81 S P I N A L D E G E N E R A T I V E D I S O R D E R S DUNCAN Q. MCBRIDE W eight bearing, flexion, extension, and rotatory movements all can cause gradual degeneration of intervertebral discs and spinal facet joints. Dehydration, degeneration, collapse, and herniation of intervertebral discs can occur. This causes failure of the spine to bear its normal axial load and can also cause compression and irritation of neural elements. The result is pain and neurologic dysfunction. The specific neurologic deficits are related to the exact location and function of the affected nerves. This chapter introduces the spectrum of degenerative disorders that involve the human spinal column. CASE 1 CERVICAL SPONDYLITIC MYELOPATHY A 66-year-old female complained of the insidious onset of neck pain radiating to her shoulders and arms, right greater than left. She was experiencing tingling, burning, and numbness in her right thumb and index finger. She also noted progressive upper and lower extremity weakness to the extent that she was unable to open a jar or turn a doorknob with her hands. She complained that her gait had become stiff and she was unable to run or to climb stairs without assistance. She denied difficulties with urination or anal sphincter control. She had mild atrophy of the thenar and hypothenar eminences, right greater than left. Her gait was slightly spastic in nature. Her mental status, cranial nerves, and cerebellar examinations were normal. She had 4+/5 weakness of the right biceps and brachioradialis muscles, and 5–/5 weakness of the wrist extensor on the right and the triceps bilaterally. She also had 5–/5 weakness of the lower extremities diffusely. There was hyperesthesia with tingling sensation on palpation in the index finger and thumb on the right side and into the radial aspect of the forearm. Pin/dull discrimination revealed error in the same distributions bilaterally, with diffuse errors in the trunk and the lower extremities. Joint position sense was intact. Deep tendon reflexes were absent at the biceps and brachioradialis. She had 2+ triceps reflexes and 3+ patellar and Achilles reflexes. The plantar reflexes were extensor bilaterally, and there was mild clonus at the ankles. Cervical spine radiographs showed the normal lordotic curve with osteophytic spurs at C4-C5 and C5-C6 with evidence of foraminal stenosis. An MRI scan demon587 5 8 8 N E U R O S U R G E R Y strated circumferential stenotic levels at C4-C5 and C5C6 due to disc herniation and osteophytes ventrally and ligamentous hypertrophy dorsally (Fig. 81.1). The stenosis at C5-C6 was quite severe with compression of the cervical spinal cord and lack of CSF signal at that level. There was marked stenosis of the neural foramina bilaterally, with greater narrowing on the right side. She was placed in a cervical collar and given dexamethasone acetate. An anterior cervical spinal decompression with discectomies at C4-C5 and C5-C6 was done urgently. The C5 vertebral body underwent corpectomy. The osteophyte and herniated disc segments were carefully elevated and removed. The posterior longitudinal ligament was opened and the dura allowed to relax forward into the defect created in the bone. Foraminotomies were performed at C5-C6 with careful excision of bony material from the neuroforamina. The spine was fused using autologous iliac crest. Postoperatively, she noted immediate improvement in her pain syndrome in the upper extremities and neck. She had residual numbness in the C5-C6 distribution, which gradually resolved. Her abnormal gait and lower extremity symptoms had begun to improve while on dexamethasone and continued to improve over the course of several months, such that she had a normal gait on long-term follow-up. At 6 weeks postoperatvely, she was able to discontinue the cervical collar orthosis. Cervical spine flexion/extension views demonstrated a good fusion of the bone graft with C4, C5, and C6 moving as one solid unit. CASE 2 LUMBAR DISC HERNIATION A 31-year-old male was lifting a couch when he felt a “pop” in his back. He suffered immediate severe lower back pain and could not straighten up properly. Within an hour, he began to note burning of the left leg from his buttock down to the dorsum of his foot. This pain was a shooting, electrical sensation that was quite severe. Whenever he tried to stand, walk, or sit or whenever he coughed or sneezed, he noted increased pain in the lower extremity. He spent 1 or 2 days in bed, which gave some relief; however, he sought medical attention when pain relievers and bed rest did not completely resolve his pain syndrome. He had not had difficulty urinating. On physical examination, he was in marked distress and unable to straighten, preferring to lie on the examination table. He had decreased light touch in the posterolateral