Spinal cord disease can result from diverse pathologic processes including trauma. Irrespective of the pathogenesis, it can lead to significant impairment of motor, sensory, or autonomic function.
This review focuses on the clinical description of common patterns of spinal cord involvement. Considerable differences exist in terms of clinical complications after traumatic and nontraumatic spinal cord injury (SCI). In this article, the general principles of management of traumatic SCI are emphasized. For specific nontraumatic neurologic diseases that affect the spinal cord, see Multiple Sclerosis, Amyotrophic Lateral Sclerosis, and other articles listed in Differentials.
Trauma to the spinal cord typically leads to a combination of symptoms and signs resulting from immediate and delayed injury.
The initial mechanical trauma is secondary to traction and compression forces. Direct compression of neural elements by bone fragments, disc material, and ligaments damages both the central and peripheral nervous systems. Blood vessel damage also leads to ischemia. Rupture of axons and neural cell membranes also occurs. Microhemorrhages occur within minutes in the central gray matter and progress over the next few hours. Massive cord swelling happens within minutes. The cord fills the whole spinal canal at the injury level and leads to further secondary ischemia. Loss of autoregulation and spinal shock cause systemic hypotension and exacerbate ischemia.
Ischemia, toxic metabolic compounds, and electrolyte changes cause a secondary injury cascade. Hypoperfusion of gray matter extends to the surrounding white matter and alters the propagation of action potentials along the axons, contributing to spinal shock. Glutamate is a key element in the excitotoxicity. Massive release of glutamate leads to overstimulation of neighbor neurons and production of free radicals, which kill healthy neurons. Excitotoxic mechanisms kill neurons and oligodendrocytes, leading to demyelination. AMPA (alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid) glutamate receptors play a major role in oligodendrocyte damage. Additionally, recent evidence has shown that a wave of apoptosis further affects the oligodendrocytes up to 4 segments from the trauma site days and weeks after the initial trauma. Syringomyelia may develop as one outcome of this cascade.
Traumatic SCI accounts for an estimated annual incidence of approximately 40 cases per million population, or approximately 12,500 new cases each year, in the United States (published figures range from 28-55 cases per million people). This number excludes those who died at the scene of an accident. Currently, 240,000-337,000 patients with SCI are alive in the United States.[1]
International
SCI incidence is estimated at 15-40 cases per million population. In Australia, recent statistics report an age-adjusted rate of 14.5 cases per million population.
Mortality/Morbidity
In 1927, Harvey Cushing described an 80% mortality rate for World War I soldiers with SCI in the first few weeks because of infections from bedsores and catheterization, with survival restricted to partial lesions. Today, in well-organized spinal cord centers, 94% of patients survive the initial hospitalization.
Recent statistics show the cost of the care of patients with C1-4 tetraplegia at approximately $1,048,259 in the first year and approximately $182,033 for each subsequent year.[1] Estimated lifetime costs for high tetraplegia are $4,651,158 for 25-year-old individuals and $2,556,197 for 50-year-old individuals. This amount does not include indirect costs such as loss of productivity, which vary with the educational background. Overall, lifetime costs range from $500,000 to $2 million, depending on the extent of injury and the location. Total direct costs for patients with SCI in the United States exceed $7 billion per year.
Life expectancy is greatly decreased, although major advances of medical management have markedly prolonged survival. Mortality rates are significantly higher during the first year after injury than during subsequent years. An Australian study found, among first-year survivors, overall 40-year survival rates were 47 and 62% for persons with tetraplegia and paraplegia, respectively. The most significant increases in mortality were seen in those with tetraplegia and American Spinal Injury Association Impairment Scale (AIS) grades A-C lesions, with SMRs between 5.4 and 9.0 for people < 50 years, reducing with advancing attained age.[2]
In the past, renal failure was the leading cause of death after SCI. Currently, pneumonia, pulmonary emboli, and septicemia surpass renal failure. For further details of the epidemiology, please see information provided by the National Spinal Cord Injury Association.
Race
Recent statistics show a rising incidence of SCI in African American people in the United States. Currently, about 24% of SCIs occur among blacks.[1]
According to the National Center for the Dissemination of Disability Research, from 1973-1978, 77.5% of the persons in the database were white people, 13.5% were black people, 5.7 % were Hispanic people, and 0.8% were Asian people. However, since 1990, only 59.1% were white people, while 27.6% were black people, 7.7% were Hispanic people, 0.4% were American Indian people, and 2.1% were Asian people.
