre-existent vertebral rotation in the normal spine: magnetic resonance
imaging study in humans with situs inversus totalis. Spine (Phila Pa 1976) 2007; May 1;32(10):1123-8.
[7] Janssen MM, Vincken KL, Kemp B, Obradov M, de Kleuver M, Viergever MA, et al. Pre-existent
vertebral rotation in the human spine is influenced by body position. Eur Spine J 2010;
Oct;19(10):1728-34.
[8] Schlösser TP, Vincken KL, Attrach H, Kuijf H, Viergever MA, Janssen MA, Castelein RM. Closure of
the neurocentral junction as related to pre-existent rotation in the normal growing spine. EPOS 31st
Annual Meeting. Journal of Children's Orthopaedics 2012;6(0):1-26.
[9] Castelein RM, van Dieen JH, Smit TH. The role of dorsal shear forces in the pathogenesis of adolescent
idiopathic scoliosis--a hypothesis. Med Hypotheses 2005;65(3):501-8.
[10] Kouwenhoven JW, Smit TH, van der Veen AJ, Kingma I, van Dieen JH, Castelein RM. Effects of
dorsal versus ventral shear loads on the rotational stability of the thoracic spine: a biomechanical
porcine and human cadaveric study. Spine (Phila Pa 1976) 2007; Nov 1;32(23):2545-50.
R.M.Castelein / Pre-Existent Rotation of the Normal Spine at Different Ages 25
Research into Spinal Deformities 8, IOS Press, Incorporated, 2012. ProQuest Ebook Central,
Copyright © 2012. IOS Press, Incorporated. All rights reserved.

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Chapter 2
Genetics and Aetiology
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Philomen, and in his lowly cabin he subsisted solely on the fruit of
his little garden, and the milk furnished him by his goat. Now, one
quiet evening, some one tapped gently at his door, and an old
man, though younger than he, entering, claimed his hospitality.
"Most willingly, my friend. My cottage is not large; my garden yields
not much fruit; my goat gives but little milk; but, even so, I share
it cheerfully with all who cross my threshold in the name of
hospitality. Enter, then, good friend, and rest after the fatigue of
the day."
"But," said the traveller hesitatingly, "I am not alone. I have twelve
companions with me, overpowered by weariness and parched with
thirst, for we have just crossed the desert."
"Let them all come; you are all welcome. All who come hungry to
my door are welcome to all I possess."
Then the stranger made a sign to his companions, who were
silently standing at the door; and he found that they were Jesus
and his twelve apostles, whom St. Peter led on their journey, ever
walking in advance, he who was one day to open the gates of
Paradise.
They entered, partook of his simple fruit, drank the milk furnished
by his goat, and rested for a time on his rough mat. When day
dawned, St. Peter said to him, "Before going hence, hast thou no
petition to make to us? Hast thou not some wish? Ask whatever
thou wilt in return for thy hospitality. All that thou shalt ask shall be
granted unto thee."
Then the old man made three wishes, and said: "My sweet Lord, I
love life so well, grant me yet five hundred years to live; the days
pass so quickly in this peaceful cabin."
"Granted," said a sweet and touching voice, which seemed to
come, as it were, from the midst of the group. "What else wilt thou

have?"
"My good Lord, I have a beautiful fig-tree in my garden, which
bears such fine fruit that they are often stolen from me. Grant me,
then, that whoever climbs into it may not be able to descend until I
give him leave; thus I will ensnare the thief."
Jesus smiled as he heard this quaint wish, and, bowing his fair
head, said: "It shall be done as thou wishest. Hast thou more still
to ask? Speak freely, for thou seest that I grant thee all that thou
hast wished for."
"My dearly loved Lord, I have a wooden chair, on which my friends
sit when they come sometimes at night to talk with me. Grant me
that whoever rests on it may not be able to rise, and must remain
there as long as I shall please."
And Jesus approved again, because he loved this guileless old man,
who was so simple of heart and made such modest wishes. St.
Peter then thanked him, and went forth, followed by his twelve
companions, among whom Jesus loved to conceal himself.
Years passed by one after the other. One century passed, then
another, until finally the last day of the last year arrived, and the
venerable Philomen saw the grim traveller Death enter his cabin,
who said to him:
"Come along, old man! Thou hast eluded me this long time—thanks
to an especial favor. Thou hast reached the years of Mathusale. If
every one lived as thou hast, I would have no work on earth. Come
along, quick. Regulate thy affairs, bid farewell to thy garden,
because, with the setting sun, I lead thee forth with me."
"O my good dame! if you would but pity me! Ah! yes, if you would
have some pity, you would let me live some few days more—only
one day, then. It is so good to live!"

"No, nothing; not one moment more," replied the sinister guest in a
harsh and dry voice.
"At least, then, let me once more eat of the fruit of my fig-tree. I
have loved them so well; it will be a last consolation to me. But I
am too weak to shake the tree, and too old to reach those highest
branches. Do you go up, and gather me that fig up there; it is so
thoroughly ripened by our eastern sun."
"Most willingly. See, old man, I will show thee that Death is not as
surly as 'tis said she is."
Then placing her hour-glass and scythe at the foot of the tree, the
unlucky dame climbed up; but scarcely had she pressed her foot
upon the branches, than, lo! they sprang up as if from her tread,
closed around and so shut in the impudent wight that she could
not even stir. She called; she cried aloud, then moaned and
supplicated. Philomen renewed his humble petition, but she
persistently refused.
"Very well! I only want five hundred years, five centuries more!"
And raising his head menacingly, he took up the hour-glass and
scythe, quietly returning to his cabin. Every morning he returned,
imposed his conditions of release, which Death, becoming more
and more irritated, as obstinately refused. Then he would go back
patiently to his cabin. On the third night he saw a dark figure, with
glittering eyes, prowling round the foot of his tree. He listened, and
heard this conversation. Now, you must know that this was the
Devil, who came to make his complaint: "What dost thou there,
thou idler? Thou no longer sendest me work to do. I am ruined by
thy delay."
But the terrible accomplice could do nothing; because he who binds
on earth as he binds in heaven had bound her so firmly that Death
herself could not undo it.

