What is Central Cord Syndrome?
There are around 300,000 people in the US living with a spinal cord injury. There are also about 18,000 new cases every year. Central cord syndrome is the most frequent form of partial spinal cord injury, and it occurs in about 11,000 cases annually in the US. This syndrome primarily causes problems with movement, affecting the arms more than the legs. It can also lead to bladder issues.
Because its symptoms are pretty unique, central cord syndrome is often included in the potential causes of the “man in a barrel” syndrome. The severity of the symptoms can widely vary, and usually, it matches the extent of the nerve root injury.
What Causes Central Cord Syndrome?
Central cord syndrome is a condition where your spinal cord doesn’t fully function and is often caused when the neck is extended too far, putting pressure on the spinal cord. It’s thought that this kind of injury causes the spinal cord to be squeezed at the front by things like bony growths or material from the discs between your vertebrae and at the back by a ligament in your spine. This idea came from early studies using X-ray images of the spinal cord taken after injecting a special dye.
At first, Schneider and his team suggested that this pressure on the spinal cord caused internal bleeding, which in turn squeezed the surrounding white matter in the spinal cord.
Moreover, Schneider thought that the arms were more affected than the legs because of the specific arrangement of nerves in a particular area of the spinal cord (the lateral corticospinal tract), with the nerves controlling arm movement located closer to the middle. However, later research showed that the nerves for the upper and lower limbs are mixed throughout this area of the spinal cord, suggesting that the distinctive symptoms of central cord syndrome might be due to the hand and arm nerves being more densely packed in the lateral corticospinal tract. More recent studies found that the grey matter in the spinal cord wasn’t damaged and there was no signs of internal bleeding, instead disruption of the white matter axons, especially in the lateral corticospinal tracts, seems to be the main feature.
Less often, this condition can be caused by other issues such as age-related changes to your spine, instability between the first and second vertebrae in your neck, a cord that is pulled too tight, bone weakening disease, and spinal joint problems. This condition can damage the large nerves, disrupting nerve signals and causing problems with movement and sensation, particularly in the upper limbs, but also to some degree in the lower limbs. How much function is lost overall depends on how much damage is caused to the nerves.
Risk Factors and Frequency for Central Cord Syndrome
A study by McKinley and his team, looking back at 839 patients with spinal injuries at a top-level trauma center, found that Central Cord Syndrome (CCS) was the most commonly experienced type of spinal cord injury. This condition is primarily seen in males and typically occurs either through falls or car accidents among younger people or from overstretching injuries in older individuals who might also have pre-existing spinal conditions like osteoarthritis or cervical spondylosis.
- In the United States, 15 to 25% of spinal cord injuries are due to CCS.
- CCS can sometimes be overlooked if a patient’s initial symptoms are mild.
- An estimated 11,000 new cases of CCS occur each year in the United States.
Signs and Symptoms of Central Cord Syndrome
People who have injured their necks, particularly older individuals who have fallen and hyperextended their neck, might display a variety of symptoms. Oftentimes, they experience more profound weakness in their arms, especially their hands, as compared to their legs. Sensory abnormalities are also common, with changes in pain and temperature sensitivity, and sometimes even light touch, occurring below the injury site. These sensory issues tend to show up in a ‘cape-like’ pattern, stretching from the upper back down the arms. Along with these sensory changes, patients might experience neck pain where the spinal cord is being pressed upon.
In addition to these symptoms, some people may exhibit signs of upper motor neuron dysfunction, which can include problems with bladder control, often urinary retention, and priapism (persistent and painful erection). Even though the sensation in the lower back is typically unaffected, it’s crucial for healthcare providers to check the rectal tone to determine the compression’s severity.
Testing for Central Cord Syndrome
If doctors suspect that a patient might have a spinal cord injury, they’ll typically go forward with a radiographic evaluation, usually using a type of scan called a CT scan. This provides a quick imaging of the spine to help determine the extent of injury. In patients where a neck injury is suspected, additional images may be obtained to show the neck’s movement. CT scans can usually show if there’s any squeezing or pressure on the spinal canal.
