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Rare Diseases and Syndromes of the Spinal Cord
Ibrahim M. Eltorai
123
Rare Diseases and Syndromes of the Spinal Cord
Rare Diseases and Syndromes of the Spinal Cord
Ibrahim M. Eltorai The Spinal Cord Injury and Disorders Healthcare Group The Veterans Affairs Long Beach Healthcare System Long Beach, CA USA
ISBN 978-3-319-45146-6 ISBN 978-3-319-45147-3 (eBook) DOI 10.1007/978-3-319-45147-3
Library of Congress Control Number: 2016959045
© Springer International Publishing Switzerland 2016 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made.
Printed on acid-free paper
This Springer imprint is published by Springer Nature The registered company is Springer International Publishing AG Switzerland The registered company address is Gewerbestrasse 11, 6330 Cham, Switzerland
This book is dedicated to my beloved wife
Salwa
who was my guardian angel, my inspiration, friend, and the light of my life. Her guidance and support, for nearly 60 years, remain in my heart and mind and will never be forgotten.

Acknowledgments

• Department of the Veterans Administration, Long Beach, for their encourage­ment as a volunteer to continue this research.
• The Spinal Cord Injury Service at the Long Beach V.A. Medical Center, where I worked and conducted my research for nearly four decades.
• My assistant, Leslie K. Shimoda, who was instrumental in doing the meticulous literature research for the updates, bookkeeping, as well as typing the manuscript.
• Many thanks to the Springer Publishing Company staff and to everyone who helped with this book, including each editor individually.
• My grandson, Adam Eltorai, who is an MD/PhD candidate, for facilitating the communications with Springer, since my eyesight is very poor.
• Many thanks to the libraries of the Long Beach VA Medical Center and the University of California, Irvine, for giving me access to the literature research.
• Stanley van den Noort (1930–2009) the former Dean of Medicine at the medical school in Irvine, and chairman of the Neurosciences Department, was a great consultant. He was always following me and my progress on this book. He prom­ised to write a forward to this book, but unfortunately passed away in an accident before the publication of this book.
vii

Contents

Part I Congenital and Familial Causes of Myelopathy
1 Cervical Myelopathy Due to Exostosis of the Posterior
Arch of the Atlas ............................................. 3
2 Myelopathy Due to Congenital Hypoplasia of the Atlas ............. 7
3 Congenital Absence of the Posterior Elements of the Axis .......... 11
4 Congenital Afibrinogenemia .................................. 13
5 Anterior Arch Aplasia with OS Odontoideum .................... 17
6 Myelopathy in Pediatric Blau Syndrome ........................ 19
7 Intradural Extramedullary Bronchgenic Cyst .................... 21
8 Intramedullary Capillary Hemangioma ......................... 25
9 Intradural Extramedullary Capillary Hemangioma ............... 27
10 Spinal Intradural Cerebellar Ectopia ........................... 31
11 Cystic Dilatation of the Conus Ventriculus Terminalis ............. 33
12 Syringomyelia in Association with Dandy-Walker Syndrome ....... 37
13 Myelopathy Due to Duplication of the Laminae .................. 39
14 Compression Myelopathy Due to an Intramedullary
Cyst Containing Ectopic Choroidal Plexus Tissue ................. 41
15 Chiari 1 Malformation and Holocord Syringomyelia
in Hunter Syndrome ......................................... 43
16 Myelopathy in Infantile Myofibromatosis ....................... 47
17 Tetraparesis in Laurence-Moon-Biedl- Bardet Syndrome ........... 51
18 Lymphangiomatosis ......................................... 53
19 Ischemic Myelopathy Due to Marfan’s Syndrome. . . . . . . . . . . . . . . . . 55
20 Myelopathy in Neurocutatneous Melanosis ...................... 57
ix
x
Contents
21 Myelopathy in Osteogenesis Imperfecta ......................... 59
22 Prader-Willi Syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 63
23 Compression Myelopathy in Proteus Syndrome .................. 65
24 Segmental Spinal Dysgenesis .................................. 69
25 Ischemic Myelopathy Due to Sickle Cell Trait .................... 73
26 Sotos Syndrome as a Cause of Compression Myelopathy ........... 77
27 Atlantoaxial Instability in Spondyloepiphyseal
Dysplasia Congenita ......................................... 79
28 Brown Séquard Syndrome Associated with Sprengel’s
Deformity .................................................. 81
29 Synovial Chondromatosis ..................................... 85
30 Myelopathy Due to Tourette’s Syndrome ........................ 87
Part II Endocrine Causes of Myelopathy
31 Adrenomyeloneuropathy Causing Spastic Paresis ................. 93
32 Copper Deficiency ........................................... 97
33 Diabetic Myelopathy ........................................ 101
34 Cervical Myelomalacia in Laron Syndrome ..................... 105
35 Compression Myelopathy due to Tophaceous Gout. . . . . . . . . . . . . . . 109
Part III Iatrogenic
36 Myelopathy due to Acupuncture .............................. 113
37 Bronchial Artery Embolization (BAE) for Severe Hemoptysis ..... 117
38 Spinal Cord Infarction Following Central Line Insertion ......... 119
39 Chemical Myelopathy due to Cervical Myelography ............. 121
40 Myelopathy due to Cervical Spinal Manipulation ................ 125
41 Paraplegia Following Chemonucleolysis ........................ 129
42 Spinal Cord Injury After Chest Compressions .................. 131
43 Cervical Myelopathy Contrast Injection ....................... 133
44 Acute Transverse Myelitis in an Infant After Dtap Vaccination .... 135
45 Paraplegia After Endovascular Repair of Abdominal
Aortic Aneurysm ........................................... 139
Contents
46 Spinal Cord Infarction After Endoscopic Esophageal
Varix Ligation ............................................. 141
47 Subacute Combined Degeneration Provoked by Nitrous
Oxide Anethesia Patients with Cobalamin Deficiency ............. 143
48 Myelopathy due to Cervical Spine Flexion for Pediatric
Tracheal Resection ......................................... 147
49 Spinal Cord Stimulator Scar Tissue ........................... 151
50 Neonatal Paraplegia Through Umbilical Catheterization ......... 153
51 Malfunction of a Ventriculo-Peritoneal Shunt ................... 157
52 Viper Antivenin Induced Brown-Séquard Syndrome ............. 159
Part IV Idiopathic
53 Baastrup’s Disease Causing Neurogenic Claudication ............ 163
54 Castleman’s Disease ........................................ 167
55 Cruciate Paralysis .......................................... 171
56 Myelopathy Due To Gorham’s Disease ......................... 175
57 Hajdu Cheney Syndrome .................................... 179
xi
58 Holocord Myelopathy with Thoracic Stenosis ................... 181
59 Myelitis due to Hypertrophic Dens ............................ 183
60 Myelopathy due to Léri’s Disease ............................. 187
61 Idiopathic Primary Spinal Myoclonus ......................... 191
62 Idiopathic Retroperitoneal Fibrosis ........................... 193
63 Isolated Spinal Neuro-Behçet Disease .......................... 197
64 Subacute Progressive Ascending Myelopathy (SPAM) ............ 203
65 Idiopathic Spinal Cord Herniation ............................ 207
66 Spontaneous Spinal Epidural Hematoma ....................... 211
67 Tumoral Calcinosis of the Spine .............................. 215
68 Transient Cervical Myeloneurapraxia ......................... 219
Part V Infectious Cause of Myelopathy
69 Bacterial Meningitis in Adults ................................ 225
70 Guillain-Barré Syndrome Following Allogeneic Bone Marrow
Transplantation ............................................ 227