Next morning, after a fresh dispute with Philomen, she yielded, and
consented to let him have five hundred years added to his life. But
as Death is treacherous, he sought his tablet, and before she came
down he made her sign the treaty. After that he set her free,
restored her baggage, her hour-glass and scythe, and let her
depart, threatening and raging as she went, vowing to cut off, at
the very moment of the promised time, the life of one who had so
pitilessly ridiculed her.
Years again passed by, one by one; the centuries were
accomplished; and yet Philomen did not grow old. Ten times had he
seen pass by that unhappy pilgrim condemned to wander for ever
round the world. Each journey marked one century as this
wandering Jew crossed the Jordan, near his little cabin, on the road
to Jerusalem, that, ascending Golgotha, he might sue for mercy on
the very spot where the blood had been shed of him whom he had
despised! The centuries had all now passed, and one evening,
when Philomen sat quietly by his hearth, the dark traveller entered
once more. Midnight was the fatal hour. She rudely accosted him:
"Come along now, old man! Thou shouldst long since have been in
thy grave. No mercy for thee this time! Thou wouldst but mock me
again, could I show pity for thee. Oh! how tired I am; so tired, so
worried! To-day I have killed nearly three thousand Christians, then
a whole race of infidels, and decimated an entire kingdom, with my
well-tempered weapon, pestilence. Rich and poor, prelates and
priests, I have upturned everything—everything. But I am horribly
tired, and while awaiting the expiration of thy time, I will rest me a
little here." Saying these words, she threw herself on the wooden
stool that Jesus had gifted with supernatural powers. Then she
began to jeer at the old man, speaking to him of the joys of life, of
youth, of love, etc. When midnight tolled, she attempted to rise
from the chair and spring at Philomen, who had wisely placed
himself beyond her reach; but, nailed down upon this wonderful
seat, she could not move! In vain she shook her glass, made
deadly thrusts with her scythe! Then the good man went to his
hearth, and kindled such a fire as nearly roasted her even at that

distance. Her hour-glass was about falling to pieces, the handle of
her scythe was nearly reduced to ashes, when, after a most
vigorous dispute, she granted Philomen a new lease of five
centuries more of life!
Now, this was, as you know, the second time she had been caught
in the same trap, and more enraged than ever, she went forth
crying aloud that she should not be caught again; and good old
Philomen lived on through the long years obtained by this trick. But
everything of time must end; everything falls; everything dies;
everything passes away. And these five centuries, too, were
gathered with all that had gone before. But Death had learned
prudence now, and did not venture near, sending a shaft from afar
that pierced the good old man and sent him at once from life to
death. But as he had lived so innocently and ever observed the
laws of holy hospitality, God had a place prepared for him in his
own beautiful Paradise.
Now, it happened that before going there our Philomen wished to
see, just a little, what was going on in hell. Since the night that he
overheard the dispute between Death and Satan, he had cherished
a great desire to do so. He quietly entered the abode of the
condemned, and when the Devil came to meet him, and would
have seized upon him, Philomen cried out: "Stop there! I am not
for thee! I am of the kingdom of the elect, and come here only to
see if all that is said of thee in the kingdom of the living be true.
Lead me everywhere!" When, conducted by his dark guide, he had
visited the bowels of the earth and witnessed all manners of
torture, he proposed to him to stake his own soul against some of
the most fearfully punished among the damned who were uttering
most terrific shrieks. The dice were brought, and shaken by each in
turn. Philomen gained twelve souls; then Satan became fearful he
might lose all with this mysterious partner, and refused to play on.
Philomen then took the road to Paradise, and, reaching the gate,
tapped gently. Saint Peter came to open for him. He at once
recognized him, and, smiling, said, "Pass on, we have expected you

all this time." "Oh! very well," said the acute old man, "but, like
you formerly, I am not travelling alone: I have with me twelve
companions, who claim your hospitality." "This is but fair," said St.
Peter, once more smiling, "so come in." And so Philomen and his
twelve ransomed souls all went to join the throng of the blessed
who will for ever sing the glory of God.
It is thus the good old man lived fifteen hundred years, and
practised the holy rules of hospitality. And it is thus that our pious
ancestors taught their children never to refuse entrance to those
who knocked at their doors, imploring shelter; and thus we, too,
see how religiously and beautifully hospitality was practised in the
former ages, in the chateaux of the rich as well as in the more
humble dwellings of the poor.

Mine Enemy.

If he could stand against me now,
With other eyes and an alien brow;
If I could break the spell that still
My will entangles with his will;
If he could laugh the while I weep;
If I could wake, and he asleep;
Could I uncoil the mystery
Where he is I, and I am he:
Then might I hide me from his face;
Or strike him down within his place;
And so, at last, my life be free
From his tormenting company.
But no; his blush my forehead burns,
His the pallor my pale cheek turns,
And when he sees the thing I do,
'Tis mine own eyes that he looks through.
When I would hate this tiresome mate,
He teaches me the way to hate;
When from his presence I would flee,
He, taunting, flies along with me.
But best I like his baser slips,
His angry eyes and impious lips;
For then, half-wrenched away from me,
Almost it seems he leaves me free.
'Tis then I raise aloft my cry:
St. Michael, to the rescue fly!
'Tis then almost my foot is prest
Upon the monster's struggling breast;