Sex
Traumatic SCI is more common in young adult males, who are usually at a higher risk for motor vehicle accidents, violence, falls, and injury from recreational activities such as diving. Approximately 79% of spinal cord injuries occur among males. The male-to-female ratio in the United States is 4:1.[1]
Age
The average age at injury has increased from 29 years during the 1970s to 42 years since 2010.[1]
The rapid onset of symptoms after trauma usually makes the diagnosis obvious. With any trauma, especially to the head or neck and with whiplash injury, spinal injury should be immediately suspected. Patients with cervical stenosis may be especially prone to SCI, and the diagnosis may be challenging in patients after high cervical lesions, when unresponsiveness may follow hypotension and respiratory failure. C2 injuries, especially odontoid fractures, must be ruled out in older patients with neck pain after even a minor injury. SCI may be overlooked in patients with concomitant trauma to the head or to multiple body parts, especially if patients are confused or only have limited SCI. Therefore, SCI must be considered after any major traumatic event, and the patient's neck should be stabilized until SCI is ruled out.
SCI in elderly patients is also challenging. When patients with underlying cervical stenosis are found unresponsive after a fall at home or in a nursing home, diagnosis may be difficult because of concomitant multiple medical problems. In addition, respiratory distress or hypotension due to spinal shock may lead to a confusional state that may deviate attention to a brain lesion, prevent immediate diagnosis, and further contribute to worsening of the spinal lesion.
A high degree of suspicion is also warranted for patients who are at high risk for SCI because of concomitant medical problems such rheumatoid arthritis, Down syndrome, neck dystonia or torticollis, and congenital neck abnormalities.
Leg claudication may indicate lumbar spinal stenosis, especially if accompanied by weakness or numbness. Patients with cervical spinal stenosis can present with arm wasting and/or atrophy (ie, lower motor neuron changes) from anterior horn cell or root involvement and leg stiffness and/or spasticity (ie, upper motor neuron changes).
Acute SCI must be suspected whenever someone presents with a combination of autonomic (ie, urinary retention, constipation, ileus, hypothermia, hypotension, bradycardia), motor (ie, hemiplegia and/or hemiparesis sparing the face, paraplegia and/or paraparesis, tetraplegia and/or tetraparesis), and sensory (ie, lack of sensation at a certain level, hemisensory loss) symptoms. They vary according to the phase of SCI, ie, acute, subacute, or chronic.
In the acute phase, physicians must be vigilant in cases of sudden onset of quadriparesis (with or without respiratory distress); paraparesis; loss of sensation or bowel or bladder control; sexual dysfunction; or symptoms of neurogenic shock such as lightheadedness, diaphoresis, and bradycardia. The classic syndromes of incomplete SCI are described below.
In the subacute phase, patients may report pain, which can be progressive depending on pathology and rapidity of the process.
Complete spinal cord transection syndrome
In the acute phase, the classic syndrome of complete spinal cord transection at the high cervical level consists of respiratory insufficiency; quadriplegia with upper and lower extremity areflexia; anesthesia below the affected level; neurogenic shock (ie, hypothermia and hypotension without compensatory tachycardia); loss of rectal and bladder sphincter tone; and urinary and bowel retention leading to abdominal distention, ileus, and delayed gastric emptying. This constellation of symptoms is called spinal shock. Horner syndrome (ie, ipsilateral ptosis, miosis, anhydrosis) is also present with higher lesions because of interruption of the descending sympathetic pathways originating from the hypothalamus.
Lower cervical level injury spares the respiratory muscles. High thoracic lesions lead to paraparesis instead of quadriparesis, but autonomic symptoms are still marked. In lower thoracic and lumbar/sacral cord lesions, hypotension is not present but urinary and bowel retention are.
The presence of priapism following the spinal shock phase indicates the presence of complete spinal cord injury and is a marker for the progression to complete cord injury.[3]
Anterior cord syndrome
The anterior cord syndrome is typically observed with anterior spinal artery infarction and results in paralysis with loss of pain and temperature sensation below the level of the lesion and relative sparing of touch, vibration, and proprioception (because the posterior columns receive their primary blood supply from the posterior spinal arteries).
Central cord syndrome
Central cord syndrome is typically observed in syringomyelia, central canal ependymoma, and trauma. It is associated with more significant arm weakness than leg weakness and variable sensory deficits; often, the most affected sensory modalities are pain and temperature because the lateral spinothalamic tract fibers cross just ventral to the central canal. This is sometimes referred to as dissociated sensory loss and is often present in a capelike distribution.
