cervical myelopathy info
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FIGURE 1. Axial computerizedtomography scan showing ventralosteophytes pressing into thespinal canal.
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Cervical Spondylotic Myelopathy: ACommon Cause of Spinal Cord Dysfunctionin Older PersonsWILLIAM F. YOUNG, M.D.,
Temple University Hospital, Philadelphia, Pennsylvania
Cervical spondylotic myelopathy is the most common cause of spinal cord dysfunction in olderpersons. The aging process results in degenerative changes in the cervical spine that, in advancedstages, can cause compression of the spinal cord. Symptoms often develop insidiously and arecharacterized by neck stiffness, arm pain, numbness in the hands, and weakness of the hands andlegs. The differential diagnosis includes any condition that can result in myelopathy, such as multiplesclerosis, amyotrophic lateral sclerosis and masses (such as metastatic tumors) that press on thespinal cord. The diagnosis is confirmed by magnetic resonance imaging that shows narrowing of thespinal canal caused by osteophytes, herniated discs and ligamentum flavum hypertrophy. Choice oftreatment remains controversial, surgical procedures designed to decompress the spinal cord and, insome cases, stabilize the spine are successful in many patients. (Am Fam Physician2000;62:1064-70,1073.)
Cervical spondylotic myelopathy (CSM) is the most common spinal cord disorder in personsmore than 55 years of age in North America and perhaps in the world. As the number of olderpersons in the United States increases, the incidence of CSM will most likely increase. In aprospective study designed to more accurately define the incidence of CSM, 23.6 percent of585 patients with tetraparesis or paraparesis admitted to a United Kingdom regionalneuroscience center had CSM.1 The overall prevalence in this population is unknown.
Pathophysiology of CSM
Spondylosis refers to the degenerative changes that occurin the spine, including degeneration of the joints,intervertebral discs, ligaments and connective tissue of thecervical vertebrae. There are three importantpathophysiologic factors in the development of CSM: (1)static mechanical; (2) dynamic mechanical; and (3) spinalcord ischemia.2 Static mechanical factors result in thereduction of spinal canal diameter and spinal cordcompression. With aging, the intervertebral discs dry outresulting in loss of disc height. This process puts greaterstress on the articular cartilage of the vertebrae and theirrespective end plates. Osteophytic spurs develop at themargins of these end plates (Figure 1). Osteophytesstabilize adjacent vertebrae whose hypermobility is causedby the degeneration of the disc.3
The disc also calcifies, further stabilizing the vertebrae. Osteophytes increase the weight-bearing surface of the end plates and, therefore, decrease the effective force being placed onthem. In addition to osteophytic overgrowth, the ligamentum flavum may stiffen and buckleinto the spinal cord dorsally. Osteophytic overgrowth ventrally and, in some cases, bucklingof the ligamentum flavum dorsally can cause direct compression of the spinal cord resultingin myelopathy (clinically evident spinal cord dysfunction). Symptoms are believed to developwhen the spinal cord has been reduced by at least 30 percent.4
Dynamic mechanical factors relate to the fact that the normal motion of the cervical spine mayaggravate spinal cord damage precipitated by direct mechanical static compression. Duringflexion, the spinal cord lengthens, thus stretching over ventral osteophytic ridges. Duringextension, the ligamentum flavum may buckle into the spinal cord causing a reduction ofavailable space for the spinal cord (Figure 2).
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Common complaints of patientswith cervical spondyloticmyelopathy include neckstiffness, crepitus in the neck withmovement, brachialgia, a dull"achy" feeling in the arm, andnumbness or tingling in thehands.
TABLE 1Clinical Presentation ofCervical SpondyloticMyelopathy
Common symptomsClumsy or weak handsLeg weakness or stiffnessNeck stiffnessPain in shoulders or armsUnsteady gait
Common signsAtrophy of the hand musculatureHyperreflexiaLhermitte's sign (electric shock-likesensation down the center of the backfollowing flexion of the neck)Sensory loss
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FIGURE 2. Dynamic mechanical factors in cervical spondylotic myelopathy. (Left) During flexion, thespinal cord is stretched over ventral osteophytic ridges. (Right) During extension, the ligamentumflavum may buckle into the spinal cord reducing space for the cord.
