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

Cover Page

Cite item

Full Text

Open Access Open Access
Restricted Access Access granted
Restricted Access Subscription Access

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.

About the authors

Vachtang G. Toriya

H. Turner National Medical Research Center for Children’s Orthopedics and Trauma Surgery

Email: vakdiss@yandex.ru
ORCID iD: 0000-0002-2056-9726
SPIN-code: 1797-5031

MD, neurosurgeon

Russian Federation, 64-68 Parkovaya str., Pushkin, Saint Petersburg, 196603

Sergei V. Vissarionov

H. Turner National Medical Research Center for Children’s Orthopedics and Trauma Surgery

Email: vissarionovs@gmail.com
ORCID iD: 0000-0003-4235-5048
SPIN-code: 7125-4930
Scopus Author ID: 6504128319
ResearcherId: P-8596-2015

MD, PhD, Dr. Sci. (Med.), Professor, Corresponding Member of RAS

Russian Federation, 64-68 Parkovaya str., Pushkin, Saint Petersburg, 196603

Margarita V. Savina

H. Turner National Medical Research Center for Сhildren’s Orthopedics and Trauma Surgery

Email: drevma@yandex.ru
ORCID iD: 0000-0001-8225-3885
SPIN-code: 5710-4790
Scopus Author ID: 57193277614

MD, PhD, Cand. Sci. (Med.)

Russian Federation, 64-68 Parkovaya str., Pushkin, Saint Petersburg, 196603

Alexey G. Baindurashvili

H. Turner National Medical Research Center for Сhildren’s Orthopedics and Trauma Surgery

Author for correspondence.
Email: turner01@mail.ru
ORCID iD: 0000-0001-8123-6944
SPIN-code: 2153-9050
Scopus Author ID: 6603212551
http://www.rosturner.ru/science_org.htm

MD, PhD, Dr. Sci. (Med.), Professor, Member of RAS, Honored Doctor of the Russian Federation