'Tis then I feel my shoulders glow
With hints of wings they yet may know,
And breathe as slaves pant, wild and sweet,
Whose chains are falling to their feet!
'Tis then I nestle, safely bound
By wings of angels circling round,
And feel the drawing of the cord
That holds my anchor in the Lord!
And most I fear when cunningly
He crouches, hidden from mine eye,
And breathes into the pipes whose keys
Hold all my spirit's melodies.
When I his hiding would betray,
He holds the lamp, and leads the way;
When I would break his hardihood,
He wields the lash that draws my blood.
So deep his guile, I scarce can know
From whose intent my actions grow;
So brightly do his tear-drops shine,
I oft mistake his grief for mine.
When veiled emotions, swift and strong,
Run all my trembling nerves along,
If 'tis his sigh or mine whose swell
Upheaves my breast, I cannot tell.
When friendship frowns, I turn to see
My foe's eyes beaming tenderly;
When friendship harshly speaks, I hear
His dulcet tones wooing mine ear.
When God is slow to hear my cry,

Behold th' insidious list'ner nigh!
When thirst has parched my vitals up,
His hand presents the sparkling cup.
If I would reason with my foe,
He lets the high-piled logic grow,
And lowly bends, in humble guise,
With silent mouth and drooping eyes.
But as, o'erflowing with content,
I view my stately monument,
Nor guess the thoughts lie side to side
In subtle, weak cement of pride,
With sudden flash of mocking wit
He plays about and shatters it,
Or some volcanic underthrust
Levels my structure with the dust.
And straight, ere I can speak for pain,
He builds my chang'd thoughts up again
In airy stretches, bright or dim,
With flower-woven cornice-rim;
With domes that melt into the sky,
Like piles of snowy cumuli;
And pinnacles where fancy sees
Stars cling and swim, like golden bees;
With long-drawn wings whose cloudy tips
The sunset kisses with red lips;
And cloudy-curtained windows bright,
Whence pours a flood of rosy light.
And with it come bewildering tunes,
Where heavenly airs bear hellish runes;

And, calling sweet and calling clear,
The voice that most I long to hear.
But if, lured by this temple fair,
Dazzled, I seek to enter there,
It clings, and burns with lurid light,
Like Glauce's bridal-garment white.
Then since my foe so potent is,
And I so weak, lest I be his,
Some friend I need, stronger than he,
To stand and keep my heart for me.
And since, though driven forth with pain,
Ever he stealeth back again,
More need have I of heavenly light
To make his lurking-places bright.
And since I stand unarmed, indeed,
Before his wrath, great is the need
I should invoke, with prayerful word,
Saint Michael of the fiery sword!
That night and day I still should cling
Beneath my hovering angel's wing;
And ne'er let slip the golden cord
That holds my anchor in the Lord!
Translated From The Revue Du Monde Catholique.

Flaminia.
By Alexandre De Bar.
Concluded.
"You will not be surprised to see that Flaminia was ignorant of the
veritable nature of the affection that she felt for Albert; but you will
be astonished to learn that he shared entirely her ignorance,
although he had seen much of life. Yet think that it is to know
nothing of the most impetuous passion of our soul if we have only
learnt the theory; for as to know the world we must have lived in
the world, so to know the heart one must have lived by the heart;
if such has not been one's experience, all is obscurity and one
takes a false route. Now, Albert had lived out of the world, and had
not yet loved aught but a glorious renown. Besides all this, if you
will look back upon that fair time of youth which has now fled from
us, you will remember that the descent which allures us is often so
gentle that we follow it without attention; until the day when an
unforeseen event, and often even an unimportant circumstance,
arouses us, and permits us by a glance to see the road that we
have already glided down. Albert, too, descended that charming
declivity, gathering the perfumed flowers which hung on the
shrubs, and intoxicating himself with perfumes, with light and
songs. His soul happy, his heart pure, dazzled by the celestial
gleams which irradiated him, how could he see where all this was
conducting him? This is how he first became aware of his position:
There was at the bottom of the gardens of the palace Balbo a long
alley, that was covered by the thick foliage of the vines, whose
stems, black and distorted, clung to and spread up the stone pillars
on each side. Here and there the jasmines displayed the silver stars
of their flowers, which shone out of the deep shade of their leaves.
From that alley the eye gazed upon a vast horizon, bounded by two
large sheets of azure, the sea and sky, between which the

mountains lifted their imposing masses, gilded by the rays of the
setting sun. It was in this perfumed gallery that, each evening,
Albert was conducted by his hosts, as soon as the refreshing
breeze of evening blew across the sea. Often it was the arm of
Flaminia that aided his yet feeble steps in this exercise. How many
charming hours thus passed for them during the calm of those
evenings, when the noises of the day ceased one by one, until the
ear brought but the sound of the whispering breeze, pure and
sweet as the breath of a sleeping child, to the touched and
softened soul! One day, the fever seemed struggling to regain its
power over the form of Albert; his wounds were scarcely closed,
and the emotions that he experienced reacted most powerfully
upon his health. Sir, man is born for suffering, and not for joy. His
body can support an immense weight of sorrow and pain without
giving way; but it is worn out by pleasure, and joy kills it. Giovanni,
uneasy about his friend, strictly forbade his leaving his room, and
that evening the family went alone to their walk. Albert returned
sadly to the saloon, become more desert for him than the sands of
Sahara, in company with Giovanni, who, in the hope of distracting
his loneliness, talked to him of battles and of victories; although
had he known how far the mind of his friend was from all such
subjects, he might have given himself far less trouble with an
equally good result. Little caring then for glory, Albert's heart was
with Flaminia under the perfumed shade of the vines and jasmines.
At their return, Flaminia held out to Albert a spray of jasmine
covered with flowers, saying to him: 'You like these flowers, so I
bring you them.' When Albert had retired to his own room, he took
this bouquet and covered it with kisses: he listened with delight to
the voice that issued from those flowers and that told him such
sweet words. A flame seemed to mingle with their perfumes that
carried a new life to his heart; but it carried there also the light.
Another voice made itself heard and showed him the truth, and he
fell from the regions of happiness where his dream had carried
him, into the implacable reality; for he then discovered with what
sort of an affection they were both animated. And he a knight of
the Order of Malta! If absence could have given the repose of