In terms of guidelines relating to imaging, the American Association of Neurological Surgeons recommends not conducting imaging before taking off any neck braces in patients who are awake and not showing symptoms. For a more detailed analysis of the spine and surrounding areas such as blood vessels, ligaments and discs between the bones, an MRI scan is preferred. MRI scans are also recommended for patients with spinal cord injuries to cover the entire spine.
On the clinical side, patients with a condition known as central cord syndrome show symptoms such as weaker upper limb functions compared to lower limbs, varying degrees of sensitivity below the injury level, and possibly issues with urination or digestion. An objective criterion for diagnosing this condition is a difference of 10 between the upper and lower limb motor scores, according to a widely accepted classification for spinal cord injuries.
It’s important to identify the exact place of the spinal cord injury to guide the treatment strategy and to set functional rehabilitation goals. The ideal examination tool for spinal cord injuries is the ISNCSCI. More serious findings on this examination could point to a neck fracture. When central cord syndrome is paired with a neck fracture, recovery can take a longer time. The doctor must also assess other issues such as difficulty with breathing, pressure sores on the skin, issues with bowel and bladder function due to nerve damage, excessive muscle stiffness, a condition called autonomic dysreflexia, and issues with body-temperature regulation.
Treatment Options for Central Cord Syndrome
When managing spinal cord injuries, the first steps taken at the scene (referred to as the primary survey) involve checking airway, breathing, circulation, and any disabilities. This also includes assessing responsiveness using the Glasgow coma scale, examining the pupils of the eyes, and looking for any signs that point to disabilities. This process should be repeated whenever the patient’s condition changes. A secondary survey should then be carried out, inspecting the patient more closely including assessing the spine, checking limbs for motor function, checking for incontinence and examining the skin. Medical responders should also try to gather as much information from the patient as possible, paying attention to any complaints of neck pain. In suspected cases of spinal cord injury, it’s crucial to limit any movement of the spine.
Once the patient has been rushed to the hospital, healthcare providers should primarily focus on stabilising the patient’s condition by ensuring proper blood flow to reduce injury. Keeping the blood pressure of the patient steady is particularly important, with a target range set for at least seven days. However, studies don’t offer conclusive evidence on a perfect range for blood pressure.
The use of corticosteroids, such as intravenous (IV) methylprednisolone, to manage spinal cord injuries, generates varying opinions. While it is believed to stop the inflammation that can worsen spinal cord injuries, it also carries a risk of negative side effects. Some health organizations recommend against its use, whereas others advise that it should be administered within 8 hours of the injury, but for no longer than 24 hours.
Removing any backboards used for transport as soon as possible is important to avoid pressure sores. All patients suspected of having a spinal cord injury should undergo a comprehensive trauma evaluation in the emergency department with their neck kept stable. Precise imaging techniques and guidelines should be used to determine the need for continued neck immobilization.
Conservative treatment, which includes physical and occupational therapy, should only be considered in cases without fracture, dislocation, disc herniation, or spinal instability. If these conditions are present, surgery is generally preferred. The decision between conservative and surgical treatment, particularly for acute central cord syndrome, is still up for debate with no set guidelines. Some studies suggest that delaying surgery might result in better outcomes, while others propose that patients could benefit from immediate decompression surgery if they experience a decline or plateau in neurological recovery.
Since there are no rigid guidelines on which surgical procedure to choose, it’s up to the surgeon’s judgement on what will give the best results with least complications. Typically, an anterior approach might be used for single-level anterior compressive lesions, while a posterior approach is suitable for those with compression at multiple levels. For patients with spinal fractures or dislocations, a combination of the two approaches may be required.
What else can Central Cord Syndrome be?
When examining patients with a condition known as central cord syndrome, doctors also need to cross out other conditions with similar symptoms. Two of these similar conditions are cruciate paralysis and cervical root avulsion.
Cruciate paralysis is a rare nerve disease affecting part of our spine in the neck region. This disease can be caused by physical injuries, metabolic issues, or complications after surgery. A person suffering from cruciate paralysis would usually experience weakness in both upper arms and hands while the lower part of the body remains unaffected in most cases. The key factor that differentiates cruciate paralysis from other conditions is that it mainly affects specific nerve fibers in our spine in the neck region.
