Atlantoaxial Subluxation in Patient with Ankylosing Spondylitis

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					                                                                   Atlantoaxial subluxation in ankylosing spondylitis   99

    Atlantoaxial Subluxation in a Patient with Ankylosing
  Spondylitis Who Presented with Progressive Quadriplegia
                    After Minor Trauma
                               Chung-Yi Wang1, Frank Du2, Shih-Ming huang1

        Atlantoaxial subluxation due to minor trauma manifesting with progressive quadriplegia is rare. We
    present a case in a 38- year-old man who had ankylosing spondylitis for 10 years. The patient initially
    had right arm weakness, which progressed to bilateral upper and lower extremity weakness within one
    month. Ten days prior to referral to the emergency department (ED), he fell and was totally bed-ridden
    with quadriplegia. Two-dimensional computed tomographic (CT) scan of the C- spine performed in the
    ED demonstrated atlantoaxial subluxation. Conservative supportive treatment was suggested and he was
    discharged in stable condition 46 days later.

    Key words: ankylosing spondylitis, guadriplegia, atlantoaxial subluxation

                  Introduction                                Herein, we report a case of AS with minor trauma
                                                              presenting with progressive quadriplegia after one
     Ankylosing spondylitis (AS) is a group of                month.
immune-mediated disorders characterized by
chronic inflammation of the axial skeleton that                                   Case Report
may progress to a rigid, osteoporotic spine(1). Neck
pain and stiffness is one of the characteristics                   A 38 year-old man was referred to our
of this advanced disease. It usually begins in                emergency department (ED) because of paresthesia
the second decade, and is more common in men                  in the limbs and trunk numbness. He had lower
than women (2) . Patients with advanced AS are                back pain for 10 years without any prior trauma.
susceptible to fracture after trauma, and the fracture        Ankylosing spondylitis was diagnosed by
tends to involve the lower cervical vertebral area(3).        radiography (squaring of the lumbar vertebrae
The most serious complication encountered in                  and bony erosion and sclerotic changes in both
AS is spinal fracture, dislocation and atlantoaxial           sacroiliac joints) and a positive result on a human
subluxation (AAS). However, AAS is considered                 leucocyte antigen (HLA)-B27 test. He was
an uncommon feature of AS without rheumatoid                  regularly followed in our orthopedic clinic and
arthritis. AAS may be symptomless, but it may                 had been managed with an oral nonsteroidal-anti-
cause severe pain or neurological symptoms (4) .              inflammatory drug (diclofenac, 25 mg three times

Received: June 3, 2009 Accepted for publication: August 24, 2009
From the 1Department of Emergency Medicine, Mackay Memorial Hospital, Taitung Branch Hospital
  Department of Nuclear Medicine, Kaohsiung Veterans General Hospital
Address reprint requests and correspondence: Dr. Chung-Yi Wang
Department of Emergency Medicine, Mackay Memorial Hospital, Taitung Branch Hospital
1 Lane 303, Changsha Street, Taitung County 95107, Taiwan (R.O.C.)
Tel: (089)310150 ext 333 Fax: (089)342203
100   J Emerg Crit Care Med. Vol. 21, No. 2, 2010

daily) and an anti-rheumatic drug (sulfasalazine,       (AAS) (Fig. 3). Because of the progression of
500 mg four times daily). However, he had neck          impending respiratory failure, nasal endotracheal
stiffness and neck pain over the past year. The neck    intubation was performed in the ED. The patient
pain was mainly related to motion but sometimes         was then transferred to the surgical intensive
was also present at rest. Therefore he was treated      care unit (SICU) after intubation. Hydration and
with acupuncture and the pain and stiffness             empirical antibiotics (ciprofloxacin, 400 mg two
subsided. One month before he was referred to           times daily) were administrated in the ED. Three
our ED, he felt right arm weakness followed by          days after admission, the antibiotics were changed
progressive weakness of the bilateral upper and         to vancomycin (500 mg four times daily) in the
lower extremities. He could not walk without            SICU because of bacteremia (Methicillin-resistant
assistance and complained of falling easily. Ten        Staphylococcus aureus in the blood culture) and
days prior to this ED visit, he fell and was totally    sepsis (leukocytes 20,900/mL). Two weeks later, the
bed-ridden because of quadriplegia. Difficulty in       sepsis was controlled, and renal function returned
urination and urine incontinence was noted several      to the normal range (BUN 8 mg/dl, Cr 0.8 mg/dl)
days later. Two days before this ED visit, he began     after hydration. A tracheostomy was performed
to feel shortness of breath, dyspnea and abdominal      because of respiratory failure and a cystostomy was
fullness.                                               performed for neurogenic bladder. Conservative
     Physical examination showed muscle atrophy,        supportive treatment for AAS was suggested
and generalized hyperreflexia in his bilateral upper    because of severe kyphoscoliosis and long-term
arms and lower legs. The numbness was most              neurologic impairment in this patient. The patient’s
prominent in the distal parts of both upper and         condition was stable and he was discharged 46 days
lower extremities in a stocking-glove distribution.     later. He was advised to receive regular follow up
In addition, the bilateral limbs were flaccid and       in the neurosurgery clinic.
proprioceptive sensation was poor even though
pain and temperature sensations were preserved.                            Discussion
Manual muscle-testing revealed generalized muscle
weakness (grade 1 of 5) throughout the upper                 Spinal cord injuries are not uncommon in
and lower extremities. Spinal cord injuries were        the ED especially in patients suffering traumatic
thus suspected. In the ED, his body temperature         injuries such as motor vehicle accidents, falls,
was 36.5°C, blood pressure 131/71 mmHg and              gunshot wounds, and sports injuries. Clinical
the heart rate 134 beats/min. Laboratory tests          presentation with limb weakness, neck pain, urinary
revealed the following results: leukocytes 17,100/mL.   incontinence and dyspnea can be seen in patients
blood urea nitrogen (BUN) 79 mg/dl, creatinine (Cr)     with spinal cord injuries. Neurological deficits
7.0 mg/dl, and potassium 7.7 mEq/L. The urinalysis      worsen quickly after injury. A detailed evaluation
showed numerous white cells. Acute renal failure,       should be performed without delay, especially
with urinary tract infection and hyperkalemia was       in patients with multiple trauma. Patients with
diagnosed. Lateral radiographs of the cervical          ankylosing spondylitis are more predisposed to
(C)-spine (Fig. 1) and tharaco-lumbar (T-L)-            spinal trauma and cord injury than the healthy
spine (Fig. 2) showed a bamboo spine. Two-              population (5,6) . Injuries to the lower cervical
dimensional computed tomographic (CT) scan of           spine (C5-C7) occur frequently in ankylosing
the C- spine demonstrated atlantoaxial subluxation      spondylitis, whereas, fracture of the upper cervical
                                                               Atlantoaxial subluxation in ankylosing spondylitis 101

                   Fig. 1      Lateral radiographs of the cervical spine showing a bamboo
                               spine and paraspinal ligament calcification

                      Fig. 2     Dilated bowel with distended gas and a bamboo spine

spine in such patients is rare(3,4). Our patient had       neurological examination should be performed to
ankylosing spondylitis for 10 years and suffered           rule out spinal cord injuries even in cases of minor
from progressive quadriplegia due to atlantoaxial          trauma.
subluxation. The diagnosis of atlantoaxial                      Spinal cord injury is a true emergency in
subluxation may be delayed because when trauma             the emergency department. A neck collar should
is minor. The physician should be alert to the             be applied in patients suspected to have cervical
patient with ankylosing spondylitis and a detailed         spine injury to prevent further injury. In our case,
102   J Emerg Crit Care Med. Vol. 21, No. 2, 2010