aspect of his left thigh down into his calf and onto the dorsum of his foot, including the lateral aspect onto the plantar surface. He had normal power in his lower extremities except for his left extensor hallucis longus, which was 4/5. He was unable to stand on his toes on the left, and had difficulty with heel standing. The deep tendon reflexes were 2+ at the knees and the right ankle and absent at the left ankle. The straight leg raising test produced severe pain in the left leg at 30 degrees. The back was nontender to palpation, and there was no paraspinous muscle spasm. An MRI of the lumbar spine showed a large disc herniation with probable extrusion of disc fragment into the spinal canal on the left side at L5–S1 (Fig. 81.2). The disk caused entrapment of the L5 nerve root exiting at that level as well as the traversing S1 root. He underwent an L4-L5 hemilaminotomy on the left with microscopic discectomy. A large free disc fragment was found to be compressing the L5 and S1 nerve roots and it was removed. Postoperatively, he had immediate resolution of his severe pain. He rapidly recovered power in his toe and ankle, although he had persistent absence of the deep tendon reflex at the left ankle on long-term follow-up. D GENERAL CONSIDERATIONS egenerative disorders of the spine are quite common. Approximately 50% of individuals over 50 years of age who are studied with lumbar or cervical magnetic resonance imaging (MRI) scans show degenerative changes. Disc herniations can occur acutely with trauma, or insidiously with dehydration and degeneration of the disc and gradual inability of the disc to carry its normal weight and motion load. This causes the disc to lose its height and results in bony osteophyte formation, where bone edges touch that previously did not. This abnormal friction causes overgrowth of bony end-plates and results in bone spur formation (spondylosis). Furthermore, abnormal motion and chronic degenerative changes in posterior facet joints results in hypertrophy of the joint and the adjacent ligamentum flavum. This commonly occurs in the lumbar spine, resulting in progressive circumferential stenosis of the spinal canal with advanced age. In the cervical spine, this can be further aggravated by degeneration and hypertrophy of the uncovertebral joints that lie at the posterolateral aspects of the disc space bilaterally. The principal manifestations of spinal degenerative disease are pain and neurologic deficit. Patients with cervical spine disc herniation present with a syndrome of neck, arm, and head pain, typically in the dermatomal pattern of the exiting nerve root. Frequently this pain is a severe burning, tingling numbness that awakens the patient at night due to repeated minor trauma to the cervical nerve root. Pain often is felt in the paraspinous muscle area and at trigger points in the interscapular region and occipital areas. Cervical disc herniation or cervical spondylosis can also cause neurologic deficit in the spinal cord (Case 1). In these circumstances, patients will have long tract upper motor neuron findings manifested below the level of cervical compression, as well as lower motor neuron periph- S P I N A L D E G E N E R A T I V E D I S O R D E R S A B FIGURE 81.1 (A) Saggital T2-weighted MRI of the cervical spine demonstrating degenerative discs with spinal cord compression maximum at C4-C5 and C5-C6. Note increased signal intensity between these discs indicative of cord edema. (B) Axial CT image through the C5-C6 disc illustrates spinal canal stenosis and spinal cord compression. 5 8 9 5 9 0 N E U R O S U R G E R Y A B FIGURE 81.2 (A) Saggital T2-weighted MRI of the lumbar spine demonstrating degenerated herniated disc at L5–S1 with nerve root entrapment. (B) Axial view through L5–S1 disc illustrates neural foramenal and nerve root compression on the right from the disc extrusion. S P I N A L D E G E N E R A T I V E eral findings in the distribution of the nerve root at the level of compression. Lumbar spinal degeneration occurs by similar mechanisms. The highest percentage of cases occur below L4, and neurologic signs are virtually always peripheral nerve in nature. Patients with lumbar degenerative disease typically present with varying degrees of a combination of low back pain and leg pain. Radiating leg pain in the distribution of affected nerve roots is often called sciatica. Because low back pain is so common (60–80% incidence) the judicious use of conservative management, diagnostic testing, and surgery is extremely important. Thus, operation is limited to those patients who fail conservative management. The importance of psychosocial issues in the treatment of spinal degenerative disease cannot be overemphasized. All studies that have examined surgical outcome have demonstrated the effect of litigation. Other