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proposed predominant pathophysiological mechanisms for
neurologic injury.
66. Which of the following is true regarding Foix-
Alajounine syndrome?
(a) is also known as subacute ascending necrotizing
myelitis
(b) is a result of symptomatic spinal dural arteriove-
nous malformations
(c) symptoms include myelopathy, radiculopathy and
sphincter dysfunction
(d) treatment consists of fistula disconnection
(e) all of the above
Answer (e)
All of the presented options are true.
Use the following figure (see Fig. 9.15) to answer ques-
tions 67–80.
67. A 55-year-old female presents to the emergency room
with multiple falls, bilateral upper extremity weakness
and bilateral positive Hoffmann signs. She has been
using a walker for the past 2 weeks due to the increased
falls. MRI of the cervical spine with and without con-
trast was performed (shown). What is the most likely
diagnosis?
(a) acute spinal cord injury
(b) magnetic artifact- CSF flow voids
(c) spinal cord arteriovenous malformation
(d) acute disc herniation
(e) epidural hematoma
Answer (c)
Notice the posterior epidural serpiginous blood vessels
and the intradural nidus at the C5 level with surrounding
myelomalacia, class ic of true arteriovenous malformations,
also known as Type 2 spinal vascular malformation or
Glomus AVM.
68. What is the next best step for management of this
patient?
(a) start high dose corticosteroids
(b) spinal angiogram
(c) emergent fistula clipping/disconnection
(d) close observation
(e) endovascular embolization of associated spinal
radicular artery
Answer (b)
A spinal angiogram is the next best step. It will provide
additional information about nidus architecture, adjacent
anatomy, associated vascular lesions and lesion flow. Cor-
ticosteroid use in this scenario is somewhat controversial.
Observation in a myelopathic patient might not be in the
patient’s best interest. Emergent treatment is usually not
necessary unless there is acute, compressive hemorrhage.
69. What is the gold standard for treatment of this lesion?
(a) clipping of the fistulous connection
(b) resection of the nidus
Fig. 9.15 Axial and Sagittal T2t MRI of the cervical spine
180 R. Nunna et al.
(c) high dose corticosteroids
(d) endovascular embolization
(e) none of the above
Answer (b)
In contrast with arteriovenous fistulas, which are treated
with precise clipping of the fistulous connection, true arte-
riovenous malformations can be potentially cured with
resection of the nidus. Embolization of feeding arteries may
make the nidus smaller and reduce the risk of hemorrhage
but is rarely curative and risks occlusion of the nidus’
venous outflow and, consequently, AVM rupture.
70. What is the Aminoff-Logue Disability Scale grade for
this patient?
(a) 0
(b) 1
(c) 2
(d) 3
(e) 4
(f) 5
Answer (e)
The Aminoff-Logue Disability Scale is a scoring system to
stratify the functional status of patients with spinal dural
arteriovenous fistulas. Grade 0 is normal. Grade 1 is weak-
ness, gait disturbances but no restriction of activities. Grade 2
is restricted activity. Grade 3 is a patient who requires a cane,
stick or some type of simple support for walking. Grade 4 is a
patient who requires crutches or a walker. Grade 5 is a patient
who cannot stand and is bed- or wheelchair-bound.
71. Which of the following is the reported annual risk of
hemorrhage for a spinal AVM?
(a) 0.5–1%
(b) 1–3%
(c) 3–5%
(d) 5–10%
(e) > 10%
Answer (c)
The average annual risk of hemorrhage from a spinal
AVM is estimated to be 4% and is markedly increased
(two-fold) in patients with prior hemorrhage and associated
aneurysms.
72. Spinal cord cavernous malformations account for what
percentage of all central nervous system cavernous
malformation?
(a) 1%
(b) 5%
(c) 10%
(d) 15%
(e) 25%
Answer (b)
5% of all cavernous malformations of the central venous
system are spinal cord cavernous malformation. 20% of
patients with spinal cord cavernous malformations also have
an intracranial malformation.
73. A 27-year-old Hispanic patient presents to your clinic
for evaluation of an abnormal spinal cord finding. MRI
revealed T2 hyperintense, non-contrast-enhancing
intramedullary lesion at the level of the conus medul-
laris. The lesion demonstrates a “popcorn” appearance.
She also has a history of seizures but has never had a
brain MRI performed. Which of the following is the
most appropriate recommendation?
