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Cervical , , and Rheumatoid Matthew McDonnell, MD, and Phillip Lucas, MD  Ce r v i ca l Sp o n y l o s i s /St e n o s i s The uncovertebral hypertrophy, Clinical Presentations Cervical spondylosis is common and which may lead to foraminal stenosis. The Axial progresses with increasing age. It is the posterior zygoapophyseal joints can also is an extremely common result of degenerative changes in the cer- become arthritic, causing foraminal nar- but nonspecific presenting symptom. vical spine, including disc degeneration, rowing. The ligamentum flavum thickens It is often associated with stiffness and facet , osteophyte formation, and sometimes buckles as a result of the headaches. The pain or soreness is usu- ligamentous thickening and loss of cervi- loss of disk height. These degenerative ally in the paramedian neck muscles cal . Spinal stenosis, or narrowing changes can result in cervical stenosis with posteriorly, with radiation toward the of the , may occur as a result compression. Concomitant occiput or into the shoulder, arm and of progression of spondylotic changes. straightening of cervical lordosis (or even periscapular regions. The referred pain Spinal cord or root function may ), disk herniations or protru- does not follow a dermatomal distribu- be affected, resulting in symptoms of sions often may accentuate the problem tion. Deep palpation of some of these or . because the spinal cord will be stretched areas results in reproducible patterns of over the posterior aspect of the disks and referred pain. Determining the source Natural History and Epidemiology vertebral bodies. of neck pain can be a diagnostic chal- Spinal cord compression resulting Cervical spondylosis will typically lenge. New pain patterns may develop from spondylotic changes is usually a slow result in stiffening of the spinal motion as a result of postural adaptations and and progressive process. Many patients segments. It is not uncommon for the compensatory overuse of normal tissues have evidence of significant compression motion segments one or two levels above further confusing the clinical picture. on imaging studies but are asymptomatic. the stiff segments to become hypermobile. Shoulder pathology sometimes presents Most cases of myelopathy develop in a The resulting instability may lead to dy- with pain referred to the neck. A careful stepwise fashion described by episodes of namic cord compression.1,4,5 history should be obtained to rule out exacerbation of symptoms and worsen- Radiculopathy is due to biochemical inflammatory arthritis or an infectious ing function followed by long periods of and biomechanical changes that occur or neoplastic process.2,4,8 static function. Fewer patients have steady with age as a result of the degenerative progressive deterioration. With vascular cascade and lead to insufficiency, acute onset may occur with disc herniation or fo- devastating, irreversible ischemic changes raminal narrowing. occurring within the cord.1,2 The Approximately 25% of individu- gradually loses height, als younger than forty years of age, posterior portions of 50% of individuals over forty years of the disc bulge into the age, and 85% of individuals over sixty spinal canal and the years of age have some degree of disc neuroforamina, the degeneration.1-3 ligamentum flavum and facet capsule Pathophysiology infold, and osteophytes The pathoanatomy of cervical spon- form. All of this leads to dylosis results from the sequelae of the ag- decreases in canal and ing process in the spine, specifically, disk foraminal size. Sublux- degeneration with hypertrophic osseous ation and hypermobil- and ligamentous changes. Disk desic- ity between vertebral cation is accompanied by biochemical bodies may occur. The changes, with a relative increase in the pain that occurs as a ratio of keratan sulfate to chondroitin result of sulfate. The loss of elasticity and total compression is thought disk substance results in a decrease in disk to be mediated by an height with annular bulging. This altered inflammatory response biomechanical environment stimulates as well as nerve root Figure 1: Lateral cervical spine xrays of a patient with 6,7 formation of chondro-osseous spurs at edema and fibrosis. degenerative cervical spondylosis showing disk space narrowing, the annular insertion near the end-plates. end-plate sclerosis and osteophytes. 105 Volume 95 No. 4 Ap r i l 2012

�������� ���������������������������������������������������������������� ���������������������������������������� � Cervical Radiculopathy be obtained in essentially all patients with Treatment Cervical radiculopathy refers to neck pain or neurologic symptoms. Typi- Axial Neck Pain symptoms in a specific dermatomal dis- cal radiographic manifestations of cervical Nonoperative treatment is the stan- tribution in the upper extremity. Severe spondylosis include disk-space narrowing, dard for discogenic and axial neck pain. neck and arm pain is typical, partially al- end-plate sclerosis, and osteophytic changes Nonsteroidal anti-inflammatory agents, leviated by holding the arm over the head at the end-plates, uncovertebral joints, and narcotic analgesics, , or tilting the head to the contralateral side. facet joints. (Figure 1) The AP view allows muscle relaxants, and antidepressants Patients may report sensory or motor loss identification of cervical ribs and scoliotic are commonly used to relieve neck pain corresponding to the involved nerve root. deformity. The lateral view demonstrates and radiculopathy. A short period of rest On physical exam, symptoms are aggra- the degree of disk narrowing, the size and the use of a soft collar with the neck vated by extension and lateral rotation of of end-plate osteophytes, the size of the in mild flexion may sometimes alleviate the head (Spurling Maneuver). Sensory spinal canal, and overall sagittal alignment acute pain and spasm. , deficits, motor deficits, and diminished which may influence the choice of surgi- including isometric exercises, active range- reflex activity may be elucidated on exam. cal procedure. Flexion-extension views are of-motion exercises, aerobic conditioning, Care should be taken to differentiate critical to diagnose instability, which may and resistive exercises, has been found to cervical radiculopathy from compressive not be evident on a neutral lateral view. be helpful for patients with chronic neck lesions of of the upper extrem- Oblique views can be used for visualizing pain.12 In one study, nonoperative man- ity (i.e. carpal tunnel syndrome, cubital foraminal narrowing, which is typically due agement resulted in complete resolution tunnel syndrome) and electrodiagnostic to uncovertebral joint spurs. studies can often be useful. Additionally, Magnetic resonance imaging (MRI), patients with metabolic disorders, such while not indicated for every patient with as diabetes, who have neuropathy may neck pain, is the next step in the evalua- be more susceptible to radiculopathy and tion of the patient with a presumed diag- compressive neuropathy.2,8 nosis of spondylosis with myelopathy or radiculopathy. Persistent neck or arm pain Cervical Myelopathy of several months duration, neurologic The patient with cervical spondylotic findings, or a worsening symptomatic myelopathy may present with subtle find- picture warrants MRI. If evidence of my- ings that have been present for years or elopathy is present on exam, MR imaging with quadriparesis that developed over the is indicated to assess for disk herniation, course of a few hours. If the cord compres- hypertrophy, buckling of the ligamentum sion and myelopathy are either moderate flavum and the degree of cord compres- or severe, patients complain of gait and sion. One of the strengths of MR imaging balance abnormalities involving the lower is the ability to visualize the spinal cord, its extremities. They also have numbness or size, shape, quality (pathologic changes) in their upper extremities. and degree of compression.10,11 Fine motor control is usually affected as Although MR imaging provides well, and they will note changes in their optimal visualization of soft tissues, CT- handwriting or ability to manipulate but- myelography offers better definition of tons or zippers. Arm weakness is common. spurs or and ossified posterior lon- Leg weakness can occur, and patients gitudinal (OPLL). The exact de- may notice problems moving their body gree of cord deformation in the transverse