forms of secondary gain should also be considered, including familial sympathy, drug seeking, and other psychosocial motivators. K E Y P O I N T S • Disc herniations can occur acutely with trauma, or insidiously with dehydration and degeneration of the disc and gradual inability of the disc to carry its normal weight and motion load; this causes the disc to lose height and results in bony osteophyte formation, where bone edges touch that previously did not • Principal manifestations of spinal degenerative disease are pain and neurologic deficit • Patients with cervical spine disc herniation present with neck, arm, and head pain, typically in the dermatomal pattern of the exiting nerve root; frequently pain is severe burning, tingling numbness that awakens patient at night due to repeated minor trauma to cervical nerve root • In circumstances of neurologic deficit of spinal cord, patients will have long tract upper motor neuron findings manifested below the level of cervical compression, as well as lower motor neuron peripheral findings in the distribution of the nerve root at the level of compression • Highest percentage of cases occur below L4, and neurologic signs are virtually always peripheral nerve in nature • Radiating leg pain in the distribution of affected nerve roots often called sciatica T D I S O R D E R S head movements, and often the patient will feel more comfortable with the head tilted away from the side of maximum root compression. The patient may also complain of weakness and numbness in the hand or arm. Specific complaints depend on the level of compression and the degree of neurologic compromise. In those with spinal cord symptoms, the complaints include weakness or abnormal sensation in the legs as well as, potentially, bowel and bladder sphincter disturbance. As in lumbar disease, maneuvers that increase the degree of neural compression cause increased symptoms. These include extreme flexion, extension, or lateral rotation of the spine or Valsalva maneuvers that increase the intrathecal pressure. Thus, the patient’s complaints might include increased pain on coughing, sneezing, laughing, or defecation. Symptoms from lumbar disc disease are similar. Those with disease producing nerve root entrapment have leg symptoms that far outweigh the low back complaints. The patients have abnormal posture, tilting away from the side of disc herniation. The pain can be so severe that they cannot straighten completely or stand upright (Case 2). Symptoms of lumbar stenosis, without acute disc herniation, differ considerably from those of the acute herniated disc with stenosis. Patients typically have an insidious onset of pain rather than acute pain. The pain is exclusively in the legs and is often bilateral. If back pain does exist, it is typically a low-level dull ache in the uncomplicated stenosis patient. The hallmark feature of lumbar stenosis is onset of leg pain on walking some distance that is relieved by rest. Usually the patient will say they can walk a block or two and then they have to sit down to relieve the burning pain in the posterolateral aspects of their thighs and calves. In cervical degenerative disease, a wide variety of physical findings may be present. These include pain and tenderness in the paracervical musculature and at trigger points in the interscapular area. Limited range of motion in the neck may also be present. A thorough motor examination of the upper and lower extremities is essential. Motor testing results are given on a five-point scale (Table 81.1). Because the motor power scale is weighted toward the weaker end, clinicians often will include the subcategories of 4+ and 5– power to indicate various degrees of opposition to applied resistance (Case 2). As individual muscle groups are tested, their bulk and tone should also TABLE 81.1 Motor power scale DIAGNOSIS he chief complaint of a patient with cervical degenerative disease is likely to be neck, shoulder, arm, and possibly hand pain. The pain is frequently described as an electrical shooting, pins-and-needles sensation in the more distal regions of the nerve root and more of an ache or stabbing pain in the neck. The pain is exacerbated by 5 9 1 0 No contraction 1 Palpable or visible contraction with no joint movement 2 Movement of joint only when gravity eliminated 3 Can overcome gravity but not added resistance 4 Can push against added resistance, but weakly 5 Normal strength 5 9 2 N E U R O S U R G E R Y be documented. The presence of atrophy should be clearly defined. Sensory testing, including light touch, pin/dull discrimination and joint position sense, aid significantly in the localization of both radicular and spinal cord problems. The testing of deep tendon reflexes, Babinski response, Hoffmann response, and clonus of the ankles is essential