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25.6 Diagnosis
Diagnosis is based on the following criteria:
1. Ethnicity
2. Symptoms of anemia
3. History of other manifestation of sickle cell anemia e.g. acute attacks, fat embolism
4. Body pains, parathesia, anesthesia, muscle weakness, paresis, sphincter dysfunction
5. Change in mental status
6. High lesions may lead to respiratory distress or even arrest
7. Blood picture: sickle cell erythrocytes, evidence of anemia, hemolysis. ESR is
elevated.
8. CSF: high protein content and presences of red blood cells
9. MRI, MRA, and CT scan to rule out any hemorrhage and epidural abscess
25.7 Management
• hydration
• blood transfusion
• antibiotics: penicillin
• Tissue plasma activator (TPA) if bleeding is ruled out by MRI
• possibly exchange transfusion to reduce vascular occlusions
• antiplatelet drugs
• rehab therapy
• Hyperbaric oxygen (HBO) is being used in some centers especially in Europe for
treatment of stroke. It may be thought of in spinal cord infarct since there is no
evidence of its use.
Updating
Márquez et al. (2012)
Spinal cord ischemia due to sickle cell disease is extremely rare. To the best of
their knowledge only one case has been reported.
The patient was initially diagnosed by diffusion weighted images (DWI); follow-
up MRI confi rmed the diagnosis.
Case Report
A 19-year-old male African American developed acute fl accid tetraplegia with
bladder dysfunction. He was diagnosed with sickle cell disease at the age of 5. He
had multiple admissions for chest syndromes and pain crises; the last of which was
Updating
76
1 month prior to the present condition. He was diagnosed 6 years ago as having a
silent cerebral infarct. On admission, MRI of the spine was normal; however, DWI
showed a high signal in the spinal cord from C2–C7 with compounding restricted
diffusion on the attenuation diffusion coeffi cient (ADC) map suggesting infarction.
Three days later another MRI showed enlargement of the spinal cord at the same
levels. There was low intensity signal in T1-weighted images and high signal intensity on T2-weighted images. Another DWI showed restricted diffusion.
Management
Exchange transfusion keeping his hemoglobin A at a higher level than 70 % to prevent further ischemia.
Outcome
The patient improved with rehabilitation and was able to walk, although the left
upper extremity remained paralyzed.
Reference
Márquez JC, Granados AM, Castillo M. MRI of cervical spinal cord infarction in a
patient with sickle cell disease. Clin Imaging. 2012;36(5):595–8.
References
Loupy A, Laissy JP, Klein I, et al. Fat emboli unleashed: an exceptional etiology of encephalitis in
sickle cell disease. Ann Hematol. 2008;87(11):939–41.
Rothman SM, Nelson JS. Spinal cord infarction in a patient with sickle cell anemia. Neurology.
1980;30(10):1072–6.
Wolman L, Hardy AG. Spinal cord infarction associated with the sickle cell trait. Paraplegia.
1970;7(4):282–91.
25 Ischemic Myelopathy Due to Sickle Cell Trait
77
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_26
2 6
Sotos Syndrome as a Cause of Compression Myelopathy
26.1 Definition
Sotos syndrome (cerebral gigantism) is a rare overgrowth syndrome, characterized
by distinct facies, above average birth weight and length, joint laxity, pes planus,
acromegaly, excessive growth rate, advanced bone age, and developmental delay.
Very rarely are the spine or vertebra involved.
26.2 Incidence
Sotos syndrome only has slightly more than 300 cases reported in the English literature to date. Prior cases report only spinal or vertebral involvement with an increased
rate of scoliosis. However in 2004, Carlo and Dormans ( 2004 ) reported a patient
with Sotos syndrome and an incidental fi nding of cervical instability, causing C3–
C4 anterolisthesis with pinching of the spinal cord.
26.3 Etiology
The disease is congenital in origin, and the genetic code has not been reported.