(a) obtain a brain MRI
(b) discuss genetic testing
(c) recommend open resection
(d) a & b
(e) a & c
Answer (d)
Familial Multiple Cavernous Malformation Syndrome
(FMCMS) is an autosomal dominant disease that results in
the occurrence of multiple brain and spinal cord vascular
malformations. There is a high incidence in patients with
Hispanic descent, classically Mexican descent. Patients with
FMCMS are more likely to have spinal cord cavernous
malformations.
74. The vascular supply for spinal arteriovenous malfor-
mations most commonly derives from which of the
following?
(a) anterior spinal artery
(b) posterior spinal artery
(c) radicular arteries
(d) paraspinal arteries
(e) none of the above
Answer (c)
The radicular arteries are the most common source of
vascular supply for these lesions. These arteries arise from
the T6-L2 levels and are branches of the artery of
Adamkiewicz.
75. Which of the following are common findings in patients
with spinal cord arteriovenous malformations?
(a) progressive myelopathy
(b) paresthesia
(c) weakness
9 Tumors, Infections, Inflammatory and Vascular Lesions of the Spine 181
(d) back pain
(e) all of the above
Answer (e)
Progressive myelopathy, weakness, paresthesia and back
are all symptoms attributable to these lesions. Neurologic
deficits can sometimes be reversible, related to vascular steal
phenomena, and can be the diagnosis elusive. A patient with
any of the above symptoms and a suspicious spinal cord
imaging finding should have an MRI of the entire spine with
and without contrast for adequate diagnosis.
76. Of the following joints, which is frequently affected in
rheumatoid arthritis (RA)?
a. Distal interphalangeal joints
b. First carpometacarpal joint
c. Atlantoaxial joint
d. Lumbosacral joint
Answer: (C) Atlantoaxial joint. The distal interphalangeal
joints and the first carpometacarpal joints, unlike the other
wrist joints and the proximal interphalangeal joints, are
rarely affected in patients with rheumatoid arthritis. The axial
skeleton is also rarely affected except for the cervical spine,
which is affected in up to 90% of RA patients.
77. A 58-year-old woman presents to the clinic concerned
about swollen and tender wrists and knees that are stiff
primarily shortly after waking up. She also complains
of an inability to completely extend her right index
finger at the distal joint. On physical exam, nontender
subcutaneous nodules about 5 mm in size were noted
on the posterior surface of the left forearm. What
pharmacologic agent would likely be indicated for
long-term control in this patient?
a. Methotrexate
b. Hydroxyurea
c. Tofacitinib
d. Rituximab
Preferred Response: (A) Methotrexate. Methotrexate is a
folate analog with anti-inflammatory properties and is rec-
ommended as the first-line treatment for patients presenting
with moderate to severe rheumatoid arthritis. It has no
contraindications for use. Hydroxyurea is a ribonucleotide
reductase inhibitor that is used as a chemotherapy for
myeloproliferative diseases and can be used to induce fetal
hemoglobin synthesis in patients with beta-thalassemia or
sickle cell disease. However, it has not been shown to benefit
rheumatoid arthritis patients. Tofacitinib is a JAK inhibitor
that is used as a second-line therapy for patients in which
methotrexate does not satisfactorily alleviate symptoms.
Rituximab is a tumor necrosis factor a inhibitor that is
indicated after methotrexate fails to satisfactorily alleviate
symptoms following at least three months of therapy.
78. What is recommended in rheumatoid arthritis patients
prior to elective surgery?
a. Discontinue methotrexate use
b. Obtain flexion–extension x-rays of the c-spine
c. Start adalimumab and continue through operation
d. Discontinue daily glucocorticoids
Answer: (B) Obtain flexion-extension x-rays of the c-spine.
Up to 90% of patients with rheumatoid arthritis will p resent
with rheumatoid cervical spondylitis, and the diagnosis is
often missed. Preoperative flexion-extension radiographs
help evaluate the position of C1 relative to C2 and gauge the
risk of spinal cord compression and injury. It is generally
recommended to continue methotrexate and glucocorticoids
throughout surgery. Tumor necrosis factor a antagonists
such as adalimumab have the potential to lead to infections
as they intervene in immunological processes. Thus, surg-
eries should be planned approximately 13 weeks after the
last dose with the agent not being restarted until 10-14 days
postoperatively.
