Rheumatoid Arthritis in the
Cervical Spine
Abstract
The cervical spine often becomes involved early in the course of
rheumatoid arthritis, leading to three different patterns of
instability: atlantoaxial subluxation, atlantoaxial impaction, and
subaxial subluxation. Although radiographic changes are common,
the prevalence of neurologic injury is relatively low. The primary
goal of treatment is to prevent permanent neurologic injury while
avoiding potentially dangerous and unnecessary surgery. Strategies
include patient education, lifestyle modification, regular
radiographic follow-up, and early surgical intervention, when
indicated. Magnetic resonance imaging is indicated when
neurologic deficit (myelopathy) occurs or when plain radiographs
show atlantoaxial subluxation with a posterior atlantodental
interval ≤14 mm, any degree of atlantoaxial impaction, or subaxial
stenosis with a canal diameter ≤14 mm. Surgery should be
considered promptly for any of the following: progressive
neurologic deficit, chronic neck pain in the setting of radiographic
instability that does not respond to nonnarcotic pain medication,
any degree of atlantoaxial impaction or cord stenosis, a posterior
atlantodental interval ≤14 mm, atlantoaxial impaction represented
by odontoid migration ≥5 mm rostral to McGregor’s line, sagittal
canal diameter <14 mm, or a cervicomedullary angle <135°.
R
heumatoid arthritis (RA) is a
chronic inflammatory autoim-
mune disorder characterized by poly-
arthritic disturbance of peripheral
joints and early involvement of the
cervical spine. RA is relatively com-

mon, affecting 0.5% to 1.5% of the
US population and twice as many
women as men.
1
The etiology of the
disease is unknown but is likely to
be multifactorial, with a relatively
strong genetic component. RA re-
duces life expectancy, and half of all
afflicted patients become disabled
within 10 years of diagnosis.
2
The
course of the disease in any given pa-
tient, however, is unpredictable and
may be relentlessly progressive or
characterized by intermittent flares
and remissions. Current medical
treatment involves early and aggres-
sive use of disease-modifying anti-
rheumatic drugs, such as methotrex-
ate, antimalarial drugs, sulfasalazine,
and gold. Early clinical studies sug-
gest that pharmacologic blockade of
tumor necrosis factor-α with etaner-
cept or infliximab can preserve joint
function and favorably affect the nat-
ural history of the disease.
3
Chronic synovial inflammation

leads to progressive destruction of
the joints, ligaments, and bone, par-
ticularly in the atlantoaxial region.
Eventually, this process leads to
clinical manifestations of pain, de-
David H. Kim, MD, and
Alan S. Hilibrand, MD
Dr. Kim is Assistant Clinical Professor of
Orthopaedic Surgery, Department of
Orthopaedic Surgery, Tufts University
School of Medicine, Boston, MA, and
The Boston Spine Group, New England
Baptist Hospital, Boston. Dr. Hilibrand is
Associate Professor, Departments of
Orthopaedic Surgery and Neurosurgery,
and Director of Medical Education for
the Department of Orthopaedic Surgery,
Jefferson Medical College, Thomas
Jefferson University, Philadelphia, PA,
and The Rothman Institute, Philadelphia.
None of the following authors or the
departments with which they are
affiliated has received anything of value
from or owns stock in a commercial
company or institution related directly or
indirectly to the subject of this article:
Dr. Kim and Dr. Hilibrand.
Reprint requests: Dr. Hilibrand, The
Rothman Institute, 925 Chestnut Street,
Philadelphia, PA 19107-4216.

JAmAcadOrthopSurg2005;13:463-
47 4
Copyright 2005 by the American
Academy of Orthopaedic Surgeons.
Volume 13, Number 7, November 2005 463
formity, instability, and neurologic
deficits. Three characteristic pat-
terns of instability resulting from
rheumatoid involvement of the cer-
vical spine have been described; each
may occur in isolation or in combi-
nation: atlantoaxial subluxation, at-
lantoaxial impaction, and subaxial
subluxation. Each form of instabili-
ty can lead to compression of or in-
jury to the spinal cord or brainstem
and can progress to paralysis or
death. Treatment strategies for the
rheumatoid cervical spine include
patient education and lifestyle mod-
ification, periodic assessment for ra-
diographic signs of increased risk of
neurologic injury, and early surgical
intervention to prevent permanent
neurologic injury.
Epidemiology
After the hands and feet, the cervical
spine is the most common site of
disease involvement in RA.
4