in the diagnosis of upper and lower motor neuron problems. Analysis of the patient’s gait and ability to stand on heels and toes, and the Romberg test, further define the neurologic deficit that may be present (Table 81.2). Lumbar degenerative disease examination of the low back for scoliosis, percussion for deep tenderness, and palpation of paraspinous musculature, sacroiliac joint, and the sciatic notch for tenderness is done. Range of motion of the back, and examination of the hip, knee, and ankle should be noted. The typical syndromes for the lumbar and sacral roots are listed in Table 81.3. In herniation, a tension test should be done by straight leg raising. The patient is placed on the back and the leg is elevated by holding the ankle. This puts significant stretch on the nerve root that is compressed by the herniated disc. Absence of straight leg raising or other tension test findings should place significant doubt on the diagnosis of lumbar disc disease that is correctable by operative decompression. In patients with lumbar stenosis, all of the above mentioned root syndromes and tension test findings, are likely to be completely absent. In these patients, careful examination of the hip joint as well as the peripheral vascular pulses in the legs are extremely important to differentiate other commonly occurring sources of similar pain. Additionally, abdominal and rectal examinations are indicated since there are significant abdominal and retroperitoneal conditions (abdominal aortic aneurysm) that may produce leg pain. A large percentage of plain radiographs of the lumbar spine fail to diagnose the pathology completely, and further imaging is required. At present, MRI scanning is the imaging test of choice for initial evaluation of spinal disorders. Neuronal elements are clearly seen on MRI, and often, disc and bone pathology is adequately imaged by this technique. Computed tomography (CT) scan is superior to MRI in TABLE 81.2 LEVEL Lumbar sacral spine levels MOTOR SENSORY REFLEX L1 Iliopsoas Upper anterior thigh Cremasteric L2 Hip flexion, leg adduction Mid-anterior thigh None L3 Hip extension Anterior thigh, medial knee None L4 Quadriceps femoris Knee, medial calf Patellar L5 Ankle dorsiflexors, toe dorsiflexors (EHL) Lateral thigh and calf, dorsum foot to great toe None S1 Ankle, toe plantar flexors Lateral calf, ankle and foot, sole Achilles S2 Hamstrings Posterior thigh None S3–4 Anal sphincter Perianal Anal wink Abbreviation: EHL, extensor hallucis longus. imaging of bony foramina and for disc versus osteophyte differentiation. On occasion, patients require both MRI and CT scanning to complement each other. The best preoperative test remains the myelogram with postmyelogram CT scan. The myelogram, with intrathecal injection of water soluble contrast material, clearly delineates individual nerve roots as well as spinal cord anatomy. The cross-sectional CT scan with the contrast dye in the thecal sac, gives a superior image of the relationship of disc, bone, ligament, and nerve. In patients with multilevel problems, those with both cervical and lumbar degenerative problems, and those with difficult-to-diagnosis pathology, the myelogram remains the ultimate preoperative imaging test. K E Y P O I N T S • Pain exacerbated by head movements; often patient will feel more comfortable with head tilted away from side of maximum root compression • Patient may also complain of weakness and numbness in hand or arm • Patients typically have insidious onset of pain rather than acute pain, exclusively in legs and often bilateral Cervical spine levels SENSORY TABLE 81.3 LEVEL MOTOR REFLEX C1–3 Neck muscles Posterior head and neck None C4 Diaphragm Shoulder Pectoral C5 Deltoid Lateral upper arm (Biceps) C6 Biceps, wrist extensors Radial forearm, thumb, and index finger Biceps, brachioradialis C7 Triceps, wrist flexors Long finger and dorsal web of hand Triceps C8 Finger flexors Ring, little fingers, and ulnar forearm None • If back pain exists, typically low-level dull ache in uncomplicated stenosis patient; hallmark feature of lumbar stenosis is onset of leg pain upon walking some distance that is relieved by rest D DIFFERENTIAL DIAGNOSIS egenerative disorders can present in a similar fashion to cervical spondylitic disease. These include acute disc herniation, gradual disc collapse, osteophyte formation, cir- S P I N A L D E G E N E R A T I V E cumferential spinal stenosis, hypertrophied ligamentum flavum, ossification of the posterior longitudinal ligament (OPLL), and segmental instability. Rheumatoid arthritis and ankylosing spondylitis must also be considered. Tumors of the cervical spine, both intramedullary and extramedullary, can cause nerve root symptoms and long track findings. Central nervous system disorders such as multiple sclerosis and peripheral