Occipital Nerve Stimulation for the Treatment of Refractory Occipital Neuralgia: A Case Series

doi:10.1016/j.wneu.2017.06.064

World Neurosurgery

Volume 105, September 2017, Pages 599-604

https://doi.org/10.1016/j.wneu.2017.06.064 Get rights and content

Background

Occipital neuralgia is a chronic pain syndrome characterized by sharp, shooting pains in the distribution of the occipital nerves. Although relatively rare, it associated with extremely debilitating symptoms that drastically affect a patient's quality of life. Furthermore, it is extremely difficult to treat as the symptoms are refractory to traditional treatments, including pharmacologic and procedural interventions. A few previous case studies have established the use of a neurostimulation of the occipital nerves to treat occipital neuralgia.

Objective

The following expands on that literature by retrospectively reviewing the results of occipital nerve stimulation in a relatively large patient cohort (29 patients).

Methods

A retrospective review of 29 patients undergoing occipital nerve stimulation for occipital neuralgia from 2012 to 2017 at a single institution with a single neurosurgeon.

Results

Of those 29 patients, 5 were repair or replacement of previous systems, 4 did not have benefit from trial stimulation, and 20 saw benefit to their trial stage of stimulation and went on to full implantation. Of those 20 patients, even with a history of failed procedures and pharmacological therapies, there was an overall success rate of 85%. The average preoperative 10-point pain score dropped from 7.4 ± 1.7 to a postoperative score of 2.9 ± 1.7. However, as with any peripheral nerve stimulation procedure, there were complications (4 patients), including infection, hardware erosion, loss of effect, and lead migration, which required revision or system removal.

Conclusion

Despite complications, the results suggest, overall, that occipital nerve stimulation is a safe and effective procedure for refractory occipital neuralgia and should be in the neurosurgical repertoire for occipital neuralgia treatment.

Introduction

Occipital neuralgia (ON) is a debilitating cranial neuropathy that is associated with lancinating pain within the distribution of the greater (∼90%), lesser (∼10), and/or third occipital nerves (rare).1, 2 It is a relatively understudied entity, and, thus, there is limited epidemiologic data on incidence and prevalence or any predisposing risk factors. The pain is typified by sharp, electric, shooting pain that radiates from the upper cervical/occipital region along the posterior scalp to the vertex of the skull, which may be triggered or unprovoked.3, 4, 5 Most commonly, this pain is felt unilaterally (90%), although ON also may be bilateral. There may be extreme hypesthesia or dysesthesia in the distribution of the affected nerve(s) as well, and occasionally, patients describe a constant component to their pain.

ON is considered to be idiopathic in nature; however, several etiologies have been implicated in the etiology of ON, including trauma (e.g., motor vehicle accident “whiplash”), compression (e.g., atlantoaxial instability secondary to degenerative changes), or nerve entrapment (myofascial or vascular).⁶ Some rare causes of ON include schwannomas and other neoplastic processes, postherpetic neuralgia, and temporal arteritis.7, 8, 9 As of yet, there is no clear standard of care in managing this condition, and many of the treatment options for ON are under active investigation.¹⁰

As in other conditions, the treatment of ON proceeds from least to most invasive. First-line treatment consists of conservative management, including rest, massage, hot/cold compresses, and physical therapy. An escalation of care can include anti-inflammatory medications and muscle relaxants, especially in the context of acute pain. In instances in which the pain is chronic, a trial of antiepileptics, antidepressants, or GABA-targeted therapies may be initiated. For those patients whose pain is resistant to these therapies, more invasive measures may be considered, including steroid injections, local anesthetic nerve blocks, and botulinum A toxin injection.¹¹ Although these procedures do have relatively high initial success rates, limitations include the need for repeat injections and loss of efficacy over time.