forgetfulness to Flaminia, Albert would not have hesitated to have
left her at once. But if there exist attachments so slight that the
simple absence of their object is sufficient to cure them, so there
are others which may be likened to those long-lived plants that
extend their roots in all directions and all depths; so that one
cannot tear them from the soil in which they have once gained a
hold. Such affections as these resist all human efforts, and absence
but serves to render their wounds more poignant and more lively.
Albert understood too well the character of Flaminia not to know
that their destiny was irrevocably fixed. Divine Providence seemed
to have drawn them together in this world but to make them merit,
by a sacrifice of their affections, the happiness that was destined
for them in the next. The ordinary remedy of absence would have
been useless in their case. Albert understood this, and the idea of
getting himself absolved from his vows of knighthood came to him.
This thought he repelled. It was not that he believed the success of
such a measure impossible, but that he saw in it a desertion of his
duty; he felt that his conscience would not be in tranquillity, and
that it would perpetually remind him that one cannot thus break his
engagements with God. He knelt down piously, and that which
passed in his soul during that cruel night, and that which he
suffered during that struggle, ever rested a secret between him and
God. For you, scholar of the eighteenth century, it is an
unpardonable weakness that of placing one's self humbly on one's
knees before the Divine Majesty. Yet, thanks only to this weakness,
Albert, in all the force of youth, resisted without failing before the
most impetuous, the most irresistible of all our passions, and came
forth victorious out of the rudest combat that he had ever given.
He loved, passionately, Flaminia: Flaminia, beautiful, rich in heart
and soul, full of all the merits, of all the virtues, that can entrance
at the same time the heart, the soul, and the senses; Flaminia,
who loved him with an equal ardor, and who confided herself to
him absolutely and without reserve. He had over her an absolute
power, and, far from using it, he subdued his passion, and,
directing by a determined will the tumultuous waves of his heart,
he traversed without shipwreck those tempests that are more

ungovernable than the rage of the ocean. The strength with which
he aided himself was that same weakness which makes you smile.
Had he trusted only in himself, he would have fallen, because he
was but a man; he implored the aid of him who is strength itself,
and he vanquished. Faith was for him what the fortifying oil was
with which the athletes rubbed their bodies before the struggle;
and, not content with aiding him to overcome himself, she knew
also how to dry his tears by the blessed aid of hope. For, at the
same time that she showed him in all their barrenness the painful
paths of duty, she let him see at the end of the journey, and as the
price of his victory, that eternal union of souls which time itself is
powerless to break. I know you to be prejudiced, my dear
Frederick, on all that which touches religious questions; but, at the
same time, I know you to be of too good faith not to acknowledge
that there is truly something superhuman in a doctrine which gives
such victories; neither shall I insist on the detail of the events
which occurred during the six months that Albert yet passed by the
side of Flaminia, for they would have no value in my recital. It
would not, perhaps, be without a certain interest to follow the
developments of that affection, so completely purified from all
earthly thoughts; but, as there are certain situations where a look,
a smile, takes the proportions of a veritable event, it would be
necessary for me to enter into the very slightest points of its
psychology. On learning the gravity of the wounds of his brother,
Adolph Shraun had come in all haste to the palace Balbo. Antonia
failed not to produce in his heart an impression as profound, but
more decisive, than that which Flaminia had already aroused in his
brother. As he knew that the project of an alliance would be joyfully
received in the two families, Antonia was not long without knowing
the sentiments which she had enkindled. The frank, impetuous, and
lively character of Adolph had already predisposed her in his favor,
so that she quickly shared the same sentiments and hopes as
himself. Joy renders us much more disposed to confidence than
does sorrow, and Antonia did not fail to feel the need of confiding
to some one both her secret and her love. This need caused her to
seek in Flaminia for sympathy, and the reciprocal confidence which

was due between these two young hearts, so well formed to love
and sustain each other, was then established for ever. The naïve
confidences of her sister enlightened Flaminia on her own
sentiments, and carried into her soul the light that she had but
caught glimpses of before. She then understood the nature of her
destiny, and, like Albert, she accepted it without a murmur. She
took refuge in the consoling thought that their union would be
accomplished in those celestial regions where only reign the eternal
laws of love; and thus placing her hopes upon a sure basis, she
resigned herself to her cross, prayed, and awaited God's will. I
think that I have quite sufficiently instructed you upon the state of
these noble hearts; so that I can arrive at that which is the object
of my story—namely, to tell you how it was that my great-
grandfather, Adolph, saw, one day, two souls." The Baron Frederick
could not here repress a deep sigh of satisfaction, and the count,
who noticed nothing, continued: "The hours, which their separation
was soon to render so long, passed away with a cruel rapidity; the
moment approached when Albert ought to leave Flaminia, that he
might report himself to the Grand-Master Coroner, who was then
preparing an expedition directed against Napoli of Roumania, and
the few days they had yet to pass together made them feel still
more strongly the happiness that they were about to lose. Giovanni
had announced his intention of following his friend, and their
approaching departure had cast a shade of sadness on that
household, lately so joyous that it had seemed a nest hidden from
the world, where alone happiness dwelt. One evening, when,
according to their usual custom, they were all grouped together
under the shadow of the vines, the conversation took a melancholy
form, and the fear that reigned in all their hearts expressed itself
by words: they were talking of death. 'Come, come,' said the Prince
Balbo, after a few minutes of discussion on the subject, 'what is the
use of these fears? When duty calls, we must obey, not only by
action, but in heart, and without regret. Besides,' he added, 'the
hour of our death is not in our own choice; and none are protected
from his stroke when God calls the angel of death and says,
"Strike!" I have, like you, my children, incurred many perils in my

