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F R O M G R A N D R O U N D S A N D O T H E R C L I N I C A L C O N F E R E N C E S O F T H E M G H P A I N C E N T E R , M A S S A C H U S E T T S G E N E R A L H O S P I T A L MASSACHUSETTS
Occipital Neuralgia
GENERAL HOSPITAL
By S T E V E N B A R N A , M . D . A N D M A L I H A H A S H M I , B . S .
Occipital neuralgia is a form of headache that involves the posterior occiput in the
greater or lesser occipital nerve distribution. Pain can be severe and debilitating, with
M G H PA I N C E N T E R
frequent paroxysms. Occipital neuralgia can be difficult to distinguish from other types of
headache and, therefore, diagnosis can be challenging. Local anesthetic block of the
Chief, Division of Pain MedicineEditor, Pain Management Rounds occipital nerves, either peripherally or more proximally at the C2 and/or C3 nerve root,
may aid in diagnosis. Treatment may include medications, minimally invasive percuta-
neous procedures, and surgical interventions. This issue of Pain Management Rounds pre-
sents the characteristics of occipital neuralgia and outlines available treatment options.
Steve Barna, M.D.
Medical Director, MGH Pain Clinic BACKGROUND
Gary Brenner, M.D., Ph.D.
Director, Pain Medicine Fellowship Headache accounts for nearly 20 million outpatient visits per year in the United States and is one of the most common complaints brought to doctors. Nearly 95% of the population will experience a headache at some point in their life. While the parenchyma of the brain is insen- sate, the scalp, head muscles, periosteum, dura, and blood vessels are all pain-sensitive; thus, there are many possible causes of head and face pain. Occipital neuralgia is a headache syndrome that may be either primary or secondary. Primary headaches have no clear structural or disease-related cause, (eg, migraine, tension, and cluster headaches). Primary headaches constitute the etiology of >90% of head and facial Jianren Mao, M.D., Ph.D.
Director, Pain Research Group pain1 and occipital neuralgia is often confused with other primary headache syndromes, includ- Secondary headaches have an underlying disease process that may include tumor, trauma, infection, systemic disease, or hemorrhage. Director, Center for Shingles andPostherpetic Neuralgia ETIOLOGY
Patients with occipital neuralgia may be divided into those with structural causes and those with idiopathic causes. Structural causes include: • trauma to the greater and/or lesser occipital nerves• compression of the greater and/or lesser occipital nerves or C2 and/or C3 nerve roots by MGH PAIN CENTER
• tumors affecting the C2 and C3 nerve roots. The editorial content of Pain Management The greater occipital nerve receives sensory fibers from the C2 nerve root and the lesser Rounds is determined solely by the occipital nerve receives fibers from the C2 and C3 nerve roots. The third occipital nerve (least MGH Pain Center, Massachusetts General Hospital.
occipital nerve) stems from the medial sensory branch of the posterior division of the C3 nerve Pain Management Rounds is approved
by the Harvard Medical School
Department of Continuing Education
to offer continuing education credit
root and travels along the greater occipital nerve. It passes Patients with a history of rheumatoid arthritis or trauma through the trapezius and splenius capitus slightly medial should receive a thorough spine work-up. Diagnostic to the greater occipital nerve. Clinically, the third occipi- occipital nerve blockade also aids in diagnosis.
tal nerve may also be involved in causing occipital neural- Occipital neuralgia often is confused with migraines gia. Cervical spine changes include spondylosis, arthritis and other headache syndromes (Table 1). In some cases, of the upper cervical facet joints, and thickening of the occipital neuralgia is misdiagnosed as fibrocytis or ligaments in that area (particularly C1-4 levels).2 Some fibromyalgia, cervical spine arthritis, or cervical disc cases of presumed occipital neuralgia may in fact be C2 or C3 radiculopathies. Compression of the greater occipitalnerve is possible as it travels up the neck, passing through TREATMENT OPTIONS
the semispinalis and trapezius muscles. Whiplash or If the cause is structural, then surgical treatment may hyperextension injury may lead to this scenario.3 Other be indicated. Because the majority of patients have no possible causes include localized infections or inflamma- clear structural cause, their treatment is usually sympto- tion, gout, diabetes, and blood vessel inflammation.4 matic. Local nerve blocks, medications, occipital nerve Although it cannot be quantified, most patients fall in the stimulator implantation, surgical decompression, or category of “unknown cause,” when no identifiable lesion lesioning of the C2 and/or C3 nerve roots, or even the greater and/or lesser occipital nerves, may be considered.