For ONS that remains refractory to these interventions, surgical interventions neurosurgical interventions are considered the final line of treatment and include a broad arsenal of surgical approaches. Ablative interventions have been published at each level of the greater, lesser, and/or third occipital nerves, including the C1–C4 dorsal roots (dorsal cervical rhizotomy), C2 ganglion (ganglionectomy), and the nerves themselves (neurectomy).12, 13, 14 In addition to mechanical neurectomy, neurolysis may be performed via radiofrequency ablation and chemical and cryo-denervation of the occipital nerve, although the long-term outcomes of these procedures are still under investigation.10, 15

In contrast to the ablative procedures, occipital nerve stimulation (ONS) constitutes a reversible, nondestructive therapy. Results from a limited number of retrospective and prospective case series with relatively small samples (mean of 9–10 patients) suggest that this therapy may have long-term efficacy and a relatively benign side-effect profile in the treatment of ON.3, 4, 5, 16, 17, 18, 19, 20, 21 To further clarify the outcomes of neuromodulation in this relatively rare condition, herein we present the largest currently published retrospective case series on the use of ONS for ON in 20 patients.

Section snippets

Patient Selection

The study was approved by the Emory University Institutional Review Board. Patients were selected for medical record review based on a diagnosis of ON (ICHD 2/3 Beta criteria). All patients were evaluated at the Emory Neurosurgery Clinic and the operations were conducted by a single neurosurgeon (N.M.B.) at in the Emory University Hospital between the years of 2012 and 2017. Typically, patients were considered candidates for surgery if they had a history of previous pharmacological, procedural,

Patient and ON Characteristics

The patient demographics and characteristics are included in Table 1. The characteristics of their ON are presented in Table 2. The majority of patients had unilateral ON, with both lancinating and constant pain components. As would be anticipated by the nature of the syndrome, the mean preoperative-reported pain scores were quite high at 7.4, with a mean preoperative maximum-reported pain score of 8. On average, the patients had symptoms for more than 11 years before undergoing a trial of ONS.

Discussion

ON is a rare and debilitating disease. Unfortunately, it is also recalcitrant to pharmacological, procedural, and surgical treatment. In our cohort, most of the patients have tried and failed more than 7 medications. Furthermore, the patients have undergone invasive procedures, often repeatedly with transient, partial effects. In these patients, we have shown that ONS is a safe and effective treatment option, with evidence of effects lasting more than 1 year postimplantation. Of the patients

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Cited by (32)