Acute traumatic central cord syndrome is typically considered to be caused by a hemorrhage that affects the central part of the spinal cord, destroying the axons of the inner part of the corticospinal tract devoted to the motor control of the hands. However, others have proposed that destruction of the motor neurons supplying the muscles of the hand was the most likely cause. A recent MRI study corroborates the first hypothesis (corticospinal tract rather than motor neuron destruction).[4] The traumatic injury is usually caused by severe neck hyperextension and is characterized by initial quadriplegia replaced over minutes by leg recovery. In addition to the distal more than proximal arm weakness (man-in-a-barrel syndrome), bladder dysfunction, patch sensory loss below the level of the lesion, and considerable recovery occur.
Brown-Séquard syndrome
Brown-Séquard syndrome is essentially equivalent to a hemicordectomy. Ipsilaterally, paralysis, loss of vibration and position sense below the level of the lesion, hyperreflexia, and an extensor toe sign are present. In addition, ipsilateral segmental anesthesia occurs at the level of the lesion. Contralaterally, loss of pain and temperature sensation occurs below the level of the lesion (beginning perhaps 2-3 segments below). Brown-Séquard syndrome is more common after trauma. However, the full spectrum of this syndrome is rarely observed in clinical settings.
Cauda equina and conus medullaris syndromes
Patients with lesions affecting only the cauda equina can present with a polyradiculopathy with pain, radicular sensory changes, asymmetric lower motor neuron–type leg weakness, and sphincter disturbances. This can be difficult to distinguish from involvement of the lumbosacral plexus or multiple nerves. Lesions affecting only the conus medullaris cause early disturbance of bowel/bladder function.
Motor weakness (especially paraparesis or quadriparesis) can be flaccid in the acute phase or when the anterior horn is involved. Identification of affected muscle and the sensory level helps with injury localization.
Reflexes are lost immediately after SCI. Superficial abdominal reflexes are elicited by running a semisharp stimulus in any abdominal quadrant (upper quadrants are best) toward the umbilicus. Then, umbilical movement toward the stimulus (ie, abdominal muscle contraction in that quadrant) is observed.
The cremasteric reflex is elicited by running a semisharp stimulus down the upper inner thigh. As this is elicited, look for contraction of the cremasteric muscle (ie, scrotal elevation).
An anal wink is contraction of the anal sphincter on irritation, elicited by a light stroke with a semisharp stimulus to the perianal area. As this is elicited, look for a characteristic puckering of the anus.
The bulbocavernosus reflex is elicited by lightly tapping the dorsum of the penis or gently moving a urinary catheter, if in place. The intact reflex results in contraction of much of the pelvic floor musculature.
To check for a sensory level, separate testing of pinprick, light touch, and vibration senses is helpful in order to discriminate conditions such as Brown-Séquard syndrome. The stimulus should be applied and moved rostrally until a change is noted in the quality or intensity of the stimulus. This may be confirmed by moving caudally as well. Usually, some physiological overlap occurs at the sensory level when the examiner first moves rostrally then caudally. This examination may be performed anteriorly or posteriorly. Sensation over occiput should be checked when high cervical lesions are suspected because this area is supplied by upper cervical dorsal roots.
With the resolution of the spinal shock phase, areflexia and hyporeflexia are replaced by hyperreflexia with increased tone and extensor great toe sign (Babinski sign) develops. In humans, the spinal shock phase lasts for a few weeks, and it can be prolonged when the patient develops complications such as bedsores and urinary tract infections.
Withdrawal reflexes may be exaggerated to the point of flexor spasms and may be accompanied by sweating, piloerection, and automatic emptying of the bladder or rectum (also called the mass reflex).
The Beevor sign is elicited by having the patient flex the neck to look at the umbilicus; if the umbilicus moves upward, it implies intact abdominal motor control down to approximately the T10 level and loss of motor function below.
The Lhermitte sign or symptom results from neck flexion, which stretches and irritates damaged fibers in the dorsal columns of the cervical spine. It results in an electroshock sensation going down the arm or down the back, indicating probable meningeal or dorsal column pathology. It is a poor localizer within the cord.
Such injuries result from motor vehicle and workplace accidents, community violence, and recreational activities. In the United States, motor vehicle accidents account for 36-48%, violence for 5-29%, falls for 17-21%, and recreational activities for 7-16% of events.