nerve entrapment syndromes commonly mimic cervical spine degenerative complaints. Peripheral nerve compression syndromes include carpal tunnel syndrome with entrapment of the median nerve in the wrist, and cubital tunnel syndrome with entrapment of the ulnar nerve at the elbow. The former is distinguished from a C6 or C7 radiculopathy by the presence of normal sensation in the radial nerve innervated portion of the dermatomes (volar aspect of the thumb, index, and long fingers). Tinel’s sign (electric shock sensation produced by tapping over the nerve) may be found at the point of entrapment. Often, however, one must perform EMG (electromyograph)/NCV (nerve conduction velocity) testing to distinguish between peripheral entrapment and cervical radiculopathy. Lumbar disc herniation or degenerative collapse, progressive lumbar stenosis with hypertrophy of the nerve roots in the lateral gutters, and spondylosis with osteophyte formation are all degenerative spinal disorders. Additionally, spondylolisthesis, when a vertebra is out of anteroposterior alignment with its neighbor (subluxation), can be degenerative, congenital, or traumatic. All these disorders can cause nerve root entrapment with varying degrees of associated low back pain. Retroperitoneal and pelvic pathologies including renal stones and neoplasms, can also mimic the lumbar pain syndrome. Back pain and neurologic symptoms and signs in the legs are occasionally caused by thoracic disc herniation. Claudication is pain in the calf on exertion due to poor arterial inflow. It can be distinguished from lumbar neurogenic claudication by the quality of the pain, which is usually a cramp or a tight sensation that progresses from distal to proximal in vascular disease. This is unlike the burning proximal to distal pain experienced by the patient with lumbar stenosis. In addition, those with vascular claudication will obtain some relief when standing up, whereas the patient with neurogenic claudication usually needs to sit or lie down to get relief from pain in the legs. Physical examination is very helpful in distinguishing these two disorders. A TREATMENT ll patients with cervical radicular signs and symptoms, without myelopathy, are given a course of conservative management in an attempt to relieve their pain. Those with profound motor weakness due to peripheral D I S O R D E R S 5 9 3 nerve entrapment who do not have myelopathy may be the exception to this rule. Conservative management of cervical spine disorders is immobilization with a cervical collar. Cervical traction is another option for pain due to a degenerative cervical spine. Application of axial traction in the neutral to slightly flexed position may open the interspace at the disc level and allow the neural foramen some relief of compression from either disc or osteophyte. Traction is applied intermittently during the day for as long as the patient can tolerate. It is used in addition to a soft cervical collar, which should be worn at all other times, especially at night. Anti-inflammatory agents, particularly nonsteroidal drugs, are most often prescribed. Analgesics can be helpful, but prolonged narcotic use should be avoided. Muscle relaxants are only indicated in patients with severe intermittent muscle spasm. Tricyclic antidepressants, particularly amitriptyline, are often used with considerable success in the nerve root entrapment syndromes. Immobilization and traction may address the cause of the patient’s pain, while other approaches merely treat the symptoms. With these modalities, a significant percentage will obtain relief of their symptoms. If significant neurologic findings are absent, the patient should be treated in this manner for at least 6 weeks. A conservative approach is indicated initially for all patients who present with the low back pain syndrome, with or without pain radiating into the leg. The exceptions to this rule are patients with acute cauda equina syndrome and those with documented progressive motor loss. Similar to cervical pathology, the mainstay of treatment is immobilization. This is best achieved in the lumbar spine by bed rest for at least 7–10 days. Patients with cervical stenosis and myelopathy, or patients with traumatic soft disc herniations with myelopathy, should be operated on without a prolonged attempt at conservative treatment. High dose steroids, either intravenous or oral, are indicated as a perioperative adjunct. Additionally, those with radicular symptoms who do not significantly improve with conservative management are in need of operation. In those cases, the degree of the patient’s pain and neurologic deficit determines the urgency of the elective operation. There are varying surgical approaches for relief of cervical degenerative radiculopathy and myelopathy. All patients with