weight, such as is necessary when rising out plane is more sharply visualized with CT- of a chair or going up stairs. The proximal myelography as well. This modality is also motor groups of the legs are more involved useful when MRI is contraindicated. than the distal groups. Changes in bowel Lastly, electrodiagnostic studies are or bladder function can occur in extremely sometimes used. For patients with cervical severe cases of myelopathy. On physical radiculopathy, electromyographic–nerve examination, the findings that establish conduction studies may be useful in con- the diagnosis are brisk reflexes, clonus, or sidering the of carpal pathological reflexes confirming an upper- tunnel syndrome, ulnar cubital tunnel motor-neuron lesion.1,9 syndrome, or thoracic outlet syndrome. Electrodiagnostic modalities may also Diagnosis help elucidate the confusing clinical pre- Plain radiographs are an important sentations of amyotrophic lateral sclerosis, part of the diagnostic workup, and an- multiple sclerosis, and severe peripheral Figure 2: AP (a) and lateral (b) views teroposterior (AP), lateral, and flexion- neuropathy.1 of a patient who had an anterior extension views of the cervical spine should cervicaldecompression and fusion at C5-C6 106 Medicine & Health/Rhode Island �������! �������������������������������������������������������������������������

of nonoperative measures. These patients mild symptoms of pain. Some may be should have neuroimaging studies dem- even symptomatic while others may go onstrating a pathological condition that on to develop severe pain and significant correlates with clinical findings and physi- neurologic deficit. cal exam. Surgery is also an option for Cervical spine involvement may be patients with a progressive motor deficit overlooked in spite of the fact that after or a disabling motor deficit from the ra- the and feet, cervical spine is the diculopathy.15 Surgical treatment options most common site of disease involving include anterior cervical discectomy and .19 The involvement fusion (ACDF) (Figures 2a and 2b), total of the cervical spine may be difficult to disc arthroplasty (Figures 3a and 3b), or detect due to systemic complaints or posterior decompression with fusion. lack of clear neurologic deficit on physi- cal exam. It is therefore imperative that Myelopathy the treating physician be well aware of Treatment of myelopathy is generally the natural history as well as the clinical surgical. Mild cases of myelopathy, usu- presentation in deciding what treatment ally consisting of mild upper extremity options are available for patients with symptoms, may respond to nonoperative rheumatoid arthritis. treatment but rarely resolves completely. In one prospective randomized study patients with mild-to-moderate nonpro- gressive or slowly progressive myelopathy were found to have similar outcomes after either nonoperative or operative treat- ment16. Another study reports that trial of nonoperative treatment did not decrease the potential for ultimate recovery of patients with mild myelopathy.17 Patients with severe or progressive myelopathy are candidates for surgical intervention. A number of factors such as the degree of neurologic dysfunction, patient disability, findings on radiographs and magnetic resonance imaging, dura- tion of symptoms, and presence of co- morbidities are considered in the decision regarding when to proceed with surgery. Figure 3: AP (a) and lateral view (b) of Surgical treatment options include a patient who had a total cervical disc anterior decompression utilizing either arthroplasty at C6-C7. discectomy and fusion (for single level disease) or corpectomy with strut graft of symptoms in 43% of the patients and fusion (for multilevel disease). Posterior partial resolution in 25%, whereas 32% decompressive procedures also can be had continued moderate or severe pain.12 used to treat cervical myelopathy. These In rare cases, some patients who fail con- procedures include (a canal servative management may improve with expanding procedure that maintains surgery for discogenic axial neck pain. stability posteriorly) (Figure 4a and 4b) or with instrumentation Radiculopathy and fusion. Cervical radiculopathy is first treated " ������������������������������������������������������������������������������#� �����������������#�������#$with conservative, nonoperative measures Rh e u m a t o i d Ar t h r i t i s o f t h e as described above. Additionally, some Ce r v i ca l Sp i n e authors have reported that epidural in- Rheumatoid arthritis of the cervical jections may be of short-term benefit to spine is not as common as degenerative patients with radiculopathy.13,14 arthritis but up to 90% of patients with Surgery is an option for patients the diagnosis of rheumatoid arthritis have Figure 4: AP (a) and lateral (b) cervical spine with persistent cervical radiculopathy and radiographic changes within the cervi- films of a patient who had laminoplasty disabling following failure cal spine.18 Many of these patients have performed for cervical myelopathy. 107 Volume 95 No. 4 Ap r i l 2012

�������� ��������������������������������������������%���������%�����$ Clinical Presentation Diagnostic Imaging Neck pain is the most Even asymptomatic patients with common presenting symptom known systemic rheumatoid arthritis in patients with rheumatoid should have periodic radiographs with arthritis. It can be present in flexion/extension views. Early detection of up to 80% of the patients.18 subluxation and close follow-up prevents Some patients with atlanto- the development of serious neurologic axial subluxation can have complications. a clunking sensation during If there is concern about the upper neck extension with reduction cervical spine with regard to the possibil- of atlanto-axial subluxation. ity of bone erosion a CT scan can be very This has been labeled a positive beneficial. If the clinical history and exam sharp-purser test.22 Addition- reveals neurologic deficit then an MRI of ally, patients may complain of the cervical spine is the diagnostic study stiffness, crepitance and pain- of choice. ful range of motion. Depending upon the lo- Treatment cation of the pathological pro- Non-surgical Treatment cess patients may also present While up to 90% of patients with with in the upper rheumatoid arthritis will have some Figure 5: Patient with rheumatoid arthritis and extremity as well as weakness involvement of the cervical spine only subaxial subluxation. involving both upper and about ten percent will become symp- lower extremities. Objective tomatic enough to warrant surgical Pathophysiology neurologic signs have been intervention. Patients with early cervical Rheumatoid arthritis is a systemic found to be present in seven to 34% of disease, intermittent pain and without disease characterized by inflammation patients.18 If significant compression of the radiographic instability or myelopathy and eventual destruction of the synovial spinal cord occurs myelopathy will develop can benefit from early aggressive medical joints. The cervical spine has 22 synovial causing significant weakness in addition treatment. This includes the use of non- joints and the inflammatory process may to gait disturbance. Subluxation may also steroidal anti-inflammatory , mirror that which occurs in synovial joints cause occlusion of vertebral arteries and mild analgesics and disease modifying throughout the body.18 The course of the vascular insufficiency to the spinal cord and anti-rheumatic drugs (DMARD).26 In disease in any patient is unpredictable. brain stem as well as the cerebellum. Pa- addition, the use of soft collars are appro- It may be very progressive. It may be tients may present with cranial nerve palsy, priate and patients seem to benefit greatly characterized by intermittent flare-ups paraplegia and even sudden death.23 from a comprehensive program of patient and remissions. In the cervical spine rheu- The classification system of Ranawat education, physical therapy with specific matoid arthritis may lead to instability, is commonly used to characterize the neu- focus on isometric strengthening of neck subluxation and spinal cord compression. rologic status of patients with rheumatoid muscles and postural training. Three characteristic patterns of instability arthritis. have been described. The most common area of involvement is the atlanto-axial or Classification System of Ranawat24 C1-C2 level. results in eventual destruction of the transverse