Russian Federation, 64-68 Parkovaya str., Pushkin, Saint Petersburg, 196603

References

  1. Mitchell SW. On a rare vaso-motor neurosis of the extremitiesand on the mala-dies with which it may be confounded. Am J Med Sci. 1878;76:17–36.
  2. Mcdonnell A, Schulman B, Ali Z, et al. Inherited erythromelalgia due to mutations in SCN9A: natural history, clinical phenotype and somatosensory profile. Brain. 2016;139(4):1052–1065. doi: 10.1093/brain/aww007
  3. Estacion M, Harty TP, Choi J-S, et al. A sodium channel gene SCN9A polymorphism that increases nociceptor excitability. Ann Neurol. 2009;66(6):862–866. doi: 10.1002/ana.21895
  4. Diatchenko L, Slade GD, Nackley AG, et al. Genetic basis for individual variations in pain perception and the development of a chronic pain condition. Hum Mol Genet. 2004;14(1):135–143. doi: 10.1093/hmg/ddi013
  5. Tang Z, Chen Z, Tang B, Jiang H. Primary erythromelalgia: a review. Orphanet J Rare Dis. 2015;10(1):127.
  6. Alhadad A, Wollmer P, Svensson A, Eriksson KF. Erythromelalgia: Incidence and clinical experience in a single centre in Sweden. Vasa. 2012;41(1):43–48. doi: 10.1024/0301-1526/a000162
  7. Reed KB, Davis MDP. Incidence of erythromelalgia: a populationbased study in Olmsted County, Minnesota. J Eur Ac Derm Vener. 2009;23(1):13–15.
  8. Smith LA, Allen EV. Erythermalgia (erythromelalgia) of the extremities. Am Heart J. 1938;16(2):175–188. doi: 10.1016/s0002-8703(38)90693-3
  9. Johnson E, Iyer P, Eanes A, Zolnoun D. Erythema and burning pain in the vulva: A possible phenotype of erythromelalgia. Case Rep Med. 2011;2011(3):374167.
  10. Messeguer F, Agusti-Mejias A, VilataCorell JJ, Requena C. Auricular erythromelalgia: report of a rare case. Dermatol Online J. 2013;19:16
  11. Heidrich H. Functional vascular diseases: Raynaud’s syndrome, acrocyanosis and erythromelalgia. Vasa. 2010;39(1):33–41. doi: 10.1024/0301-1526/a000003
  12. Parker LK, Ponte C, Howell KJ, et al. Clinical features and management of erythromelalgia: long-term follow-up of 46 cases. Clin Exp Rheumatol. 2017;35:80–84.
  13. Davis MDP. Immersion foot associated with the overuse of ice, cold water, and fans: a distinctive clinical presentation complicating the syndrome of erythromelalgia. J Am Acad Dermatol. 2013;69(1):169–171.
  14. Kirby RL. Erythromelalgia-notsobenign. Arch Phys Med Rehabil. 1987;68:389.
  15. Tamrazova OB, Molochkov AV, Koren’kova OV, Novikov KA. Angiotrofonevrozy. Eritromelalgiya u 11-letnego rebenka. Al’manakh klinicheskoy meditsiny. 2016;44(1):52–57. (In Russ.). doi: 10.18786/2072-0505-2016-44-1-52-57
  16. Chan MKH, Tucker AT, Madden S, et al. Erythromelalgia: an endothelial disorder responsive to sodium nitroprusside. Arch Dis Child. 2002;87(3):229–230.
  17. Zsoylu S, Coskun T. Sodium nitroprusside treatment in erythromelalgia. Eur J Pediatr. 1984;141(3):185–187.
  18. Friberg D, Chen T, Tarr G, van Rij A. Erythromelalgia? A clinical study of people who experience red, hot, painful feet in the community. Int J Vasc Med. 2013;2013(2):864961.
  19. Toriya VG, Savina MV, Vissarionov SV, Baindurashvili AG. Nasledstvennaya eritromelalgiya u podrostka. Klinicheskoye nablyudeniye redkogo zabolevaniya. Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2022;10(1):85–92. doi: 10.17816/PTORS90396
  20. Tham SW, Giles M. Current Pain management strategies for patients with erythromelalgia: a critical review. J Pain Res. 2018;30(11):1689–1698. doi: 10.2147/JPR.S154462
  21. Graziotti PJ, Goucke CR. Control of intractable pain in erythromelalgia by using spinal cord stimulation. J Pain Symp Manag. 1993;8(7):502–504. doi: 10.1016/0885-3924(93)90194-z
  22. Matzke LL, Lamer TJ, Gazelka HM. Spinal cord stimulation for treatment of neuropathic pain associated with erythromelalgia. JAPM. 2016;41(5):619–620. doi: 10.1097/aap.0000000000000457
  23. Chakravarthy K, Malayil R, Kirketeig T, Deer T. Burst spinal cord stimulation: A systematic review and pooled analysis of real-world evidence and outcomes data. Pain Medicine. 2019;20:S47–S57. doi: 10.1093/pm/pnz046

Supplementary files

Supplementary Files
Action
1. JATS XML
Download
2. Fig. 1. Patient A. at the age of 14 years. Ulcerative skin lesion of the lower extremities

Download (170KB)
Indexing metadata
3. Fig. 2. Appearance of the lower extremities at hospitalization

Download (294KB)
Indexing metadata
4. Fig. 3. Intraoperative radiography and the position of the eight-junction epidural electrode at the Th11–Th12 vertebrae level

Download (76KB)
Indexing metadata
5. Fig. 4. Data of intraoperative neurophysiological monitoring using an epidural electrode with muscular activity registration of the lower extremities on the left and right: channel I: m. vastus lateralis, channel II: m. tibialis ant., channel III: m. gastrocnemius, and channel IV: m. abd. hallucis brevis. The muscular activity during the period of choosing the epidural electrode position is presented, and the muscular activity of the feet on both sides is recorded in presence of rhythmic stimulation with electrical impulses when the electrode is connected

Download (137KB)
Indexing metadata
6. Fig. 5. Patient A. at 15 years old. The appearance of the lower extremities on day 7 of stimulation, regression of hyperemia, and leg and feet swelling

Download (157KB)
Indexing metadata

Copyright (c) 2022 Toriya V.G., Vissarionov S.V., Savina M.V., Baindurashvili A.G.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.
 


Согласие на обработку персональных данных

 

Используя сайт https://journals.rcsi.science, я (далее – «Пользователь» или «Субъект персональных данных») даю согласие на обработку персональных данных на этом сайте (текст Согласия) и на обработку персональных данных с помощью сервиса «Яндекс.Метрика» (текст Согласия).