A 44-year-old female patient presented to the emergency department after being found on the floor of her sober living home, stuck between a table and her bed. The patient has a history of inhalant use disorder and alcohol use disorder and, at presentation, had been sober for 100 days. She was not taking any medications and had no other pertinent past medical history. She stated that she experienced a restless sleep cycle and fell; though the nature of the position from which she fell (standing, sitting, lying, etc.) remained unclear. The patient reported struggling to free herself from between her table and bed for several hours before someone came to help. Her chief complaints included acute pain of the head, neck, and lower back. She also described muscle spasms in her lower extremities that she believed to be secondary to her previous inhalant use. The emergency room physician ordered a stat Computed Tomography (CT) head and Cervical spine (C-spine) to elucidate possible traumatic and/or coagulopathic etiologies of her acute pain.

The CT head was negative for any focal lesions; however, the CT C-spine was positive for a C2 fracture that extended from the right base of the dens, across the body and left lateral mass, traversing the foramen transversarium. This was classified as a Type III dens fracture with potential injury to the left vertebral artery. The CT also showed a fracture of left lateral body, pedicle, and facet of C7 on the left, as well as a fracture of the anterior endplate osteophytes at C6-C7. This scan also showed diffuse osteosclerosis. Due to these findings, a CT angiography was ordered to further evaluate the vertebral artery. The results of the CT angiography indicated a left vertebral artery Grade 1 injury, and confirmed the cervical spine fractures and diffuse osteosclerosis seen on the C-spine imaging. A CT of the abdomen was ordered for evaluation of internal injury. This scan returned negative, however it confirmed previous findings of diffuse osteosclerosis.

Neurosurgeon was consulted, and upon evaluation of the patient and review of the imaging studies, the patient was diagnosed with central cord compression at C6-C7. They recommended emergent cord decompression and vertebral fusion. Neurosurgeon also stated that the patient’s most extensive areas of stenosis were found at C3-T1, and further recommended emergent decompression and vertebral fusion to prevent further injury. These procedures were performed within twenty-four hours of diagnosis, in accordance with the AO Spine’s Clinical Practice Guidelines [1].

Following a successful operation, the patient was admitted to the surgical intensive care unit (SICU). She remained intubated for twenty-four hours due to concern for significant edema at the level of C3 and potential airway compromise. Four days after extubation, she was transferred to the internal medicine service. Here, the team assisted in managing her postoperative pain and chronic muscle spasms. While recovering, the patient’s pain and muscle spasms worsened. Additionally, in the last three of her twenty days on the internal medicine service, the patient became tachycardic, hypotensive, and febrile. Despite independent management of her hypotension, fever, and tachycardia, the patient’s pain remained poorly controlled. A CT and Magnetic Resonance Imaging (MRI) were conducted to rule-out Deep Vein Thrombosis and Pulmonary Embolism. Though the CT was negative, the results of the MRI indicated a spinal cord lesion not seen on prior imaging. Per the radiologist, this new lesion was suggestive of a reperfusion injury, otherwise termed “White Cord Syndrome” (WCS).

The two major differential diagnoses, as compared to the MRI findings of WCS, are the clinical diagnoses termed Compressive Myelopathy (CM) and Central Cord Syndrome (CCS). Both CM and CCS exhibit symptoms based on the neurological distribution of deficits.

CM presents with pain, weakness, and/or numbness and tingling. These deficits are the result of the compression of the spinal cord and often resolve after the area of compression is released [2-4]. Because CM most commonly occurs in the cervical spinal cord, [5] this differential diagnosis was strongly considered; however, CM is a chronic condition. This information renders this diagnosis unlikely due to the patient’s apparent new spinal cord lesion.

CCS is a traumatic spinal cord injury that damages the central portion of the spinal cord [6]. This injury frequently occurs in older adults with a history of cervical stenosis but can also occur in younger patients with sports-like injuries (e.g., blunt force applied to the crown of the head, as is often seen in football [6]). In patients with CCS, the most common presentation is some form of either complete or partial quadriplegia with greater weakness of the upper extremities than in the lower extremities.