A surgical decision aid for occipital neuralgia with literature review and single center case series
2024, Clinical Neurology and Neurosurgery
Occipital neuralgia (ON) is a debilitating headache disorder. Due to the rarity of this disorder and lack of high-level evidence, a clear framework for choosing the optimal surgical approach for medically refractory ON incorporating shared decision making with patients does not exist.
A literature review of studies reporting pain outcomes of patients who underwent surgical treatment for ON was performed, as well as a retrospective chart review of patients who underwent surgery for ON within our institution.
Thirty-two articles met the inclusion criteria. A majority of the articles were retrospective case series (22/32). The mean number of patients across the studies was 34 (standard deviation (SD) 39). Among the 13 studies that reported change in pain score on 10-point scales, a study of 20 patients who had undergone C2 and/or C3 ganglionectomies reported the greatest reduction in pain intensity after surgery. The studies evaluating percutaneous ablative methods including radiofrequency ablation and cryoablation showed the smallest reduction in pain scores overall. At our institution from 2014 to 2023, 11 patients received surgical treatment for ON with a mean follow-up of 187 days (SD 426).
Based on these results, the first decision aid for selecting a surgical approach to medically refractory ON is presented. The algorithm prioritizes nerve sparing followed by non-nerve sparing techniques with the incorporation of patient preference. Shared decision making is critical in the treatment of ON given the lack of clear scientific evidence regarding the superiority of a particular surgical method.
Occipital Nerve Stimulation for Recurrent Trigeminal Neuralgia Without Occipital Pain
2023, Neuromodulation
Citation Excerpt :
However, it may be offered either as a salvage treatment when these treatments have failed or cannot be performed again or as a less invasive alternative to MVD when contraindicated by the patient’s status. ONS has shown various degrees of effectiveness in refractory headaches, including occipital neuralgia,21–24 migraine,21,25,26 and chronic cluster headache.21,27–31 The mechanisms underlying ONS are still unclear, but it probably acts through a non–disease-specific mechanism by modulating nociceptive integration in the trigemino-cervical complex (TCC).
Trigeminal neuralgia (TN) is a severe, debilitating pain condition causing physical and emotional distress. Although the management of TN is well codified with medical and then surgical treatments, 15% to 30% of patients will experience intractable pain. Neuromodulation techniques have been scarcely used for refractory TN, with only small case series and short-term follow-up.
We conducted a retrospective study of patients treated with occipital nerve stimulation (ONS) for medically and surgically resistant TN without painful trigeminal neuropathy. The effectiveness of the ONS was evaluated using the Barrow Neurological Institute (BNI) pain score and the pain relief (0%–100%) at best and at last follow-up.
Seven patients who have refractory TN were included. The mean age at ONS was 49 years. The mean pain duration was 8.6 years. The mean number of medical and surgical treatments before ONS was six and five, respectively. A percutaneous trial was performed in five of seven patients; all responded (pain relief > 40%), and four of five patients experienced pain recurrence after explantation. Eventually, six patients had a permanent ONS implantation. The average BNI pain score before implantation was V. The mean follow-up after implantation was 59 months. All patients reported an improvement after implantation. The average BNI score and mean pain relief at best were IIIa and 86.7%, respectively. At last follow-up, the average BNI score and mean pain relief were IIIa and 58.0%, respectively, with three patients experiencing pain recurrence. Adverse events were reported for four patients who required surgical revision for lead breakage (1), erosion (1), migration (1), or hardware-related discomfort (1). One patient finally underwent explantation because of infection.
Although ONS is not validated in this indication, these results suggest that it can induce an improvement in TN recurring after several surgical treatments, and the benefit of the stimulation can be sustained in the long term.
The Clinicaltrials.gov registration number for the study is NCT01842763.
Long-Term Experience with Occipital and Supraorbital Nerve Stimulation for the Various Headache Disorders—A Retrospective Institutional Case Series of 96 Patients
2021, World Neurosurgery
Citation Excerpt :
The results from our study are well within these reported ranges. Varying definitions of responder status have been used; thus, we reported both the ≥30%23,29 and ≥50%2,6,9-12,14,17,19,23,27,29-31 reduction in headache severity scores. However, a direct comparison was limited because only slightly more than one half of our population had undergone exclusive ONS implantation.
We have provided long-term data on clinically meaningful pain alleviation for drug-refractory headache disorders using occipital (ONS) and supraorbital nerve stimulation (SONS).
We performed a retrospective review of 96 patients with migraine, cervicogenic headache, cluster headache, neuropathic pain of the scalp, tension-type headache, and new daily persistent headache who had undergone ONS (61.5%), SONS (11.5%), or combined ONS plus SONS (27.1%) trial implantation and definitive implantation from 2007 to 2017. Changes in pain perception over time were monitored using the visual analog scale (VAS) for pain.
The cohort consisted of 60.4% women and 39.6% men, with a mean age of 46.9 ± 11.5 years and pain duration of 14 ± 14.1 years. Of the 96 patients, 65 (67.7%) were treatment responders to a trial (≥30% amelioration in the average or maximum VAS score for pain and/or number of headache days) that had lasted 22.5 ± 8.8 days. The reduction in their average VAS score for pain was to 37% ± 24.4% of baseline compared with 99.1% ± 24.1% of baseline for those without a response (P < 0.01). Of the 56 patients who had undergone implantation and had long-term follow-up data available for ≤10 years, 32 (57.1%) reported a ≥50% reduction in their average VAS score for pain. Four patients (6.5%) had requested hardware explantation. Stage II complications included 1 infection (1.6%) and 6 electrode dislocations (9.7%). The study limitations included the retrospective nature, lack of controls receiving placebo intervention, and randomization.
After careful patient selection according to a positive response to a trial of ONS and/or SONS, clinically meaningful long-term benefit was achieved in 57.1% of our patients with various chronic headache conditions.
Neuromodulation in headache and craniofacial neuralgia: guidelines from the Spanish Society of Neurology and the Spanish Society of Neurosurgery
