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HEADACHE CURRENTS

Headache Currents

Occipital Neuralgia Diagnosis and Treatment:


The Role of Ultrasound
Samer Narouze, MD, PhD

ETIOLOGY OF OCCIPITAL NEURALGIA


Background.—Occipital neuralgia is a form of neuropathic
Most cases of occipital neuralgia are idiopathic with no defi-
type of pain in the distribution of the greater, lesser, or third
occipital nerves. Patients with intractable occipital neuralgia do nite structural etiology. Possible etiologies are listed below.
not respond well to conservative treatment modalities. This  Occipital nerve entrapment: The greater occipital
group of patients represents a significant therapeutic challenge
and may require interventional or invasive therapeutic nerve (GON) arises from the C2 dorsal ramus with
approaches. minor contribution from C3 and can follow a very
Purpose.—Occipital neuralgia frequently occurs as a result variable anatomic course.2–5 It curves around the
of nerve entrapment or irritation by a tight muscle or vascular
inferior border of the inferior oblique muscle to
structure, or nerve trauma during whiplash injury.
Although the entrapment theory is most commonly accepted, ascend on its superficial surface. Then it penetrates
it lacks strong clinical evidence to support it. Accordingly, the the semispinalis capitis and invariably the splenius
available interventional approaches have been targeting the muscle to end subcutaneously near the nuchal line by
accessible part of the occipital nerve rather than the entrapped
part.
penetrating the trapezius muscle or the fascia. The
Conclusion.—Bedside sonography is an excellent imaging GON can be entrapped anywhere from its origin at
modality for soft tissue structures. Ultrasound not only allows the C2 nerve root until it becomes subcutaneous at
distinguishing normal from abnormal entrapped occipital the trapezius apponeurosis.
nerves, it can identify the level and the cause of entrapment as
well. Ultrasound guidance allows precise occipital nerve blocks  Postoperative: especially after Arnold-Chiari malfor-
and interventions at the level of the “specific” entrapment mation surgery or other craniocervical junction sur-
location rather than into the site of “presumed” entrapment. geries. Occipital neuralgia and neuritis have been
reported after radiofrequency ablation (RFA) and
Key words: occipital neuralgia, diagnostic ultrasound, entrapment cryoablation.6
neuropathy  Atlanto-axial joint (AAJ) rheumatoid arthritis and
subluxation as the C2 dorsal root ganglion lies poste-
riorly to the medial aspect of the joint.
 C2 nerve root and dorsal root ganglion (DRG)
lesions: for example, meningioma, vascular
INTRODUCTION malformation.
Occipital neuralgia is defined as a unilateral or bilateral  Trauma, infection, space occupying lesions, and
paroxysmal, shooting, or stabbing pain in the posterior tumors.
part of the scalp, in the distribution of the greater, lesser,
or third occipital nerves. It is commonly associated with DIFFERENTIAL DIAGNOSIS OF OCCIPITAL
tenderness over the involved nerve and sometimes accom- NEURALGIA
panied by diminished sensation or dysesthesia in the Occipital neuralgia must be distinguished from primary and
affected area.1 The pain of occipital neuralgia may be secondary headache disorders as well as tender trigger points
referred to the fronto-orbital area through the trigemino- in the suboccipital and upper neck muscles.
cervical interneuronal connections in the trigeminal spinal Occipital pain can be referred pain arising from the atlanto-
nucleus (Table 1). occipital, atlanto-axial, or upper cervical zygapophyseal (facet)
joints. Clinically, this can be differentiated from the typical
Address all correspondence to Samer Narouze, MD, PhD, Clinical Professor of Anesthesiol-
ogy and Pain Management, Ohio University; Clinical Professor of Neurological Surgery,
Ohio State University; Chairman, Center for Pain Medicine; Western Reserve Hospital,
1900 23rd St, Cuyahoga Falls, OH 44223, USA.
Accepted for publication February 8, 2016.
.............
Headache .............
C 2016 American Headache Society
V Conflict of Interest: Advisory board; SJM.