79. What laboratory test is the strongest indicator of
rheumatoid arthritis?
a. Rheumatoid factor
b. Anti-cyclic citrullinated peptide antibodies
c. Anti-nuclear antibodies
d. C-reactive protein
Answer: (B) Anti-cyclic citrullinated peptide antibodies. In
the past, rheumatoid factor (RF) was used as a marker for
rheumatoid arthritis. However, anti-cyclic citrullinated pep-
tide (anti-CCP), although having similar sensitivity for RA
(*70% compared to 60-80% for RF), is much more specific
for the disease than RF (>90% for anti-CCP vs. 85% for RF).
Anti-nuclear antibodies and C-reactive protein can also be
elevated and reflective of the severity of the disease (espe-
cially CRP).
80. Which of the following is true regarding the etiology of
rheumatoid arthritis?
a. Autoimmune attack primarily occurs against carti-
lage and bone
b. Mononuclear cells mediate tissue destruction
182 R. Nunna et al.
c. IL-1 receptor antagonists are ineffective in mitigat-
ing the disease process
d. Genetics is not believed to be a risk factor for
developing the disease
Answer: (B) Mononuclear cells mediate tissue destruction. There is
substantial evidence for a role for CD4
+
helper T cells in rheumatoid
arthritis, which is characterized by autoimmune attack beginning in
the soft tissues that then extends to cartilage and bone later in the
course of the disease. T cells activated through interactions between
the T cell receptor and class II MHC-peptide antigens can in turn
activate B cells, some of which are destined to dif ferentiate into
autoantibody-producing plasma cells. This process leads to the
formation of immune complexes that could include rheumatoid
factors and anti-cyclic citrullinated peptide antibodies inside the
joint, amplifying inflammation. Effector T cells stimulate macro-
phages and fibroblasts in the synovial joint to secrete proinflam-
matory mediators, including tumor necrosis factor a (TNF-a).
TNF-a upregulates adhesion molecules on endothelial cells, pro-
moting leukocyte influx and production of inflammatory mediators
such as interleukin 1 (IL-1) and IL-6. Therefore, IL-1 receptor
antagonists (such as anakinra) are effective in alleviating
RA-associated inflammation. TNF-a also serves to inhibit bone
formation and, with other pathways, induce osteoclastogenesis and
therefore bone erosion. Major histocompatibility complex alleles
HLA-DR1 and HLA-DR4 are associated with an increased risk for
RA as well as a host of other autoimmune diseases including type I
diabetes mellitus and Addison’s disease.
81. Which of the following is true regarding rheumatoid
arthritis therapies?
a. Glucocorticoids are recommended for long-term
symptom control
b. Chosen DMARD therapy should be re-evaluated if
minimal disease activity or remission is not achieved
by six months
c. Newer targeted and biologic DMARDs are now
preferred to conventional long-term therapies
d. Targeted DMARDs have few er immunological side
effects than conventional thera pies
Answer: (B) Chosen DMARD therapy should be
re-evaluated if minimal disease activity or remission is not
achieved by six months. Glucocorticoids should be used at
the lowest dose possible for the shortest amount of time
possible due to its significant potential for toxicity. Con-
ventional long-term DMARDs (such as methotrexate) is still
the first line treatment for moderate to severe RA, and
methotrexate monotherapy is strongly recommended over all
other synthetic and biologic DMARDs in patients with
severe disease. Furthermore, patients taking targeted
DMARDs have the same potential to acquire severe infec-
tions as patients on traditional methotrexate therapy.
82. Which of the following is true regarding subaxial sub-
luxation in rheumatoid arthritis?
a. Lower c-spine instability is more common than
atlantoaxial instability
b. Atlantoaxial c-spine instability is typically not seen
with subaxial subluxation
c. Steroid therapy can help prevent lower c-spine
instability
d. Subaxial subluxation is often observed in patients
with subcutaneous rheumatoid nodules
Answer: (D) Subaxial subluxation is often observed in
patients with subcutaneous rheumatoid nodules. Lower
c-spine instability is observed in approximately 20% of
patients with rheumatoid arthritis while the incidence of
atlantoaxial instability is 50-80%. Upper c-spine instability
is often seen in patients presenting with lower c-spine
instability. Steroid therapy can cause decreased bone mineral
density resulting in c-spine instability and is a significant risk
factor for subaxial subluxation.