De-
pending on the population and diag-
nostic criteria, between 17% and
86% of patients with RA have evi-
dence of cervical spine disease.
5
Sev-
eral studies of patients with RA sug-
gest that the cervical spine becomes
involved early in the course of the
disease, often within the first 2 years
following diagnosis.
6
A prospective
study of 103 Finnish patients with
rheumatoid factor–positive RA re-
vealed a 20-year incidence of atlan-
toaxial subluxation of 23%, atlanto-
axial impaction of 26%, and subaxial
subluxation of 19%.
7
Pathophysiology
The cervical spine is composed of 22
separate synovial joints, all potential
targets of rheumatoid disease.
5
The
synovial joints between the trans-
verse atlantal ligament and the odon-
toid process, as well as those between

the anterior atlas arch and the odon-
toid, are frequently involved. The oc-
cipitoatlantal and atlantoaxial artic-
ulations are the only segments in the
spine without intervertebral disks,
which may account for the high prev-
alence of disease in the upper cervi-
cal spine in patients with RA.
Atlantoaxial Subluxation
Rheumatoid involvement of the
synovial joints in the cervical spine
is characterized by formation of pan-
nus, an inflammatory tissue with
variable fibrous content. Synovitis
and pannus formation can weaken
the transverse, alar, and apical liga-
ments. The weight of the head, par-
ticularly with neck flexion, contrib-
utes to the repetitive strain of these
ligaments, leading to stretching or
rupture and the onset of atlantoaxi-
al subluxation. Transverse ligament
weakening and rupture occur most
commonly at the site of a synovial
bursa separating the ligament from
the posterior surface of the odontoid.
Inflammation also leads to decalcifi-
cation and occasional rupture of the
ligamentous insertion sites on the
atlas.

Erosion of the odontoid process, a
hallmark of RA, may occur anterior-
ly at its synovial joint with the arch
of C1, posteriorly at its synovial
joint with the transverse ligament,
and at the tip of the odontoid in re-
lation to the apical ligament inser-
tion. Asymmetric patterns of ero-
sion can lead to various radiographic
instability patterns, including an-
teroposterior, rotatory, or lateral sub-
luxations (Table 1). Both rotatory
and lateral subluxation patterns can
result in torticollis.
Atlantoaxial subluxation may oc-
cur in up to 49% of RA patients and
usually manifests as anterior sub-
luxation of the atlas (C1) on the ax-
is (C2).
8
This increased anterior at-
lantodental interval and decreased
posterior atlantodental interval is
apparent on lateral cervical spine ra-
diographs, particularly with the
neck in flexion (Figure 1). As sublux-
ation increases with time, the space
available for the spinal cord decreas-
es, which may compress or injure
the spinal cord. However, in patients

with a large spinal canal, an anterior
atlantodental interval >10 mm may
be seen without any apparent neuro-
logic sequelae.
The reducibility of atlantoaxial
subluxation, either with extension
positioning or external traction, is
important in planning treatment.
Initially, subluxation may be purely
dynamic, appearing only on flexion
views. Eventually, pannus may be-
come interposed between the anteri-
or atlas arch and the odontoid, con-
verting a dynamic subluxation into a
fixed one.
Atlantoaxial Impaction
Involvement of the atlanto-
occipital and atlantoaxial joints can
lead to weakening and collapse of
the lateral masses, with rostral mi-
gration of the odontoid process and
atlantoaxial impaction. Atlantoaxial
impaction also has been referred to
as superior migration of the odon-
toid, cranial settling, and basilar in-
vagination. The prevalence of atlan-
toaxial impaction is reportedly
between 5% and 32% and generally
is thought to follow the appearance
of atlantoaxial subluxation.

5
Com-
pared with other instability patterns,
atlantoaxial impaction appears to
carry the worst prognosis and a
much higher risk of myelopathy.
The symptomatology of atlanto-
axial impaction is highly variable.
Compression of the C1 and C2 nerve
roots leads to occipitocervical pain.
Ventral pressure on the medulla ob-
longata can injure local cranial nerve
nuclei or cause sudden death by
Table 1
Patterns of Rheumatoid Arthritis in
the Cervical Spine
Atlantoaxial subluxation
Anterior
Posterior
Lateral
Rotatory
Atlantoaxial impaction
Subaxial subluxation
Rheumatoid Arthritis in the Cervical Spine
464 Journal of the American Academy of Orthopaedic Surgeons
compressing the respiratory center.
The anterior spinal artery and verte-
bral arteries also can be compro-
mised, leading to neurologic deficits,
vertebrobasilar insufficiency, or tran-

sient ischemic attacks.
9,10
With pro-
gression of atlantoaxial impaction
and greater penetration of the odon-
toid process into the foramen mag-
num, the degree of atlantoaxial sub-
luxation may decrease in a process
referred to as “pseudostabilization.”
5
However, any reduction in atlan-
toaxial subluxation with the pro-
gression of atlantoaxial impaction
actually carries a worse prognosis
because of the increased risk of
brainstem injury and sudden death.
11
Subaxial Subluxation
Destabilization of the facet joints
as a result of weakening of the facet
capsules and interspinous ligament
results in anterior subaxial sublux-
ation. Although marked degenera-
tive disk changes are consistently
present, synovitis has not been ob-
served in disk or annular tissue.
Therefore, anterior spinal disease
does not appear to contribute direct-
ly to the development of subaxial
subluxation. This instability pattern

is a relatively late manifestation of
cervical spine disease and is ob-
served in 20% to 25% of patients
with RA.
5
It is also the most fre-
quently observed new instability
pattern following upper cervical fu-
sion in RA patients. Subaxial sub-
luxation occurs most frequently at
the C2-3 and C3-4 levels and typical-
ly affects multiple adjacent levels,
yielding a characteristic “staircase”
deformity and associated kypho-
sis.
6,12
Spinal cord compression oc-
curs anteriorly from the proximal
edge of the vertebral body distal to
the slip or posteriorly from the neu-
ral arch of the slipped vertebrae. Sub-
axial subluxation may not be appar-
ent on radiographs with the patient
in neutral position but should be-
come apparent, when present, on
flexion or extension views.
Clinical Presentation
Rheumatoid involvement of the cer-
vical spine is often asymptomatic.
Neck pain, the most frequent com-