The spinal cord is located inside the vertebral canal, which is formed by the foramina of 7 cervical, 12 thoracic, 5 lumbar, and 5 sacral vertebrae. Cervical and lumbar spondylosis are particularly common in elderly patients, making them prone to SCI. Cervical SCI is common with relatively minor trauma in patients older than 65 years. Return of functional motor recovery in this group is delayed.[5]
Laboratory studies are performed as indicated for the evaluation of a patient with trauma. While no specific tests for the evaluation of SCI are indicated for acute cases, these may be required with long-term medical complications of SCI. Analysis of spinal fluid may be necessary for the evaluation of nontraumatic causes of SCI such as transverse myelitis and to rule out other conditions when diagnosis is uncertain.
Anteroposterior, lateral, and special view (eg, odontoid and neuroforaminal) radiography is important to show alignment of bony structures. If cervical fracture is suspected, visualizing the T1 is important to avoid missing low cervical fractures or subluxation. Radiography can miss facet fractures, and dynamic radiographic views are often warranted. However, in the immediate setting, they are contraindicated and computerized tomographic (CT) imaging and magnetic resonance imaging (MRI) are the preferred methods. If one fracture is found, other levels should be carefully checked for additional injury. The absence of fractures does not ensure spinal column stability.
CT imaging is better for bone definition and is important when radiography shows injury or when an area is poorly visualized. CT imaging can also show soft tissue changes, cord edema, demyelination, cysts, abscesses, hemorrhage, and calcifications. CT myelography is preferred for better evaluation of spinal canal abnormalities.
MRI is the best method for definition of neural tissues. MRI findings correlate with neurologic status and help to establish prognosis. Patients with spinal cord injury without radiographic abnormality (SCIWORA) should undergo MRI testing of the suspected area, radiographic screening of the entire spinal cord, assessment of spinal stability with flexion-extension radiographs (in the acute setting and late follow-up, even with negative MRI), but neither spinal angiography or myelography is recommended.
See the image below.
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A T1-weighted MRI that depicts a lesion with high signal enhancement inside the cervical spinal cord. This type of signal enhancement is consistent wi....
Studies such as functional MRI, magnetoencephalography, positron emission tomography, transcranial magnetic stimulation, somatosensory evoked potentials, electromyography, and nerve conduction studies are used as research tools but are not indicated routinely.
Discussing each therapeutic strategy separately is difficult because of the diversity of etiologic processes and manifestations. Instead, this article focuses on general guidelines for the management of patients with spinal cord injury (SCI), especially after traumatic SCI.
Important advances in the medical and surgical management of SCI have occurred in recent years. The primary goal is to limit secondary injury.
Spinal stabilization, immobilization, and management of hemodynamic and/or autonomic disturbances are crucial in the acute injury phase, while management of gastrointestinal (eg, ileus, constipation, ulcers), genitourinary (eg, urinary tract infections, hydronephrosis), dermatologic (eg, bed sores), and musculoskeletal (eg, osteoporosis, fractures, overuse syndromes, acute and chronic pain) complications is the long-term goal.
Patients with acute traumatic central cord syndrome should have intensive care unit management, particularly if severe neurological deficits are present. Medical management should include hemodynamic and respiratory monitoring with maintenance of mean arterial pressure at 85-90 mmHg for the first week after injury to improve spinal cord perfusion.
If trauma is suspected, stabilize the head and neck manually or with a collar. Move the patient very carefully using the logroll technique to prevent lateral displacement. A spine board with restraints is recommended, but other items, such as head blocks, pillows, and cushions, may be useful. Careful and fast transportation of patients from the site of injury to the nearest medical facility is recommended; whenever possible, transportation to a specialized acute spinal cord injury treatment center is preferred.
Emergent radiation therapy may be required for metastatic disease. For spinal tumors causing mass effect, a few anecdotal protocols use dexamethasone in high doses of 10-100 mg IV followed by 6-10 mg IV q6h for 24 hours, potentially tapered intravenously or orally over 1-3 weeks.
A multicenter study reported reduced mortality rates after SCI with high doses of methylprednisolone administered within 8 hours, and this practice has been considered the standard of care in the United States.[6] However, this remains controversial because of increased risk of gastric bleeding and wound infection.
