A 68-year-old portly male is walking home after having a few drinks at a local north pole watering hole. He stumbles and tries to catch himself, but falls face first onto the ground. Luckily, his trusty red-nosed designated driver sees this happen and takes him to the closest emergency department where you happen to be working your sixth night in a row. While your schedule clearly indicates you are on your chief’s naughty list, you hope your astute diagnostic abilities will help you get on the nice list of your newest VIP customer.

Your primary survey indicates that the patient’s ABCs are intact, and your team is getting the patient on the monitor while placing an IV and drawing labs. You start doing a secondary survey and notice the patient cannot feel and is noticeably weak in his upper extremities. He lifts his legs off the bed with no problem and has intact sensation in both lower extremities. After a complete exam, the patient is rushed to radiology. The Head and C-Spine CT show no acute findings, but there are degenerative cervical spine changes. You suspect “badness” and order an MR cervical spine, which ultimately reveals the diagnosis.

 

What happened?

Our patient has acute traumatic central cord syndrome (ATCCS). ATCCS results from an incomplete spinal cord injury and involves a range of weakness and sensory deficits. These deficits are worse in the upper extremities compared to the lower extremities, with occasional bladder dysfunction. Sensory changes include loss of pain and temperature sensation and occasionally hyperesthesia in the upper extremities. In order to officially diagnose ATCCS, the patient’s American Spinal Injury Association (ASIA) motor score in the upper extremities must be ten points lower than the lower extremities. The score is determined by assessing motor strength in five upper extremity and five lower extremity muscle groups on a scale of zero to five as seen in the image of the scoring sheet below. A score of zero indicates total paralysis, and five indicates full range of motion against resistance. The sum for the upper extremities and lower extremities, respectively, is 50 for a normal score.1,2

The syndrome has a bimodal distribution. Younger patients present after high-velocity trauma any may have underlying congenital spinal canal narrowing, while older patients present after minor, low-velocity trauma and typically have underlying cervical stenosis or degenerative changes.3 ATCCS is the most common spinal cord injury and makes up approximately 9% of all traumatic spinal cord injuries.4

 

What causes the characteristic symptoms?

ATCCS results from hyperextension injury. Neck extension causes the ligamentum flavum to protrude into the spinal canal and pinch the spinal cord. After the initial injury, secondary factors cause further damage to the cord. These include edema and hematomyelia that progress to Wallerian degeneration in which the distal aspect of the injured nerves degenerate. In animal models, secondary injury is mediated by ischemia, neurotransmitter and electrolyte derangements, and lipid peroxidation that can cause further inflammation. The symptoms of central cord syndrome are due to compression of the spinothalamic and corticospinal tracts. The symptom pattern is thought to be related to upper extremity tracts being more medial to the lower extremity tracts; thus, upper extremity tracts are exposed to greater injury as shown in the image below. More recent evidence shows that the corticospinal tract may be preferentially involved in upper extremity motor function compared to lower extremity motor function, accounting for the symptoms.3,5

Moore D. Central Cord Syndrome. https://www.orthobullets.com/spine/2008/incomplete-spinal-cord-injuries

 

How do I manage these patients in the ED?

ED management of patients with ATCCS should follow basic trauma resuscitation guidelines. After stabilizing the ABCs, looking for neurologic deficits is important. Patients with suspected spinal fracture or spinal cord injury should be placed in a cervical collar, and spinal precautions should be maintained during the exam and imaging to prevent further damage.6 As discussed, clinicians should be especially vigilant when assessing elderly patients after minor falls and MVCs as lower-velocity traumas in the setting degenerative cervical spine changes increases the risk for ATCCS.1 While most patients will undergo initial CT, it will often be negative or show only cervical stenosis and/or degenerative changes. Patients with neurological deficits concerning for spinal cord injury must get an MR to identify ATCCS.3

 

Should I give them steroids?

Short answer, no. The use of steroids for spinal cord injury was a controversial topic for many years. A Cochrane review from 2012 recommended that initial management of patients with spinal cord injury should include high dose methylprednisolone started within eight hours of injury.7 However, those recommendations stemmed from a post-hoc subgroup analysis that was not reproduced in subsequent studies. In 2013, the American Association of Neurologic Surgeons and the Congress of Neurologic Surgeons updated their guidelines for pharmacologic therapy for acute spinal cord injury. They recommended against giving methylprednisolone, as there is no Class I or II evidence supporting its use. While Class III evidence exists, its low quality and the greater levels of evidence showing harm make steroid use illogical.8 Therefore, while steroid infusion was prevalent and considered the standard of care at one time, it has fallen out of favor and should be avoided in ATCCS.

