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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_6042_Библиотеки_им_академика_М_И_Перельмана.pdf
xii
Contents
71 Myelopathy due to Cladosporium trichoides
or Cladophialophora bantiana ................................ 231
72 Coxsackie Transverse Myelitis ................................ 235
73 Cytomegalovirus Myelitis .................................... 239
74 Degos Disease (DD) ......................................... 243
75 Dengue Myelitis ............................................ 247
76 Dental Extraction .......................................... 251
77 Holospinal Epidural Abscess ................................. 253
78 Imported Vaccine-Associated Paralytic Poliomyelitis ............. 255
79 Lemierre’s Syndrome ....................................... 259
80 Listeriosis ................................................. 261
81 Melioidosis ................................................ 265
82 Mumps Viraemia ........................................... 269
83 Myelitis due to Sparganosis .................................. 271
84 Toxocara canis ............................................. 275
85 Toxoplasmosis of the Spinal Cord ............................. 279
86 Trypanosomiasis ........................................... 283
87 Compression Myelopathy due to Typhoid Spondylodiscitis ........ 287
88 West Nile Virus Infection .................................... 289
89 Zoster Myelitis ............................................. 293
Part VI Neoplastic Causes of Myelopathy
90 Adamantinoma of the Spine .................................. 299
91 Amyloidosis ............................................... 301
92 Acute Paraplegia Secondary to Brown Tumor ................... 305
93 Myelopathy due to Chondroblastoma of the Spine ............... 309
94 Costal Osteochondroma Causing Compression Myelopathy ....... 313
95 Cutaneous Adenocystic Carcinoma ............................ 317
96 Myelopathy due to Intraspinal Desmoid Tumor ................. 319
97 Intramedullary Ectopic Adrenal Cortical Adenoma .............. 323
98 Spinal Tanycytic Ependymoma ............................... 325
99 Follicular Dendritic Cell Sarcoma (FDCS) ...................... 331
Contents
xiii
100 Left Atrial Papillary Fibroelastoma ........................... 335
101 Gliofibroma ............................................... 339
102 Glioneuronal Tumor ........................................ 343
103 Myelopathy in Primary Spinal Epidural Mantle
Cell Lymphoma ............................................ 345
104 Malingnant Eccrine Poroma ................................. 349
105 Melanoma ................................................ 353
106 Intramedullary Meningioma of the Cervical Spinal Cord ......... 357
107 Metastatic Retinoblastoma (Pediatric) ......................... 361
108 Infantile Myofibromatosis ................................... 363
109 Myopericytoma of the Thoracic Spine Causing Myelopathy ....... 367
110 Natural Killer T-Cell Lymphoma of the Cauda Equina ........... 369
111 Myelopathy in Neurocutatneous Melanosis ..................... 373
112 Anaplastic Oligodendroglioma ............................... 375
113 Oncocytoma in Melanocytoma ............................... 379
114 Osteosarcoma ............................................. 381
115 Myelopathy Due to Spinal Paraganglioma ...................... 385
116 Plasma Cell Leukemia (PCL) ................................ 389
117 Pleomorphic Xanthoastrocytoma ............................. 391
118 Primary Intraspinal Primitive Neuroectodermal Tumor (PNET) ... 395
119 Intramedullary Schwannoma Associated
with Neurofibromatosis ..................................... 399
120 Secondary Syringomyelia due to Spinal Intramedullary
Metastasis ................................................. 403
121 Spinal Hemangioblastomas in Von Hippel Lindau Disease ........ 407
122 Spinal Artery Steal Syndrome in Paget’s Disease of the Bone ...... 411
123 Intramedullary Teratoma .................................... 413
Part VII Toxic Causes of Myelopathy
124 Benzene Myelitis ........................................... 419
125 Myelopathy due to Flexion Drug Overdose ..................... 423
126 Myelopathy due to Heroin Addiction .......................... 427
127 Myelopathy due to Wasp Sting ............................... 431
xiv
Contents
Part VIII Vascular Causes of Myelopathy
128 Anterior Spinal Artery Syndrome ............................. 437
129 Cervical Myelopathy Caused by Bilateral Persistence
of the First Intersegmental Arteries ........................... 441
130 Myelopathy in Cobb Syndrome ............................... 445
131 Eale’s Disease .............................................. 449
132 Ectopic Choroidal Plexus Tissue .............................. 453
133 Myelopathy due to Fibrocartilaginous Spinal Cord Embolism ..... 455
134 Intravascular Papillary Endothelial Hyperplasia ................ 459
135 Posterior Spinal Artery Syndrome ............................ 463
136 Myelopathy due to Spinal Cord Vasculitis ...................... 465
137 Epidural Varix ............................................. 469
Part IX Miscellaneous Causes of Myelopathy
138 Paraplegia of Cerebral Origin ................................ 477
139 Decompression Sickness ..................................... 481
140 Exertional Myelopathy due to Congenital Kyphosis Type II ....... 485
141 Thoracic Compression Myelopathy due to Facet
Joint Osteophyte ........................................... 487
142 Hysterical Paralysis ........................................ 489
143 Differential Diagnosis: Hypermagnesemia ...................... 493
144 Spinal Epidural Lubricant Grease ............................ 495
145 Myelopathy due to Intramedullary Herniated Nucleus Pulposus ... 497
146 Spinal Cord Injury Without Radiologic Abnormality (Sciwora) .... 499
147 Surfer’s Myelopathy ........................................ 503
148 Trampoline Spinal Cord Injuries in Pediatrics .................. 507
149 Traumatic Tethering ........................................ 509
Conclusion .................................................... 513
About the Author
Dr. Ibrahim Eltorai completed his medical education at Cairo University in Egypt
(formerly King Fouad University, affiliated with London University) and then completed postgraduate training in Neurosurgery and Cardiovascular surgery at multiple European institutions. He was appointed Professor of Surgery at the Cairo
University School of Medicine. He has published nearly 150 articles in Egyptian
and European journals.