26.4 Clinical Picture
In Sotos syndrome the child has a head circumference and height above normal. The
palms and feet length are at the 97th percentile. Generalize ligamentous laxity will
be noticed. In Carlo and Dormans’ case There is scoliosis and in the cervical spine
Abstracted from Carlo and Dormans ( 2004 )
78
there was 50 % anterolisthesis at C3–C4, which was incompletely reducible on
extension of the neck. There was impingement on the spinal cord for which the
patient had surgery manifested by hyperrefl exia in the lower extremities. In general,
children have higher rates of cervical laxity than adults because of the disproportionally large heads, horizontal orientation of cervical facet joints, and greater neck
weakness.
26.5 Diagnosis
The syndrome has some similarity to Downs except for the anterolisthesis at the
cervical region. Radiography reveals the anterolisthesis at C3–C4 in the case
described; magnetic resonance imaging (MRI) shows anterolisthesis causing mild
spinal canal stenosis with evidence of chronic compression and mild atrophy of the
spinal cord.
26.6 Management
Because of the cervical anterolisthesis and spinal cord compression surgery is indicated. Surgery was done by posterior approach and posterior spinal wire and fusion
of C3–C4 by wiring with titanium sublaminar cables and iliac crest autograft. The
postoperative was somewhat stormy but 10 weeks after surgery there was radiologic
evidence of stable reduction and arthrodesis of the fused segment. Carlo and
Dormans recommend awareness of the syndrome for early diagnosis and management before neurological injury.
Reference
Carlo W, Dormans JP. Cervical instability in Sotos syndrome: a case report. Spine.
2004;29(7):E153–6.
26 Sotos Syndrome as a Cause of Compression Myelopathy
79
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_27
2 7
Atlantoaxial Instability in Spondyloepiphyseal Dysplasia Congenita
27.1 Definition
Spondyloepiphyseal dysplasia congenita (SEDC) is a rare systemic skeletal disease
characterized by congenital dwarfi sm, a short trunk, and epiphyseal dysplasia of the
long bones and vertebral bodies and frequently suffers from atlantoaxial instability
associated with os odontoideum, leading to spinal cord compression and occasionally to sudden death.
27.2 Incidence
It is a rare disease. Eleven cases of SEDC were reviewed by Sawaizumi and
Kitamura and added a case of their own. The ages ranged from 5 to 56; the male to
female ratio is even.
27.3 Etiology
Congenital anomaly genetically not yet known.
27.4 Pathology
• Atlantoaxial instability
• Os odontoideum
• Spinal stenosis
• Scoliosis
Abstracted and reported by Gembun et al. ( 2001 )
80
27.5 Clinical Picture
Neurological symptoms may occur without trauma. Trauma may cause much damage to the spinal cord which may not recover after decompression.
27.6 Diagnosis
Diagnosis is based on the following criteria:
1. Radiography will reveal:
(a) Atlantoaxial instability
(b) Flexion extension views
(c) Os odontoideum
(d) Spinal stenosis
(e) Scoliosis
(f) Flat vertebrae
(g) Epiphyseal dysplasia of the long bones
2. Urine glucosamnogly level is normal to differentiate from Morquio’s disease
3. MRI: high signal lesion in T2 weighted images.
27.7 Management
Laminectomy of C1. Occipitocervical fusion using SS rod and bone iliac graft
Reference
Gembun Y, Nakayama Y, Shirai Y, Miyamoto M, Sawaizumi T, Kitamura S. A case report of spon-
dyloepiphyseal dysplasia congenita. J Nippon Med Sch. 2001;68(2):186–9.
27 Atlantoaxial Instability in Spondyloepiphyseal Dysplasia Congenita
81
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_28
2 8
Brown Séquard Syndrome Associated with Sprengel’s Deformity
28.1 Definition
Sprengel’s deformity (SD) is a congenital condition in which there is failure of the
scapular descent during intra-uterine growth. The neurological syndrome was due
to spina bifi da of C4–6 and defect of posterior arch of the fi fth cervical vertebra and
the presence of an ectopic bone on the left side of the spina bifi da. There was an
omovertebral bridge attached to cervical spine. The congenital anomalies lead to
Brown Séquard syndrome in the case described by Hauman et al.
