Neuromodulation: Technology at the Neural Interface Received: January 26, 2015
Accepted: February 10, 2015
(onlinelibrary.wiley.com) DOI: 10.1111/ner.12295
Spinal Cord Stimulation for the Treatment of Neuropathic Pain Associated With Leprosy: A Case Report Nicholas J. Brandmeir, MD; Michael D. Sather, MD Background: Leprosy is a major source of nerve damage and may lead to neuropathic pain as well as complex regional pain syndrome (CRPS). Spinal cord stimulation is an effective treatment for CRPS, but there are no reports of this treatment in a patient with leprosy. Case Presentation: The patient is a 55-year-old man who presented with CRPS in the arms and legs secondary to leprosy that persisted despite multidrug therapy, steroid treatment, and intravenous immunoglobulin. His pain and opioid use were both decreased with insertion of cervical and thoracic spinal cord stimulators. Conclusion: Spinal cord stimulation may be a valuable intervention for patients with leprosy-induced CRPS. Keywords: Hansen’s disease, leprosy, spinal cord stimulation Conflicts of Interest: The authors reported no conflict of interest.
INTRODUCTION
762
Leprosy represents a major source of nerve impairment and nerve damage despite the presence of multidrug therapy (MDT) (1). Leprosy usually presents with combined dermatologic and neurologic symptoms, but a pure neural form is common and may delay diagnosis. Typically, leprosy presents as a cutaneous syndrome with macules/plaques that have a raised edge, central hypopigmentation, and reduced sensation. Peripheral nerve involvement is present in 12–55% of new leprosy diagnoses and presents alone in 4–8% of cases. Symptoms are varied and may include autonomic, motor, and sensory deficits. Most commonly, leprosy presents with negative symptoms like anhidrosis, numbness, and weakness. Less commonly, positive symptoms such as paresthesia, hyperhidrosis, pain, and allodynia may occur. To confirm a diagnosis of leprosy, one of three cardinal features must be present: a skin lesion with abnormal sensation, enlarged peripheral nerves, or acid fast bacilli on skin smear or biopsy (2). In developed countries, diagnosis can be delayed, with a mean delay in diagnosis of 1.8 years (3). This delay, combined with the natural history of the disease, leads to significant levels of nerve impairment and morbidity (3). A rare, but serious sequela of leprosy, is complex regional pain syndrome (CRPS) type 2 (4). CRPS is described as a persistent pain not restricted to a particular distribution that is disproportionate to the stimulus (4,5). CRPS can be divided into two types based on the pathophysiology; type I involves no nerve injury, while type II involves some nerve injury. When patients with CRPS are refractory to medical management, spinal cord stimulation (SCS) has been beneficial, although experts disagree on the strength of the evidence supporting this intervention (5). Aside from MDT, many treatments exist for the nerve symptoms of leprosy. These include steroids, surgical decompression, and www.neuromodulationjournal.com
immunosuppressive therapy. All have shown promise, although randomized controlled trials have shown variable results (2). In this report, we present a case of a patient with severe pain and CRPS type II secondary to leprosy who was treated successfully with SCS. A review of PubMed, Google, and pertinent bibliographies indicates that this is the first report of the successful use of SCS for the treatment of neuropathic pain in leprosy.
CASE REPORT The patient is a 55-year-old man with a medical history of polymyositis, Hashimoto’s thyroiditis, type 2 diabetes mellitus, and nonalcoholic steatohepatitis who presented with complaints of unremitting burning in his hands and feet worsened by heat, with some hyperalgia that began in 2011 but reached their zenith in 2012 (the time of presentation) when his hands, feet, legs, and buttocks were all encompassed by the pain. In 2013, the patient was found to have a multiple peripheral nerve abnormalities, including a neuropathic right phrenic nerve, abnormal QSART and Valsalva tests, and an electromyogram (EMG) that demonstrated length-dependent axonal neuropathy. During this workup, it was discovered that the
Address correspondence to: Nicholas Brandmeir, MD, Department of Neurosurgery, Penn State Milton S Hershey Medical Center, Mail Code EC110, 30 Hope Drive, Hershey, PA 17033, USA. Email: [email protected] Penn State Milton S Hershey Medical Center, Hershey, PA, USA For more information on author guidelines, an explanation of our peer review process, and conflict of interest informed consent policies, please go to http:// www.wiley.com/bw/submit.asp?ref=1094-7159&site=1 Funding: Internal Departmental funding.