 

Do I really have to wake up the neurosurgeons?

Neurosurgery should evaluate patients with central cord syndrome to determine if they may benefit from early surgery. Recent data has shown improved neurologic outcomes from early decompression. The Surgical Timing in Acute Spinal Cord Injury Study (STASCIS) was a prospective, multicenter cohort study that looked at early decompression surgery within 24 hours of injury compared to surgery after 24 hours. The primary outcome was change in ASIA impairment scale at 6-month follow up. The study showed that early surgery may result in improved neurologic outcomes.9 However, a subsequent systematic review of studies evaluating early decompression found that data was limited due to inconsistent definitions for ATCCS, highly heterogeneous patient populations, and no clear time limit for early surgery. As a result, they concluded that there was only low level evidence to support early surgery.5

 

Where do I send these patients?

All ATCCS patients should be admitted to an ICU setting for close cardiorespiratory and neurologic monitoring. Medical management for these patients focuses on maintaining their MAP between 85 to 90 mmHg for the first week to ensure adequate spinal cord perfusion.1 The Consortium of Spinal Cord Medicine recommends dopamine or norepinephrine for cervical or high thoracic injuries and phenylephrine for lower thoracic injuries. However, recent data has shown increased complications with dopamine use, making norepinephrine the preferred vasopressor.10

 

“Doc, will I ever get better?”

Patients with ATCCS usually have improvements in neurologic function with good outcomes. Early physical and occupational therapy should focus on maintaining remaining motor function and improving lower extremity and core muscle strength. This will allow earlier ambulation despite the more severe upper extremity symptoms preventing the use of assistive devices for ambulation.6 During their recovery, patients may experience complications such as spasticity and neuropathic pain. Neurologic improvement and positive outcomes are associated with high level of education, young age at injury, higher ASIA score at admission, no comorbidities, and no spasticity.3 While recovery is slow and patients may initially worsen during the first week, long-term prognosis is good with neurologic function improving over months to a year.

 

Summary:

  • ATCCS has a bimodal distribution and is the most common of the spinal cord injury syndromes
  • Patients with concerning neurologic findings after a trauma should undergo MR
  • Patients should be admitted to the ICU and evaluated by neurosurgery
  • Patients should be fluid resuscitated and vasopressors used to maintain a MAP of 85 to 90 mmHg
  • ATCCS improves with time and patients typically have neurologic recovery with good outcomes

 

References:

  1. Aarabi B, Hadley MN, Dhall SS, et al. Management of Acute Traumatic Central Cord Syndrome. Neurosurgery 2013;72:195–204.
  2. Singh AP. What Is ASIA Score and How It Helps In Classification of Spinal Injury [Internet]. Bone and Spine. 2017 [cited 2017 Nov 25];Available from: http://boneandspine.com/what-is-asia-score-and-how-it-helps-in-classification-of-spinal-injury/.
  3. Molliqaj G, Payer M, Schaller K, Tessitore E. Acute Traumatic Central Cord Syndrome: A Comprehensive Review. Neurochirurgie 2014;60(1-2):5–11.
  4. McKinley, W, et al. “Incidence and Outcomes of Spinal Cord Injury Clinical Syndromes.” Journal Spinal Cord Medicine, vol. 30, no. 3, 3 Jan. 2007, pp. 215–224., Accessed 1 Dec. 2017.
  5. Anderson KK, Tetreault L, Shamji MF, et al. Optimal Timing of Surgical Decompression for Acute Traumatic Central Cord Syndrome: A Systematic Review of the Literature. Neurosurgery 2015;77:S15–32.
  6. Stobart Gallagher MA, Gillis CC. Central Cord Syndrome. StatPearls [Internet] 2017 [cited 2017 Nov 12];Available from: https://www.ncbi.nlm.nih.gov/books/NBK441932.
  7. Bracken MB. Steroids for acute spinal cord injury. Cochrane Database of Systematic Reviews 2012.
  8. Hurlbert RJ, Hadley MN, Walters BC, et al. Pharmacological Therapy for Acute Spinal Cord Injury. Neurosurgery 2013;72(3):93–105.
  9. Fehlings MG, Vaccaro A, Wilson JR, et al. Early versus delayed decompression for traumatic cervical spinal cord injury: results of the Surgical Timing in Acute Spinal Cord Injury Study (STASCIS). PLoS One 2012;7(2).
  10. Saadeh YS, Smith BW, Joseph JR, et al. The impact of blood pressure management after spinal cord injury: a systematic review of the literature. Neurosurgical Focus 2017;43(5).

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