Background & Aims
Neurocardiovascular dysautonomia can result in postural orthostatic tachycardia syndrome (POTS) and disproportionally affects patient with Ehlers-Danlos Syndrome (EDS) and hypermobility spectrum disorders.1 Pain in the upper extremities also disproportionally affects these populations and is commonly due to sympathetically maintained pain. Sparse literature characterizes the pain symptomatology and treatment for these conditions. Cervical spinal cord stimulation (SCS) for upper extremity complex regional pain syndrome (CRPS) is an established therapy for sympathetically maintained pain in the upper extremities refractory to conservative, comprehensive management.2 SCS also can improve autonomic dysfunction-related orthostasis.3 In this study, patients with EDS, POTS and upper extremity CRPS were treated with cervical spinal cord stimulation over a three-year time period in an interdisciplinary pain medicine clinic. The aim of the study was to characterize patient experience and the myriad of dysautonomia and pain symptoms before and after cervical spinal cord stimulation.
Methods
After obtaining IRB approval, patients receiving cervical spinal cord stimulation for CRPS with a formal diagnosis of autonomic dysfunction and POTS were identified via billing records and review of the electronic health record (EHR). Written consent was obtained from all patients willing to participate in the study. Patients were excluded if age <18 years old or without formal dysautonomia diagnosis confirmed with testing. Via EHR review demographics, medical history, treatment approach and response to treatment were documented. Patients were contacted and surveyed on their experience with spinal cord stimulation therapy at the time of consent along with a Patient Global Impression of Change (PGIC) scale. All data was collected by the research team to limit treatment bias. Pre and post neuromodulation treatment symptoms were organized tabularly as were patient survey responses. Degree of pain reduction after stimulation treatment was evaluated as a percentage and presented as averages with standard error of the mean. Oswestry Disability Index and % disability were compared before and after spinal cord stimulation.
Results
Nine sequential patients met criteria for inclusion from June 2020 through December 2023. All were reached via phone, and 7 of the 9 patients consented for the study at time of abstract and poster submission. Patients were all female gender with an average age of 36.3 ± 3.7 years, and moderate or severe disability on Oswestry Disability Index prior to implant. All 7 patients proceeded to implant after successful trial with leads being placed epidurally at C2 superiorly. All patients were responsive to stellate ganglion block prior to SCS, had trialed multiple medications, participated in physical and occupational therapy, and were evaluated for stimulation treatment by pain psychology. All patients had significantly widespread pain in addition to their upper extremity CRPS Type 1 and POTS diagnoses and all had a diagnosis of hypermobility. Three patients received tonic stimulation and 4 received passive recharge sub perception burst. The average time of pain prior to implantation was 15.7 ± 7.9 years. After stimulation, average pain reduction was 64.3% ± 18.1% after 16.6 ± 9 months time since implant. ODI and % disability pre and post implant were not significantly changed. All patients described meaningful improvement in chronic pain and would recommend spinal cord stimulation to patients experiencing similar symptoms. A wide range of symptoms were meaningfully improved in all subjects the respondent’s PGIC scores were all 6 or greater indicating patients all felt better, that the treatment provided definite improvement, and has made a real and worthwhile difference.
Conclusions
Patients with POTS, CRPS, and EDS experience a multitude of unpleasant, disabling symptoms. This small but unique retrospective case-series study, characterizes the medical complexity of this population while illustrating the patient described benefits of cervical spinal cord stimulation. From this data, the surgical experience and treatment appear consistent with other patient populations in regards to risks and benefits. Patient reported outcomes suggest significant reduction of patient reported symptoms and improvement of function and quality of life with a high level of patient satisfaction with the therapy on PGIC. The response of both pain and neurologic symptoms in this study suggests a common or intersecting pathway in the neurocircuity of neurocardiovascular dysautonomia and chronic pain with sympathetically maintained features. Future prospective study should elucidate via sham control effect size, optimal waveform, and durability of outcomes over time as well as potential disease modification.
References
- Cook G , Sandroni P. Management of headache and chronic pain in POTS. Auton Neurosci. 2018 Dec;215:37-45
- Burgher A, Kosek P, Surrett S, et al. Ten kilohertz SCS for Treatment of Chronic Upper Extremity Pain (UEP): Results from Prospective Observational Study. J Pain Res. 2020 Nov 10;13:2837-2851.
- Squair J, Berney M, Jiménez M, et al. Implanted System for Orthostatic Hypotension in Multiple-System Atrophy. The New England Journal of Medicine. 2022 386(14), 1339–1344.
- Dworkin RH, Turk DC, Farrar JT, et al.; IMMPACT. Core outcome measures for chronic pain clinical trials: IMMPACT recommendations. Pain. 2005 Jan;113(1-2):9-19.
Presenting Author
David Dickerson
Poster Authors
David Dickerson
MD
Endeavor Health
Lead Author
Noah Ben-Isvy
Endeavor Health
Lead Author
Andrew Locke
Endeavor Health
Lead Author
Candy Gonzalez
Endeavor Health
Lead Author
Natalie Schmitt
PhD
Endeavor Health
Lead Author
Mary Kiolbasa
OTL/R
Endeavor Health
Lead Author
Susan Lesher
PhD
Endeavor Health
Lead Author
Matthew Bowersox
MD
Endeavor Health
Lead Author
Octavia Kincaid
MD
Rush University Medical Center
Lead Author
Topics
- Treatment/Management: Interventional Therapies – Neuromodulation