© 2015 International Neuromodulation Society
Neuromodulation 2015; 18: 762–764
SCS IN LEPROSY patient had been traveling to India and working with patients with leprosy since 2006 as part of his occupation as a minister. The patient was referred to a clinic specializing in leprosy and a sural nerve biopsy was recommended. The Fite stain from this biopsy demonstrated acid-fast bacilli, a finding pathognomonic for leprosy. After confirming the diagnosis, minocycline and dapsone were started. The patient’s neuropathic pains did not improve with treatment. He was also treated with prednisone and intravenous immunoglobulin, with only temporary relief of his severe pain. Secondary to his corticosteroid therapy, the patient’s diabetes became difficult to control. At this time, the patient required 5 mg of methadone tid for pain control. The patient was diagnosed with CRPS type II, a known but rare sequela, of leprosy infection based on the failure of the pain to respond to MDT and immunosuppressive regimens that are the mainstay of treating neurologic leprosy symptoms. Because of the nature of this case, a painful neuropathy with atypical response to treatment or a small fiber painful neuropathy cannot be definitively excluded (4). The patient was treated with 5 mg of methadone every 8 hours, nonopioid pharmacologic agents and cognitive-behavioral therapy. A lidocaine infusion was entertained, but ultimately abandoned after insurance issues. Sympathetic blocks and transcutaneous nerve stimulation were not undertaken because of the multiple limb involvement of the patient’s pain. Ultimately, percutaneous SCS trial was planned after a psychological evaluation to ensure the patient was appropriate for stimulation. The trial was placed at the T10-T11 level and lowered the patient’s pain from 9/10 to 2/10. After his good response, the patient wished to proceed with both permanent cervical and thoracic SCS in lieu of a dedicated cervical trial so as to avoid undergoing two surgeries. The risks and benefits of this approach were discussed with the patient and plans were made to go forward with surgery. The surgery was performed under general endotracheal anesthesia. The cervical spine was implanted at the C2-4 level with a 2 × 8 epidural paddle lead. The thoracic spine was implanted at the T10T11 level with a 5-6-5 epidural paddle lead, which was not feasible at the cervical spine because of the narrowness of the canal (both leads from Medtronic Inc., Minneapolis, MN, USA). Because the patient is implanted under general anesthesia, we prefer the width of coverage and stability offered by the paddle electrode even though the trial was done with a percutaneous electrode. Both implants were placed in the midline and confirmed in position with fluoroscopy and x-ray (Fig. 1). The patient recovered well from surgery and was discharged home on postoperative day two. At his two-month follow up, the patient reported that his pain was a 5/10 from a 9/10 in his hands and legs, and that he was no longer requiring methadone.
Figure 1. a,b. Lateral and A-P x-rays of cervical spine showing midline placement of 2 × 8 electrode at C2-4. c,d. Lateral and A-P x-rays of thoracic spine showing placement of 5-6-5 electrode midline at T10-11.
leprosy and its associated conditions. As technology advances and economies develop, new healthcare technologies can be introduced to these populations; and as immigration continues in the global economy, those populations will have access to new healthcare technologies. As this occurs, observational and interventional trials will be able to be conducted to determine the true effectiveness of SCS for neuropathic pain associated with leprosy. Until such evidence can be gathered effectively, SCS should remain a treatment option for patients with medically refractory neuropathic pain/CRPS secondary to leprosy.
Authorship Statement DISCUSSION
www.neuromodulationjournal.com
How to Cite this Article: Brandmeir N.J., Sather M.D. Spinal Cord Stimulation for the Treatment of Neuropathic Pain Associated With Leprosy: A Case Report. Neuromodulation 2015; 18: 762–764
© 2015 International Neuromodulation Society
Neuromodulation 2015; 18: 762–764
763
Our report describes the successful treatment of CRPS type II secondary to leprosy. This is the first report of SCS for this purpose. This demonstrates that SCS has the potential to be an effective treatment for pain associated with leprosy. SCS is largely a treatment modality restricted to the developed world, but it has been demonstrated to be economically favorable in the treatment of many types of neuropathic pain syndromes (5). The majority of cases of leprosy remain in the developing world (2), and access to this technology among the populations most affected is extremely rare. Further, the immigration from endemic areas to more developed economies will necessitate treatment options for
Dr. Michael D. Sather performed the surgery described in the article. Dr. Nicholas J. Brandmeir performed the literature review and prepared the manuscript with intellectual input from Dr. Sather. Dr. Michael D. Sather approved the manuscript.
BRANDMEIR & SATHER
REFERENCES 1. Sharma P. Disabilities in multibacillary leprosy patients: before, during and after multidrug therapy. Indian J Lepr 1996;68:127–136. 2. Wilder-Smith EP, Van Brakel WH. Nerve damage in leprosy and its management. Nat Clin Pract Neurol 2008;4:656–663. doi: 10.1038/ncpneuro0941 3. Lockwood DNJ, Reid AJC. The diagnosis of leprosy is delayed in the United Kingdom. QJM 2001;94:207–212. doi: 10.1093/qjmed/94.4.207 4. Ghia D, Gadkari R, Nayak C. Complex regional pain syndrome secondary to leprosy. Pain Med 2012;13:1067–1071. 5. Taylor RS, Van Buyten J-P, Buchser E. Spinal cord stimulation for complex regional pain syndrome: a systematic review of the clinical and cost-effectiveness literature and assessment of prognostic factors. Eur J Pain 2006;10:91–101. doi: 10.1016/ j.ejpain.2005.02.004
764 www.neuromodulationjournal.com
© 2015 International Neuromodulation Society
Neuromodulation 2015; 18: 762–764
Accepted: February 10, 2015
(onlinelibrary.wiley.com) DOI: 10.1111/ner.12295
Spinal Cord Stimulation for the Treatment of Neuropathic Pain Associated With Leprosy: A Case Report Nicholas J. Brandmeir, MD; Michael D. Sather, MD Background: Leprosy is a major source of nerve damage and may lead to neuropathic pain as well as complex regional pain syndrome (CRPS). Spinal cord stimulation is an effective treatment for CRPS, but there are no reports of this treatment in a patient with leprosy. Case Presentation: The patient is a 55-year-old man who presented with CRPS in the arms and legs secondary to leprosy that persisted despite multidrug therapy, steroid treatment, and intravenous immunoglobulin. His pain and opioid use were both decreased with insertion of cervical and thoracic spinal cord stimulators. Conclusion: Spinal cord stimulation may be a valuable intervention for patients with leprosy-induced CRPS. Keywords: Hansen’s disease, leprosy, spinal cord stimulation Conflicts of Interest: The authors reported no conflict of interest.
INTRODUCTION
762
Leprosy represents a major source of nerve impairment and nerve damage despite the presence of multidrug therapy (MDT) (1). Leprosy usually presents with combined dermatologic and neurologic symptoms, but a pure neural form is common and may delay diagnosis. Typically, leprosy presents as a cutaneous syndrome with macules/plaques that have a raised edge, central hypopigmentation, and reduced sensation. Peripheral nerve involvement is present in 12–55% of new leprosy diagnoses and presents alone in 4–8% of cases. Symptoms are varied and may include autonomic, motor, and sensory deficits. Most commonly, leprosy presents with negative symptoms like anhidrosis, numbness, and weakness. Less commonly, positive symptoms such as paresthesia, hyperhidrosis, pain, and allodynia may occur. To confirm a diagnosis of leprosy, one of three cardinal features must be present: a skin lesion with abnormal sensation, enlarged peripheral nerves, or acid fast bacilli on skin smear or biopsy (2). In developed countries, diagnosis can be delayed, with a mean delay in diagnosis of 1.8 years (3). This delay, combined with the natural history of the disease, leads to significant levels of nerve impairment and morbidity (3). A rare, but serious sequela of leprosy, is complex regional pain syndrome (CRPS) type 2 (4). CRPS is described as a persistent pain not restricted to a particular distribution that is disproportionate to the stimulus (4,5). CRPS can be divided into two types based on the pathophysiology; type I involves no nerve injury, while type II involves some nerve injury. When patients with CRPS are refractory to medical management, spinal cord stimulation (SCS) has been beneficial, although experts disagree on the strength of the evidence supporting this intervention (5). Aside from MDT, many treatments exist for the nerve symptoms of leprosy. These include steroids, surgical decompression, and www.neuromodulationjournal.com
immunosuppressive therapy. All have shown promise, although randomized controlled trials have shown variable results (2). In this report, we present a case of a patient with severe pain and CRPS type II secondary to leprosy who was treated successfully with SCS. A review of PubMed, Google, and pertinent bibliographies indicates that this is the first report of the successful use of SCS for the treatment of neuropathic pain in leprosy.
CASE REPORT The patient is a 55-year-old man with a medical history of polymyositis, Hashimoto’s thyroiditis, type 2 diabetes mellitus, and nonalcoholic steatohepatitis who presented with complaints of unremitting burning in his hands and feet worsened by heat, with some hyperalgia that began in 2011 but reached their zenith in 2012 (the time of presentation) when his hands, feet, legs, and buttocks were all encompassed by the pain. In 2013, the patient was found to have a multiple peripheral nerve abnormalities, including a neuropathic right phrenic nerve, abnormal QSART and Valsalva tests, and an electromyogram (EMG) that demonstrated length-dependent axonal neuropathy. During this workup, it was discovered that the
Address correspondence to: Nicholas Brandmeir, MD, Department of Neurosurgery, Penn State Milton S Hershey Medical Center, Mail Code EC110, 30 Hope Drive, Hershey, PA 17033, USA. Email: [email protected] Penn State Milton S Hershey Medical Center, Hershey, PA, USA For more information on author guidelines, an explanation of our peer review process, and conflict of interest informed consent policies, please go to http:// www.wiley.com/bw/submit.asp?ref=1094-7159&site=1 Funding: Internal Departmental funding.
© 2015 International Neuromodulation Society
Neuromodulation 2015; 18: 762–764
SCS IN LEPROSY patient had been traveling to India and working with patients with leprosy since 2006 as part of his occupation as a minister. The patient was referred to a clinic specializing in leprosy and a sural nerve biopsy was recommended. The Fite stain from this biopsy demonstrated acid-fast bacilli, a finding pathognomonic for leprosy. After confirming the diagnosis, minocycline and dapsone were started. The patient’s neuropathic pains did not improve with treatment. He was also treated with prednisone and intravenous immunoglobulin, with only temporary relief of his severe pain. Secondary to his corticosteroid therapy, the patient’s diabetes became difficult to control. At this time, the patient required 5 mg of methadone tid for pain control. The patient was diagnosed with CRPS type II, a known but rare sequela, of leprosy infection based on the failure of the pain to respond to MDT and immunosuppressive regimens that are the mainstay of treating neurologic leprosy symptoms. Because of the nature of this case, a painful neuropathy with atypical response to treatment or a small fiber painful neuropathy cannot be definitively excluded (4). The patient was treated with 5 mg of methadone every 8 hours, nonopioid pharmacologic agents and cognitive-behavioral therapy. A lidocaine infusion was entertained, but ultimately abandoned after insurance issues. Sympathetic blocks and transcutaneous nerve stimulation were not undertaken because of the multiple limb involvement of the patient’s pain. Ultimately, percutaneous SCS trial was planned after a psychological evaluation to ensure the patient was appropriate for stimulation. The trial was placed at the T10-T11 level and lowered the patient’s pain from 9/10 to 2/10. After his good response, the patient wished to proceed with both permanent cervical and thoracic SCS in lieu of a dedicated cervical trial so as to avoid undergoing two surgeries. The risks and benefits of this approach were discussed with the patient and plans were made to go forward with surgery. The surgery was performed under general endotracheal anesthesia. The cervical spine was implanted at the C2-4 level with a 2 × 8 epidural paddle lead. The thoracic spine was implanted at the T10T11 level with a 5-6-5 epidural paddle lead, which was not feasible at the cervical spine because of the narrowness of the canal (both leads from Medtronic Inc., Minneapolis, MN, USA). Because the patient is implanted under general anesthesia, we prefer the width of coverage and stability offered by the paddle electrode even though the trial was done with a percutaneous electrode. Both implants were placed in the midline and confirmed in position with fluoroscopy and x-ray (Fig. 1). The patient recovered well from surgery and was discharged home on postoperative day two. At his two-month follow up, the patient reported that his pain was a 5/10 from a 9/10 in his hands and legs, and that he was no longer requiring methadone.
Figure 1. a,b. Lateral and A-P x-rays of cervical spine showing midline placement of 2 × 8 electrode at C2-4. c,d. Lateral and A-P x-rays of thoracic spine showing placement of 5-6-5 electrode midline at T10-11.
leprosy and its associated conditions. As technology advances and economies develop, new healthcare technologies can be introduced to these populations; and as immigration continues in the global economy, those populations will have access to new healthcare technologies. As this occurs, observational and interventional trials will be able to be conducted to determine the true effectiveness of SCS for neuropathic pain associated with leprosy. Until such evidence can be gathered effectively, SCS should remain a treatment option for patients with medically refractory neuropathic pain/CRPS secondary to leprosy.
Authorship Statement DISCUSSION
www.neuromodulationjournal.com
How to Cite this Article: Brandmeir N.J., Sather M.D. Spinal Cord Stimulation for the Treatment of Neuropathic Pain Associated With Leprosy: A Case Report. Neuromodulation 2015; 18: 762–764
© 2015 International Neuromodulation Society
Neuromodulation 2015; 18: 762–764
763
Our report describes the successful treatment of CRPS type II secondary to leprosy. This is the first report of SCS for this purpose. This demonstrates that SCS has the potential to be an effective treatment for pain associated with leprosy. SCS is largely a treatment modality restricted to the developed world, but it has been demonstrated to be economically favorable in the treatment of many types of neuropathic pain syndromes (5). The majority of cases of leprosy remain in the developing world (2), and access to this technology among the populations most affected is extremely rare. Further, the immigration from endemic areas to more developed economies will necessitate treatment options for
Dr. Michael D. Sather performed the surgery described in the article. Dr. Nicholas J. Brandmeir performed the literature review and prepared the manuscript with intellectual input from Dr. Sather. Dr. Michael D. Sather approved the manuscript.
BRANDMEIR & SATHER
REFERENCES 1. Sharma P. Disabilities in multibacillary leprosy patients: before, during and after multidrug therapy. Indian J Lepr 1996;68:127–136. 2. Wilder-Smith EP, Van Brakel WH. Nerve damage in leprosy and its management. Nat Clin Pract Neurol 2008;4:656–663. doi: 10.1038/ncpneuro0941 3. Lockwood DNJ, Reid AJC. The diagnosis of leprosy is delayed in the United Kingdom. QJM 2001;94:207–212. doi: 10.1093/qjmed/94.4.207 4. Ghia D, Gadkari R, Nayak C. Complex regional pain syndrome secondary to leprosy. Pain Med 2012;13:1067–1071. 5. Taylor RS, Van Buyten J-P, Buchser E. Spinal cord stimulation for complex regional pain syndrome: a systematic review of the clinical and cost-effectiveness literature and assessment of prognostic factors. Eur J Pain 2006;10:91–101. doi: 10.1016/ j.ejpain.2005.02.004
764 www.neuromodulationjournal.com
© 2015 International Neuromodulation Society
Neuromodulation 2015; 18: 762–764