28.2 Incidence
Sprengel’s deformity (SD) occurs in 1–2 per 3,000 births. The condition is usually
sporadic, but may be inherited as an autosomal dominant trait. Brown-Séquard syndrome (BSS) was only once reported and our search brought no other cases reported
since 1986
28.3 Etiology
SD is congenital and rare familial (Carno Disease). BSS was found to be due to
cervical spina bifi da, absence of hemi-arch of C5 and small mass of bone causing
compression of the cord on the left side.
Abstracted from Hauman et al. ( 1986 )
82
28.4 Pathology
There was cord compression which when relieved a neurological recovery was
attained. The deformity may be associated with syringomyelia, Klippel-Feil syndrome, or a cervical rib, hemivertebra, diastematomyelia, aortic cortication, hyperplasia of the shoulder muscles, pectoris major, serratus anticus, trapezius, and
latissus dorsi.
28.5 Clinical Picture
The patient recorded was 13 year old with inherited SD through his mother. He
developed right hemithermoanalgesia and pyramidal syndrome on the left. He was
diagnosed with BSS with C6 sensory level.
28.6 Diagnosis
Diagnosis is based on the following criteria:
1. right congenital elevated scapula
2. left pyrmadial hemiparesis
3. Radiography shows the following:
• Elevated right scapula
• Omovertebral bridge with upper angle of the scapula linked to the cervical
spine and is imbedded in the right half of the a large posterior arch defect of
C5
• Spina bifi da of C4 and C6
• To the left of the midline there is a boney fragment included in the spina bifi d a
4. Myelogram: showed a block at C4
5. CT scan showed:
• aplasia of the medial part of the left posterior arch and defi cit of C5
• aberrant boney fragment embedded spina bifi da
• compression of postrolateral aspect of the cervical dura
• spinal cord compression by the small left fragment at C4
• MRI was not performed
28.7 Management
Surgery consisted of:
• Removal of the boney fragment responsible for the compression from the cleft in
the posterior arch of C4
• Vertebral omo connection was not removed
28 Brown Séquard Syndrome Associated with Sprengel’s Deformity
83
The result of the surgery was regression of the spinal cord compression. The
authors recommend surgery to be done between 3 and 7 years of age; in older children there is an increased risk to the brachial plexus from stretching or compression
by the clavicle.
Reference
Hauman H, Wilms G, Roussel JM, Van den bergh R. Congenital elevation of the scapula and
Brown-Sequard syndrome. Clin Neurol Neurosurg. 1986;88(4):289–92.
Reference
85
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_29
2 9
Synovial Chondromatosis
29.1 Definition
This is a condition of cartilaginous proliferation with synovium and may be calcifi ed or ossifi ed. It may be extruded from the synovium, forming a loose body.
29.2 Incidence
The condition occurs in large joints most commonly in the knees. In the spine, only
nine cases have been reported. Median age is 39 years. There is a male to female
ratio of 5:4.
29.3 Etiology
There is no defi nite causative factor; however, the condition may be primary arising
in healthy joints or secondary in pathologic joints: osteoarthritic, rheumatoid and
neuropathic joints, osteonecrosis, tuberculosis, and osteochondral fractures.
Whether spinal cord is metaplastic or neoplasic the former is more rare.
29.4 Pathology
The tumor is well defi ned proliferating within the subsynovial space. The synovium
is preserve, but may be attenuated. The chondrocytes have small round nuclei, as
seen in benign chondromas. Some chondrocytes may have larger irregular nuclei.
Incomplete ablation results in recurrence (15 %). Gallia et al. ( 2004 ) reported two
Abstracted from Abdelwahab et al. ( 2008 ).