plaint, may be present in 40% to 80%
of patients.
5
Characteristically, pa-
tients describe the pain as a deep ache
radiating into the occipital, retro-
orbital, or temporal areas. The tem-
poral pattern is typically mechanical
and readily distinguishable from the
typical pain of r heumatoid s ynovitis.
Pain referred to the face, ear, or sub-
occiput originates from irritation of
the C2 nerve root supply to, respec-
tively, the nucleus of the spinal
trigeminal tract, greater auricular
nerve, or greater occipital nerve.
13
Some patients with atlantoaxial
subluxation report the sensation of
the head falling forward during neck
flexion.
14
A clunking sensation also
can occur during neck extension
with spontaneous reduction of atlan-
toaxial subluxation; this has been la-
beled a positive Sharp-Purser test.
15
Stiffness, crepitus, and painful range
of motion also are common com-

plaints. Sleep apnea may be caused
by brainstem compression associat-
ed with atlantoaxial impaction.
16
Objective neurologic signs are
present less frequently than pain but
may be found in 7% to 34% of pa-
tients.
17,18
In contrast to radiograph-
ic changes, which appear early in the
course of RA, neurologic deficits
usually appear later, most common-
ly in late middle-age patients. They
may be difficult to elicit in patients
with more advanced disease. Ex-
tremity weakness is thought to re-
sult from advancing joint involve-
ment with neurologic symptoms.
Signs of cervical myelopathy, the
clinical manifestation of spinal cord
Figure 1
A 65-year-old woman with rheumatoid arthritis and progressive cervical myelopathy. A, Lateral radiograph of the cervical spine
reveals atlantoaxial subluxation. The anterior atlantodental interval measures 4 mm and the posterior atlantodental interval
measures 14 mm. Sagittal (B) and axial (C) T2-weighted MRI scans reveal significant additional reduction in space available for
the cord to 7 mm because of a large soft-tissue pannus posterior to the odontoid process.
David H. Kim, MD, and Alan S. Hilibrand, MD
Volume 13, Number 7, November 2005 465
compression, should be sought, in-
cluding a wide-based spastic gait,

clumsy hands, a visible change in
handwriting, or difficulty manipu-
lating buttons or handling coins.
Other classic physical findings asso-
ciated with myelopathy include hy-
perreflexia, a positive Babinski test,
and a positive Hoffmann sign. Mild
motor and reflex deficits may be im-
possible to identify in patients with
significant pain and deformity of ex-
tremity joints. Lhermitte’s sign, the
sensation of electric shocks traveling
down the torso or upper extremities
on neck flexion, suggests myelopa-
thy. Urinary retention, followed by
overflow incontinence, is a symp-
tom of severe myelopathy. Occa-
sionally, injury to the pyramidal
tract decussation can manifest as a
“cruciate paralysis,” with varying
degrees of upper extremity weakness
that may be symmetric or asymmet-
ric.
10
This injury pattern is often dif-
ficult to distinguish from central
cord syndrome.
Radiographic
Evaluation
Plain Radiography

Appropriate preoperative screen-
ing for cervical spine disease in pa-
tients with RA is a controversial
subject. No radiographic screening
protocol is universally accepted.
Nevertheless, certain groups of pa-
tients should be considered strong
candidates for preoperative radio-
graphic screening (Table 2). All pa-
tients with RA should undergo an
initial series of cervical spine radio-
graphs, including an anteroposterior
view and lateral views with the pa-
tient in the neutral position as well
as in flexion and extension. These
radiographs are recommended before
any surgical procedure requiring in-
tubation. One study of 113 RA pa-
tients undergoing elective hip or
knee arthroplasty found that 61%
demonstrated evidence of instabili-
ty, defined by at least 3 mm of dy-
namic atlantoaxial subluxation, by
atlantoaxial impaction (according to
Ranawat’s method), or by subaxial
subluxation, although only 50% of
patients were symptomatic.
19
Atlantoaxial Subluxation
Traditionally, an anterior atlanto-

dental interval >5 mm was consid-
ered diagnostic of atlantoaxial sub-
luxation. However, several studies
have demonstrated that the anterior
atlantodental interval does not cor-
relate with the risk of neurologic
injury.
19-21
Boden et al
20
showed that
measurement of the posterior atlan-
todental interval appears to be a
more reliable predictor of neurolog-
ic deficit in patients with atlantoax-
ial subluxation; a value ≤14 mm has
been suggested as an indication for
surgical stabilization (Figure 2). The
posterior atlantodental interval also
may be used to predict neurologic re-
covery after surgery.
Atlantoaxial Impaction
Several radiographic measure-
ment techniques have been suggest-
ed as means to gauge the severity of
atlantoaxial impaction. The original
definition of atlantoaxial impaction
was protrusion of the odontoid
above the margins of the foramen
magnum, also known as McRae’s