83. Which radiographic finding is most consistent with
cranial settling in rheumatoid arthritis?
a. Superior tip of the dens is 5 mm above McGregor’s
line
b. C2 is 15 mm below the line connecting the anterior
and posterior arches of C1
c. McRae’s line is 2 mm above the superior tip of the
dens
Answer: (A) Superior tip of the dens is 5 mm above
McGregor
’s line. McGregor’s line is defined as the line from
the posterior edge of the hard palate to the caudal posterior
occiput curve. Cranial settling is present when the superior
tip of the dens is greater than 4.5 mm above this line. The
Ranawat C1-C2 index is defined as the distance from the
center of C2 pedicle to a line connecting the anterior and
posterior C1 arches. Normal measurement in men is around
17 mm and 15 mm in women, and a distance of less than 13
mm is consistent with impaction. McRae’s line defi nes the
opening on the foramen magnum. The tip of the dens may
protrude slightly above this line, but if the dens is below this
line, then impaction is not present (see Fig. 9.16).
84. A 68-year-old woman with rheumatoid arthritis pre-
sents with a Ranawat C1-C2 index of 11 mm and an
9 Tumors, Infections, Inflammatory and Vascular Lesions of the Spine 183
odontoid process protruding 2 mm above McRae’s line
on radiograph. What would be the next steps in man-
agement for this patient?
a. C2 to occiput fusion
b. Posterior fusion
c. Start high dose glucocorticoid therapy
d. Obtain CT and MR images
Preferred Response: (D) Obtain CT and MR images.
A Ranawat index less than 13 mm and odontoid process
above McRae’s line are strongly suggestive of atlantoaxial
subluxation with cranial settling. In such patients,
occiput-C2 fusion is indicated. However, it is prudent to
obtain further imaging prior to operating. CT is useful to
better delineate bony anatomy. MRI will help evaluate the
degree of spinal cord compression and identify myeloma-
lacia, thus determining whether resection of the posterior C1
arch is necessary for complete decompression.
85. Which of the following is indicated in patients pre-
senting with Ranawat class IIIB myelopathy?
a. Posterior C1-C2 fusion
b. Transoral retropharyngeal odontoid resection
c. C2 to occiput fusion with resection of posterior C1
arch
d. No surgical intervention is indicated
Preferred Response: (D) No surgical intervention is indi-
cated. Ranawat classifications for cervical myelopathy range
from I (pain, no neurologic deficit) to IIIB (objective
weakness, long tract upper motor nerve signs, and an
inability to ambulate). Patients with class IIIB myelopathy
have severe basilar invagination. Surgical intervention is
extremely risky and associated with a poor prognosis. C2 to
occiput fusion or odontoid resection is indicated in patients
with basilar invagination, and posterior C1-C2 fusion is
indicated to resolve atlantoaxial instability. However, the
three options would be contraindicated due to poor likeli-
hood of success.
85. When should a resection of the posterior C1 arch be
considered to resolve symptomatic atlantoaxial
subluxation?
a. Subaxial subluxation > 4 mm
b. Anterior atlanto-dens interval > 10 mm
c. Posterior atlanto-dens interval < 14 mm
d. Ranawat C1-C2 index < 13 mm
Answer: (D) Ranawat C1-C2 index < 13 mm. Subaxial
subluxation greater than 4 mm indicates cord compression
and is treated by posterior fusion and wiring at the level of
subluxation. An anterior atlanto-dens interval (AADI)
greater than 10 mm and posterior atlanto-dens interval
(PADI) less than 14 mm are general indications for surgery
but are not in and of themselves an indication for resection
of the posterior C1 arch. Often, a posterior C1-C2 fusion is
sufficient to resolve symptom s and decompress the spinal
cord. Subaxial subluxation does not call for decompression
at the C1 level. A Ranawat index less than 13 mm suggests
impaction due to basilar invagination. A C2 to occiput
fusion is the recommended approach, but C1 posterior arch
resection may be required if the atlantoaxial subluxation is
not reduci ble.
86. Which of the following is not an indication for a pos-
terior fusion?
a. Anterior atlanto-dens interval of 11 mm between
flexion and extension views
b. Ranawat C1-C2 index of 7 mm and indications of
brain stem compromise
c. Subaxial subluxation of 5 mm
d. Cervicomedullary angle of 120º on MRI
Answer: (B) Ranawat C1-C2 index of 7 mm and indications
of brain stem compromise. Whenever there are indications of
brain stem compromise with basilar invagination, a transo-
ral, anterior retropharyngeal or endonasal odontoid resection
Fig. 9.16 Lateral XR of the cervical spine with depiction of cervico
occipital parameters (from: Wasserman, Bradley R. et al. “Rheumatoid
arthritis of the cervical spine–clinical considerations.” Bulletin of the
NYU hospital for joint diseases 69 2 (2011): 136–48) [93]
184 R. Nunna et al.
is suggested. For atlantoaxial subluxation (as suggested by
an AADI greater than 3.5 mm between flexion and extension
views), posterior C1-C2 fusion is indicated. Posterior fusion
of affected cervical levels is indicated with subaxi al sub-
luxations greater than 4 mm. A cervicomedullary angle of
less than 135º in the context of basilar invagination suggests
impending neurologic impairment which would be surgi-
cally treated by a C2 to occiput fusion or an odontoid
resection.