801
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Table 1.—ICHD-3 Beta Diagnostic Criteria of Occipital


Neuralgia†
A. Unilateral or bilateral pain fulfilling criteria B-E
B. Pain is located in the distribution of the greater, lesser and/or third
occipital nerves
C. Pain has two of the following three characteristics:
1. recurring in paroxysmal attacks lasting from a few seconds to
minutes
2. severe intensity
3. shooting, stabbing or sharp in quality
D. Pain is associated with both of the following:
1. dysesthesia and/or allodynia apparent during innocuous stimulation
of the scalp and/or hair
2. either or both of the following:
(a) tenderness over the affected nerve branches
(b) trigger points at the emergence of the greater occipital nerve or in
the area of distribution of C2
E. Pain is eased temporarily by local anesthetic block of the affected Fig. 2.—Short axis sonogram at C1-2 level showing a normal
nerve greater occipital nerve (arrows) as it runs between the inferior
F. Not better accounted for by another ICHD-3 diagnosis. oblique muscle (IOM) and the semispinalis capitis (SSC). Trap,
trapezius muscle; Spl, splenius muscle.

neuropathic pain of occipital neuralgia, as the pain will be 1. Diagnosis of occipital nerve entrapment: by demon-
aggravated with movement of the affected joint.
strating enlarged, abnormal, swollen nerve8 (Figs. 2
Figure 1 illustrates a proposed algorithm to guide appropri-
ate diagnosis and treatment for occipital pain.7 and 3). The normative sonographic data indicate
that the GON cross-sectional area is 2.0 6 0.1 mm2
at C1-2 level. The size of the GON typically
remains the same until it branches in the occipital
THE ROLE OF ULTRASOUND IN DIAGNOSIS
THE ETIOLOGY OF OCCIPITAL NEURALGIA area. The mean GON cross-sectional area in
Diagnostic Ultrasound symptomatic patients following entrapment was
The GON can be traced with ultrasound from its origin at 4.1 6 2.6 mm2.8,9
C2 nerve root all the way until it becomes subcutaneous at
the trapezius aponeurosis.

Fig. 3.—Short axis sonogram at C1-2 level showing a swollen


greater occipital nerve with edema surrounding the nerve
Fig. 1.—Occipital headache algorithm. *There is lack of evidence (arrows) as it runs between the inferior oblique muscle (IOM)
supporting the use of BoNT for tension type headache (TTH). and the semispinalis capitis (SSC).
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Fig. 4C.—Short axis sonogram at the occipital level showing the


Fig. 4A.—Short axis sonogram at C1-2 level showing the greater greater occipital nerve (arrows) as it pierces the trapezius muscle
occipital nerve (arrows) as it runs between the inferior oblique (Trap). Occ, occiput. Notice the swollen enlarged nerve.
muscle (IOM) and the semispinalis capitis (SSC). Trap, trapezius
muscle; Spl, splenius muscle. 2. Diagnosis of the cause of entrapment:

 entrapment within the suboccipital muscles


(Fig. 4A-C)
 impingement by arterial vessels (Fig. 5)
 impingement by venous aneurysms/malformations
(Fig. 6)
 muscle space occupying lesion (Fig. 7)

3. Lesions at the level of the C2 nerve root and dorsal


root ganglion (DRG) may be identified as well (Fig. 8).

THE ROLE OF ULTRASOUND IN THE


TREATMENT OF OCCIPITAL NEURALGIA
Interventional Modalities
1. Ultrasound guided occipital nerve block
The procedure can be performed either distally at the
nuchal line (Fig. 9, position A) or more proximally
between C1 and C2 (Fig. 9, position B).10 I prefer block-
ing the GON at C1-2 level where it runs between the infe-
rior oblique muscle (IOM) and the semispinalis capitis
muscle (SSC). The GON is well identified here and can
Fig. 4B.—Short axis sonogram at C1 level showing the greater be easily targeted rather than trying to identify the termi-
occipital nerve (arrows) entrapped within the belly of the semi-
nal subcutaneous branches at the nuchal line. However, it
spinalis capitis (SSC). Trap, trapezius muscle; Spl, splenius mus-
cle; IOM, inferior oblique muscle. Notice the enlarged nerve with should be noted that are no available studies directly com-
enhanced fascicular pattern. paring blocking the neve at these two locations.
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Fig. 5.—Short axis sonogram at C1-2 level showing an abnormal Fig. 7.—Short axis sonogram at C1-2 level showing a cyst
artery (A) impinging the greater occipital nerve (arrow) as it runs (arrows) within the semispinalis capitis (SSC). IOM, inferior
between the inferior oblique muscle (IOM) and the semispinalis oblique muscle.
capitis (SSC).
laterally until the suboccipital muscles are seen in the
The procedure can be performed with the patient either view. To better differentiate the IOM from the SSC, the
in the prone or sitting position. A high-frequency ultra- lateral end of the transducer is tilted cephalad to be in line
sound transducer is usually used (low-frequency trans- with the orientation of the IOM as it stretches between C1
ducer may be used depending on body habitus). First, a and C2. By changing the transducer orientation in this
transverse short axis view is obtained by applying the manner, the sonogram will show the IOM in its long axis,
transducer in the midline over the occiput and then while obtaining a short axis view of the SSC and thus
scanning caudally to identify C1 and C2 levels. C1 lacks a clearly differentiating the two muscles and the fascial
spinous process, and the first bifid spinous process plane in between where the GON can be searched for.
encountered is C2 (Fig. 10). Then, the transducer is moved