cervical spine pathology require extremely careful intubation, using an awake fiberoptic nasal technique with minimal head manipulation. In addition, they must be positioned very carefully to ensure that there is no exacerbation of cervical compression while anesthetized. Those with myelopathy should be monitored with intraoperative electrophysiologic evoked potentials. The typical approach in the presence of ventral cervical compression from a disc or osteophyte is an anterior cervical discectomy. This is done through a small incision 5 9 4 N E U R O S U R G E R Y in the anterior aspect of the neck, with removal of the intervertebral disc in a piecemeal fashion. Posterior cervical procedures include a focal laminectomy and foraminotomy for relief of unilateral radiculopathy due to a laterally placed disc or osteophyte. This small operation is often highly effective in relieving compressive symptoms of the root. Occasionally the ventral disc material can be removed as well. The major advantage of this approach is that it does not require cervical fusion. A more extensive bilateral laminectomy is indicated in patients with myelopathy who have greater than two levels of compression, especially those with circumferential compression that includes narrowing of the canal posteriorly. Operation for a lumbar disc should be performed acutely in those with cauda equina syndrome (loss of urinary control, weakness, sensory loss) or progressive motor loss. In addition, when degenerative lumbar stenosis is present, a significant response to conservative management is unlikely. Lumbar disc decompression should be performed only on those with pain syndromes predominantly in the leg. In addition, these patients must have failed a course of conservative management and be free of severe psychosocial problems. For some, litigation revolving around the disc herniation and its consequences is a relative contraindication to operation. A new, less invasive approach is currently being advocated. This is known as endoscopic discectomy and is performed through a similar posterolateral approach; however, this instrumentation is placed into the neuroforamen. With direct visualization of the disc and root through a microendoscope, the surgeon is able to remove disc material both from the spinal canal and from the intervertebral disc space. As this promising technique is quite new, determination of its safety and efficacy is yet to be made. P FOLLOW-UP atients who are motivated and unencumbered by secondary gain issues, who have clear-cut pathology and whose operation is successful, should have a very high percentage of cure from their pain syndrome. In those with myelopathy, the patient’s age and the duration and severity of the myelopathy will determine the outcome. Peripheral nerve injury generally has a favorable long term recovery rate due to wallerian regeneration. After anterior cervical approach and alignment of the cervical spine, the position of the bone graft and the ultimate fusion of the graft must be assessed. These patients are kept in a cervical collar until the graft is fused. When this occurs at 6 weeks to 2 months postoperatively, the patient is taken out of the collar and flexion/extension lateral cervical spine views are taken to ensure that the fused segment moves as one block. Those who have myelopathy or a significant motor loss from radiculopathy are treated postoperatively with physical and occupational therapy to maximize their recovery. Patients who have undergone simple lumbar laminectomy are generally mobilized to their feet on either the first or the second postoperative day and go directly into a physical therapy program to exercise their back and abdominal musculature, and assist them in ambulation. Postlaminectomy lumbar instability and recurrent disc herniations are both potential postoperative problems. SUGGESTED READINGS Chevrot A, Vallee C: Imaging of degenerative disk diseases. Curr Opin Radiol 4:103, 1992 A very useful reference on current modalities of diagnostic imaging. Hardin JG, Halla JT: Cervical spine and radicular pain syndromes. Curr Opin Rheumatol 7:136, 1995 A good general review of radicular pain syndromes. QUESTIONS 1. Lumbar degenerative disease most commonly presents with? A. Altered control of voiding. B. Sciatica. C. Neurogenic impotence. D. Lower extremity spasticity. 2. Lumbar stenosis, in contradistinction to acute disc herniation? A. Primarily has lower extremity complaints. B. Has pain with ambulation not relieved by rest. C. Has slower onset of symptoms. D. Is associated with severe and constant back pain. 3. The imaging test of choice for the initial diagnosis of spinal disorders is? A. Myelography. B. CT. C. MRI. D. Plain films of the spine. (See p. 605 for answers.)
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