Occipital neuralgia is often difficult to manage because it CLINICAL FEATURES
can easily be mistaken for other headache syndromes.8 Occipital neuralgia symptoms include aching, Management of occipital neuralgia follows the usual burning, and throbbing pain that is often unilateral and course, starting with the recommended conservative treat- continuous with intermittent, shocking, shooting pain.
ment, conventional therapy, and medications such as The pain usually originates in the suboccipital area and non-steroidal anti-inflammatory drugs (NSAIDs), neuro- radiates to the posterior and/or lateral scalp. Occasionally, pathic medications (seizure medications, tricyclic anti- patients report pain behind the eye on the affected side.
Pain may also be perceived over the neck, temple, andfrontal regions.5 Pressure over the occipital nerves may Conservative treatment
amplify the pain, but there is usually no clear trigger.
Physical therapy, massage, acupuncture, and heat are Furthermore, some patients may have a positive Tinel’s other treatments that can be used for the treatment of sign over the occipital nerve. Occasionally, neck move- ments (eg, extension and rotation) may trigger pain. Attimes, patients with occipital neuralgia may experience Medications
symptoms similar to migraine or even autonomic changes Medications that may help relieve pain in occipital characteristic of cluster headaches. Associated symptoms neuralgia include gabapentin 300-3600 mg/day, carba- include posterior scalp paresthesias, photophobia, and mazepine 400-1200 mg/day, phenytoin 300-600 mg/day, dizziness. Many patients with occipital neuralgia report a valproic acid 500-2000 mg/day, and baclofen 40-120 mg/day. NSAIDs and opioids may also be beneficial. DIAGNOSIS
NERVE BLOCKS
Thorough history-taking and a complete physical and Nerve blocks consisting of steroids and local anes- neurological examination are necessary in diagnosing thetics may also be considered for treatment of occipital headache.7 A diagnosis is usually made based on the characteristic area of the pain. In addition, finding tenderareas that exacerbate the pain aids in diagnosis. It is Occipital nerve block
important to clarify whether the cause of occipital neural- Occipital nerve block is indicated for the diagnosis gia is structural or idiopathic. Abnormal findings on neu- or treatment of occipital neuralgia. The greater occipital rologic exam usually indicate a structural cause, in which nerve is 2.5 to 3 cm lateral to the external occipital pro- case, computed tomography (CT) or magnetic resonance tuberance and medial to the occipital artery. The third imaging (MRI) of the head and cervical spine may be occipital nerve is medial to the greater occipital nerve indicated. The work-up of occipital neuralgia should and the lesser occipital nerve is about 2.5 cm lateral to include assessment for atlanto-axial joint instability.
TABLE 1: Differential diagnosis of common headaches
Clinical features
Epidemiology
Pathophysiology
Migraine
headache
headache
May affect frontal, fronto-occipital, occipital, orbital area. headache
orbital pain. If untreated, may last from 15 to 180 minutes. At least one autonomic sign on painful side (eg, lacrimation, nasal congestion, rhinorrhea, miosis, eye edema, ptosis, conjunctival injection).
May occur from once a day to 8 times a day in cycles from 1 week to every year.
Cervicogenic May have similar presentation as
headache
occipital neuralgia, cluster, tension, and neck movement or change in head position. Ipsilateral shoulder, neck, or arm pain that is nonradicular. Usually unilateral, and can involve neck, occiput, temple, or periorbital region. Typically constant or intermittent, but rarely throbbing or lancinating. May have associated nausea and dizziness.
Occipital
neuralgia
usually. May be unilateral or bilateral. nerves or C2 and/or C3 nerve rootsby degenerative cervical spine changes, cervical disc disease, andtumors affecting the C2 and C3nerve roots.