2021, Neurologia
En los últimos años han surgido numerosos dispositivos de neuromodulación, invasivos y no invasivos, que se han aplicado en pacientes con cefaleas y neuralgias sin que exista una revisión actualizada de su eficacia y seguridad, ni recomendaciones de ninguna institución sanitaria sobre su uso específico en cada entidad nosológica.
Neurólogos del Grupo de Cefaleas de la Sociedad Española de Neurología (SEN) y neurocirujanos expertos en neurocirugía funcional seleccionados por la Sociedad Española de Neurocirugía (SENEC), hemos realizado una revisión exhaustiva en el sistema Medline sobre neuromodulación en cefaleas y neuralgias.
Presentamos una revisión actualizada y establecemos por primera vez unas recomendaciones consensuadas entre la SEN y la SENEC sobre el uso de la neuromodulación en cefaleas y neuralgias, adjudicando niveles de evidencia sobre su eficacia actual, específicamente en cada entidad nosológica.
Los resultados actuales de los estudios proporcionan evidencias para la indicación de técnicas de neuromodulación en casos refractarios de cefaleas y neuralgias (sobre todo en migraña, cefalea en racimos y neuralgia del trigémino), seleccionados por neurólogos expertos en cefaleas, tras comprobar el agotamiento de las opciones farmacológicas. Adicionalmente, en el caso de la neuromodulación invasiva, se recomienda que los casos sean debatidos en comités multidisciplinarios y la cirugía sea realizada por equipos de neurocirujanos expertos en neurocirugía funcional y con una morbimortalidad aceptable.
Numerous invasive and non-invasive neuromodulation devices have been developed and applied to patients with headache and neuralgia in recent years. However, no updated review addresses their safety and efficacy, and no healthcare institution has issued specific recommendations on their use for these 2 conditions.
Neurologists from the Spanish Society of Neurology's (SEN) Headache Study Group and neurosurgeons specialising in functional neurosurgery, selected by the Spanish Society of Neurosurgery (SENEC), performed a comprehensive review of articles on the MEDLINE database addressing the use of the technique in patients with headache and neuralgia.
We present an updated review and establish the first set of consensus recommendations of the SEN and SENC on the use of neuromodulation to treat headache and neuralgia, analysing the current levels of evidence on its effectiveness for each specific condition.
Current evidence supports the indication of neuromodulation techniques for patients with refractory headache and neuralgia (especially migraine, cluster headache, and trigeminal neuralgia) selected by neurologists and headache specialists, after pharmacological treatment options are exhausted. Furthermore, we recommend that invasive neuromodulation be debated by multidisciplinary committees, and that the procedure be performed by teams of neurosurgeons specialising in functional neurosurgery, with acceptable rates of morbidity and mortality.
Peripheral trigeminal branch stimulation for refractory facial pain: A single-center experience
2020, Clinical Neurology and Neurosurgery
Citation Excerpt :
However, the latter is considered a simpler procedure with potentially fewer complications [6–9]. The use of peripheral nerve stimulation has been shown to be effective in a number of published cases for various indications including facial and occipital pain [9,10]. Previous studies have largely focused on the combined stimulation of the occipital and trigeminal nerve branches, while the safety and efficacy of sole supraorbital nerve (SON) and infraorbital nerve (ION) stimulation remains unclear [9,11,12].
Facial pain refractory to medical treatments may benefit from neurosurgical interventions. Only a few studies have reported on the efficacy of peripheral trigeminal stimulation and more specifically supraorbital nerve (SON) and infraorbital nerve (ION) stimulation for the treatment of facial pain.
In the present study, we identified all patients at our institution who underwent SON and/or ION stimulation for treatment of facial pain due to post-herpetic, traumatic or idiopathic etiology. Relevant pre and post-operative outcomes were analyzed.
We identified 15 patients who underwent SON and/or ION stimulation. Among them, 12 (80 %) endorsed >50 % pain relief during the trial stimulation period. After a median follow-up of 5.8 months with permanent implantation, 1 patient (8.3 %) was diagnosed with lead erosion and IPG migration, two patients had lead infections (16.7 %) and one (8.3 %) had wound dehiscence. No lead migrations were identified during the long-term follow-up. The VAS score showed a statistically significant reduction from a median pre-operative score of 7 to a post-operative score of 1.8 (p = 0.011), which corresponded to a 74.3 % average pain reduction.
SON and/or ION stimulation can be an effective treatment for intractable facial pain due to post-herpetic, traumatic or idiopathic etiology; however the complication rate is relatively high. Future prospective studies with longer follow-up periods are warranted.
Occipital neuralgia: A neurosurgical perspective
2020, Journal of Clinical Neuroscience
Citation Excerpt :
Trans-electrical nerve stimulation (TENS) has provided relief in more than 50% of patients up to 5 years [25]. In recent years, permanent implantation of occipital nerve stimulation devices have grown in popularity, with one retrospective study showing success in >80% [26]. In addition, botulinum toxin injection and pulsed radiofrequency have been tried with limited success [27–29].
Occipital neuralgia typically arises in the setting of nerve compression by fibrosis, surrounding anatomic structures, or osseous pathology, such as bone spurs or hypertrophic atlanto-epistropic ligament. It generally presents as paroxysmal bouts of sharp pain in the sensory distribution of the first three occipital nerves. Due to the long course of the greater occipital nerve (GON), and its peculiar anatomy, and location in a mobile region of the neck, it is unsurprising that the GON is at high risk for compression. Little is known how to diagnose or treat this neuropathic pain syndrome. The objective of this paper is to isolate the etiology involved, and treat this condition promptly. After all nonoperative efforts are exhausted, surgical transection of the nerve is the treatment of choice in these cases. An isolated C2 neurectomy or ganglionectomy is performed for an optimal pain relief. C1-2 instrumented fusion can be considered if, extensive facet arthropathy with instability is identified. Authors review the spectrum of treatment options for this debilitating condition, and discuss the case example of a patient who required conversion to a C1-C2 instrumented fusion following C2 ganglionectomy due to an underlying extensive degenerative disease and intraoperative findings suggestive of atlantoaxial instability.