87. What finding is most suggestive of neurologic com-
promise in subaxial subluxation due to rheumatoid
arthritis?
a. Cervical height index < 2.0
b. Subluxation > 4 mm/20%
c. Presence of > 1 subluxation
d. Presence of subcutaneous rheumatoid nodules
Answer: (A) Cervical height index < 2.0. The cervical height
index is a method for assessing subaxial subluxation that
accounts for the contribution of subluxations at multiple
levels along with the loss of disk height and bony collapse. It
is calculated by first determining the distance from the center
of the C2 sclerotic ring to the tip of the C2 spinous process.
This distance is then divided by the distance from the center
of the C2 sclerotic ring to the midpoint of the inferior border
of the C7 vertebral body by the distance. A cervical height
index less than 2.0 is almost 100% sensitive and specific for
predicting neurologic compromise. Subluxation greater than
4 mm or 20% and the presence of more than one subluxation
may increase the risk for neurologic compromise but are not
particularly sensitive nor specific. The presence of subcuta-
neous rheumatoid nodules, while associated with subaxial
subluxation, has not been shown to be associated with
neurologic compromise.
88. When is conservative management NOT advised over
surgical intervention?
a. 5 mm subaxial subluxation without concomitant
pain or neurologic symptoms
b. Ranawat C1-C2 index of 15 mm with neurologic
symptoms
c. Posterior atlanto-dens interval of 12 mm without
neurologic symptoms
d. Lateral subluxation of C1 on C2
Answer: (C) Posterior atlanto-dens interval of 12 mm
without neurologic symptoms. A PADI of less than 14 mm
is an indication for posterior C1-C2 resection regardless of
the presence of neurologic symptoms due to the significantly
increased risk for injury. The other answer choices indicate
alterations of the vertebral canal that may result in neuro-
logic compromise but can be conservatively managed as
long as evidence of compromise does not manifest.
89. A 65-year-old woman diagnosed with rheumatoid
arthritis ten years ago comes into the ED with ataxia,
headache, neck pain and stiffness. Physical exam
reveals bilateral upper and lower extremity weakness,
patellar hyperreflexia, and loss of sensation in the hands
and toes although she is still ambulatory. Flexion–ex-
tension XR reveals a space available for cord (SAC) of
12 mm. She had a posterior C1-C2 fusion a little less
than one year ago. What intervention would be rec-
ommended in this case?
a. Occiput-C2 fusion with resection of posterior C1
arch
b Odontoidectomy
c. Posterior C1-C2 fusion
d. Conservative management with steroid therapy
Preferred Response: (B) Odontoidectomy. Odontoidec-
tomies are rarely indicated for simple atlantoaxial instability
but are used as a secondary procedure when there is residual
anterior cord compression following a posterior spinal
fusion. In this case, the residual compression is likely due to
pannus formation that failed to resolve with time as it usually
would following posterior fusions. Occiput-C2 fusion is
typically only indicated with basilar invagination. Posterior
C1-C2 fusion, which failed once, is unlikely to work. Con-
servative management is not indicated in this patient as the
space available for the spinal cord (SAC, which is equivalent
to PADI) is less than 14, indicating a significant risk for
neurologic injury without surgical intervention.
90. Which MHC allele is commonly found in patients with
ankylosing spondylitis (AS) ?
a. HLA-DQ8
b. HLA-DR4
c. HLA-B27
d. HLA-A3
Answer: (C) HLA-B27. Greater than 90% of patients pre-
senting with ankylosing spondylitis present with the
HLA-B27 allele of major histocompatibility complex
(MHC) I. However, fewer than 10% of HLA-B27-positive
patients have symptoms of AS. The exact mechanism by
which HLA-B27 contributes to AS is unknown, but it is
speculated that HLA-B27 can aggregate with peptides in the
joints to lead to a degenerative cascade. Alternatively, it also
9 Tumors, Infections, Inflammatory and Vascular Lesions of the Spine 185
has been speculated that HLA-B27 can cause a cytotoxic
T-cell autoimmune reaction, leading to joint inflammation
and degeneration. HLA-DQ8 is a MHC I allele associated
with gluten-sensitive enteropathies–such as Celiac disease.