line. Because identifying the mar-
gins of the foramen magnum on
plain radiographs is difficult, alter-
native landmarks have been estab-
lished (Figure 3). McGregor’s line is
defined on a l ateral radiograph by the
hard palate and the base of the oc-
ciput (opisthion). Protrusion of the
tip of the odontoid above McGre-
Table 2
Indications for Anteroposterior and Lateral Radiographs of the Cervical
Spine* in Patients With Rheumatoid Arthritis
Prolonged cervical symptoms >6 months
Neurologic signs or symptoms
Scheduled procedures requiring endotracheal intubation in patients who
have not had cervical radiographs in the last 2 to 3 years
Rapidly progressive carpal or tarsal bone destruction
Rapid overall functional deterioration
* Neutral, flexion, and extension
Figure 2
Diagrammatic representation of
atlantoaxial subluxation typically seen in
patients with rheumatoid arthritis. The
posterior atlantodental interval (PADI)
is measured from the posterior margin
of the odontoid process to the anterior
margin of the posterior arch of C1.
(Reproduced with permission from
Boden SD, Dodge LD, Bohlman HH,
Rechtine GR: Rheumatoid arthritis

of the cervical spine: A long-term
analysis with predictors of paralysis
and recovery. J Bone Joint Surg Am
1993;75:1282-1297.)
Rheumatoid Arthritis in the Cervical Spine
466 Journal of the American Academy of Orthopaedic Surgeons
gor’s line >4.5 mm is considered
diagnostic of atlantoaxial impac-
tion.
20
The presence of odontoid erosion
can make all of these radiographic
measurements inaccurate. For this
reason, other techniques have been
developed to diagnose atlantoaxial
impaction on plain radiographs,
even with significant odontoid ero-
sion.
22
These include the Ranawat
method, designed to assess the ex-
tent of collapse at the atlantoax-
ial articulation, and the Redlund-
Johnell method, which uses the dis-
tance between the anterior axis base
and McGregor’s line
22
(Figure 4).
Subaxial Subluxation
The radiographic appearance of

subaxial subluxation is characteris-
tic and includes not only sagittal
plane listhesis of sequential verte-
bral bodies but also posterior ele-
ment changes, including facet joint
erosions and widening, whittling, or
spindling of the spinous processes
(Figure 5). There are multiple def-
initions of subaxial subluxation.
Figure 3
Radiographic landmarks for assessing atlantoaxial impaction in patients with
rheumatoid arthritis. On a lateral radiograph, atlantoaxial impaction is diagnosed by
protrusion of the odontoid tip proximal to McRae’s line or 4.5 mm above
McGregor’s line. (Adapted with permission from Riew KD, Hilibrand AS, Palumbo
MA, Sethi N, Bohlman HH: Diagnosing basilar invagination in the rheumatoid
patient: The reliability of radiographic criteria. J Bone Joint Surg Am
2001;83:194-200.)
Figure 4
Methods to assess atlantoaxial impaction on plain radiographs. A, Ranawat method. A line (a) is drawn across the transverse
axis of the atlas, and a connecting line (b) is drawn through the vertical axis of the odontoid from the center of the C2 pedicle
radiographic shadow. Values (x)<15 mm in men and <13 mm in women are diagnostic for atlantoaxial impaction. B, Redlund-
Johnell method. A line (a) is drawn between McGregor’s line (b) and the midpoint of the inferior end plate of C2 (c). A value
(x)<34 mm in men and <29 mm in women is diagnostic for atlantoaxial impaction. (Adapted with permission from Riew KD,
Hilibrand AS, Palumbo MA, Sethi N, Bohlman HH: Diagnosing basilar invagination in the rheumatoid patient: The reliability of
radiographic criteria. J Bone Joint Surg Am 2001;83:194-200.)
David H. Kim, MD, and Alan S. Hilibrand, MD
Volume 13, Number 7, November 2005 467
Yonezawa et al
12
described subaxial

subluxation as >4 m m o r 20% listhe-
sis of vertebral body diameter.
Magnetic Resonance
Imaging
Although plaintomography,cine-
radiography, computed tomography
(CT), and CT myelography have
been used to follow the rheumatoid
cervical spine, these studies have
been supplanted by the use of mag-
netic resonance imaging (MRI). MRI
provides the most detailed defini-
tion of the craniocervical junction,
including the size of rheumatoid
pannus. The finding of odontoid ero-
sion on MRI is considered highly
specific for RA and may clarify the
diagnosis in otherwise nonspecific
cases.
23
The space-occupying effect of the
periodontoid pannus can be visual-
ized clearly on MRI. Both T1- and
T2-weighted images provide excel-
lent visualization of brainstem or
spinal cord contour. Increased signal
intensity within the spinal cord on
T2-weighted images may represent
edema, gliosis, or myelomalacia and
has been associated with poor neuro-

