Reduction of central neuropathic pain with ketamine infusion in a patient with Ehlers–Danlos syndrome: a case report

Abstract

ObjectiveEhlers–Danlos syndrome frequently causes acute and chronic pain because of joint subluxations and dislocations secondary to hypermobility. Current treatments for pain related to Ehlers–Danlos syndrome and central pain syndrome are inadequate. This case report discusses the therapeutic use of ketamine intravenous infusion as an alternative.Case reportA 27-year-old Caucasian female with a history of Ehlers–Danlos syndrome and spinal cord ischemic myelopathy resulting in central pain syndrome, presented with severe generalized body pain refractory to multiple pharmacological interventions. After a 7-day course of ketamine intravenous infusion under controlled generalized sedation in the intensive care unit, the patient reported a dramatic reduction in pain levels from 7–8 out of 10 to 0–3 out of 10 on a numeric rating scale and had a significant functional improvement. The patient tolerated a reduction in her pain medication regimen, which originally included opioids, gabapentin, pregabalin, tricyclic antidepressants, and nonsteroidal anti-inflammatory drugs.ConclusionKetamine infusion treatment has been used in various pain syndromes, including central neuropathic pain, ischemic pain, and regional pain syndrome. Reports have suggested that ketamine modulates pain by the regression of N-methyl-D-aspartate receptor to a resting state. As such, propagation of nociceptive signal to brain is interrupted allowing for the restoration of physiological balance between pain inhibition and facilitation. The present report shows that this treatment option can be used in patients with refractory central pain syndrome in the setting of spinal cord myelopathy secondary to Ehlers–Danlos syndrome. In addition, as seen in this case, this protocol can potentially decrease the chronic use of pain medication, such as opioids.

Consensus Guidelines on the Use of Intravenous Ketamine Infusions for Acute Pain Management From the American Society of Regional Anesthesia and Pain Medicine, the American Academy of Pain Medicine, and the American Society of Anesthesiologists

“…Other acute pain conditions outside the perioperative setting in which ketamine has anecdotally been reported to be effective include postendoscopic retrograde cholangiopancreatography pancreatitis pain, 23 renal colic, 24 and exacerbation of central pain from Ehlers-Danlos syndrome. 25 …”

Intravenous Ketamine Administered as Patient Controlled Analgesia and Continuous Infusion for Central Pain Syndrome

“…Ketamine, on the other hand, was recently approved by the FDA as a treatment for major depression and demonstrates a lasting antidepressant efficacy (12). The mechanism behind ketamine's antidepressant effects are unknown but are thought to be related to the inhibition of NMDA receptors, leading to increased production of proteins that are normally decreased in patients with major depression (13). The advantage of ketamine is that it can simultaneously treat a patient's physical pain as well as their emotional pain.…”

Identification of a de novo case of COL5A1‐related Ehlers‐Danlos syndrome in an infant in the West Indies leading to improved targeted clinical care

“…This educational material was then placed into the child's medical file. We were also able to include advice for combined management of pain including analgesics, heat, and physical therapy 23, 24, 25, 26, 27, 28. Lastly, dental problems have been reported for patients with classical EDS,29 and the family have been advised as to the importance of good dental hygiene.…”