ligament as Grade I No neurologic deficit well as bone erosion of the odontoid pro- Grade II Subjective weakness, hyperreflexia, dysethesia cess. Atlanto-axial subluxation occurs in up to 49% of patients.20 Sub-axial sublux- Grade III Subjective weakness and long tract signs ation is the second most common type and a.) Ambulatory b.) Quadriparetic non-ambulatory it is due to destruction of the facet joints below the C2 level. (Figure 5) It results in the characteristic staircase deformity and occurs in approximately 30% or patients Indications for radiographs of cervical spine in with rheumatoid arthritis.21 The third type rheumatoid arthritis patients25 of subluxation is atlanto-axial impaction with vertical subluxation of the axis. It 1. Prolonged cervical symptoms greater than 6 months occurs as C1, C2 and the occiput settle 2. Neurologic signs or symptoms due to erosion of the joints. It can lead to 3. Scheduled operative procedure requiring endotracheal intubation brain stem compression as the odontoid 4. Rapid progressive destruction of carpal or tarsal enters the foramen magnum. This occurs 5. Rapid overall functional deterioration. in 12-30% of rheumatoid patients.21 108 Medicine & Health/Rhode Island Surgical Treatment Re f e r e n c e s 21. Neva MH, Kaarela K, Kauppi M. Prevalence As with any surgery it is imperative 1. Emery, SE. Cervical Spondylotic Myelopathy: of radiographic changes in the cervical spine. A cross-sectional study after 20 years from that a thorough pre-operative assessment Diagnosis and Treatment. J Am Acad Orthop Surg. 2001;9(6):376–88. presentation of rheumatoid arthritis. J Rheum. be carried out in what is typically a very 2. Lees F, Turner JWA. Natural history and 2000;27:90–3. frail patient population. Serious consider- prognosis of cervical spondylosis. BMJ. 22. Sharp, J., Purser, D.W. Spontaneous atlanto- ation should be given to surgery in patients 1963;2:1607–10. axial dislocation in anklylosing and rheumatoid arthritis. Ann Rheum Dis. with progressive neurologic deficit as a 3. Lee MJ, Cassinelli EH, Riew KD. Prevalence of cervical spine stenosis: Anatomic study in cadav- 1961;20:47–50. result of spinal cord compression due to ers. J Bone Joint Surg (Am). 2007;89(2):376–80. 23. Zeidman SM, Ducker TB. Rheumatoid arthri- subluxation. In addition surgery for pa- 4. Bohlman HH. Cervical spondylosis and myel- tis; neuroanatomy, compression and grading of opathy. Instr Course Lect. 1995;44: 81–97. deficit.Spine. 1994;19:2259–66. tients experiencing severe unrelenting pain 24. Ranawat CS, O’Leary P, Pellicci P. Cervical spine unresponsive to are surgical 5. Wang B, Liu H, Wang H, Zhou D. Segmental insta- bility in cervical spondylotic myelopathy with severe fusion in rheumatoid arthritis. J Bone Joint Surg candidates. Relative indications for surgery disc degeneration. Spine. 2006;31: 1327–31. Am. 1979;61:1003–10. include patients who show radiographic 6. Chabot MC, Montgomery DM. The pathophys- 25. Kim PA, Hilibrand AS. Rheumatoid arthritis in the cervical spine. J Am Acad Orthop Surg. risk factors of impending neurologic injury, iology of axial and radicular neck pain. Semin Spine Surg. 1995;7:2–8.72. 2005;13:463–74. particularly when the space available for 7. Cooper RG, Freemont AJ, Hoyland JA, Jenkins 26. Hamilton JD, Gordon MM, McInnes JB, John- the spinal cord is 14 mm or less due to JP, West CG, Illingworth KJ, Jayson MI. Herni- ston RA, Madhok R, Capell EJ. Improved medi- subluxation. ated intervertebral disc-associated periradicular cal and surgical management of the cervical spine disease in patients with rheumatoid arthritis over Goals of surgical treatment in rheu- fibrosis and vascular abnormalities occur without inflammatory cell infiltration. Spine. ten years. Ann Rheum Dis. 2000;59:434–8. matoid arthritis of the cervical spine are 1995;20:591–8. 27. Boden, SD. Rheumatoid arthritis of the cervi- to decompress the spinal cord, achieve 8. Rao RD, Currier BL, Albert TJ, Bono CM, cal spine: surgical decision making based on spinal stability through fusion across Marawar SV, Poelstra KA, Eck JC. Degenerative predictors of paralysis and recovery. Spine. 