                  Fig. 3    Two-dimensional computed tomographic scan of the cervical
                            spine reveals severe atlantoaxial subluxation

a neck collar was used by the ambulance crew and       sacroilitis over several years (1,2). Early diagnosis
the cervical spine was immobilized in the neutral      is not easy. The initial symptom is pain generally
position when the patient was transferred to our       felt deep in the buttock or in the lower lumbar
ED. The neck collar was removed by the family          regions(1,2). Even minor trauma to the rigid, fragile
because of patient discomfort. After relief from       spinal column can cause severe damage (1,3). The
the neck collar, the pain lessened. In our case,       mechanisms explaining AAS include transverse
neck deviation to the right side had already been      ligament damage by a periodontoidal proliferative
found one year previously. Changes to the normal       pannus or sequela of ossification of the anterior
position by the hard collar were equivocal because     and posterior longitudinal ligaments, associated
of this procedure which may have increased the         inflammatory lesions (cervical spine osteoarthritis,
neck pain and induced further spinal cord injury.      atlantodental synovitis, erosions of the dens and
Papadopoulos et al(7) reported a case of AS in which   adjacent ligaments), and physical stress (kyphosis
a hard cervical collar was used to normalize a fixed   of the dorsal spine and weight of the head at the
flexion deformity of the neck. Unfortunately, the      C1-C2 level)(2,8-10). Osteoporosis is another possible
hard cervical collar increased the neck pain and       mechanism of AAS since reduced mineral content
caused paraesthesia, followed by quadriplegia,         may result in more fragile bones. Patients often
shortness of breath, hypotension and bradycardia.      have a history of a hyperextension injury and prior
They suggested immobilization in a flexed position     alcohol use.
by placing sandbags under the occiput which may             There are no established guidelines for the
reduce cervical fracture and prevent further cord      management of AAS in patients with AS. Ramos-
damage(7).                                             Remus et al concluded that surgical stabilization
    Ankylosing spondylitis is a complex and            should be recommended when displacement
potentially debilitating disease that is insidious     between the anterior aspect of the odontoid and the
from the onset, and progresses to radiological         posterior aspect of the anterior arch of the atlas is
                                                                    Atlantoaxial subluxation in ankylosing spondylitis 103

greater than 5mm in the lateral maximal flexion                  4. S h i r a d o O, A z u m a H, Ta k e d a N, e t a l.
view(11). Also, severe pain cannot be controlled by                 Quadriparesis complicating atlantoaxial
a collar if displacement of the sagittal diameter                   subluxation and ossification of the posterior
of the spinal canal is 30% or greater or there are                  longitudinal ligament in a patient with
neurologic symptoms or signs. In our case, because                  rheumatoid arthritis. A case report. J Bone
of severe kyphoscoliosis and long-term neurologic                   Joint Surg Am 2005;87:1354-7.
impairment, the patient received rehabilitation and              5. Ludwig SC, Vaccaro AR, Balderston RA, et
conservative treatment only.                                        al. Immediate quadriparesis after manipulation
                                                                    for bilateral cervical facet subluxation: a case
                    Conclusion                                      report. J Bone Joint Surg Am 1997;79:587-90.
                                                                 6. Chou LW, Lo SF, Kao MJ, et al. Ankylosing
     In conclusion, the risk of spinal cord injury in               spondylitis manifested by spontaneous anterior
AS is higher than in the healthy population. Careful                atlantoaxial subluxation. Am J Phys Med
neurologic and radiographic evaluation of patients                  Rehabil 2002;81:952-5.
with this disease should be performed to monitor                 7. Papadopoulos MC, Chakraborty A, Waldron
possible myelopathy. A detailed examination of the                  G, Bell BA. Lesson of the week: exacerbating
entire spine should be performed to rule out spinal                 cervical spine injury by applying a hard collar.
cord injuries in all patients with underlying disease               BMJ 1999;319:171-2.
of the spine even if only minor trauma is noted.                 8. Takasita M, Matsumoto H, Uchinou S, et
The present report illustrates the importance of                    al. Atlantaxial subluxation associated with
appropriate neurologic and radiographic evaluation                  ossification of posterior longitudinal ligament
of patients with AS to help prevent severe                          of cervical spine. Spine 2000;25:2133-6.
neurological complications.                                      9. Martinez-Berenguer L, Gomez-Calcerrada D,
                                                                    Martinez-Lopez JJ. Cervical cord compression
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104    J Emerg Crit Care Med. Vol. 21, No. 2, 2010

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