logic recovery following surgery.
24
The cervicomedullary angle can
be measured on MRI by drawing a
line along the anterior aspect of the
cervical spinal cord and the medulla.
This angle normally is between 135°
and 175° (Figure 6). With progressive
craniocervical disease, the brain-
stem angulates ventrally over the
displaced odontoid process, leading
to increased obliquity of the cervi-
comedullary angle. One study re-
ported a 100% correlation between a
cervicomedullary angle <135° and
neurologic signs of cervicomedullary
compression, myelopathy, or C2 ra-
diculopathy.
25
The utility of flexion-extension
or so-called dynamic MRI evaluation
has been debated.
26
A theoretic ad-
vantage is the ability to identify,
when the patient is in the neutral
position, potentially significant dy-
namic cord compression before sta-
tic compression is apparent. One
study suggested that cord diameter

in cervical flexion <6 mm is a risk
factor for neurologic deficit.
27
How-
ever, a theoretic risk of sudden death
exists as a result of prolonged cervi-
cal flexion in a patient with severe
instability; therefore, the recommen-
dation for routine dynamic MRI in
these patients may need to change.
Natural History
Understanding of the natural histo-
ry of the rheumatoid cervical spine
is limited. Most studies have been
handicapped by small sample size or
retrospective design. Oda et al
28
re-
viewed the records of 49 patients fol-
lowed radiographically for a mini-
mum of 5 years and identified a
progressive pattern of cervical sub-
luxations. The earliest instability
pattern is most often a reducible at-
lantoaxial subluxation.
6
Transition
from reducible subluxation to an ir-
reducible subluxation often ac-
companies atlantoaxial impaction,

which appears, on average, 6 years
after atlantoaxial subluxation.
11
Sub-
axial subluxation occurs less fre-
quently than do the other two pat-
terns and generally develops after
atlantoaxial impaction.
29
Forty percent to 80% of patients
with rheumatoid subluxations dem-
onstrate radiographic progression.
17
Neurologic deficits occur much less
frequently and do not correlate well
with radiographic progression.
17
Rana
30
retrospectively reviewed the
records of 41 patients with atlanto-
axial subluxation who were followed
for 10 years; 61% of patients had no
change, 27% had progressive sublux-
ation, and 12% had improvement at
final follow-up. Boden et al
20
retro-
spectively reviewed the records of 73
patients followed for an average of 7

years. In 31 patients, all treated non-
surgically, no significant neurologic
deficit developed during the observa-
tion period. Of the 42 patients (58%)
in whom paralysis developed, 7 were
managed nonsurgically. Six of the
Figure 5
Lateral radiograph demonstrating
subaxial subluxation across the C3
through C5 segments, with associated
facet joint erosions and spinous
process changes.
Figure 6
Midsagittal T-2-weighted MRI scan of
the craniocervical junction in a patient
with atlantoaxial impaction. The
cervicomedullary angle (CMA) is
defined by the angle subtended
between a line parallel to the long axis
of the brainstem (a) and a line parallel
to the cervical spinal cord (b).
Rheumatoid Arthritis in the Cervical Spine
468 Journal of the American Academy of Orthopaedic Surgeons
seven patients treated nonsurgically
experienced progressive neurologic
deterioration, and all seven died
within 4 years of the onset of paral-
ysis, five from cord compression. Of
the 35 patients who underwent sur-
gery, 25 (71%) experienced marked

neurologic improvement.
Despite the general impression
that rheumatoid involvement of the
cervical spine does not contribute to
increased mortality in this patient
population, the high rate of sudden
death observed in patients with un-
treated myelopathy argues against
this conclusion.
31
When myelopathy
appears, the mortality rate increases
dramatically; without surgery, most
patients die within 1 year.
32
An au-
topsy study of 104 patients with RA
identified 11 with atlantoaxial sub-
luxation and notable spinal cord
compression; most had experienced
sudden death.
33
This finding sug-
gests that the mortality rate from
cervical spine instability in patients
with RA may be as high as 10%.
Grading systems have been devel-
oped to assess disease severity as
well as treatment outcomes. The
Ranawat grading system is widely

used in clinical studies but has been
criticized for lacking the ability to
discriminate mild degrees of myel-
opathy
34
(Table 3). The Zeidman and
Ducker modification of the Nurick
myelopathy scale provides more dif-
ferentiation, with an assessment of
gait and hand function, and may be
more useful
10
(Table 4). However , its
failure to account for severity of ra-
diculopathic complaints significant-
ly limits this system for guiding sur-
gical decision making.
Risk Factors and
Predictors of Recovery
Several risk factors for progression of
atlantoaxial subluxation have been
suggested, including male sex, rheu-
matoid factor seropositivity, higher
initial C-reactive protein level,
presence of subcutaneous nodules,
and advanced peripheral joint dis-
ease, specifically rapid loss of carpal
height.
6,18,28,36,37
HLA-DR4 and B-27

seropositive antibodies do not ap-
pear to be significant risk factors.
6
Whether corticosteroid treatment
represents an independent risk fac-
tor remains controversial.
38
Patients
with any degree of atlantoaxial im-
paction or cord compression on MRI
are at significant risk of neurologic
injury and should be considered
strong candidates for prophylactic
decompression and/or stabiliza-
tion.
37,39
Plain radiography is an efficient
and inexpensive means of monitor-
ing disease progression. Boden et
al
20
found that the posterior atlanto-
dental interval and subaxial sagittal
canal diameter correlated with the
presence and severity of paralysis,
whereas the anterior atlantodental
interval did not. Using a standard
tube distance of 6 feet (1.8 m), 96%
of patients with atlantoaxial sublux-
ation and paralysis demonstrated a