life, and yet sixty winters have whitened my head; and how many
have I not seen of those whose life was peaceable—of flourishing
youth—sheltered from all harm, who have been struck down before
their time! Let us confide in God, my children; let us resign
ourselves beforehand to his will, which is always just, always good
—since he is eternally just and good.'
"Flaminia, crushed by the grief of a separation that snatched away
from her for ever the half of her soul, had, until these last words of
her father, remained silent; but then, lifting her head and leaning
slightly toward Albert, said to him in a tone that was audible only
to him, 'Yes, happily, one dies at every age.'
"Albert understood her thought.
"'Do you not, then, think on the grief of those who are left?'
answered he, in a voice of low reproach.
"'Oh!' replied she quickly, 'if I die first, I will come to seek you.'
"Before that cry, uttered from the heart, before that affection that
felt itself sufficiently strong to vanquish the laws of death,
sufficiently holy that God should grant it a miracle, silence could be
the only answer; but a glance of Albert replaced with all the
eloquence of the heart the powerless word. On the morrow of that
evening, Albert left Flaminia. I will not paint to you their affliction.
It was immense. But a hope that is too ill known in this, our
century, sustained their courage and energy. At the moment of an
adieu so cruel to both, not a tear fell from their eyes. That they did
flow, and most abundantly and bitterly, there is no doubt, since
grief never loses its rights, and human force, even the best
sustained, has its bounds; but they flowed in silence and in secret,
and he who was their only witness treasured them up. The days,
the months, the seasons passed on; three times the trees had lost
their foliage and renewed their leaves; three times had the alley of
vines seen the winter's sun pass unobstructed through their naked

branches. All had changed around them; their hearts alone
changed not. The renown of Albert grew each day, with his valor,
more brilliant; but it was no longer renown that he sought, it was a
death that would have opened before him that wide field where
impatience dies away before the eternity that then commences;
death that he desired because it would have brought him near to
Flaminia; and death would not listen to him. In vain did he fling
himself into the thickest of the danger; in vain did he accomplish
prodigies that had caused the bravest to turn pale; he passed
through all these without even a wound. Although he had but very
rare occasion of knowing what passed in that cherished spot where
ever rested his heart and thoughts, still he doubted not but that
the tenderness of Flaminia was as lively and as deep as his own;
nor did he deceive himself. Flaminia had refused under different
pretexts the offers that had been made to her; and notwithstanding
all the desire they felt to establish their daughter, I would dare to
affirm that it was not without a certain secret joy that the Prince
and Princess Balbo looked upon the prospect before them, the
hope of keeping her always by their side. Do not blame them too
quickly, my friend; for it is a painful thought that during twenty
years a child should have been the object of your affection and of
your solicitude; that she should have taken the best and largest
portion of your life and heart, in order that, one day, a stranger,
under the title of a new-born love, should carry away from you all
your joy; leaving you to see your much-loved child place herself
under another protection than thine, and quit without regret the
house where she leaves a blank that nothing else can fill.
"I had almost forgotten to tell you that Antonia had married
Adolphus, and lived happy and peaceful in this same castle where
we now are finishing our career. Albert, tired of war, and freed from
all further illusions of glory, had come, after having refused the
highest distinctions of the order, to seek some repose by his
brother's side. Ambition was dead in him; his soul, that had been
so severely proved, had need of recollection and calm; and he
found this by the side of him whom, after Flaminia, he loved the

best in the world. Moreover, although he himself scarcely ever
spoke of her who filled all his thoughts, still he felt a lively pleasure
in hearing her spoken of so frequently by his brother and his wife.
Albert was then calm and composed; he marched courageously
forward in life as does the traveller who climbs with difficulty the
bare paths of a desolate and arid mountain, sure to find in the
evening the joys of the fireside and the shelter of his friends' roof.
"Three years, day by day, had passed away since the moment
when Albert had quitted the palace Balbo. It was the evening;
Adolphus and Antonia were by his side, in this same saloon where
we now are. Contrary to his custom, Albert, for whom that
anniversary was a day of mourning, felt his soul full of a
penetrating and serene joy, when ten o'clock sounded from that
same clock that—"
Here the recital of the count was interrupted by the sound of the
clock which resounded in the vast apartment. One would have said
that it affirmed the words of the count, by repeating the ten
strokes which it had caused to be heard at the moment of which
he was speaking. That metallic sound seemed to have in it an
unusual power; there was something solemn in its grave slowness;
in the deep noise of the wheel drawn round by the falling lead,
which accompanied with its heavy base the more piercing sound
that traversed the thick oaken case. Both the count and his friends
were seized by an impression which they did not seek to dispel or
resist. Both instinctively uncovered their heads, and while the count
waited almost respectfully until its last vibrations were lost in
silence, the baron, more moved than perhaps he was willing to
show, placed on the table his pipe, yet fully charged with tobacco,
and, an event that certainly had not occurred with him once in ten
years, he left that inseparable companion of his leisure hours,
without touching the tankard that in vain offered to his gaze its
brown and golden tints.

"Ten o'clock had then sounded," continued the count, "and that
being the moment when each was accustomed to separate for their
bedrooms, Adolphus had got up and looked at his brother, who had
been for some time previous motionless and in an attitude of
profound attention, resembling a man who follows with his ear the
scarcely perceptible sounds of some distant harmony.
"All is finished,' murmured Albert at the moment when the clock
had finished striking; and, placing his hand on his brother's arm,
'Remain here,' said he, and turning toward Antonia: 'Pardon me, my
sister, if I thus detain Adolphus; but I have need of him to-night,
and to-morrow it will be too late.'
"'You frighten me,' answered Antonia; 'what then is going to
happen?'
"'You will know very soon,' replied Albert. 'Poor sister! your eyes
will shed many a tear; but they will be dried by the thought that
the motive which causes them to flow assures for ever the
happiness of those who are dear to you.'
"He then kissed her forehead, and, followed by Adolphus, went to
his own room, the same which is now yours, dear Frederick.
"'What is the matter with you?' asked Adolphus of his brother, as
soon as they were alone.
"'I am sad and happy at the same time; sad because I am going to
leave you alone for a short time; but very happy because I go at
last to rejoin her, and for this time not again to leave her!'
"'Explain yourself; why do you leave us?'
"'Listen: for that you may understand what is going to happen here
this night, it is necessary that you should know what I have felt
and suffered during the past three years.'

"Albert then told him of all that which I have just described to you;
of his love for Flaminia, of his struggles, and of his victory, over
himself; and Adolphus, who already knew through his wife of what
Flaminia had suffered, saw with astonishment that all which had
been felt by the one had also been by the other, in the same
degree and at the same moment. Never had the most profound
sympathy established between two beings a more complete identity
of sensations and thoughts; near or separated, their two existences
had formed but a single life, as their two souls seemed to form but
a single soul. When Albert had finished his recital, he added:
"'"If I die first, I shall come to seek you!" Flaminia had told me,
and now Flaminia has just died. Do not ask me how I know it, for I
am ignorant myself of the reason; but I do know it. I have
followed, moment by moment, the progress of her death; at the
end I have felt her die, and now I await her coming. In a few
instants more she will be here, and we shall depart together for
that blessed home where nothing can again oppose itself to our
eternal union. It seems to me that already I feel my soul
disengaging itself from its bonds; I no longer regard the sufferings
that I have endured, except with that sentiment of thankfulness
and joy which one feels at the recollection of perils that have been
overcome; my past sufferings have no longer their sting, my tears
no longer their bitterness! At the solemn moment when I am about
to quit a life that has been most painful in its trials for the happy
life of triumph, I have wished to have you by my side, that I might
say to you my last farewell in this world, and press for a last time
your hand before going to await you in eternity.' I leave you to
think, my dear Frederick, what must have been the astonishment of
Adolphus at receiving this strange confidence.
"'I have too much confidence in the firmness of your reason,' he
answered to his brother after a short silence, 'to believe that it has
become weakened, were it only for a moment; but do you not fear
to have been the victim of some mental illusion, and to have taken

for a reality that which was in reality only the dream of your heart
exalted by sadness and solitude?'
"'I understand your incredulity,' answered Albert, 'for I have myself
shared in it. Each time that the recollection of that promise
presented itself to my memory, my reason revolted against such an
evident impossibility; the soul cannot again appear in this world
once that it has quitted it, thought I, and yet I counted on the
premise even while I disbelieved its possibility. Only an hour ago, I
yet doubted, but now that doubt has passed away, since the
moment when her dying voice sounded in my ears uttering her last
words: "You have waited for me; I am here!" Then I understood
that it was not merely the strong desire of a soul overexcited by
the desire to be reunited to the second half of itself that I felt, but
that it was really a mysterious warning; and the accomplishment of
a promise that God himself had blessed, and that he permitted to
be fulfilled.'
"'But how to explain this miracle?'
"'I am unable to explain it; I tell you what is about to happen, that
is all that I can do. In a few minutes Flaminia will be present, and
in seeing her you will believe me. For the rest,' added he, after a
moment's pause, 'all is a mystery in this world, but the grand end
of all is sufficient to enlighten our paths. Do you think that it would
be more easy for me to tell you how it is that, notwithstanding we
have never said anything to each other that could divulge the
mutual state of our hearts, we have yet, in spite of our separation,
lived by the same life and the same love? That you cannot believe
me, I know, but only wait a little time, and you shall see.'

"In truth, Adolphus did not believe, although the evidently profound
conviction of Albert shook his mind and caused in him an
impression that he would gladly have shaken off, so contrary to
reason did it seem to him. 'Let us make haste, the time presses,'
said Albert. He then arranged in order, with rapidity and calm,
several important affairs with which he was charged, relating to the
principal commanderies of Germany; then, kneeling down, he
offered up a short prayer; scarcely had he finished, than, rising up
quickly, he seized the hand of his brother, and cried: 'Look! she is
come.' Adolphus turned round, and saw Flaminia standing by the
side of Albert. You who have lost some one who was dear to you,
Frederick, you have remarked that, at the moment when the last
sigh escapes and before the work of decay begins, the face is
possessed of a calm beauty, supernatural and indefinable in its
expression, that inspires an awed respect for that now lifeless form
which just a moment before contained a soul. Such looked
Flaminia; her figure, surrounded by a luminous atmosphere, had
received from immortality an august expression. It was perfectly
the form of Flaminia, such as Adolphus had known her, but it was
no longer the creature that is imperfect, and subject to the attacks
of time and life. It was the being imperishable who, coming forth
victorious from her many trials, bore in her all the splendors of her
glory. Her beauty was not that which charms by the uniformity,
more or less complete, of its lineaments; no, it was the celestial
beauty whose type is graven in ourselves; the beauty a single ray
of which suffices to illuminate the face that hides a pure soul: this
was the beauty sublime that enveloped her with its divine wings,
and transfigured her face while changing its lineaments. Adolphus
bent his knee before the vision. 'Had I not told you that she would
come?' said Albert to his brother. 'Yes!' replied a harmonious voice,
which issued from the then incorruptible lips of Flaminia. 'Yes! our
love was too pure not to merit its recompense. God has permitted
it; you waited for me, and I am come.' She bent slightly toward
him to whom she at length was about to be united, and,
surrounding him with her arms, she drew his face closer to her

own, that gleamed with a celestial joy. Behind them, and
contemplating them, stood Death, not under the form of fleshless
skeleton, but as a radiant angel who changes bitterness into joy,
and tears into smiles. His beautiful face bore the impress of grave
majesty rather than of severity, softened by that infinite mercy
which gives hope to repentance. The mercy and goodness of the
Master who sends him shone in his look, which is so sweet to the
contemplation of the soul wearied by the painful journey of life.
The hour was come! At the moment when Flaminia, in a manner,
took possession of Albert, the angel of Death drew near him, and
while with one hand he touched his shoulder, with the other he
pointed toward heaven. Albert's body fell back into the arm-chair,
which, living, he had just occupied; and when Adolphus, drawn
forward by an instinctive motion, ran to support him, he saw by the
side of Flaminia the form of his brother, that shone forth
surrounded by the same glory and the same joy. He passed the
rest of the night by the side of his brother's body, and wept,
though not over him whom he had just seen pass away to heaven.
The man whom faith sustains with its sweet consolation weeps not
the loss of his friend, but his absence. He wept because every
separation, even the shortest, is a grief, and his tears were dried by
the certainty that Albert was in the possession of a happiness that
could neither diminish nor fade, and which he hoped one day to
share with him."
The count here left off his story. The baron had listened to him
with a sustained attention, and although he preserved his
imperturbable calm, yet the recital had so much moved him, that
he remained silent; and the count, after waiting a few minutes,
continued: "Such is the history of my great-uncle Albert, as it has
been transmitted to us by him who was the witness. Do you find it,
then, surprising that the faith should be hereditary in a family
where such facts happen? What can you reply to this history?"
"Nothing," answered the baron, "except that, to draw the
consolations which it contains, one must have the faith; and

besides, in supposing that God, if he exists, interferes with the
affairs of this world, he is unjust, since he refuses to me the
consolation that he gives to others."
"Have you ever asked him for it?" answered the count with a
friendly severity. "Have you not, on the contrary, repulsed by a
determined obstinacy the solicitations of divine Providence? Pardon
me, my friend, if I awaken a painful recollection for you, but have
you not even resisted the awful voice of Death?"
"What is the good of my asking?" replied the baron, eluding the
second part of his friend's demand. "If faith be necessary, God
owes it to me without asking him."
"Food is also necessary," answered the count, "and does man find
it ready for him, unless he works? No, no, my friend; labor and
prayer, such is the destiny of man upon the earth. His material life
is bought by the sweat of his brow, as his spiritual life is the price
of his efforts. 'Seek, and you shall find; knock, and it shall be
opened unto you,' has said the divine Master. Ah! if you had ever
knelt before that God whom you blaspheme; if you had with
perseverance exposed to him your doubts, your miseries, you
would have known that he never leaves without help the soul that
sincerely implores him; you would have known that he never hides
himself from him who seeks him with a humble and contrite spirit
and a pure heart. Pray, my dear Frederick, pray, I tell you, and you
will feel that he is near to you; that his arms are open to receive
you, and his hands ready to shed on you all the sweet consolations
and hopes with which they are filled!"
It was now late; the two friends then separated, and, without
doubt, the count that night in his prayers demanded with more
than usual fervor the conversion of the man he so warmly loved.
Ordinarily, on gaining his room, the baron was accustomed to install
himself as comfortably as possible in an immense old leathern arm-
chair, whose age dated back for two or three centuries, which he

placed in front of the wood fire that burnt noisily on the hearth;
and after having again lit his pipe, that inseparable friend, he used
to take a book, and, stretching out his feet upon the copper fire-
dogs, wait until he felt sleepy, which invariably occurred as soon as
there remained no more tobacco in the sculptured wooden bowl of
his pipe.
But on that night he cast around him many a curious look, and
examined with as great an attention, one after the other, the
several pieces of antique furniture with which his room was
furnished, as though it had been the first time that he had seen
them; then, in place of sitting down in that ancient arm-chair,
which he preferred to all the others on account of its large
dimensions, he placed it in front of him, and, sitting down on the
most modern of his chairs, he regarded with a questioning curiosity
and certain respect that mute witness of an adventure, the mere
recital of which had caused so great a trouble in his mind; seeming
to ask of it a solution to his doubts and fears. After a long and
silent contemplation, he let fall his forehead on his hands, and,
plunging his fingers among his hair, whitened rather by sorrow than
by age, began to meditate profoundly. The agitation of his mind
was so great, and the flow of his thoughts so rapid, that, without
knowing it, he began to think aloud. "If what he said were true, if
there were something within us that outlived our bodies, I could
see thee again, my dearest and best-beloved Gertrude; and I could
again find the joys of our too short union, and this time for always,
unchanging and eternal! And ought I to repulse that thought
through the childish fear of abandoning myself to a false hope? Of
these two ideas were it not better to follow that which gives us
consolation and causes us to live, rather than that which thickens
around us the already too profound shades of life, and changes our
grief into despair? What consolation have I ever found in the
reason of which I am so proud? None! If pride has withheld my
tears before men, yet since twenty years they have flowed on in
silence without their source being yet dry. If I have blushed to let
my weakness be seen by men, have I not felt it a thousand times

within me, implacable, and terrible, before my vain revolts against a
destiny that broke my heart and that I was forced to submit to?
When beside her death-bed I felt but a sterile despair, when my
will, my love, were powerless to retain for a single moment the last
sigh of that life that I would have been willing to prolong at the
expense of my own days, what have I been able to do? Nothing!
not even to die! Since twenty years I implore the oblivion which
flies before me! Since twenty years, I recoil before the thought to
precipitate myself therein! Is it fear that hinders me? No! I have
faced the peril when my duty demanded it, and I would do it
again: I have too often seen Death to fear him. The reason is that
a secret voice speaks within me higher than all the sophisms of my
grief, and tells me that I have not the right to destroy the life
which I did not give myself. Yet if there is nothing beyond the
tomb, why should I fear it, and what have I to dread from oblivion?
Have I not the most absolute right on myself, since all ends but in
a dreamless sleep? Is it really a sleep? Ah! there is the truth, both
for me and for all others; it is that in secret I doubt as often of
that oblivion that I so loudly affirm, as I do whether that God does
not exist whose existence I so deny. Yet again, if God is but an
imaginary being, and if immortality is but a dream, what does one
risk to have thought the contrary? One would have lived fortified
against the ills and crosses of this life by a thought that sweetens,
even the terrors of death. One would not even feel the loss of that
hope, since the hour of our disenchantment would be the one
which should plunge us into the deep repose of oblivion! The lie,
then, would have done that which the truth could not do; it would
have given us happiness. If, on the contrary, immortality is not a
vain chimera, but a reality, is it not a terrible responsibility to have
shut one's heart to its evidence and to have misunderstood the
sublime Author of all things? Yes! in truth terrible; for in that
momentous question, doubt is not to be permitted. On all human
questions indifference follows uncertainty, but here indifference is
itself a fault—one must deny or believe. But how am I to believe?
When from earliest childhood you have had your aspirations broken
or wounded under the repeated blows of contempt, and when you

have been taught but to laugh at in others that faith whose
absence you shall one day so bitterly deplore, how then to believe?
Pray, he told me. Pray! Can I pray? Oh! happy are they who,
arrived like me at that sad epoch in life when one drags painfully
along the burden of one's worn-out days, have not to curse those
who held them away from that source of strength and consolation!
Yes, they are happy whom a pious mother taught from their cradle
to bend the knees and join the hands in prayer! Gertrude also, she
too prayed; and many a time have I felt myself touched in seeing
her bend her head before the God of whom she asked for me the
light of the faith. How many times have I not felt the desire to
share her belief, and to kneel down like her and say: 'My dear
Gertrude, there exists no place on this earth where we ought to be
separated; there is not a thought, a belief, an affection, that ought
not to be shared by us. Whatever may be the destiny that awaits
us after the destruction of our being, whether it be oblivion or
immortality, I wish to share it with you. Let your convictions be
mine also, even as your life is mine. After having given me
happiness in this world, show me the road that leads to that
eternity I wish to believe in because you believe; make me to know
that God whom I wish to love because you love him!' But alas! held
by a false shame, I resisted that voice which spoke in the depth of
my heart, and which, perhaps, was the voice of God: for is it not
possible that such feelings as these are those by which Providence
calls us to the truth? And I, how have I responded to that voice?
Why, by rallying her on her belief. I caused her tears to flow, the
only ones most certainly, but today they fall heavily upon my heart.
And now friendship speaks to me this day the very same language
that did of old her love. Shall I yet remain deaf? Ought I to cede to
or resist the voice which now speaks to me? O Albert! you on
whom was accomplished, in the room where I am, and in that arm-
chair that I now look on, so incomprehensible a mystery, cannot
you come in aid of the most faithful friend that your family ever
had!" And the excellent baron, letting himself be carried away by
his emotion, found himself, without knowing how, on his knees
before that chair in whose arms Albert had died; and the head

covered by his hands, and the heart filled with the thirst for truth,
he prayed: "O my God!" prayed he, "if it is true that you are not a
vain creation of the weakness or of the pride of man; if it is true
that you continue to watch with solicitude over the creature who
has issued from your hands, you will not see without pity the heart
full of trouble that I lift up toward you. Led astray by the habits
begun in childhood, I have perhaps followed error thinking to follow
the truth; but I have done it in all sincerity and through love of the
truth. If I am deceived, O God! enlighten my trembling soul,
dissipate the doubt which is crushing me, and draw toward you the
soul that seeks you and desires you! And you, Gertrude, dear
companion too soon lost to me, if you see my regrets that time
cannot extinguish; and the tears that your memory costs me, ask
of your God that he make himself known to me; ask him that I
may adore him as you adored him, and, above all, ask him that I
may again be united to you."
His voice died away then, and yet his prayer continued. His soul,
overexcited with the emotions of that night, poured itself out before
God without following any line of thought. It was an immense
lifting up of his whole being toward the truth—an ardent thirst for
hope; it was the twenty years of a mute despair that resumed itself
into a supreme cry; it was the heart, so pure and so good, of that
worthy man, that opened itself completely and mounted full of
desires and tears, carrying with it the most fervent prayer that had
ever reached the immovable throne of the Eternal. At last the baron
arose, but in place of at once laying himself down to sleep on the
bed, whose soft pillows vainly invited him to repose, he retook his
former position and began to reflect. The thoughts pressed so
tumultuously in his brain, ordinarily so calm, and succeeded each
other with so great a rapidity, that he could but vaguely seize them.
His eyes, fixed upon the light flame that yet burned on the hearth,
saw not that they expired one by one. The last played yet some
time on the log covered with white ashes, disappearing for a
moment to again reappear in another spot; at length it died out.
The lamp burned with a reddened glare through its lack of oil, and

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