WCS results from spinal cord decompression and is considered to be a radiologic diagnosis meaning that it is a diagnosis placed on a patient based on imaging alone. Its proper identification is due to the integral presence of newly developed lesions found on T2 MRI imaging and the absence of other factors in terms of the development of the patient’s biopathophysiological state [7]. The presentation of this syndrome can differ by area of injury, and can result in the clinical diagnosis of CCS, Brown-Sequard Syndrome, or other lesions of the spinal cord [7]. Because WCS is the radiological evidence of a process rather than a specific neurological pattern of deficits, it can present like multiple neurological syndromes. Nevertheless, unlike other neurological syndromes, WCS is the direct result of spinal cord decompression, not traumatic injury.

The patient’s neurological deficits were measured ssing the standardized physical examination skills detailed in Bate’s guide to the physical exam [8]. This patient demonstrated ? strength in all four extremities with markedly diminished grip strength. She exhibited autonomic instability, muscular spasms that were more apparent in her lower extremities, and fever. Her pain continued, uncontrolled even with the administration of opioid medications (5 mg of Oxycodon by oral intake every four hours, 5 mg of Oxycodon by oral intake with a maximum total dose of 15 mg in a 4-hour period, and 100 mg of Trazadone by oral intake PRN every night). In the context of this patient’s subjective symptoms and clinical course, imaging evidence suggested the most likely diagnosis was White Cord Syndrome (radiological diagnosis) causing Central Cord Syndrome (clinical diagnosis).

The patient was treated with Baclofen 5 mg taken orally (PO) three times daily (TID) for muscle spasm, Gabapentin 300 mg PO TID for pain, 5 mg of Oxycodon by oral intake every four hours for pain, 5 mg of Oxycodon by oral intake with a maximum total dose of 15 mg in a 4-hour period for pain, and 100 mg of Trazadone by oral intake PRN every night to help with sleep and pain. During her hospital course, she developed neurogenic bladder, neurogenic bowel, and an infection at the surgical site that was later drained by neurosurgery. Her grade 1 injury of the vertebral artery was treated with 81 mg of Aspirin to prevent the development of blood clots. The patient developed a urinary tract infection as a result of her neurogenic bladder and was treated with a seven-day course of Bactrim, dosed at 800 mg PO twice daily. The patient stated that by forcibly yawning she was able to partially overcome her neurogenic bladder and micturate on command.

One week following her spinal cord decompression surgery, the patient recovered bowel and bladder function, indicating that her neurogenic bowel and bladder resulted from edema within the spinal cord. She continued to experience muscular spasms, especially of her lower extremities, for which she was treated with Baclofen. When stable, the patient was discharged to a neurorehabilitation center to ensure that she would receive the appropriate physical and occupational therapy to help with her recovery.

WCS is a radiological diagnosis that results from a reperfusion injury of the spinal cord. An accompanying clinical diagnosis is made based on the symptoms that develop as the result of this injury. A reperfusion injury occurs when the blood supply is restored to an organ that has undergone hypoxic injury secondary to inadequate perfusion. When oxygen is reintroduced to the ischemic tissue, reactive oxygen species are formed, causing additional cellular damage and death. Reperfusion injuries involving the spinal cord have been shown to cause rapid deterioration of a patient’s neurologic status. The pathophysiology is as follows: the increased pressure within the spinal cord caused by the initial vertebral injury decreases upon injury repair [9]. This pressure deficit causes the cord size to expand and blood supply to return to the region, which leads to a breakdown of the blood-spinal cord barrier, resulting in edema and acute ischemia to the spinal cord [9]. The spinal cord ischemia is the direct result of the edema increasing the distance of the tissue from the blood supply. In addition to the ischemia, reactive oxygen species are formed during the reperfusion of the area, leading to damage of cellular machinery and cellular death within the region [10].

