Surgical treatment of a patient with erythromelalgia (Mitchell’s syndrome) using invasive spinal cord stimulation: A Clinical case

Abstract

BACKGROUND: Erythromelalgia is a rare hereditary disorder manifesting the basic triad of symptoms: erythro redness, melos limb, and algos pain. It was first described by the American neurologist, S. Weir Mitchell in 1878. Clinical manifestations of the disease worsen the physical and psychological condition of the patient leading to reduced quality of life, increased morbidity and mortality. Currently, etiotropic therapy for erythromelalgia that demonstrates high efficacy in individuals with this pathology, has not been developed. Moreover, there is no consensus on treatment strategies for this category of patients, emphasized by the absence of clinical guidelines for the treatment of erythromelalgia. Treatment of patients with erythromelalgia is currently based on sequential pharmacotherapy in order to select the most effective therapy. CLINICAL CASE: We presented the result of surgical treatment of erythromelalgia in a 15-year-old adolescent using invasive spinal cord stimulation. DISCUSSION: Erythromelalgia remains an understudied condition with the lack of sufficient understanding of its etiology and pathogenesis. For the first time in Russia, a technique of invasive spinal cord stimulation was used in a pediatric patient with erythromelalgia, which resulted in a significant reduction of neuropathic pain, restoration of vasomotor regulation in the form of reduced edema and hyperemia. CONCLUSIONS: In a patient with prolonged and pronounced refractory neuropathic pain caused by erythromelalgia, spinal cord stimulation was the only effective treatment technique alternative to symptomatic and drug therapy. Spinal cord stimulation should be considered as a method of treating neuropathic pain associated with pharmacoresistant forms of erythromelalgia.