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: Conflict of interest statement: Nicholas Boulis has compensated intellectual property rights with NeuralStem under an exclusive license from Cleveland Clinic; is a compensated consultant for NeuralStem, MRI Interventions, Agilis, and Biomedica; has compensated research funding from ALSA, DOD, NIH, and Tubman Research Institute; and has uncompensated stock options with Boston Scientific, Switch Bio, and Code Runner.

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Original ArticleOccipital Nerve Stimulation for the Treatment of Refractory Occipital Neuralgia: A Case Series

Background

Objective

Methods

Results

Conclusion

Introduction

Section snippets

Patient Selection

Patient and ON Characteristics

Discussion

Neuromodulation

Neuromodulation

Neuromodulation

Neuromodulation

Neurochirurgie

The International Classification of Headache Disorders, 3rd edition (beta version)

Cephalalgia

Occipital neuralgia: anatomic considerations

Clin Anat

Occipital nerve electrical stimulation via the midline approach and subcutaneous surgical leads for treatment of severe occipital neuralgia: a pilot study

Anesth Analg

Peripheral neurostimulation for treatment of intractable occipital neuralgia

Neurosurgery

Occipital nerve stimulation for intractable occipital neuralgia: an open surgical technique

Clin Neurosurg

Occipital neuralgia diagnosis and treatment: the role of ultrasound

Headache

Occipital (Arnold) neuralgia secondary to greater occipital nerve schwannoma

Headache

Occipital neuralgia evoked by facial herpes zoster infection

Headache

Original Article
Occipital Nerve Stimulation for the Treatment of Refractory Occipital Neuralgia: A Case Series