In this patient, the surgery performed to repair her C3-T1 injuries caused an unanticipated reperfusion injury. Consequently, she developed the neurological deficits often associated with CCS; however, her mechanism of injury differed. Rather than being the result of her initial fall, the neurological deficits were due to the decompression surgery. Typically, the neurological deficits seen in traumatic CCS are permanent because the shearing forces damage neurological fibers. In this patient, though, recovery was predicted to be more likely, given the mechanism of injury and the consequent lack of fiber disruption. With proper symptomatic management, her neurologic deficits were predicted to improve.

One area of particular interest focuses on an acute symptom described by the patient following her decompression surgery-her ability to induce a micturition response through yawning. In 1998, researchers demonstrated that 30 mg of Oxytocin injected intrathecally in rats resulted in both bladder contractions and a yawn response [11]. These researchers concluded that the descending pathway for the release of Oxytocin is likely linked to the Nitric Oxide pathway that stimulates the micturition response [11]. Based on the subjective statements from this patient, it is possible that the link between the descending pathways for Oxytocin and Nitric Oxide release might in fact be found in humans as well; however, a greater number of patients would need to be included in formal analysis to demonstrate an actual link.

Other case studies have been written about this particular syndrome (total of 8) with varying presentations of neurological deficits [7,12-17]. The authorial team wants to stress, that WCS is a radiological diagnosis, which means that it is a visual evidence of the infarction of the cord which can be seen on CT or MRI. Depending on the location of the infarction, the actual set of neurological deficits can change just as an infarction of the brain can have different presentations depending on location of the injury [7,12-17]. In examining the case studies available, no observable difference was noted between gender [7,12-17], instead all differences in the presentation were due to differences in location and in time to treatment [7,12-17]. The key reason that WCS must be considered after neurosurgical intervention, is that the patient’s treatment strategy is different than other types of spinal cord injuries and must be started as soon as possible with high dose corticosteroids to prevent further destruction of the irreplaceable spinal cord tissue [12].

While strongly contested in the neurosurgical specialty, the current treatment strategy for WCS includes the use of high dose corticosteroids (Methylprednisolone 30 mg/kg/15 min + 5.4 mg/kg/23 h) [6] and, if deemed necessary at the time of diagnosis, further decompressive surgery [12]. Other treatment strategies, such as symptomatic management, have also been implemented with success. Unfortunately, due to the lack of several case studies and larger trials (only 8 cases reported at time of writing), authoritative consensus regarding treatment strategy has not yet been established [7]. It is the opinion of the writing team that further studies be conducted, with focus on the use of anti-inflammatory medication, such as Methotrexate or Hydroxychloroquine, to reduce swelling and mitigate further injury to the spinal cord. These drugs reduce the immune response to the exposed central nervous system, caused by regional edema. Other medications to consider include nonsteroidal anti-inflammatory drugs (NSAIDs) which decrease the release of Prostaglandins and other arachidonic acid products, reducing the amount present in circulation and limiting the ability of these products to promote regional edema.

In the case of this patient, the final treatment strategy of symptomatic management and high dose corticosteroids was chosen. While this strategy is not ideal, as it can feel as though the team is not doing anything for the patient’s suffering, symptomatic management can be applied while trying to parse out the specific disorder. It is the recommendation of the team that further studies be conducted to determine a gold standard treatment technique for the benefit of patients diagnosed with “White Cord Syndrome”.

M. Payton Hughes: Nothing to disclose; Emily Courtois: Nothing to disclose; Rachel Daum: Nothing to disclose; Thomas C. Varkey: Thomas is a faculty member with the National Multiple Sclerosis Society’ Monthly Fellows Difficult Case Webinar, receives payment as a faculty member from the Colangelo College of Business at Grand Canyon University, and serves on the board of editors for ProClinS Cardiology.

All the authors are involved in conceptualization and critical revision of manuscript for publication.