The greater and third occipital nerves are blocked 10 to 20 minutes. The most serious complication is pierc- slightly above the superior nuchal line, just medial to the ing the occipital artery and bleeding. Compression of the occipital artery, which is easily palpated. After antiseptic occipital artery is usually effective in avoiding any preparation, a 25 gauge 11/2 inch needle attached to a 5 ml syringe is placed just medial to the artery at the C2 and/or C3 ganglion block
above location. For diagnostic indications, 1 ml of localanesthetic is injected. For treatment, 3-5 ml of local anes- C2 and/or C3 ganglion block has proven successful in thetic combined with steroid is injected. Anesthesia in the treating some patients. One case report demonstrated region of the greater occipital nerve usually occurs within that a patient with severe intractable occipital neuralgia RADIOFREQUENCY THERMOCOAGULATION
became pain-free for >2 months when given a C2ganglion block.12 However, repeat blocks with steroids may have adverse effects. A case report pub- another widely used method to treat occipital lished in 2001 demonstrated that a 39-year-old neuralgia. It has many advantages, including safety, female who had 6 bilateral greater occipital nerve efficacy, a rapid recovery period, and no permanent blocks over a period of 3 months developed signs of scarring. C2 ganglionotomy by RF lesion generator Cushing’s syndrome. Signs and symptoms were has also been performed and resulted in cases of intermittent hypertension, severe muscle weakness, significant pain relief. Pulsed radiofrequency (PRF) is yet another technique used to treat occipitalneuralgia. In a case report, a patient was treated BOTULINUM TOXIN
with PRF and, after a 12-month follow-up, was Botulinum Toxin Type A (botox) is an accepted pain-free.21 Recently, a new surgical treatment was treatment for migraine headache and muscle spasm- reported consisting of neurolysis of the greater related pain with relief up to 4 months.14 Botox was occipital nerve and sectioning of the inferior oblique originally used to treat strabismus and cervical dysto- nia.15 One trial demonstrated that botox helpedchronic daily headache and appeared to have a OCCIPITAL NERVE
cumulative effect with subsequent injections.16 STIMULATOR IMPLANTATION
Treatment with botox is generally well-tolerated; side Surgical implantation of a subcutaneous elec- effects are minimal and include minor discomfort or trode along the C1-C3 nerve level has been shown to bleeding at the time of injection.17 Clinical trials have significantly reduce the pain of occipital neuralgia in shown that botox injections for migraine headaches patients who have failed conservative therapies.23 reduced the duration, length, and severity of the In one study of 19 patients, 95% reported improve- headaches, as well as the intake of migraine medica- ment in their quality of life and would undergo the tions.18 Botox has been shown to be effective in the procedure again.24 In another study of 13 patients, treatment of whiplash-associated disorders that often 12 reported good-to-excellent pain control at up to cause occipital neuralgia. It improved the pain and 6 years of follow-up.25 The benefit of this procedure increased the range of motion in these patients.
is that it is minimally invasive and there is no perma- Because of its success in the treatment of muscle nent destruction of nerves or other vital structures.
spasms and migraines, botulinum toxin may prove to Another advantage is that patients can first undergo a be a reasonable treatment option for occipital neural- percutaneous trial of temporary lead placement for several days prior to permanent lead implantation.
Depending on the results of the temporary percuta- SURGICAL OPTIONS
neous trial, patients may or may not undergo the Occipital neuralgia can occasionally be treated more invasive permanent lead implantation. It has successfully with microvascular nerve decompression.
been postulated that a successful temporary percuta- Surgical procedures such as epifacial electric stimula- neous lead trial, in combination with a successful tion, dorsal cervical rhizotomy, neurolysis of the diagnostic occipital nerve block, may predict a highly greater occipital nerve, and radiofrequency rhizo- effective permanent occipital nerve stimulator tomy may also be considered. Selective C2 and/or C3 dorsal rhizotomy is another option, although fewpapers have been published assessing its utility.
CONCLUSION
Dubuisson followed 14 patients over a period of 33 Occipital neuralgia is a headache syndrome that months after partial posterior rhizotomy at C1-3. He requires careful attention to enable proper diagnosis found that 10 of 14 patients (71%) had continuing and treatment. Typically, there is no clear structural significant relief over that period of time.19 CT or cause, although appropriate work-up should be con- fluoroscopy-guided percutaneous C2 and/or C3 sidered in order to rule-out pathologic structural nerve block is also useful for confirmation of occipi- causes. The occipital nerve block is a valuable, tal neuralgia and as a preoperative guide for dorsal simple, and safe diagnostic and therapeutic tool that should be considered early in the course of treatment.
If the pain persists despite preliminary therapies, 16. Ondo WG, Vuong KD, Derman HS. Botulinum toxin A including occipital nerve blockade with local anes- for chronic daily headache: a randomized placebo-controlled, parallel design study. Cephalalgia 2004;24(1):60.
thetic and steroid, then botulinum toxin or perma- 17. Freund BJ, Schwartz M. Use of botulinum toxin in chronic nent implantation of a percutaneous occipital nerve whiplash-associated disorder. Clin J Pain 2002;18(6 Suppl): stimulator should be considered before destructive C2 and/or C3 root surgical procedures are imple- 18. Binder WJ, Blizter A. Treatment of migraine headache with botulinum toxin type A. Facial Plast Surg Clin North Am 19. Dubuisson D. Treatment of occipital neuralgia by partial posterior rhizotomy at C1-3. J Neurosurg 1995;82(4):581-6.