When Dr. Eltorai moved to the United States, he joined the Spinal Cord Injury
Service at the Veterans Administration Medical Center, Long Beach. Dr. Eltorai
worked closely with Dr. Ernst Bors and Dr. A. Estin Comarr on laying the foundations of spinal cord injury treatment as a medical specialty, which was taught not
only in the VA system, but also in hospitals nationwide. Dr. Eltorai practiced SCI
medicine in Long Beach, California, for more than 30 years until his retirement in
2004. After retirement, Dr. Eltorai served as a SCI medicine consultant and histo-
rian. Dr. Eltorai continues to research and document the history and the evolution of
SCI medicine until the present.
Dr. Eltorai published numerous clinical articles on SCI medicine and edited a
book on emergencies in spinal cord injury patients. He is a founding member and a
past President of the American Paraplegia Society, as well as, a Fellow of the
International Spinal Cord Society. Dr. Eltorai’s research on the evolution of SCI
medicine is currently displayed at the Long Beach V.A. Medical Center and will
soon be published in a book.
xv
Introduction
My interest in this subject goes back, close to 50 years, when I first emigrated to the
United States. At that time, searching for rare diseases I relied on the Index Medicus
monthly. I then copied them and currently they are stored at the Historical Archives
of SCI Medicine in Long Beach VA Medical Center, the first SCI historical library
in the world. The articles are in all languages, including English, French, German,
Dutch, Italian, and Spanish. Upon my retirement, I started working on updating
these subjects.
In order to fully understand the scope of this book, one must first look at what
defines a rare disease.
Statistically, there is no single cutoff number has been agreed upon for which a
disease is considered rare. A disease may be considered rare in one part of the
world, or in a particular group of people, but still be common in another. In the
United States, a disease might be considered rare, but common in other parts of the
world, which is especially true of genetic diseases and infectious diseases.
The exact definition of rare diseases is not well established and varies widely.
Some cases depend on the number of people living with the disease, while others
include other factors, such as the existence of adequate treatment or severity of the
disease. Globally, in the United States, the Rare Diseases Act of 2002 defines rare
disease strictly according to prevalence, specifically “any disease or condition that
affects fewer than 200,000 people in the United States,” or about 1 in 1,500 people.
This definition is essentially like that of the Orphan Drug Act of 1983, a federal law
that was written to encourage research into rare diseases and possible cures. In
Japan, the legal definition of a rare disease is one that affects fewer than 50,000
patients in Japan, or about 1 in 2,500 people. However, the European Commission
on Public Health defines rare diseases as “life-threatening or chronically debilitating diseases which are of such low prevalence that special combined efforts are
needed to address them.” The term “low-prevalence” is later defined as generally
meaning fewer than 1 in 2,000 people.
Diseases that are statistically rare, but not also life threatening, chronically debil-
itating, or inadequately treated, are excluded from their definition. The European
Organization for Rare Diseases (EURORDIS) estimates that as many as 5,000–
7,000 distinct rare diseases exist, and as much as 6–8 % of the population of the
European Union is affected by one.
xvii
xviii
Introduction
Rare diseases are usually genetic and are therefore chronic. EURORDIS esti-
mates that at least 80 % of them have identified genetic origins. Other rare diseases
are the result of infections and allergies or due to degenerative and proliferative
causes. Symptoms of some rare diseases may appear at birth or in childhood,
whereas others only appear once adulthood is reached.
Research publications emphasize rare diseases that are chronic or incurable,
although many short-term medical conditions are also rare diseases. The definitions
used in the medical literature and by national health plans are similarly divided,
with definitions ranging from 1 in 1,000 to 1 in 200,000.