HLA-DR4 is an MHC II allele that is associated with
rheumatoid arthritis (RA), type 1 diabetes mellitus, pem-
phigus vulgaris, and Addison’s disease. HLA-A3 is an
MHC I allele associated with hemochromatosis.
91. Which is true regarding the pathogenesis of ankylosing
spondylitis?
a. Soft tissue, bone, and cartilage are destroyed in a
TNF-mediated autoimmune reaction
b. Decreased levels of endogenous bone morpho-
genetic protein (BMP)-inhi bitors may contribute to
syndesmophyte formation
c. A hyperinsulinemic state may lead to an increase in
calcium deposits leading to syndesmophyte formation
d. Bony erosion occurs due to pervasive RANKL-
expressing cells
Answer: (B) Decreased levels of endogenous BMP-
inhibitors may contribute to syndesmophyte formation.
Recent resear ch has demonstrated that levels of sclerostin,
DKK1, endogenous inhibitors of BMP are decreased in AS
patients. In rheumatoid arthritis, a TNF-mediated autoim-
mune reaction results in the destruction of soft tissue, bone,
cartilage. The TNF-mediated reaction leads to a signaling
cascade that results in the infiltration of RANKL-expressing
cells, resulting in non-osteoclast-mediated bony erosion. In
diffuse idiopathic skeletal hyperostosis, hyperinsulinemia is
believed to lead to an increase in calcium deposits which in
turn leads to nonmarginal syndesmophyte formation.
92. What treatment is considered to be most vital in the
treatment of ankylosing spondylitis?
a. NSAIDs
b. Regular physical therapy
c. Infliximab
d. Tofacitinib
Answer: (B) Regular physical therapy. Regular physical
therapy best facilitates the maintenance of range of motion
and posture— a critical goal for AS patients. NSAIDs are the
first-line pharmacological therapy for AS; however, their
utility is largely to help control joint pain and inflammation.
Infliximab, a tumor necrosis factor (TNF) a inhibitor, is a
second-line therapy used in AS that blocks TNF-a, a key
protein in the inflammatory chain reaction that leads to joint
inflammation, pain, and injury. Tofacitinib, a Janus kinase
(JAK) inhibitor, has been shown to significantly increase the
ASAS40 response rate (the rate at which patients achieve an
improvement of at least 40% on the Assessment in Anky-
losing Spondylitis). However, it typically only considered
for use in patients who do not respond to NSAIDs, TNF-a
inhibitors, or antiinterleukin-17 antibodies.
93. What labs are useful for establishing a diagnosis of
ankylosing spondylitis?
a. Erythrocyte sedimentation rate
b. Rheumatoid factor
c. Anti-nuclear antibodies
d. Urine phosphoethanolamine
Answer: (A) Erythrocyte sedimentation rate. Labs generally
have little diagnostic value for ankylosing spondylitis. Diag-
nosis of AS is primarily made from a clinical and radiographic
standpoint. According to the Assessment of Spondyloarthritis
International Society, diagnosis of AS can be made in a
patient with lower back pain for greater than three months in
patients less than 45 years of age and presenting with either:
(1) sacroiliitis confirmed by XR or MRI and at least one
typical clinical or laboratory finding or (2) a positive
HLA-B27 test and at least two typical clinical or laboratory
findings. Relevant clinical findings include inflammatory back
pain, dactylitis, arthritis, enthesitis, uveitis, IBD, psoriasis,
positive family history for AS, symptom relieved in 24-48
hours by NSAIDs. Laboratory fi
ndings include positive
HLA-B27 and elevated ESR and CRP.
Rheumatoid factor and ANA are negative—which is why
AS is often referred to as a seronegative spondyloarthropa-
thy. Urine phosphoethanolamine is elevated in hypophos-
phatasia and is not useful for diagnosing AS.
94. A 40-year-old male presents with lower back stiffness
and pain that has progressively gotten worse over the
past year. A radiograph has already been obtained and
is shown below (see Fig. 9.17). What additional finding
would support a diagnosis of ankylosing spondylitis?
a. Subcutaneous nodules
b. Uveitis
c. Type 2 diabetes mellitus
d. Psoriatic lesions on the elbows and knees
Answer: (B) Uveitis. Acute anterior uveitis and iritis is a
common systemic manifestation of ankylosing spondylitis.