The Third National Acute Spinal Cord Injury Randomized Controlled Trial (NASCIS III) revealed that patients with acute spinal cord injury who receive methylprednisolone within 3 hours of injury should be maintained on therapy for 24 hours. When methylprednisolone is initiated 3-8 hours after injury, patients should be maintained on therapy for 48 hours.[7]
A small, 2-year, prospective study from Japan with 79 patients found no benefit from acute treatment and reported a higher incidence of pneumonia in the treated group.[8]
Several societies, including the Canadian Association of Emergency Physicians are no longer recommending this protocol as a standard of care for acute spinal cord injury management. The second iteration of guidelines for the management of acute cervical spine and spinal cord injuries, published in Neurosurgery in 2013, also did not recommend the use of methylprednisolone and down-graded the evidence to level III, as well as reporting that class I, II, and III evidence that steroids are associated with harmful effects, including death.[9, 10]
GM1 ganglioside, naloxone, and tirilazad had no benefit in a multicenter trial despite beneficial results in experimental animals.
Early surgery to remove damaging bone, disk, and foreign bodies is controversial unless severe compromise of the canal is clear, but surgical decompression of the spinal cord, particularly if the compression is focal and anterior, is recommended. Early reduction of fracture-dislocation injuries is also recommended. Surgical interventions in the subacute phase (ie, 24-72 h later) have yielded unsatisfactory results because most tissue damage is irreversible at that point.
In the acute phase, severe SCI, especially after high lesions, requires the attention of a specialized trauma team.
For long-term management, consultations with many specialists are often necessary because of the multiple organ complications that follow SCI.
Specifically, referral to a urologist, a gastroenterologist, a psychiatrist, a plastic surgeon, a dermatologist, and a pain management specialist may be necessary. Rehabilitation specialists such as physiatrists or neurologists become involved after the immediate hospitalization.
Clinical Context:
For various inflammatory diseases. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability.
For spinal tumors causing mass effect, a few anecdotal protocols use dexamethasone in high doses of 10-100 mg IV followed by 6-10 mg IV q6h for 24 h, potentially tapered IV or PO over 1-3 wk.
Have both anti-inflammatory (glucocorticoid) and salt-retaining (mineralocorticoid) properties. Glucocorticoids have profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.
A multicenter study demonstrated improved neurologic outcome, possibly by preventing the cascade of inflammatory events following SCI.
Rehabilitative efforts include physical, occupational, vocational, speech and recreational therapies.
In the immediate setting, at least passive range of motion activities to prevent contractures are required if the patient cannot participate actively.
Issues that must be addressed in the long term include contractures, postural abnormalities, spasticity, self-care, mobility, psychosocial adaptation, vocational and recreational skills, and adaptive equipment.
Spasticity can be addressed with drugs, including intrathecal baclofen, and nonpharmacologic approaches.
Special adaptive devices may allow patients with SCI to drive. For further information, the reader is referred to Patient Education.
In general, these interventions are more fruitful if family members and other caregivers are included.
Rehabilitative efforts also focus on long-term hemodynamic and other medical issues, including autonomic dysreflexia, respiratory care and conditioning, bowel and bladder continence, preventive gastrointestinal and genitourinary care, sexual dysfunction, depression, and skin integrity.
Various techniques to restore spinal cord function are under study, including cell transplantation. Despite promising results from animal studies, to date, no strategy has been proven effective in humans.
Repetition of task-specific movements can reactivate central pattern generators in the spinal cord and may help with ambulation and endurance. Training is currently under study in a multicenter trial. Techniques include partially supported treadmill walking and electrical stimulation of the extremity muscles to promote gait patterns.
Computer controlled transcutaneous activation of leg muscle can help with strength training and cardiovascular conditioning.
The Food and Drug Administration (FDA) –approved implantable Brindley-Finetech and Vocare bladder systems activate anterior sacral roots to regulate bladder and large bowel and urethral/anal sphincter contraction.
Sexual dysfunction must be systematically approached and treated with mechanical and pharmacologic interventions and psychosocial counseling. Testicular sperm retrieval and intracytoplasmic sperm injection are alternative techniques for failed conservative reproductive treatments in men with SCI seeking to conceive children.
Procreation is also an important issue because semen quality and motility is reduced in men because of repeated urinary tract infections. Women may experience life-threatening autonomic hyperreflexia during delivery.
