White cord syndrome
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White cord syndrome refers to the sudden onset of neurological deterioration following spinal decompressive surgery. The condition is believed to be a form of reperfusion injury of the spinal cord, not to be confused with central cord syndrome.
White cord syndrome is rare with only a few case reports published (c. 2019). All patients reported were above the age of 50 and 75% of published cases were males 1,3,4. No specific surgical approach has been correlated, both anterior and posterior decompression approaches were equally reported 3,4.
The clinical manifestation is non-specific and often described as unexplained neurologic deficits following cervical spinal cord decompression. The deficits are acute in nature and tend to manifest intraoperatively.
Neurological deficits in white cord syndrome tend to be motor in nature ranging from acute hemiparesis to quadriplegia. The severity of the neurological deficits depends on the size of the reperfusion injury and is usually consistent with the location of the hyperintensity signal on T2 MRI.
The cause is attributed to rapid cord expansion with acutely increased blood supply to the affected area following surgical decompression of a chronically compressed part of the spinal cord (e.g. due to tumor). These sudden changes are believed to result in a disruption in the blood-brain barrier (BBB) and the blood–spinal cord barrier (BSB). Such breakdown of the BBB and BSB may lead to increased permeability of inflammatory factors, resulting in neuronal apoptosis 1-5. The other well-established consequence of reperfusion is that significant amounts of free radical oxygen species (oxidants) are generated during the reperfusion, and thus, oxidative stress plays a crucial role in neural elements damage.
A 2015 rat model study explored the pathogenesis of reperfusion spinal cord injury. Their theory was that grey matter could be dislodged when acute cord compression was released 2. However, this understanding may change with the emergence of new scientific evidence as only a few clinical studies have been published 3-5.
The hallmark of white cord syndrome is the presence of intramedullary hyperintense signal on T2-weighted MRI sequences in a patient with unexplained neurologic deficits, following spinal cord decompression surgery 1-5.
Treatment and prognosis
Patients in all published case reports were treated with high-dose steroid protocols and showed remarkable improvement in symptoms. Improvement can be evident within the first 24-48 hours of treatment. Physical therapy was also shown to be effective in combination with steroid therapy 5.
History and etymology
White cord syndrome is a rare syndrome that was first described in 2013 1. Four case reports have been published to this date, all of which were associated with surgical decompression of the cervical cord.
The differential diagnosis generally includes other causes of signal change in the cervical spinal cord, however, the onset and course of these conditions tend to be quite delayed/chronic. These may include: