|Year : 2016 | Volume
| Issue : 2 | Page : 239-242
Combined occipital and supraorbital nerve stimulation for treatment of ptosis associated with chronic cluster headache
Steven M Falowski1, Jeffrey Scott Berger2, Jason E Pope3
1 Department of Neurosurgery, St. Luke's University Health Network, Bethlehem, PA, USA
2 Premier Sports and Spine Rehabilitation, Chadds Ford, PA, USA
3 Summit Pain Alliance, Santa Rosa, CA, USA
|Date of Submission||05-Mar-2016|
|Date of Acceptance||17-May-2016|
|Date of Web Publication||28-Dec-2016|
Steven M Falowski
Department of Neurosurgery, St. Luke's University Health Network, 801 Ostrum Street, Bethlehem, PA 18017
Source of Support: None, Conflict of Interest: None
Cluster headache (CH) is a neurological disorder characterized by recurrent, severe headaches on one side of the head, typically around the eye that shares a component of parasympathetic autonomic symptoms. We report a 40-year-old female who presented with migraine headaches, occipital pain and experienced daily headaches behind the right eye. It was associated with lacrimation of her right eye, erythema, and ptosis, in which her eye would partially to fully close. After failure of multiple conservative measures, she was treated with a combination of bilateral occipital nerve and right supraorbital stimulation. The patient obtained significant relief from neurostimulation. She returned to work and initially was able to stop all medications. On 1-year follow-up, the patient is on preventive medication which includes Depakote. The patient continues with significant relief and has had no return of her autonomic features, including ptosis. Neurostimulation is a viable option in the treatment of CHs, including those with disabling autonomic features. The use of combined occipital nerve and supraorbital stimulation administers relief for both headache component and autonomic symptoms.
The following core competencies are addressed in this article: Medical knowledge and patient care. The article addresses the lack of awareness surrounding neuromodulation, as well as expanding indications for an established therapy.
Keywords: Cluster headache, migraines, neuromodulation, occipital nerve stimulation, peripheral nerve stimulation, supraorbital stimulation
|How to cite this article:|
Falowski SM, Berger JS, Pope JE. Combined occipital and supraorbital nerve stimulation for treatment of ptosis associated with chronic cluster headache. Int J Acad Med 2016;2:239-42
|How to cite this URL:|
Falowski SM, Berger JS, Pope JE. Combined occipital and supraorbital nerve stimulation for treatment of ptosis associated with chronic cluster headache. Int J Acad Med [serial online] 2016 [cited 2022 Jan 19];2:239-42. Available from: https://www.ijam-web.org/text.asp?2016/2/2/239/196864
| Introduction|| |
Cluster headache (CH) is a neurological disorder characterized by recurrent, severe headaches on one side of the head, typically around the eye. CH represents one subtype of headache under the classification of trigeminal autonomic cephalgias (TACs). The TACs all share a component of parasympathetic autonomic symptoms which are generally ipsilaterally lateralized to the side of the headache. The autonomic symptoms related to sympathetic dysfunction include ptosis, miosis, anhidrosis, and symptoms associated with parasympathetic overdrive including rhinorrhea, lacrimation, and nasal congestion. These symptoms are believed to be secondary to activation of the trigeminal-parasympathetic reflex with secondary sympathetic dysfunction. These are all confined to the side of the head with the pain. CH attacks often occur periodically with spontaneous remissions that may interrupt active periods of pain although about 10–15% of chronic CH never remit.
The onset of attack is rapid and most often without preliminary signs that are characteristic in migraine. For CH, the pain occurs on one side only (unilateral), around the eye (orbital), particularly above the eye (supraorbital), in the temple (temporal) and may involve any combination. The pain is described as stabbing and may be located near or behind the eye and at the back of the head or neck. The headache attack can be accompanied by autonomic symptoms such as drooping eyelid, pupil constriction, redness of the conjunctiva, lacrimation, rhinorrhea, and less commonly, facial blushing, erythema, or sweating.
While there is no cure, treatment for acute attacks can include oxygen or a fast-acting triptan. Primary prevention can include verapamil. Steroids may be used to prevent a recurrence until verapamil takes effect, utilizing these medications concurrently. Occipital nerve blockade with steroid and local anesthetic serves a short-term therapeutic role in the treatment of CH. While occipital blockade is a low-risk therapeutic option, therapeutic benefit is modest and duration of efficacy may be low, limiting its utility for chronic CH. More invasive, high-risk surgical options may include trigeminal ganglion neurolysis, radiofrequency ablation, microvascular decompression as well as sphenopalatine ganglion blockade or radiofrequency ablation. However, due to associated risks including nerve injury, corneal anesthesia as well as contralateral headache development, the risk profile may outweigh the benefits of these destructive therapies.
Nerve simulators may be an option for those who do not improve with medications. Neurostimulation therapies may target multiple areas including the brain, cervicomedullary junction, spinal cord, or peripheral nervous system. The inherent advantage of neurostimulation over other surgical options is nondestructive and often reversible nature of these therapies. In addition, neurostimulation often affords a trial period during which patients can fairly assess efficacy prior to committing to added risks, recovery, and cost associated with permanent implantation. Occipital nerve stimulation has been useful, and early experience shows a benefit in about 60% of cases.,,
We highlight the use of combined occipital and supraorbital stimulation in a patient suffering from CH that included migraine components and occipital pain after failure of more conservative treatment options. Most significant was the patient's prominent autonomic symptoms including debilitating ptosis which prevented the patient from working and performing daily activities, more so than the headaches themselves.
| Case Report|| |
We report a 40-year-old female in otherwise normal health presented with no significant medical history with a >5-year history of frontal and occipital headaches, unilateral in location. Over a 2-year period, she began experiencing near daily headaches focused behind the right eye. Uncharacteristic of CHs, the pain would start in her right neck radiating into her temporal region and subsequently behind her right eye. She would develop lacrimation of her right eye, erythema, rhinorrhea, and ptosis, in which her eye would partially to fully close. These symptoms would awaken her from sleep. The development of ptosis prevented her from working and performing daily activities such as driving. Headaches could last from 15 min to several hours while her ptosis would last for several hours even after her headaches would subside. Daily episodes could occur for several months at a time.
She was treated conservatively with medical management including verapamil, topiramate, and steroids. She would gain some relief from oxygen therapy and dihydroergotamine mesylate injections. She also underwent occipital and sphenopalatine nerve blocks with temporary 2 days relief. However, none of the therapies gave relief of her ptosis or autonomic symptoms [Figure 1].
After discussion with the patient, the decision was made to proceed with a trial of neurostimulation. Prior to trial placement, the patient underwent routine neuropsychological screening. Electrodes were placed in the bilateral occipital region as dictated by the technique of crossing the occipital artery at a 45° angle, as well as a right supraorbital electrode. Three wide-spaced quadripolar leads were utilized (St. Jude medical) with trial duration of 5 days. During that period, the patient obtained significant relief of her symptoms with a 50–60% decrease in headache intensity and frequency. The patient only reported two discrete CHs during the trial, which were also relieved with an adjustment in programming parameters of the stimulation. She had complete relief of her ptosis during the trial stimulation [Figure 2]. Based on the success of the trial, she proceeded with permanent implantation obtaining the same results.
At this point, she has been followed for 1-year following her permanent implant. She has continued to obtain significant relief from her neurostimulation. She returned to work and continued at this point to perform her daily activities without assistance. Although initially being able to stop all medications, she has developed intermittent breakthroughs of headaches in which she is treated with a course of steroids. These could occur 1–2 times per month. At approximately 6 months postoperative, the decision was made to place the patient on preventive medication which included valproic acid. With this combination, she has obtained significant relief. Of important note is that since implantation of her stimulator, she has had no return of her autonomic features, including ptosis which was the disabling symptom for the patient. The addition of neurostimulation has led to improved quality of life with a decrease in her medications and other interventions.
Initial programming of the patient in the immediate postoperative period included utilizing only the right occipital and right supraorbital lead. The occipital lead was used in all anode configuration (1+, 2+, 3+, 4+) with the supraorbital in an all cathode configuration (9−, 10−, 11−, 12−). The program was run at a PW 150 µs and rate 40 Hz. This demonstrated immediate relief of her ptosis.
She returned for evaluation at a 2-week postoperative visit. She reported relief of her ptosis, as well as autonomic features, but was experiencing breakthrough headaches. At this point, programming was adjusted to include the left occipital lead. After several adjustments, it was determined that the supraorbital lead needed to maintain a cathode in one of its four contacts to deliver relief of her ptosis. Her main program at this point included an alternating cathode/anode configuration (1+, 2−, 3+, 4−, etc.,) on each lead running at PW 150 µs and rate 40 Hz. This administered relief of her autonomic symptoms and headaches.
A single program was given to the patient utilizing a double-guarded cathode solely on the supraorbital lead with contact 9+, 10−, 11+. Although this program did not administer headache relief, the patient would use it daily to ensure no return of her ptosis.
The patient continued to do well over the ensuing months requiring minor adjustments to her programming. At 6 months postoperative, she had experienced significant breakthrough headaches but maintained relief of her autonomic symptoms, including ptosis. At this point, it was determined that the paresthesias began to feel weak for the patient and head movements would decrease the intensity. This was overcome with an increase in PW ranging from 250 to 300 µs and subsequent decrease in frequency to 20 Hz. Contact configuration was maintained in most cases, with each program ensuring a cathode on the supraorbital lead. At 1-year postoperative, her contact configuration utilized a single bipole on the supraorbital lead with contact 10+, 11− running with bilateral occipital leads. The right occipital leads utilized alternating cathode/anode configuration with 1+, 2−, 3+, 4− while the left lead utilized only two anodes contact 5+, 6+ which decreased the coverage on the left side and delivered increasing coverage over the right side of her head.
| Discussion|| |
Peripheral nerve stimulation of the occipital nerves was introduced by Weiner and Reed in 1999 as a treatment for intractable chronic neuralgia. At this point, stimulation of the occipital nerves is becoming more widely accepted in the treatment of occipital neuritis and migraine disorders. There are several articles describing the use of occipital nerve stimulation in the treatment of migraine headaches with multiple studies demonstrating the effective treatment of occipital neuralgia with stimulation of the occipital nerve.,,, Slavin et al. have described benefits in 70% of the patients treated for occipital neuralgia. Similar results were reported by Weiner and Reed, in which patients treated for C2-mediated occipital headaches reported reduction in medication use as well as experiencing reduction in the number of headaches.
The exact mechanism of CH is lacking but appears to be hypothalamic in origin. The suprachiasmatic nucleus in the hypothalamic gray serves as a “pacemaker” involved in neuroendocrine hypothalamic regulation, specifically low testosterone levels and dysregulation of circadian rhythms of luteinizing hormone, follicle stimulating hormone, cortisol, growth hormone, prolactin, thyroid stimulating hormone, and melatonin. Positron emission tomography and functional magnetic resonance imaging have demonstrated increased ipsilateral activity congruent with headache in hypothalamic gray during attacks and electric stimulation of this area aborts/inhibits attacks. Interestingly, modulation of the trigeminocervical complex, seemingly far removed anatomically and spatially, has been shown to help both primary and secondary headache.,
There are scattered reports of occipital nerve and supraorbital nerve stimulation for the use of CHs. Migraine headache has been reportedly treated with the combination of supraorbital and CH. There are a few well-designed clinical trials evaluating preventive measures for migraine and/or CHs. At present, there is only one Food and Drug Administration-approved interventional treatment which is onabotulinumtoxinA. Present-day literature, although describing efficacy for the treatment of CH and migraine attacks, does not evaluate the efficacy of the treatment for the autonomic symptoms associated with the disease process which was the most disabling feature for our patient.
Our case report describes a patient in which the autonomic symptoms led to significant disability. The development of ptosis had led to her being unable to perform her duties at work as well as activities of daily living such as driving. Interestingly, the ptosis became as disabling if not more so than the headache syndrome itself. The use of combined occipital nerve and supraorbital stimulation administered relief for her headache component. The supraorbital stimulation afforded relief of her autonomic symptoms, allowing her to resume normal daily activities. The addition of neurostimulation improved her quality of life when other therapeutic options were exhausted.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]