posterior atlantodental interval ≤13
mm. In contrast, 60% of the same
patients had an anterior atlantoden-
tal interval <9 mm. All patients with
subaxial subluxation and paralysis
demonstrated a subaxial sagittal ca-
nal diameter ≤13 mm.
Boden et al
20
found the posterior
atlantodental interval to be a predic-
tor of surgical outcome. Postopera-
tively, no patient experienced signif-
icant neurologic recovery when the
preoperative posterior atlantodental
interval measured <10 mm. In pa-
tients with isolated atlantoaxial sub-
luxation, a posterior atlantodental
interval of at least 10 mm predicted
improvement of at least one Rana-
wat class. In the setting of atlanto-
axial subluxation and atlantoaxial
impaction, recovery required a pre-
operative posterior atlantodental in-
terval ≥13 mm. All patients with a
preoperative posterior atlantodental
interval and subaxial sagittal canal
diameter measuring at least 14 mm
experienced complete motor recov-
ery.

Multiple studies have suggested
that the degree of preoperative neu-
Table 4
Zeidman and Ducker Modification of Nurick Grading Scale for
Myelopathy
10
Grade Radiculopathy Myelopathy Gait Hand Function
0 Present Absent Normal Normal
I Present Present Normal Slight
II Present Present Mildly abnormal Functional
III Present Present Severely abnormal Unable to button
IV Present Present With assistance
only
Severely limited
V Present Present Nonambulatory Useless
Table 3
Ranawat Grading Scale for Myelopathy
35
Grade Severity
I Normal
II Weakness, hyperreflexia, altered sensation
IIIA Paresis and long-tract signs, ambulatory
IIIB Quadriparesis, nonambulatory
David H. Kim, MD, and Alan S. Hilibrand, MD
Volume 13, Number 7, November 2005 469
rologic deficit is also a strong predic-
tor of postoperative neurologic re-
covery.
40,41
Factors with no apparent

predictive value in terms of recovery
include age, sex, duration of paraly-
sis, and preoperative anterior atlan-
todental interval.
42
Nonsurgical Treatment
Patients with rheumatoid involve-
ment of the cervical spine benefit
from early, aggressive medical inter-
vention as well as regular follow-up.
Because of the prevalence and early
appearance of cervical spine involve-
ment, screening cervical spine radio-
graphs should be considered in all
patients with RA. Regular follow-up
radiography should then be per-
formed in patients with any evi-
dence of cervical spine involvement,
especially when they have advanced
peripheral joint disease. At least one
study has demonstrated that early,
aggressive combination disease-
modifying antirheumatic drug ther-
apy can prevent or delay develop-
ment of atlantoaxial subluxation.
43
Soft cervical collars are appropri-
ate treatment of symptomatic pa-
tients with relatively minor occipi-
tocervical pain and may occasionally

represent the treatment of choice in
elderly or debilitated patients who
are poor surgical candidates.
44
Unfor-
tunately, orthoses may be problem-
atic in patients with temporoman-
dibular joint involvement. Although
they offer excellent relief of symp-
tomatic neck pain, soft cervical col-
lars do not provide much limitation
of motion and probably do not alter
the natural history of cervical spine
disease.
5
Progressive neurologic de-
terioration has been observed in pa-
tients with spinal cord myelopathy
treated with a soft collar.
20
A comprehensive program of pa-
tient education, physical therapy,
collars, practical aids, symptomatic
treatment, and disease-modifying
antirheumatic drugs achieves signif-
icant (P < 0.001) lasting pain relief in
most patients.
45
Patients should be
taught to avoid cervical flexion.

Physical therapy should focus on
isometric strengthening of neck
muscles and overall postural train-
ing. Narcotic medication may be ap-
propriate for short-term relief of
acute pain, but when chronic narcot-
ic use is required for pain relief, then
surgical treatment should be consid-
ered.
Surgical Management
The goals of surgical treatment of
rheumatoid disease of the cervical
spine are to achieve spinal stability
through a solid fusion and to decom-
press involved neural structures.
The most commonly accepted indi-
cations for surgical treatment of RA
in the cervical spine include neuro-
logic deterioration and intractable
pain with spinal instability (Table 5).
In addition, Boden et al
20
proposed
surgery, even without neurologic
findings, when patients demonstrate
one of three radiographic risk fac-
tors: (1) atlantoaxial subluxation
with a posterior atlantodental inter-
val ≤14 mm; (2) atlantoaxial impact
represented by odontoid migration