Steven Barna, M.D., is the Medical Director of the MGH
20. Kapoor V, Rothfus WE, Grahovac SZ, Amin Kassam SZ, Pain Clinic and an Instructor at Harvard Medical School. Horowitz MB. Refractory occipital neuralgia: preoperative Dr. Barna’s major clinical and academic interest is mini- assessment with CT-guided nerve block prior to dorsal mally invasive interventional treatment of chronic pain. cervical rhizotomy. Am J Neuroradiol 2000;24(10):2105-10.
21. Park CH, Jeon EY, Chung JY, Kim BI, Roh WS, Cho SK.
Maliha Hashmi, BS, is a clinical researcher at the MGH
Application of pulsed radiofrequency for 3rd occipital neu- Pain Center and Neural Plasticity Research Group of ralgia: A case report. J Korean Pain Soc 2004;17(1):63-65.
22. Gille O, Lavignolle B, Vital JM. Surgical treatment of greater occipital neuralgia by neurolysis of occipital nerveand sectioning of the inferior oblique muscle. Spine References
1. Martelletti P, Suijlekom HV. Cervicogenic headache: 23. Stojanovic, M. Stimulation methods for neuropathic pain Practical approaches to therapy. CNS Drugs 2004;11(18): control. Current Pain and Headache Reports 2001;5:130- 2. Trancredi A, Caputti F. Greater occipital neuralgia and 24. Oh M, Ortega J, Bellote JB, Whiting DM, Alo K. Periph- arthrosis of C1-C2 lateral joint. European J Neurology eral nerve stimulation for the treatment of occipital neural- gia and transformed migraine using a C1-2-3 subcutaneous 3. Kuhn WF, Kuhn SC, Gilberstadt H. Occipital neuralgias: paddle style electrode: A technical report. Neuromodulation clinical recognition of a complicated headache. A case series and literature review. J Orofac Pain 1997;11(2):158-65.
25. Weiner RL, Reed KL. Peripheral neurostimulation for control of intractable occipital neuralgia. Neuromodulation 4. Loeser JD. The management of pain. In: Cranial Neuralgias.
5. Sulfaro MA, Gobetti JP. Occipital neuralgia manifesting as orofacial pain. Oral Surg Oral Med Oral Pathol Oral Radiol Abstracts of Interest
Botulinum neurotoxin for the treatment of
6. Kondev L, Minster A. Headache and facial pain in children migraine and other primary headache disorders.
and adolescents. Otolaryngol Clin North Am 2003;36(6):1153-70.
B L U M E N F E L D A M , D O D I C K D W, S I L B E R S T E I N S D , 7. Anthony M. Headache and the greater occipital nerve. Clin Neurol Neurosurg 1992; 94(4):297-301. Clinical data and experience to date have demonstrated 8. Rifat SF, Lombardo JA. Occipital neuralgia in a football that BoNT-A is an effective and well-tolerated therapy player: a case report. Clin J Sport Med 1995;5(4):251-3.
for the prevention of migraine and other headache 9. Decheng C, Gale S. Diseases treated by single point of disorders. It has a long duration of action that may last acupuncture and moxibustion. Foreign Languages Press;Beijing: 2001.
over 4 months with no systemic or serious AEs. Several 10. Xie Z. 51 cases of occipital neuralgia treated with acupunc- issues remain to be defined, however, including dosing, ture. J Tradit Chin Med 1992;12(3):180-1. location, and number of injections; optimal dilution of 11. Gawel MJ, Rothbart P. Occipital nerve block in the BoNT-A; specific headache types that respond best to management of headache cervical pain. Cephalalgia 1992; BoNT-A; and long-term efficacy and safety. Data from ongoing well-designed trials that include a larger 12. Lim SY, Kim SG, Shin KM, Soon HY. Percutaneous C2 ganglionotomy in the management of occipital neuralgia patient population investigating these issues may con- report. J Korean Pain Soc 1995; 009(1):200-5.