Spinal cord ischemia probably plays a role in the development of CSM, particularly in laterstages.5,6 Histopathologic changes in the spinal cord consistent with ischemia have beenobserved in patients with CSM. However, the precise mechanism for spinal cord ischemia isnot completely understood. Other factors associated with the development of spondylosisinclude heavy labor, posture and genetic predisposition.7,8 Also, 70 percent of patients withDown syndrome have an increased incidence of spondylosis by 50 years of age.9
Clinical History
Patients with CSM will generally have these symptoms:neck stiffness; unilateral or bilateral deep, aching neck,arm and shoulder pain; and possibly stiffness orclumsiness while walking (Table 1). CSM usuallydevelops insidiously. In the early stages of CSM,complaints of neck stiffness are common because ofthe presence of advanced cervical spondylosis.10 Othercommon complaints include crepitus in the neck withmovement; brachialgia, which is characterized as astabbing pain in the pre- or postaxial border of the arm,elbow, wrist or fingers; a dull "achy" feeling in the arm;and numbness or tingling in the hands.
Pain following a stereotypical dermatomal distribution is referred to as a radiculopathy ratherthan a myelopathy. For example, in patients with a disc herniation between the sixth andseventh vertebrae, pain radiates into the shoulder, upper arm, elbow, and index and middlefingers. It is typically unilateral. Numbness and weakness follow the same distribution. Somepatients will exhibit signs and symptoms of radiculopathy and myelopathy.
The hallmark symptom of CSM is weakness or stiffness in the legs.10,11 Patients with CSMmay also present with unsteadiness of gait. Weakness or clumsiness of the hands inconjunction with the legs is also characteristic of CSM. Symptoms may be asymmetricparticularly in the legs. Loss of sphincter control or frank incontinence is rare; however, somepatients may complain of slight hesitancy on urination.
Physical and Neurologic Examination
The physical and neurologic examination is usedto confirm the presence of spinal corddysfunction. Flexion of the neck may cause ageneralized "electric shock-like" sensation downthe center of the back,10 referred to as Lhermitte'ssign (Table 1). Atrophy of the hands, particularlythe intrinsic musculature, may be present.
Sensory abnormalities have a variable pattern onexamination. Loss of vibratory sense orproprioception in the extremities (especially thefeet) can occur. Superficial sensory loss may beasymmetric and persons are variably affected.The sensory examination may be confounded bythe presence of diabetes mellitus and aconcurrent peripheral neuropathy.
A characteristic physical finding of CSM ishyperreflexia. The biceps and supinator reflexes(C5 and C6) may be absent, with a brisk tricepsreflex (C7). This pattern is almost pathognomonicof cord compression because of cervical
spondylosis at the C5-C6 interspace.12 Ankle clonus and Babinski's sign (pathologicextension of the great toe elicited by stroking the foot) in the feet may also be revealed.Hoffmann's sign (a reflex contraction of the thumb and index finger after nipping the middlefinger) is a subtle indicator of spinal cord dysfunction. A stiff or spastic gait is alsocharacteristic of CSM in its later stages.
When cervical spondylosis is isolated to the C6-7, C7-T1 spinal levels, the arm reflexes maybe normal. A hyperactive pectoralis muscle reflex elicited by tapping the pectoralis tendon inthe deltopectoral groove causing adduction and internal rotation of the shoulder is a sign ofcord compression in the upper cervical spine (C2-3, C3-4 spinal levels).13 The "dynamic"Hoffmann's sign (when a typical Hoffmann's sign is elicited after having the patient flex andextend the neck multiple times) may be an indicator of early CSM.14 Hyperreflexia may beabsent in CSM patients who have concurrent diabetes, causing a peripheral neuropathy(Table 1).
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FIGURE 3. Sagittal magnetic resonance imagingshowing narrowing of the spinal canal as a result ofanterior herniated discs/osteophytes ( left) andposterior buckling of hypertrophied ligamentumflavum (r ight).