Subcutaneous nodules can be found in patients with
rheumatoid arthritis but not in AS patients. Diffuse idio-
pathic skeletal hyperostosis is often found in patients with
type 2 diabetes mellitus and in other conditions associated
186 R. Nunna et al.
with increased insulin levels in the blood and can present
similarly to AS. Type 2 DM has not been shown to be
associated with AS. Psoriatic lesions indicate the presence of
psoriasis which can cause psoriatic arthritis, which is distinct
from AS.
95. Which finding is the most specific for diagnosing
ankylosing spondylitis?
a. Decreased spine flexion
b. Positive Faber test
c. Positive HLA-B27
d. Limitation of chest wall expansion to < 2 cm
Answer: (D) Limitation of chest wall expansion to < 2 cm.
In the context of inflammatory back pain with insidious
onset in the 3
rd
decade of life, limitation of chest wall
expansion to less than two centimeters is the most specific
indicator of ankylosing spondylitis; it is even more specific
than the presence of the HLA-B27 allele, which is presen t in
90-95% of AS patients. Decreased spine motion is often
found in AS patients, and the Schober test can be used to
evaluate lumbar stiffness. The Faber test, a sacroiliac
provocative test in which the hip is flexed, abducted, and
externally rotated, can be used to determine if there is
inflammation of the sacroiliac joint. A positive Faber test, in
which the flexion, abduction, and external rotation of the hip
causes pain, is a typical finding in AS.
96. In patients with ankylosis spondylitis, spinal fractures
can occur after minor trauma or even spontaneously.
What should NOT be done in treatment following
trauma in an AS patient?
a. Place the patient in a cervical collar
b. Perform a full neurological examination
c. Perform CT to assess for spinal fractures
d. Maintain the patient’ s preinjury spinal alignment
Answer: (A) Place the patient in a cervical collar. As many
AS patients have a fixed kyphosis, attempting to reposition
the spine into a neutral position by placing the occiput,
shoulders, and hips in alignment can result in spinal cord
injury. As a result, it is best to attempt to maintain the
patient’s preinjury alignment. This can be achieved using
pillows or wedges underneath the head and upper back.
Performing a full neurological examination and getting a CT
or MRI to assess for fractures is standard protocol for trauma
suspected to involve the spine. Spinal fracture should have a
higher index of suspicion in patients with known AS.
97. A 35-year-old man comes into the ED after slipping
while shoveling snow off his driveway. He reports new
sharp, severe pain in his lower neck, but neurological
exam reveals no deficits with + 2 DTRs throughout and
strength 5/5. Past medical history is significant for
ankylosing spondylitis for which he is taking meloxi-
cam and etanercept. A CT was obtained which revealed
an extension-type fracture at C8 with no evidence of an
epidural hematoma. Twelve hours later, the patient
reported feeling numbness in his arms and legs, and
neurological exam revealed a decreas e in strength of
plantar and dorsiflexion. An MRI was emergently
Fig. 9.17 AP XR of the sacro
iliac joints. Credit to www.
radiologykey.com
9 Tumors, Infections, Inflammatory and Vascular Lesions of the Spine 187
obtained which revealed an epidural hematoma located
posteriorly to the spinal cord. What is the appropriate
next step in management?
a. Start high-dose IV methylprednisolone
b. Anterior decompression with fusion
c. Posterior laminectomy without fusion
d. Posterior laminectomy with fusion
Preferred Response: (D) Posterior laminectomy with fusion.
High-dose methylprednisolone has been shown to have
neuroprotective effects following spinal cord injury. It is
believed that this effect is mediated by limiting posttraumatic
oxygen free radical-induced lipid peroxidation. However,
given that this patient is experiencing acute neurologic
compromise due to spinal cord compression urgent surgical
intervention is indicated in this situation. Given that the
source of compression is located posteriorly to the spinal
cord, the surgical approach should be posterior. Instru-
mented fusion is recommended in patients with AS due to
long lever arms of the ankylosed spine and likely presence of
osteoporosis.
98. A 67-year-old man presents with an inability to lift his
head and look ahead. Radiographs reveal a severe cer-
vicothoracic “chin-on-chest” deformity (see Fig. 9.18).