Recently, the role of central pattern generators and the possibility of activating standing and stepping circuits after SCI even in chronic injury phase has been addressed.[11]
In 1914, Graham Brown demonstrated the existence of central pattern generators for walking in animals. This concept refers to neuronal networks capable of creating rhythmic motor activity in the absence of phasic sensory input.[12]
Theoretically, a similar system exists in humans and can be activated by repeated exercise or stimulation of the walking pathways. In cats, the spinal cord "learns" how to generate a continuous walking pattern known as fictive locomotion.[13] Based on these theoretical grounds, exercise programs have been developed, including suspended body weight support system over a treadmill to facilitate walking and bicycles designed for SCI. Reports of return of function several years after high cervical SCI in one patient (Christopher Reeves) have received a lot of attention, and further promising research is underway.[11]
After rehabilitation, most of the patients are treated by nonspecialists, but yearly follow-up should address all the topics detailed above and should ideally involve a physiatrist or SCI specialist.
Recent guidelines recommend that adults with a SCI should engage in (1) at least 20 minutes of moderate-to-vigorous aerobic activity 2 times per week and (2) strength training exercises 2 times per week, consisting of 3 sets of 8-10 repetitions of each exercise for each major muscle group.[14]
The main goal in long-term care is to prevent medical complications, which are the reason for which 30% of patients with spinal cord injury (SCI) require hospital admission every year. For a detailed discussion of medical management, see Sugarman's[15] 1985 article Medical complications of spinal cord injury and the 2002 article by McKinley et al[16] , Comparison of medical complications following nontraumatic and traumatic spinal cord injury. General medical guidelines include the following:
Administer empiric antibiotics as indicated, especially if the patient is febrile and epidural abscess is suspected.
Always maintain adequate perfusion to prevent further ischemic damage. Mean arterial pressure should ideally be above 70 mm Hg.
Intubate the patient if respiratory function is compromised either because of injuries (eg, C3-5, phrenic nerve, neck and/or chest) or because of easy fatigue of respiratory musculature.
Prevent nosocomial infections; treat aggressively and early if they occur.
Skin care to prevent decubitus ulcers includes vigilance, early treatment, use of air mattresses, frequent rolling and movement, and therapy.
Deep vein thrombosis and pulmonary embolism prophylaxis is paramount as well.
Bladder and bowel care includes preventing distention, discomfort, impaction, and infection.
Foley or intermittent sterile catheterization and/or rectal tube or stool softeners (eg, docusate) or stool bulking agents (eg, psyllium), depending on the extent of voluntary control present, may be needed; furthermore, enemas or suppositories may be needed to prevent or treat impactions.
Ascorbic acid (1 g PO qid) may help prevent infections by acidifying the urine; it may also help prevent urinary calculi.
Avoiding medications such as anticholinergic agents that can adversely affect bladder emptying is useful. Medications useful in the management of bladder function include bethanechol (used for flaccid paralysis but of questionable efficacy) and oxybutynin (for spastic paralysis). Avoidance of drugs with anticholinergic effect also helps in neurogenic bladder management.
Both the bowel and bladder sphincter reflexes can be trained to provide reflex emptying if lesions spare the lower motor neurons involved in micturition.
Judicious management of fluids, electrolytes, and nutrition is mandatory.
Pain and anxiety control is often required but may be difficult. Narcotics must be used judiciously or be avoided because of bowel and bladder adverse effects. Benzodiazepines, barbiturates, or any drugs causing depression of the CNS should also be used with caution because they can alter respiratory drive and exacerbate respiratory failure in patients with high spinal cord lesions. Such patients already have weak intercostal muscles and may experience further restriction of respiratory movements because of limitation of volitional movements.
Gastrointestinal prophylaxis against ulcers is also mandatory. Patients with SCI have a high incidence of stress ulcers, which can also be exacerbated by the concomitant use of steroids in the acute phase. The use of nonsteroidal anti-inflammatory drugs should also be avoided because of the risk of GI bleeding exacerbation.
Psychological and emotional support throughout the patient's disease course is necessary and is best provided informally and continuously by the caretakers; however, formal intervention by specialists may be required.
Referral to a regional trauma center or to a center with expertise on SCI may be advised because of the constellation of complications following SCI. Admission to an intensive care unit may be necessary for close hemodynamic monitoring or if concomitant head or abdominal trauma or multiple fractures are found.
Education programs teaching the general population and paramedics how to handle patients with an unstable neck and to think early about the possibility of SCI after trauma can prevent unnecessary worsening of SCI after injury.
In addition, educational programs for adolescents and families demonstrating common causes of SCI and the severity of SCI may help decrease its incidence.