This unique text is presented in a brief synopsis of the collected rare spinal cord
cases from the V.A. Long Beach SCI Historical Library. The references collected
range from 1930 to present. The information is collected from various languages,
including English, French, German, Dutch, Portuguese, Spanish, Italian, and even
abstracts in Russian, Japanese, and Chinese. Each case is illustrated with definition,
incidence, etiology, pathology, clinical picture, diagnosis, and management. These
subjects are divided into congenital (20 %), endocrinal (3 %), iatrogenic (11 %),
idiopathic (11 %), infectious (13.5 %), neoplastic (23 %), toxic (2 %), vascular (7 %),
and miscellaneous (8 %). This book is presented without illustrations or photos
mostly for space and for the reader focus on each case, individually.
References
Aymé S, Schmidtke J. Networking for rare diseases: a necessity for Europe. Bundesgesundheitsblatt
Gesundheitsforschung Gesundheitsschutz. 2007;50(12):1477–83.
Solly J, Shyndriayeva G. Managing research advances into a rare disease. Case study of the
Myrovlytis Trust. In: Sireau N, editor. Rare diseases, challenges and opportunities for social
entrepreneurs. Sheffield: Greenleaf Publications; 2013.
Van de laar FA, Bor H, Van de lisdonk EH. Prevalence of zebras in general practice: data from the
Continuous Morbidity Registration Nijmegen. Eur J Gen Pract. 2008;14 Suppl 1:44–6.
P a r t I
Congenital and Familial Causes of Myelopathy
Cervical Myelopathy Due to Exostosis of the Posterior Arch of the Atlas
1.1 Definition
Hereditary multiple exostosis (HME) is also called diaphyseal aclasis, multiple
echondromatosis, Ehrenfried disease, hereditary deforming chondrodysplasia, and
osteogenic disease. First described by Boyer in 1814, this condition has higher tendency to invade the spine and lead to myelopathy more than solitary osteochondromas, which occur mostly in the appendicular skeleton than the axial one (Mikawa
et al. 1997; Palmer and Blum 1980; Schmale et al. 1994).
1.2 Incidence
1
Incidence of HME is about 0.9 and 2 per 100,000 in Caucasian population, but not
quite accurate, some cases do not seek medical advice. Most vertebral exostosis
grow from the external parts of the laminae. Only one case was presented arising
from the posterior arch (Chooi et al. 2005). Spinal involvment is 7–9 % and usually
posterior elements leading to myelopathy.
1.3 Etiology
It is an autosomal dominant disorder, with full penetrance and has an equal gender
dominance (Peterson 1989).
Abstracted from Chooi et al. (2005)
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_1
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1 Cervical Myelopathy Due to Exostosis of the Posterior Arch of the Atlas
1.4 Pathology
These benign tumors usually arise from the metaphyseal regions of long bones of
the legs and occasionally from the ribs. During bone growth in childhood and adolescence the actively growing cartilaginous cap continues to grow. The incidence of
malignant transformation is 4–11 % (Decker and Wei 1969). Histiology shows
mature trabecular bone with a cartilaginous cap plate. The case described in the
posterior arch of the atlas, grew into the spinal cord and compressed the cord at C1.
1.5 Clinical Picture
The symptoms are delayed due t slow growth of the tumor up to 10 years. However,
sudden increase in size and pain or neurological changes may suggest malignancy.
In the case described by Chooi et al. (2005):
Progressive numbness and paresis of one arm and both legs over six months, neck pain
radiating to the arm, impaired functions of the hand, electrical shock radiating pain, bowel
and bladder incontinence, and unsteady gait. Her family history of HME.
1.6 Diagnosis
Diagnosis is based on
1. History of familial HME
2. Slow onset of neurological deficit, sensory-motor.
3. Plain radiography: Increased sclerosis in the region of the posterior arch of the
atlas.
4. MRI: exostosis from the right side of the posterior arch anteriorly and compressing the cord at C1.
5. CT Scan: pedunculated bony tumor from the posterior arch anteriorly encroaching on the spinal canal.
1.7 Management
A posterior hemi-laminectomy decompression procedure, the right posterior arch of
the atlas removed with a burr. Recovery was uneventful and neurological improvement was noted.
References
Chooi YS, Siow YS, Chong CS. Cervical myelopathy caused by an exostosis of the posterior arch
of C1. J Bone Joint Surg [Br]. 2005;87(B):257–9.
References
Decker R, Wei W. Thoracic cord compression from multiple hereditary exostosis associated with
cerebellar astrocytoma: case report. J Neurosurg. 1969;30:310–2.
Mikawa Y, Watanabe R, Nakashima Y, Hayashida T. Cervical spinal cord compression in heredi-
tary multiple exostoses: report of a case and review of the literature. Arch Orthop Trauma Surg.
1997;116:112–7.
Palmer F, Blum P. Osteochondroma with spinal cord compression. J Neurosurg. 1980;52:842–5.
Peterson H. Multiple hereditary osteochondromata. Clin Orthop. 1989;239:222–30.
Schmale GA, Conrad EU, Raskind WH. The natural history of hereditary multiple exostoses.
J Bone Joint Surg Am. 1994;76(A):986–92.
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