Cervical root avulsion, on the other hand, is a serious form of nerve injury that often results from intense trauma to the neck or one of the arms. The nerve damage can range from a slight loss of muscle function to complete paralysis requiring surgery to fix.
What to expect with Central Cord Syndrome
The natural pattern of central cord syndrome shows potential for recovery, even without surgery. However, the extent of recovery varies among individuals. Young patients who get immediate medical help usually have better chances of recovery. Factors like a person’s age, the initial severity of the condition, and findings from the initial MRI scan can inform the prognosis. Researchers found that patients below 50 years old often have better outcomes.
In most cases, patients with central cord syndrome regain a significant amount of their neurological function after the injury, including the ability to walk. However, some neurological issues may persist. Motor functions in the legs usually improve first, followed by bladder control, then the arms, with hand function being the last to improve. MRI scans can help predict the potential for neurological recovery.
Recovery usually slows after two years from the injury. By three years after the injury, patients generally regain at least 90% of their motor function, regardless of whether they underwent surgery or not. However, those who had surgical treatment were found to have better functional scores and were more likely to return to their pre-injury mobility status.
A study comparing central cord syndrome patients with other incomplete spinal cord injury patients found that the former group showed greater improvement and capability to walk after one year. Yet, they scored lower in “self-care” during that first year, likely due to impaired hand function. Those admitted to acute rehab with normal leg strength and motor recovery in both limbs, demonstrated the greatest improvements in executing daily activities at the end of their rehab. Even patients who didn’t show objective neurological recovery showed substantial functional improvements after acute rehab.
A national study showed that falls were the leading cause of this syndrome, mainly involving people with an average age of 60 years. More than half of cases were managed non-surgically, while the rest underwent surgery. The mortality rate stood at 2.6%, with older and those with greater health-related issues being more at risk.
Evidence indicates that performing surgery within 12 hours of the injury could improve recovery and is linked with fewer complications after surgery. However, the overall outcomes didn’t seem to differ between early and late surgical interventions. While early surgery might speed up recovery in some patients, long-term follow-ups didn’t highlight significant differences between those who had surgery and those who did not.
Certain factors like a lower preoperative Japanese Orthopedic Association score, long segment signal changes, and impingement suggest a poor prognosis. The severity of AIS at admission was found to be the strongest predictor of functional outcomes. Improvements should occur within the first 6 to 9 months, with the most significant improvement occurring in the first three months.
Neurological recovery in patients with central cord syndrome is influenced by the interactions between neurons, as well as networks affected by the lesion and those associated with recovery. Newer technologies like diffusion tensor imaging may prove useful. Additionally, therapies involving induced pluripotent stem cells might be a future therapeutic route.
Possible Complications When Diagnosed with Central Cord Syndrome
Patients with central cord syndrome, a type of spinal cord injury, are susceptible to a variety of complications.
Respiratory Issues:
Respiratory problems are a leading cause of illness and death in those with spinal cord injuries. Patients with damage to the cervical spinal cord are particularly at risk, especially if the injury is above the fifth vertebra (C5). This is because the injury can weaken the breathing muscles. Common respiratory conditions in these patients include acute respiratory failure, pneumonia, fluid around the lungs (pleural effusion), collapsed lung (pneumothorax), blood clots in the lungs (pulmonary embolism), and mucus plugs obstructing airways.
Cardiovascular Complications:
Heart-related issues often result from disrupted nerve function. Patients with minimally affected leg function are less likely to experience these complications. However, patients with reduced leg function may face issues like persistently low blood pressure, significant drop in blood pressure upon standing (orthostatic hypotension), and blood clots in veins (venous thromboembolism). To manage these conditions, patients are given fluid therapy for low blood pressure, and medications like fludrocortisone, ephedrine, and midodrine for orthostatic hypotension. Potential preventive measures include seeking medical emergencies only when necessary, avoid overheated environments, and eating smaller meals to prevent excessive blood flow to the digestive system.
Blood Clotting:
Patients with spinal cord injuries can develop venous thromboembolism, a serious condition involving the formation of blood clots in the veins. The risk can vary greatly among patients, ranging from 12 to 100%. Patients with central cord syndrome who have functional lower limbs are less likely to develop venous thromboembolism but it can also happen in upper limbs. To reduce the risk of such blood clot formation, doctors recommend prophylaxis treatment for a minimum of eight weeks after the injury.