In the acute phase, the classic syndrome of complete spinal cord transection at the high cervical level includes a constellation of symptoms called spinal shock. This syndrome consists of the following symptoms:
Respiratory insufficiency
Quadriplegia with upper and lower extremity areflexia
Anesthesia below the affected level
Neurogenic shock (ie, hypotension without compensatory tachycardia)
Loss of rectal and bladder sphincter tone
Urinary and bowel retention leading to abdominal distention, ileus, and delayed gastric emptying
Horner syndrome (ie, ipsilateral ptosis, miosis, anhydrosis): This is also present with higher lesions because of interruption of the descending sympathetic pathways originating from the hypothalamus.
Lower cervical level injury spares the respiratory muscles. High thoracic lesions lead to paraparesis instead of quadriparesis but autonomic symptoms are still marked. In lower thoracic and lower lesions, hypotension is not present but urinary and bowel retention are.
In the subacute phase, spinal shock is replaced by the return of intrinsic activity of spinal neurons. This usually happens in humans within 3 weeks. However, the spinal shock phase may be prolonged by other medical complications, such as infections.
Patients have persistent quadriplegia and sensory loss below the level, but spinal reflexes return.
Because modulation from supraspinal centers is lost, hyperreflexia with increased tone and extensor plantar responses are noted.
At any given level with more extensive involvement of the anterior horn, flaccidity with loss of reflex activity and atrophy are present in a lower motor neuron pattern, which is common in diseases such as poliomyelitis.
Autonomic hyperreflexia is also present in the subacute phase. Usually, the initial hypotension after high lesions resolves, although orthostatic hypotension persists.
For lesions above the lumbar/sacral centers for bladder control, the initial urinary retention is replaced by the development of an automatic spastic bladder.
Lower lesions lead to permanent atonic bladder (lower motor neuron pattern).
In humans, constipation persists and may contribute to delayed gastric emptying.
Autonomic hyperreflexia in this phase is characterized by massive firing of sympathetic neurons after distention, stimulation, or manipulation of the bladder and bowels.
Cutaneous stimulation with painful or cold stimuli can also lead to massive sympathetic firing.
This is a life-threatening condition because blood pressure may increase up to 300 mm Hg, leading to intracerebral hemorrhage, confusional states, and death.
A response to the massive sympathetic discharge is generated at the brainstem level. However, interruption of descending projections to the spinal cord can prevent the inhibition of the spinal cord sympathetic centers, which continue to fire inappropriately until the stimulus is removed.
A vagal inhibitory reflex to the heart is also generated, which leads to bradycardia and worsening symptoms.
Long-term, autonomic and somatic hyperreflexia cause severe spasticity and contractures in patients with high SCI. In the early 20th century, the chronic phase was called the third phase. It was characterized by the accumulation of bladder, skin, and bowel disorders, which eventually caused severe wasting and death. Fortunately, modern medical and nursing care have substantially prevented most of the complications.
Spasticity is a major complication of SCI, accompanying the other signs of upper motor neuron syndrome (see Spasticity). Its pathophysiology is incompletely understood, but synaptic reorganization within the spinal cord and loss of modulation by descending tracts play an important role.
Several strategies are available and should take into account the age, side effects of each treatment, and individual goals. The sedative properties of antispasticity drugs may interfere with learning in children. In addition, the increased lower extremity tone may allow the patient to stand and a decrease in tone may jeopardize mobility.
Physiotherapy is the most traditional form of treatment. Oral medications are usually helpful, but sedation is usually a limiting barrier. Benzodiazepines improve passive range of motion, hyperreflexia, painful spasms, and anxiety by affinity to the gamma aminobutyric acid (GABA) type A receptor complex. Baclofen is a GABA agonist, which must be started slowly to prevent sedation. It may be administered orally or through intrathecal infusion, the latter involves lesser adverse effects. Tizanidine is a new alternative. It is an alpha2 agonist and causes less weakness than baclofen and diazepam. Physiotherapy should also take into account the changes in muscle fiber proportion after spinal cord injury. Transformation away from type I fibers starts about 4-7 months after injury, reaching a steady state with predominantly fast glycolytic IIX fibers years after the injury.[17]
Injections of botulinum toxin, phenol, alcohol, and lidocaine can also be used in selected patients.
Surgical treatments are usually the last option and include selective posterior rhizotomy, lengthening or release of muscles and tendons, and procedures such as osteotomy, used to correct deformities.
Sexual dysfunction is common, especially with complete lesions. Many options are available, but 2 recent efficacy and safety trials in men with traumatic SCI revealed that tadalafil[18] (doses of 10 mg and 20 mg) or vardenafil[19] (10-20 mg) were good options for the treatment of erectile dysfunction secondary to traumatic SCI.