≥5 mm rostral to McGregors’s line;
or (3) subaxial subluxation with sag-
ittal canal diameter ≤14 mm.
Patients with RA are a challenging
surgical population. Those requiring
surgery suffer from a systemic illness
and may be significantly malnour-
ished and debilitated. In addition to
cervical spine disease, micrognathia
and temporal mandibular disease
make standard intubation difficult
and anesthesia hazardous. Excessive
trauma caused by standard intuba-
tion has been associated with a 14%
incidence of upper-airway obstruc-
tion following extubation.
46
This rate
can be reduced to 1% with fiberoptic
assistance. Skin lesions and corticos-
teroid use notably increase the rate of
wound complications and infections.
Poor structural bone quality may ren-
der standard surgical fixation tenu-
ous and unreliable.
Atlantoaxial Subluxation
When atlantoaxial subluxation is
reducible, a variety of posterior fu-
sion techniques are possible, includ-
ing Gallie or Brooks wiring, Magerl

transarticular screws, or Harms
C1-2 lateral mass fixation.
4
Fixa-
tion strategies incorporating Magerl
screws appear to provide particular-
ly stable fixation; a fusion rate of
95% has been reported with use of
transarticular screws.
47
The need for
postoperative halo-vest stabilization
depends on the quality of surgical
fixation.
When atlantoaxial subluxation is
nonreducible, transar ticular screw
Table 5
Indications for Surgery in Patients With Rheumatoid Arthritis With Cervical
Spine Involvement
Progressive neurologic deficit (eg, weakness, gait disturbance, loss of fine
motor coordination)
Mechanical neck pain unresponsive to nonnarcotic pain medication (in the
setting of radiographic evidence of AAS, AAI, or SAS)
Radiographic risk factors of impending neurologic injury
PADI ≤14 mm in the setting of AAS
AAI represented by odontoid migration ≥5 mm rostral to McGregor’s line
Sagittal canal diameter ≤14 mm in patients with SAS
Any degree of AAI or cord stenosis
A cervicomedullary angle <135°
AAI = atlantoaxial impaction, AAS = atlantoaxial subluxation, PADI = posterior

atlantodental interval, SAS = subaxial subluxation
Rheumatoid Arthritis in the Cervical Spine
470 Journal of the American Academy of Orthopaedic Surgeons
fixation in combination with C1
laminectomy should be considered,
especially when the patient has pos-
terior cord compression. An occipi-
tocervical fusion is an alternative.
MRI studies revealing significant
pannus resorption after spinal stabi-
lization indicate that, if bone is not
impinging anteriorly on the cord,
then odontoid resection may be un-
necessary.
48
Sublaminar wire fixa-
tion is contraindicated when atlan-
toaxial subluxation cannot be
reduced.
Atlantoaxial Impaction
Because the risk of neurologic in-
jury is high with atlantoaxial impac-
tion, early surgery following identi-
fication of this condition has been
recommended.
41
Neurologic deficit
or evidence by MRI of cord compres-
sion is a strong indication for sur-
gery.

13
The use of preoperative halo
traction has been recommended to
reduce the degree of atlantoaxial im-
paction and obviate foramen mag-
num decompression or odontoid re-
section. An occipitocervical fusion
can be performed using wires or
screws attached to the occiput below
the superior nuchal line and con-
nected to fixation in the subaxial
spine (Figure 7). When traction is un-
successful, symptomatic decompres-
sion with a transoral odontoid resec-
tion or C1 laminectomy, along with
posterior stabilization, is required.
4
An alternative technique for multi-
level instability including atlantoax-
ial impaction is a long rod loop fixed
with occipital and cervical laminar
wires without attempted fusion.
Subaxial Subluxation
Evidence from clinical studies
suggests that patients with subaxial
subluxation and neurologic deficits,
or asymptomatic patients with a
subaxial canal diameter ≤14 mm,
should be considered for surgical sta-
bilization.

20
Preoperative halo-vest
immobilization can provide tempo-
rary stabilization and reduction of
displacement, often with immediate
improvement in neurologic defi-
cits.
49
Reducible subluxations can be
fused anteriorly or posteriorly, but
optimal treatment of irreducible
subluxations is anterior decompres-
sion and fusion.
23,34
Posterior fusion
should be strongly considered fol-
lowing any laminectomy
50
(Figure
8). The extent of fusion required is
often not apparent but is a critical
decision because RA is progressive.
Instability patterns can occur in
combination at multiple levels of
the cervical spine. When atlantoax-
ial subluxation and subaxial sublux-
ation occur concurrently, fusion
should be extended to the lowest in-
volved level to minimize the risk of
junctional degeneration.

51
Some in-
vestigators recommend more exten-
sive fusions, at times including the
entire cervical spine and extending
caudally to T1 or T2.
52,53
The extent of postoperative im-
mobilization depends on the type of
surgical fixation used. Wiring tech-
niques typically require rigid immo-
bilization in a halo-vest or four-
poster orthosis for 8 to 12 weeks.
5
Following plate and screw fixation, a
cervical orthosis may be sufficient.
New plate, rod, and screw instru-
mentation techniques with less rig-
id fixation appear to have improved
fusion rates and maintenance of
alignment but may increase the risk
of neurovascular injury.
Outcomes of Surgery
Over the past 10 years, outcomes of
surgery on the rheumatoid cervical
spine have improved markedly,
largely because of e arlier diagnosis of
myelopathy and surgical referral.
54
More aggressive medical manage-