A characteristic physical finding ofcervical spondylotic myelopathy ishyperreflexia.
TABLE 2Diagnostic Criteria for CervicalSpondylotic Myelopathy
Characteristic symptoms (leg stiffness,hand weakness)
Characteristic signs (hyperreflexia, atrophyof hands)
MRI or CT (showing spinal stenosis andcord compression as a result ofosteophyte overgrowth, disc herniation,ligamentum hypertrophy)
MRI = magnetic resonance imaging; CT =computed tomography.
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Imaging and Diagnostic Studies
Magnetic resonance imaging (MRI) of thecervical spine is the procedure of choiceduring the initial screening process of patientswith suspected CSM.15 MRI is noninvasive andprovides images of the spine and spinal cordin several planes (Figure 3). In addition togiving an assessment of the degree of spinalcanal stenosis, an MRI can identify intrinsicspinal cord lesions that can also present withmyelopathy (e.g., tumors). High signalchanges seen in the spinal cord of patientswith CSM may indicate myelomalacia orpermanent spinal cord damage.
Computed tomography (CT) is complementaryto MRI (Table 2). CT may give a more accurateassessment of the amount of canalcompromise because it is superior to MRI inevaluating bone (osteophytes).16 Myelographyor the intrathecal injection of a contrast agentis used in conjunction with CT. Since theadvent of MRI, the use of myelography hasdecreased; however, it still provides usefulinformation in some instances for surgicalplanning. Plain radiographs alone are of littleuse as an initial diagnostic procedure.
Electromyography is rarely useful in most patients with CSM; however, it may help in theexclusion of specific syndromes such as peripheral neuropathy. Somatosensory evokedpotentials (SSEPs) provide a more direct assessment of spinal cord function (e.g., dorsalcolumn function) than electromyography.17 However, SSEPs are nonspecific and thereforetheir use as a diagnostic tool is undetermined.
Differential Diagnosis
The presence of myelopathy on neurologic examinationis not unique to CSM. Therefore, it is important toexclude other diagnoses that present in a similarfashion. In one study, it was found that 14.3 percent of
patients who underwent surgery for CSM were later found to have other diagnoses.18 Thisfinding could be an explanation for the lack of neurologic improvement after surgery in somecases.
Because cervical spondylosis is a universal finding in the elderly population, it is important tocorrelate cervical spondylotic changes with sensorimotor abnormalities identified onexamination.10 If there is a lack of correlation, there may be a demyelinating process (e.g.,multiple sclerosis). MRI is useful in this situation for identifying areas of demyelination in thespinal cord and cerebrum. In addition, a cerebrospinal fluid examination (e.g., oligoclonalbands) and visual evoked responses are important diagnostic adjuncts.
Amyotrophic lateral sclerosis (ALS) is anotherneurodegenerative disorder that can be confusedwith CSM. The absence of extremity sensoryabnormalities on neurologic examination of apatient with myelopathy should alert the physicianto the possibility of ALS. The presence offasciculations on examination and a denervationpattern on electromyography serve asconfirmatory evidence for ALS (Table 3).
Other conditions that can mimic CSM onpresentation are primary spinal cord tumors,syringomyelia, extramedullary conditions (e.g.,metastatic tumors), subacute combineddegeneration of the spinal cord (vitamin B12
deficiency), hereditary spastic paraplegia, normalpressure hydrocephalus and spinal cordinfarction (Table 4).10 Most of these conditions caneasily be distinguished from CSM based oncharacteristic MRI findings.
Treatment
Evaluating the efficacy of any particular treatment strategy for CSM is difficult becausereports show that as many as 18 percent of patients with CSM will improve spontaneously, 40percent will stabilize and approximately 40 percent will deteriorate if no treatment is given.19
Unfortunately, the current understanding of CSM does not allow physicians to predict thecourse of a patient. Also, the literature regarding various treatment strategies (surgical andnonsurgical) for CSM is flawed because of a lack of prospective controlled studies.