Fig. 8.—Short axis sonogram at C2 level showing the C2 dorsal


Fig. 6.—Short axis sonogram at C1-2 level showing an abnormal root ganglion (DRG), the vertebral artery (VA), and the greater
vein impinging the greater occipital nerve (arrows) as it runs occipital nerve (GON) between the inferior oblique muscle
between the inferior oblique muscle and the semispinalis capitis. (IOM) and the semispinalis capitis (SSC).
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Fig. 11.—Short axis sonogram at C1 level showing the lead


(arrow heads) placed subcutaneously superficial to the semispina-
lis capitis muscle (SSC) and the inferior oblique muscle (IOM).
SC, subcutaneous.

and whether the blocks should be done with local anes-


thetic alone or with steroids. Different recommendations
Fig. 9.—The position and orientation of the ultrasound transducer were made for different headache disorders, including
for greater occipital nerve block. A: nuchal line; B: C1-2 level. occipital neuralgia, migraine, and cluster headache.11,12
2. Ultrasound guided botulinum toxin type-A injec-
The American Headache Society Special Interest tions
Group expert consensus and systematic reviews on occipi- Recent studies have shown that injection of onabotu-
tal and other nerve blocks, published in 2010 and 2013, linumtoxinA into the “presumable” sites of GON
addressed other considerations for the blocks, including entrapment may provide some short-term relief in
the frequency of injections, as well as the type of injectate, symptomatic patients.13,14

Fig. 12.—Short axis sonogram at C1 level showing the lead


Fig. 10.—Short axis sonogram at C2 level. Note the bifid spinous (arrow heads) placed between the semispinalis capitis muscle
process of C2 (arrows). Trap, trapezius muscle; SSC, semispinalis (SSC) and the inferior oblique muscle (IOM). Med, medial; lat,
capitis muscle; IOM, inferior oblique muscle. lateral.
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Our observations indicate that botulinum toxin may tissue structures, ultrasound-guided technique will ena-
provide sustained relief in patients with occipital neu- ble the lead to be placed subcutaneously superficially
ralgia when injected into a “specific” entrapment loca- to the occipital muscles (Fig. 11). The GON can also
tion (rather than into the site of potential or be recognized, and the lead can alternatively be
“presumed” entrapment). The appropriate site for placed intentionally between the inferior oblique and
injection may be identified with bedside ultrasound semispinalis muscle (where the nerve runs) at the C1-
imaging. The normalization of biomechanics after the 2 level (Fig. 12).20 In this latter case, the GON can be
release of the occipital nerve is likely the source of the stimulated with minimal settings, which can save the
long-term recovery, rather than prolonged action of the life of the battery.
botulinum toxin itself.8
3. Ultrasound guided occipital peripheral nerve Acknowledgment: All figures are printed with permis-
pulsed radiofrequency sion from Ohio Pain and Headache Institute; Samer Nar-
There is an interest in pulsed radiofrequency (PRF) ouze, MD, PhD.
as a minimally invasive percutaneous treatment for
References
intractable occipital neuralgia. At the time of this writ-
1. Headache Classification Committee of the International Headache
ing in February 2016, there are 2 prospective studies Society (IHS). The International Classification of Headache Disor-
and one retrospective multicenter study with promising ders, 3rd edition (beta version). Cephalalgia. 2013;33:629-808.
results.15–17 However, the lack of randomized controlled 2. Mosser SW, Guyuron B, Janis JE, Rohrich RJ. The anatomy of
the greater occipital nerve: Implications for the etiology of
studies and the variation in the technique with land- migraine headaches. Plast Reconstr Surg. 2004;113:693-697
mark versus imaging guidance limit the wide applica- 3. Becser N, Bovim G, Sjaastad O. Extracranial nerves in the poste-
tion of this treatment modality. rior part of the head. Anatomic variations and their possible clin-
ical significance. Spine. 1998; 23:1435-1441.
4. Bovim G, Bonamico L, Fredriksen TA, Lindboe CF, Stolt-
4. Ultrasound guided occipital peripheral nerve Nielsen A, Sjaastad O: Topographic variations in the peripheral
stimulation course of the greater occipital nerve. Autopsy study with clinical
Occipital neurostimulation (ONS) or greater occipi- correlations. Spine. 1991;16:475-478.
5. Tubbs RS, Watanabe K, Loukas M, Cohen-Gadol AA. The intra-
tal peripheral nerve stimulation offers the potential for
muscular course of the greater occipital nerve: Novel findings
a minimally invasive, relatively low risk, nerve preserv- with potential implications for operative interventions and occi-
ing, and reversible approach to managing intractable pital neuralgia. Surg Neurol Int. 2014;31;5:155.
occipital neuralgia as well as other headache disorders. 6. Kim CH1, Hu W, Gao J, Dragan K, Whealton T, Julian C.
Cryoablation for the treatment of occipital neuralgia. Pain Physi-
There is a growing body of literature regarding the
cian. 2015;18:E363-368.
efficacy and safety occipital nerve stimulation, and the 7. Narouze S. Algorithms for the diagnosis and management of
Congress of Neurological Surgeons released a state- head and face pain. In: Narouze SN, ed. Interventional Manage-
ment in 2015 supporting the use of ONS for the treat- ment of Head and Face Pain. Springer; 2014: 9-14.
8. Narouze S, Souzdalnitski D. Occipital nerve entrapment within
ment of intractable occipital neuralgia, however
the semispinalis capitis muscle diagnosed with ultrasound. Ceph-
demanding more research and a unified improved alalgia. 2013;33:1358-1359.
technical approach.18 9. Cho JC, Haun DW, Kettner NW. Sonographic evaluation of the
The level and depth of lead placement are very cru- greater occipital nerve in unilateral occipital neuralgia.
J Ultrasound Med. 2012;31:37-42.
cial for successful ONS results. Traditionally, the lead
10. Greher M, Moriggl B, Curatolo M, Kirchmair L, Eichenberger
is placed with fluoroscopy, and if the lead is too U. Sonographic visualization and ultrasound-guided blockade of
superficial patients may experience unpleasant dysthe- the greater occipital nerve: A comparison of two selective techni-
sias in the overlying skin area. If the lead is placed ques confirmed by anatomical dissection. Br J Anaesth. 2010;
104:637-642.
deep, it may penetrate the occipital muscles, which
11. Blumenfeld A, Ashkenazi A, Napchan U, et al. Expert consensus
usually leads to painful muscle spasms upon stimula- recommendations for the performance of peripheral nerve blocks
tion.19 As ultrasound is a great tool in visualizing soft for headaches–a narrative review. Headache. 2013;53:437-446.
807 | Headache | April 2016
Headache Currents