He was diagnosed with ankylosing spondylitis ten years
ago, but despite pharmacologic management with
ibuprofen, infliximab, and tofacitinib, his condition
progressively got worse. What is the most appropriate
management for this patient?
a. Posterior fusion in situ
b. Anterior osteotomy with posterior fusion and
instrumentation
c. Posterior extension osteotomy with fusion and
instrumentation
d. Halo immobilization
Preferred Response: (C) Posterior osteotomy with fusion
with instrumentation. This patient suffers from a progres-
sively worsening kyphotic deformity of his cervicothoracic
spine (see XR below). Posterior extension osteotomy will
correct the deformity and subsequent fusion with instru-
mentation will prevent subluxation of the osteotomy site. It
is recommended that the osteotomy be performed at the
C7-T1 junction due to various anatomic advantages,
including that the vertebral vessels are anterior to the spine
and not located in the transverse foramina, and a wider
spinal canal. Posterior fusion in situ would prevent further
kyphosis but would fail to correct the existing deformity. An
anterior approach to the osteotomy would be technically
challenging and fail to allow access to the C7 pedicle which
is essential to correct the cervicothoracic kyphosis. Halo
immobilization would also fail to correct the kyphosis.
99. Which radiographic finding is most indicative of
ankylosing spondylitis?
a. Nonmarginal syndesmophytes
b. Osteopenia
c. Scalloping of vertebral bodies
d. Subaxial subluxation
Answer: (C) Scalloping of vertebral bodies. Scalloping of
vertebral bodies is a characteristic radiographic finding of
AS and is referred to as “bamboo spine” due to the spine of
patients with advanced AS having a striking resemblance to
a bamboo tree (see Fig. 9.19). Although syndesmophytes are
present in AS, they are marginal in nature (they originate
from the margin between the intervertebral disc and the
vertebral body). Nonmarginal syndesmophytes are seen in
diffuse idiopathic skeletal hyperostosis. Osteopenia,
although seen with AS, is a nonspecific finding. Subaxial
subluxation does not typically occur in AS and is more
commonly seen with rheumatoid arthritis.
100. A 71-year-old male diagnosed with ankylosing
spondylitis 31 years ago comes into office with sagittal
imbalance and an inability to stand straight. Until eight
years ago, his condition was well-managed through
ibuprofen initially with etanercept added to his treat-
ment regimen later. Since then, he has been experi-
encing progressive stiffness and lower back pain.
Radiographs reveal a thoracolum bar kyphotic defor-
mity. What is the best choice in management for this
patient?
a. Multi-segment opening wedge osteotom y
b. Single-level opening wedge osteotomy
c. Vertebral body resection
d. Closing wedge osteotomy
Preferred Response: (D) Closing wedge osteotomy. Over a
multi-segment opening wedge osteotomy (multi-level
OWO), single-level opening wedge osteotomy (single-level
OWO), or a vertebral body resection, a closing wedge
osteotomy (CWO) offer the greatest deformity correction–of
30 to 40 degrees per level versus 20–
30 degrees with the
other techniques. CWO also offers the best fusion and sta-
bility due to direct bony apposition. A multi-level OWO has
advantages over a single-level OWO in that due to dis-
tributing the correction over multiple levels, a multi-level
OWO has less bone loss than a CWO or a single-level OWO
and preserves the anterior longitudinal ligament unlike a
single-level OWO.
188 R. Nunna et al.
101. Which of the following is the strongest indicator for
surgical intervention for a kyphotic deformity caused
by ankylo sing spondylitis?
a. Chin-brow-to-vertical angle of greater than 40º
b. Dagger sign on AP XR
c. Ankylosis of intervertebral joints
d. Significant pain and physical disability
Preferred Response: (D) Significant pain and physical dis-
ability. The preferred surgical therapy for AS-induced
kyphosis is spinal osteotomy, which is considered a rela-
tively high-risk procedure. For most patients, the risks
outweigh the potential benefits. Therefore, a compelling rea-
son–such as unbearable pain or significant physical disability–
is required to consider an osteotomy. A chin-brow-to-vertical
angle > 40º,daggersignonAPXR(indicatingossification of
the vertebral ligaments), an ankylosis of intervertebral joints
are all indicators of advanced AS, but in and of themselves,
they are not indications for surgical intervention.
102. What imaging modality is preferred for the early
detection of inflammatory changes in AS?
a. CT
b. MRI with gadolinium contrast
Fig. 9.18 Lateral XR
demonstrating chin on chest
deformity (from Dr.
Gragnaniello’s personal case)
9 Tumors, Infections, Inflammatory and Vascular Lesions of the Spine 189