ment of RA and a decrease in corti-
costeroid use may be additional con-
tributing factors.
Most studies favor surgery over
nonsurgical management for pa-
tients with neurologic deficits.
55,56
One of the strongest predictors of
postoperative neurologic recovery
Figure 7
A 56-year-old woman with rheumatoid arthritis and atlantoaxial impaction.
A, Preoperative midsagittal T2-weighted MRI scan demonstrating penetration of
the odontoid through the foramen magnum with impingement on the brainstem.
B, Postoperative radiograph demonstrating reduction of the occipitoatlantoaxial
relationship and occipitocervical instrumented fusion using a plate-and-screw
system.
David H. Kim, MD, and Alan S. Hilibrand, MD
Volume 13, Number 7, November 2005 471
appears to be preoperative neurolog-
ic status. Nonambulatory patients
demonstrate higher complication
rates and lower overall survival.
57
Boden et al
20
reported a retrospective
review of the records of 35 patients
and found that all Ranawat class II
patients had notable neurologic im-
provement after surgery compared

with 62% of class III patients.
Casey et al
58
reported results from
their prospective study of 116 pa-
tients with atlantoaxial impaction.
Transoral odontoid resection was
performed for anterior bony com-
pression; otherwise, laminectomy
and instrumented fusion were done.
A higher revision surgery rate was
noted in patients without fusion to
the occiput. Occipitocervical fusions
failed because subaxial instability
below the level of fusion progressed.
Satisfactory results were more like-
ly in patients with better preopera-
tive neurologic function. Overall,
marked neurologic improvement oc-
curred in 45% and significant pain
relief in 97% of patients. A high rate
of perioperative complications was
attributed to the general debility of
the patient population; the perioper-
ative mortality rate was 10%.
Surgical results for subaxial sub-
luxation may be less favorable.
Olerud et al
52
reported a small retro-

spective study of 16 patients with
subaxial subluxation and varying de-
grees of myelopathy who underwent
anterior and/or posterior decompres-
sion and fusion. Although neck pain
was typically relieved, arm pain im-
proved less reliably. Patients with
myelopathy had the worst prognosis,
and four of five with severe myelop-
athy died within 3 months of surgery.
The authors recommend early sur-
gery for subaxial subluxation, before
significant myelopathy develops.
The overall complication rate is
markedly increased in patients with
RA. Wound complications, includ-
ing infection, may occur in up to
25% of patients. Generalized os-
teopenia correlates with systemic
disease activity and compromises
fixation strength, leading to in-
creased rates of instrumentation f ail-
ure.
52
Successful outcomes are pred-
icated on achieving solid fusions,
and pseudarthrosis has been associ-
ated with a less favorable clinical re-
sult because of persistent instabili-
ty.

20
Recurrent instability in the
form of subaxial subluxation at
more caudal levels may occur either
as a result of the natural progression
of the disease or as a result of in-
creased stress from an adjacent fu-
sion. Current perioperative mortali-
ty rates have been reported to be
between 5% and 10%, with in-
creased rates in patients having coin-
cident cardiovascular disease or at-
lantoaxial impaction.
34,41,58,59
Summary
In most patients with RA, neck pain
occurs without significant neurolog-
ic deficit. Multimodality therapy, in-
cluding patient education, physical
therapy, and active medical treat-
ment, can be very effective in con-
trolling symptoms and limiting dis-
Figure 8
A, Preoperative lateral cervical radiograph of a 46-year-old woman with RA myelopathy and subaxial subluxation of C4 on C5.
B, Preoperative sagittal T1-weighted MRI scan revealing cervical cord compression at level of subluxation. C, Postoperative
radiograph following laminectomy and instrumented fusion using C1-2 transarticular screws and lateral mass screws from C3
to C5.
Rheumatoid Arthritis in the Cervical Spine
472 Journal of the American Academy of Orthopaedic Surgeons
ease progression. The presence of

neurologic findings should prompt
consideration for early surgical treat-
ment because the results of surgery
are related directly to the severity of
preoperative neurologic deficits.
Despite the high prevalence of ra-
diographic changes associated with
rheumatoid involvement of the cer-
vical spine, the incidence of signifi-
cant neurologic deficits is relatively
low.
6,28
Similarly, progression of ra-
diographic changes does not corre-
late well with the appearance of neu-
rologic deficits. Therefore, the
primary treatment goal is preventing
or arresting irreversible neurologic
deterioration while avoiding danger-
ous, costly, and unnecessary sur-
gery.
To screen effectively for clinical-
ly significant deformity or instabili-
ty, plain radiographs and regular
follow-up examinations are critical.
The presence of neurologic deficit,
atlantoaxial subluxation with a pos-
terior atlantodental interval ≤14
mm, any amount of atlantoaxial im-
paction, or subaxial stenosis with a

canal diameter ≤14 mm indicates
the need for further evaluation by
MRI and possible surgery. A canal
diameter <14 mm on MRI or a cervi-
comedullary angle <135° should
prompt consideration of surgical
treatment.
Surgical treatment of the unstable
rheumatoid cervical spine is one of
the most challenging problems in
spine surgery. Medical comorbidi-
ties, poor bone quality , and impaired
wound healing increase the risk of
surgical complications and perioper-
ative mortality. Nevertheless, with
appropriate patient selection, proper-
ly performed surgery undertaken by
a spinal surgeon experienced in
treating these patients, and with ad-
vanced anesthetic and perioperative
management, most patients may an-
ticipate marked neurologic improve-
ment and pain relief as well as nota-
ble improvement in overall quality
of life.
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