Autonomic Dysreflexia:
This is a potentially life-threatening condition that often occurs in spinal cord injury patients. It is characterized by a disruptive response of the nerve system that controls functions like heart rate and constriction of blood vessels. Typically, this happens during the first month after the injury. Signs and symptoms may include high blood pressure, severe headaches, and increased anxiety. Managing this condition involves eliminating any identifiable causes and careful monitoring of the patient’s blood pressure.
Neuropathic Pain:
People with central cord syndrome can experience chronic pain associated with nerve damage at the injury level and beneath it. This pain is generally described as burning, tingling, sharp, or electric-like. Treatment often includes medications like gabapentin and tricyclic antidepressants, with additional consideration for SSRIs and SNRIs.
Spasticity:
Some patients with central cord syndrome experience spasticity, a condition involving disordered movement control resulting from nerve damage. Several treatments are available to manage spasticity, such as different forms of physical therapy and medications like GABA agonists and alpha-2-adrenergic agonists.
Genitourinary Complications:
Neurogenic bladder is often seen in central cord syndrome patients. This condition affects the bladder’s sensation and control, causing urgency, spasms, and frequent urination, along with incontinence. Treating this involves using a catheter; its long-term use may either require intermittent self-catheterization or having an indwelling catheter, with the former being the preferred method.
Pressure Injury:
About one-third of patients with central cord syndrome may develop pressure ulcers, which are sores that form due to tissue damage often at bony prominences. They are usually managed by avoiding immobility, regularly checking the prone parts, using an emollient on the part exposed to friction, using cushions, and maintaining adequate weight and nutrition.
Additional Complications:
The risk of traumatic brain injury (TBI) is found to be high among patients with traumatic spinal cord injury. Studies show that about 40 to 50% of such patients are also diagnosed with a TBI. Importantly, motor vehicle collisions are more likely to cause comorbid TBI compared to falls.
Recovery from Central Cord Syndrome
Currently, there aren’t any studies that compare people with central cord syndrome who have received rehabilitation services in a hospital to those who have not. However, after being discharged from the hospital, it’s suggested that these patients undergo occupational and physical therapy. These rehabilitation programs should be specifically designed based on the patient’s needs and the goals they want to achieve.
Physical therapy generally focuses on maintaining a good range of movement, building up strength, and improving coordination mainly in the lower body and middle section of the body. This helps increase walking ability (with or without using support equipment), aids in shifting movements and maintains balance. The therapy includes activities like stretching, therapeutic exercise, aerobic conditioning, training to climb stairs and shift positions, gait training, functional electrical stimulation, and water therapy.
Since central cord syndrome affects the upper body strength and function more than the lower body, occupational therapy should concentrate on improving hand strength and skill. This would help patients carry out daily activities like eating, dressing, bathing, and personal hygiene more independently. Using a splint when not in therapy could also help avoid the development of stiff, bent joints.
Preventing Central Cord Syndrome
People with central cord syndrome face both physical and mental challenges. It’s really important for different kinds of health professionals to communicate effectively with each other to ensure the best possible outcome for the patient. These professionals can include brain doctors (neurologists), general medical doctors (internists), brain surgeons (neurosurgeons), mental health doctors (psychiatrists), doctors who help with physical recovery (physiatrists), physical and occupational therapists, and nurses. Those suffering from this condition are often at risk of succumbing to depression and thoughts of suicide. Additionally, if a patient lacks motivation, it can slow down their recovery and may even result in permanent disability.
To help patients recover better, the medical team might use strategies like:
* Regularly checking in with the patient’s brain doctor, physiatrist, and general doctor.
* Teaching patients how to properly use a catheter (a tube for draining urine) to prevent urinary tract infections and other complications.
* Advising patients against staying immobile for long periods and recommending that they change positions every 2 to 4 hours.
* Regular sessions with a physical therapist for exercises that strengthen muscles to prevent them from wasting away.
* Checking blood pressure at home.
* Regularly meeting with a mental health doctor who can identify signs of depression and suicidal thoughts.