1994;19:2275–80. the unstable segment, and to prevent ir- cervical spondylosis: clinical syndromes, patho- genesis, and management. J Bone Joint Surg 28. Boden SD, Dodge LD, Bohlman HH, Recht- reversible neurologic deficit and to avoid Am. 2007;89:1360–78. inger GR. Rheumatoid arthritis of the cervical catastrophic functional decline.27,28 9. Clarke E, Robinson PK. Cervical myelopathy: spine. Long term analysis with predictors of Surgical treatment of patients with A of cervical spondylosis. Brain. paralysis and recovery. J Bone Joint Surg Am. 1993;75:1282–97. rheumatoid arthritis of the cervical spine 1956;79:483–510. 10. Lehto IJ, Tertti MO, Komu ME, Paajanen HE, 29. Clark DR, Goetz DD, Menezes AH. Arthrod- can be successful. Over the past decade Tuominen J, Kormano MJ. Age-related MRI esis of the cervical spine in rheumatoid arthritis. outcomes have improved considerably changes at 0.1 T in cervical discs in asymptomatic J Bone Joint Surg Am. 1989;71:381–92. as a result of earlier diagnosis of my- subjects. Neuroradiology. 1994;36:49–53.3649 30. Peppelman WC, Kraus DR, Donaldson 11. Matsumoto M, Fujimura Y, Suzuki N, Nishi Y, WF, Agarwal A. Cervical spine surgery in elopathy and more aggressive medical Nakamura M, Yabe Y, Shiga H. MRI of cervical rheumatoid arthritis: Improvement of neuro- 29,30 management. intervertebral discs in asymptomatic subjects. J logic deficit after cervical spine fusion. Spine. Patients with rheumatoid arthritis Bone Joint Surg (Br). 1998;80:19–24.8019 1993;18:2375–79. 31. Reiter MF, Boden SD. Inflammatory disorders of have an overall higher complication rate 12. Chiu TT, Lam TH, Hedley AJ. A random- ized controlled trial on the efficacy of exercise the cervical spine. Spine. 1998;23, 2755-2766. than the general population. Complica- for patients with chronic neck pain. Spine. tions include infection, instrument failure 2005;30:E1–7.30E1. Matthew McDonnell, MD, is a Chief and lack of solid fusion in up to 25% of 13. Gore DR, Sepic SB, Gardner GM, Murray Resident in the Department of Orthopaedic the patients. The peri-operative mortality MP. Neck pain: a long-term follow-up of 205 patients. Spine. 1987;12:1–5. Surgery at the Warren Alpert Medical School rate is five to ten percent.30 14. Cicala RS, Thoni K, Angel JJ. Long-term results of Brown University and Rhode Island of cervical epidural steroid injections. Clin J Hospital. Summary Pain. 1989;5:143–5 Phillip Lucas, MD, is a Clinical The majority of patients with rheu- 15. Albert TJ, Murrell SE. Surgical management of cervical radiculopathy. J Am Acad Orthop Surg. Associate Professor in the Department of matoid arthritis involving the cervical 1999;7:368–76. Orthopaedic Surgery at the Warren Alpert spine can be managed non-operatively. 16. Kadanka Z, Mares M, Bednanik J, Smrcka V, Medical School of Brown University, and These patients should be monitored Krbec M, Stejskal L, Chaloupka R, et al. Ap- an Attending Surgeon at Rhode Island closely by the treating physician for the proaches to spondylotic cervical myelopathy: conservative versus surgical results in a 3-year Hospital. development of neurologic symptoms or follow-up study. Spine. 2002;27:2205–11. subluxation on radiographs. Non-surgical 17. Matsumoto M, Chiba K, Ishikawa M, Maruiwa treatment in the majority of patients H, Fujimura Y, Toyama Y. Relationships be- Disclosure of Financial Interests tween outcomes of conservative treatment and The authors and/or their spouses/ has a definite role. Several studies now magnetic resonance imaging findings in patients significant others have no financial inter- suggest early surgical intervention in with mild cervical myelopathy caused by soft ests to disclose. patients with progressive instability and disc herniations. Spine. 2001;26:1592–8. neurologic deficit is indicated to prevent 18. Rawlins BA, Jerardie FP, Boachie Adjei. Rheumatoid arthritis of the cervical spine. Corresponden c e significant morbidity and mortality in Rheumatoid Disease. Clinics of North America, 31 Matthew McDonnell, MD these patients. 1998;524:55–65. 19. Crockard HA. Surgical management of cervical 2 Dudley Street rheumatoid problems. Spine. 1995;20:2584–90. Providence, RI 02903 20. Morizono Y, Sakou T, Kawaida H. Upper cervi- [email protected] cal spine involvement in rheumatoid arthritis, Spine. 1987;12:721–5. 109 Volume 95 No. 4 Ap r i l 2012