firm a role for BoNT-A as a first-line agent for migraine 13. Lavin PJ, Workman R. Cushing syndrome induced by serial prevention. Neurotoxin therapy is part of a broader occipital nerve blocks containing corticosteroids. Headache headache management approach. Because the injection techniques for headache are unique and vary depending 14. Loder E, Biondi D. Use of botulinum toxins for chronic on the primary headache disorder being treated and the headaches: a focused review. Clin J Pain 2002;18(6 Suppl): location and pattern of pain referral, the use of BoNT- 15. Blumfeld AM, Dodick DW, Silberstein SD. Botulinum A for headache is not simply an extension of its use for neurotoxin for the treatment of migraine and other primary cosmesis. The use of BoNT-A in the overall manage- headache disorders. Dermatol Clin 2004; 22(2):167-75.
ment of primary headache disorders should be reserved for medical practitioners who not only have experience with explained by gate control theory in the past, it seems that BoNT-A injections, but possess the expertise in the diagno- neuromodulation acts also by modulation of neurotransmit- sis and management of complex headache disorders.
ters in the central nervous system. Three neurostimulation Educating patients and addressing headache triggers and methods are currently used in clinical practice: spinal cord optimizing acute treatment improve the outcome of any stimulation (SCS), peripheral nerve stimulation (PNS), and deep brain stimulation (DBS). The SCS and PNS are excel- Dermatol Clin 2004;22(2):167-75.
lent treatment choices for certain forms of neuropathic pain.
The new indications for SCS are end-stage peripheral vascu- Peripheral neurostimulation for
lar disease and ischemic heart disease, whereas PNS is used control of intractable occipital neuralgia
for the treatment of occipital neuralgia and chronic pelvicpain. DBS is reserved for carefully selected patients in whom W E I N E R R L , R E E D K L , D A L L A S , T E X A S the other treatment modalities have failed. In a minority of OBJECTIVE: To present a novel approach for treatment of
patients the "tolerance" to neurostimulation develops after intractable occipital neuralgia using percutaneous peripheral long-term use. Further research is needed to establish better nerve electrostimulation techniques.
outcome predictors to neurostimulation and possibly METHODS: Thirteen patients underwent 17 implant
procedures for medically refractory occipital neuralgia. A Curr Pain Headache Rep 2001;5(2):130-7.
subcutaneous electrode placed transversely at the level of C1across the base of the occipital nerve trunk produced pares- Upcoming Scientific Meetings
thesias and pain relief covering the regions of occipital nerve pain. RESULTS: With follow-up ranging from 1-1/2 to 6 years,
Spotlight on Migraine: Real Patients – Real Answers
American Headache Society
12 patients continue to report good to excellent response with greater than 50% pain control and requiring little or no additional medications. The13th patient (first in the series) was subsequently explanted following symptom resolution.
CONCLUSIONS: In patients with medically intractable
occipital neuralgia, peripheral nerve electrostimulation subcutaneously at the level of C appears to be a reasonablealternative to more invasive surgical procedures following failure of more conservative therapies.
24th Annual Meeting of the American Pain Society
American Pain Society
Neuromodulation 1999;2(3):217-221.
Hynes Convention Center, Boston, MassachusettsCONTACT: www.ampainsoc.org Stimulation methods for neuropathic pain control
Neurostimulation methods for control of chronic neuro- 23-25 June 2005
47th Annual Scientific Meeting of the
pathic pain have recently gained in popularity. The reasons American Headache Society
for this are multifactorial. As opposed to nerve ablation, these methods are minimally invasive and reversible. The improvements in hardware design simplified implantation techniques and prolonged equipment longevity. Stimulation trials have become less invasive, allowing patients to test its effects before final implantation. Finally, the scientificevidence has shown good outcomes of neurostimulationmethods for chronic neuropathic pain control. Recentresearch efforts have revealed new potential mechanisms ofaction of neurostimulation. Whereas its action was widely This publication is made possible by an educational grant from 2004 The MGH Pain Center, Massachusetts General Hospital, which is solely responsible for the contents. The opinions expressed in this publication do not necessarily reflect those of the publisher or
sponsor, but rather are those of the authoring institution based on the available scientific literature. Publisher: SNELL Medical Communication Inc. in cooperation with the MGH Pain Center,
Massachusetts General Hospital. All rights reserved. The administration of any therapies discussed or referred to in Pain Management Rounds should always be consistent with the recognized prescribing
information as required by the FDA. SNELL Medical Communication Inc. is committed to the development of superior Continuing Medical Education.

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