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The primary goal of surgery is todecompress the spinal cord, thusgiving the neural elements moreroom.
FIGURE 4. Postoperative magnetic resonanceimaging of patient in Figure 3 showing adequatedecompression of the spinal cord after multilevelposterior laminectomy.
TABLE 3Differential Diagnosis: CSM vs.ALS
Feature CSM ALS
Age Older than 55 Older than 55MRI findings Spondylosis SpondylosisFasciculations Absent PresentAtrophy of arms Present PresentAtrophy of legs Absent PresentDenervation Absent Present
CSM = cervical spondylotic myelopathy; ALS =amyotrophic lateral sclerosis; MRI = magneticresonance imaging.
TABLE 4Conditions That Mimic CervicalSpondylotic Myelopathy onPresentation
Amyotrophic lateral sclerosis
Extrinsic neoplasia (metastatic tumors)
Hereditary spastic paraplegia
Intrinsic neoplasia (tumors of spinal cordparenchyma)
Multiple sclerosis
Normal pressure hydrocephalus
Spinal cord infarction
Syringomyelia
Vitamin B12 deficiency
Nonsurgical TreatmentIn patients who are mildly affected by CSM, a "careful watching" approach can be taken. Avariety of nonsurgical strategies have been used with variable success for the treatment ofCSM. These include cervical traction, cervical immobilization (collar or neck brace), skulltraction and physical therapy. Cervical immobilization is the most commonly used treatment inthe United States. Some studies demonstrate the benefits of wearing a brace, while otherstudies show that immobilization does not improve the patient's condition.20 It has also beenreported that symptomatic patients may deteriorate neurologically during bracing, causingmany to advocate earlier surgical intervention.20,21 A nonsurgical approach is usuallyinadvisable.
Surgical TreatmentOnce frank myelopathy occurs, surgical intervention isnecessary. The primary goal of surgery is todecompress the spinal cord, thus giving the neuralelements more room. Traditionally, cervicallaminectomy, a posterior approach, has been used forsurgical treatment of CSM. However, over the past 20
years, it has been increasingly recognized that laminectomy is not appropriate for all patients.Further neurologic deterioration after laminectomy is attributed to a development of latentinstability of the spine with development of kyphotic spinal deformities and to the inability ofposterior approaches to directly address anterior vector compression secondary toosteophytic overgrowth.
For this reason, anterior approaches to thespine have been increasingly used.22 Throughan anterior cervical approach, one can directlyaddress and remove osteophytes and discmaterial for decompression of the spinal cord.Also, with the addition of interposition bonegrafts and, in some cases, cervical plates(instrumentation) to promote spinal fusion, thedevelopment of instability of the neck can beprevented. A variety of factors must beconsidered when deciding whether to use ananterior or posterior approach, but the primarygoal of both approaches is to provideadequate space for the spinal cord (Figure 4).
Many surgical series show "improvement," orat least stabilization of symptoms withposterior and anterior approaches. Afterreviewing the surgical literature, oneinvestigator found that the rate of successfuloutcome after surgery was at best 50 percentwith the potential for significant postsurgical morbidity.22 The older surgical literature hasbeen criticized because of the uncertainty of whether nonspondylotic myelopathy conditions(e.g., multiple sclerosis, ALS) had been sufficiently excluded before surgery.22
A variety of factors determine success after surgery. Factors that may portend a less thansatisfactory surgical outcome include severe preoperative neurologic deficits, abnormal signalchanges within the spinal cord and/or spinal cord atrophy seen on MRI, and severity of cordcompression seen on radiographic studies.23-25
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Final Comment
CSM is a common cause of disability in older persons. Because spondylosis is a universalfinding as patients age, it is important to correlate clinical history and neurologic findings withradiographic studies. MRI is the most useful radiographic study for quantifying the degree ofstenosis and excluding other pathologies. Current treatment remains controversial with regardto surgical and nonsurgical management. In the future, prospective randomized trials may berequired to definitively establish treatment guidelines. Currently, surgical decompression isappropriate for many symptomatic patients.
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