For a more detailed view of all medical complications in patients with SCI, see Sugarman's 1985 article.[15]
Spinal trauma and other spinal cord diseases often cause severe physical impairment secondary to motor, sensory, and autonomic impairment.
Overall, life expectancy is greatly decreased, although major advances of medical management have markedly prolonged survival.
The ability to predict clinical outcome after SCI based on early examination is limited. The most important predictor of improved outcome is retention of sacral sensation (S4-5), especially pinprick, 72 hours to 1 week after injury. In general, most individuals regain one level of motor function, mostly within the first 6 months, although further improvement can be observed years later. Age is also a prognostic factor. In central cord syndrome, 91% of patients younger than 50 years regain ambulation, whereas only 41% of people older than 50 years develop a similar outcome.
Transient or chronic reactive mild or severe depression is very common after SCI. In the case of trauma, younger patients often have greater functional improvement from acute spinal injury. The suicide rate among individuals with SCI is nearly 5 times higher than in the general population and is lower for men than for women. In 1998, Hartkopp et al also observed a 2 times higher suicide rate in marginally disabled persons compared to more severely affected individuals.[20]
Education is an essential element for efficient rehabilitation and for prevention of the multiple complications following SCI. Multiple Web sites and written literature are available for the education of the patient and family.
For more details about available programs, please see the following:
The National Spinal Cord Injury Association
ThinkFirst National Injury Prevention Foundation
Spinal Times
For excellent patient education resources, see eMedicineHealth's patient education article Bladder Control Problems.
Francisco de Assis Aquino Gondim, MD, PhD, MSc, FAAN, Professor Adjunto of Neurology and Clinical Skills, Department of Internal Medicine, Universidade Federal do Ceará, Brazil
Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Consultant for Pfizer, PTC Therapeutics and Alnylam<br/>Serve(d) as a speaker or a member of a speakers bureau for: Speaker for Shire, PTC Therapeutics and BSL Beringer<br/>Received travel grants from for: Aché, Biogen, Genzyme, Ipsen, Novartis, Baxter, Teva, Pfizer.
Coauthor(s)
Florian P Thomas, MD, PhD, MA, MS, Chair, Neuroscience Institute and Department of Neurology, Director, National MS Society Multiple Sclerosis Center and Hereditary Neuropathy Foundation Center of Excellence, Hackensack University Medical Center; Founding Chair and Professor, Department of Neurology, Hackensack Meridian School of Medicine at Seton Hall University; Professor Emeritus, Department of Neurology, St Louis University School of Medicine; Editor-in-Chief, Journal of Spinal Cord Medicine
Disclosure: Nothing to disclose.
Specialty Editors
Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Received salary from Medscape for employment. for: Medscape.
Howard S Kirshner, MD, Professor of Neurology, Psychiatry and Hearing and Speech Sciences, Vice Chairman, Department of Neurology, Vanderbilt University School of Medicine; Director, Vanderbilt Stroke Center; Program Director, Stroke Service, Vanderbilt Stallworth Rehabilitation Hospital; Consulting Staff, Department of Neurology, Nashville Veterans Affairs Medical Center
Disclosure: Nothing to disclose.
Chief Editor
Stephen A Berman, MD, PhD, MBA, Professor of Neurology, University of Central Florida College of Medicine
Disclosure: Nothing to disclose.
Additional Contributors
Stephen A Berman, MD, PhD, MBA, Professor of Neurology, University of Central Florida College of Medicine
Disclosure: Nothing to disclose.
References
National SCI Database. Spinal Cord Injury (SCI) Facts and Figures at a Glance. The National SCI Statistical Center. Available at https://www.nscisc.uab.edu/PublicDocuments/fact_figures_docs/Facts%202014.pdf. August 2014; Accessed: December 28, 2015.
Edgerton VR, Harkena SJ, Dobkin BH. Retraining the human spinal cord. Lin VW, ed. Spinal Cord Medicine: Principles and Practice. New York, NY: Demos; 2003. 817-26.
A T1-weighted MRI that depicts a lesion with high signal enhancement inside the cervical spinal cord. This type of signal enhancement is consistent with blood and is most commonly observed secondary to cord trauma.
A T1-weighted MRI that depicts a lesion with high signal enhancement inside the cervical spinal cord. This type of signal enhancement is consistent with blood and is most commonly observed secondary to cord trauma.
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