12. Ashkenazi A, Blumenfeld A, Napchan U, et al. Interventional 16. Choi, HJ, Oh IH, Choi SK, Lim YJ. Clinical outcomes of
procedures special interest section of the American. Peripheral pulsed radiofrequency neuromodulation for the treatment of
nerve blocks and trigger point injections in headache manage- occipital neuralgia. J Korean Neurosurg Soc. 2012;51:281-285.
ment–a systematic review and suggestions for future research. 17. Huang JHY, Galvagno SM Jr, Hameed M, et al. Occipital
Headache. 2010;50:943-952. nerve pulsed radiofrequency treatment: A multi-center study
13. Kapural L, Stillman M, Kapural M, McIntyre P, Guirgius M, Mekhail evaluating predictors of outcome. Pain Med. 2012; 13:489-497.
N. Botulinum toxin occipital nerve block for the treatment of severe 18. Sweet JA, Mitchell LS, Narouze S, et al. Occipital nerve stimula-
occipital neuralgia: a case series. Pain Pract. 2007;7:337-340. tion for the treatment of patients with medically refractory occipi-
14. Taylor M, Silva S, Cottrell C. Botulinum toxin type-A tal neuralgia: Congress of neurological surgeons systematic review
(BOTOX) in the treatment of occipital neuralgia: A pilot study. and evidence-based guideline. Neurosurgery. 2015;77:332-41.
Headache. 2008;48:1476-81. 19. Hayek SM, Jasper J, Deer TR, Narouze S. Occipital
15. Vanelderen P, Rouwette T, De Vooght P, Puylaert M, Heylen R, neurostimulation-induced muscle spasms: Implications for lead
Vissers K, Van Zundert J. Pulsed radiofrequency for the treat- placement. Pain Physician. 2009;12:867-876.
ment of occipital neuralgia: A prospective study with 6 months 20. Narouze S. Ultrasonography in pain medicine: Future directions.
of followup. RegAnesth Pain Med. 2010;35:148-151. Tech Reg Anesth Pain Manage. 2009;13;198-202.

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