Neuralgias of The Head: Occipital Neuralgia: Review

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REVIEW

Neuroscience

http://dx.doi.org/10.3346/jkms.2016.31.4.479 • J Korean Med Sci 2016; 31: 479-488

Neuralgias of the Head: Occipital Neuralgia


Il Choi1 and Sang Ryong Jeon2 Occipital neuralgia is defined by the International Headache Society as paroxysmal
1
shooting or stabbing pain in the dermatomes of the greater or lesser occipital nerve.
Department of Neurological Surgery, Dongtan
Sacred Heart Hospital, University of Hallym
Various treatment methods exist, from medical treatment to open surgical procedures.
University, Hwaseong, Korea; 2Department of Local injection with corticosteroid can improve symptoms, though generally only
Neurological Surgery, Asan Medical Center, temporarily. More invasive procedures can be considered for cases that do not respond
University of Ulsan College of Medicine, Seoul, adequately to medical therapies or repeated injections. Radiofrequency lesioning of the
Korea
greater occipital nerve can relieve symptoms, but there is a tendency for the pain to recur
Received: 13 November 2015 during follow-up. There also remains a substantial group of intractable patients that do
Accepted: 20 January 2016 not benefit from local injections and conventional procedures. Moreover, treatment of
occipital neuralgia is sometimes challenging. More invasive procedures, such as C2
Address for Correspondence:
Sang Ryong Jeon, MD
gangliotomy, C2 ganglionectomy, C2 to C3 rhizotomy, C2 to C3 root decompression,
Department of Neurological Surgery, Asan Medical Center, neurectomy, and neurolysis with or without sectioning of the inferior oblique muscle, are
University of Ulsan, College of Medicine, 88 Olympic-ro 43-gil,
Songpa-gu, Seoul 05505, Korea
now rarely performed for medically refractory patients. Recently, a few reports have
E-mail: [email protected] described positive results following peripheral nerve stimulation of the greater or lesser
occipital nerve. Although this procedure is less invasive, the significance of the results is
hampered by the small sample size and the lack of long-term data. Clinicians should
always remember that destructive procedures carry grave risks: once an anatomic structure
is destroyed, it cannot be easily recovered, if at all, and with any destructive procedure
there is always the risk of the development of painful neuroma or causalgia, conditions
that may be even harder to control than the original complaint.

Keywords:  Occipital Neuralgia; Third Occipital Nerve; Greater Occipital Nerve; Lesser
Occipital Nerve; Cervicogenic Headache

INTRODUCTION ETIOLOGY AND PATHOPHYSIOLOGY

According to the definition of the International Headache Soci­ Neuralgia is pain in one or more nerves caused by compression
ety (IHS), occipital neuralgia (ON), also known as C2 neuralgia, and/or irritation of peripheral nerve structures. In ON, irrita­
involves paroxysmal shooting or stabbing pain in the derma­ tion of the GON and/or LON by chronically contracted muscles
tomes of the greater occipital nerve (GON or nervus occipitalis and spondylosis of the upper cervical spine is often implicated
major) and the lesser occipital nerve (LON or nervus occipitalis (4,5). In addition, compression from intra- or extra cranial ves­
minor). From an origin in the suboccipital region, the pain spre­ sels, giant cell arteritis, callus formations after vertebral frac­
ads throughout the vertex, particularly the upper neck, back of tures, schwannomas, and other masses are rare causes of ON.
the head, and behind the eyes. The pain may be accompanied The etiologies are summarized in Table 1.
by hypesthesia or dysesthesia in the affected areas. The most
common trigger is compression of the GON or LON (1), with CLINICAL PRESENTATION
the GON more frequently involved (90%) than the LON (10%)
(2). Patients with ON suffer from a shooting or stabbing pain in the
neck that radiates over the cranium. The pain is characterized
EPIDEMIOLOGY as persistent, paroxysmally aggravating, and of variable distri­
bution; can be perceived in the retro-orbital area due to the con­
ON is a well-known disorder, but its incidence remains to be vergence of the C2 dorsal root and the nucleus trigeminus pars
accurately determined. A study in the Dutch general popula­ caudalis (6). Due to connections with the VIII, IX, and X cranial
tion reported a relatively low incidence of 3.2 per 100,000. Fe­ nerves and the cervical sympathicus, vision impairment/ocular
male dominance was present but not significant, and no time pain (67%), tinnitus (33%), dizziness (50%), nausea (50%), and
and seasonal variation was found (3). congested nose (17%) can also be present (7). On physical ex­

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Choi I, et al.  •  Occipital Neuralgia

Table 1. Known possible causes of irritation: vascular, neurogenic, muscular, and os- Diagnostic block
teogenic
The clinical presentation (i.e., tenderness over the occipital ner­
Category Causes of irritation ves) and a temporary improvement in the headache with a lo­
Vascular • Irritation of the C1/C2 nerve roots by an aberrant branch of the cal anesthetic diagnostic block of the occipital nerve on the af­
posterior inferior cerebellar artery (69)
• Dural arteriovenous fistula at the cervical level (70)
fected side confirm the diagnosis (1). Occipital nerve block, as
• Bleeding from a bulbocervical cavernomas (71) well as an essential diagnostic tool, can also be a good treatment
• Cervical intramedullar cavernous hemangioma (72) option for ON. Thus, the anatomy of the occipital nerve and the
• Giant cell arteritis (73-75)
• Fenestrated vertebra artery pressing on C1/C2 nerve roots (76) location of the exact target site are very important. Clinicians
• Aberrant course of the vertebra artery (77) should keep in mind that occipital nerve block relief is not spe­
Neurogenic • Schwannoma in the area of the craniocervical junction: schwan- cific for ON and that false-positive results occur with migraine
noma of occipital nerve (78,79)
• C2 myelitis (80)
and cluster headaches (7,10).
• Multiple sclerosis (81)
Osteogenic • C1/C2 arthrosis, atlantodental sclerosis (82) Anatomy of the occipital nerve
• Hypermobile C1 posterior arch (83)
The GON is the largest pure sensory nerve in our body. The GON
• Cervical osteochondroma (84)
• Osteolytic lesion of the cranium (85) is the medial branch of the dorsal ramus of the C2 spinal nerve.
• Exuberant callus formation after C1/C2 fracture (86) The GON initially courses in a downward, lateral direction, makes
the bend along the inferior oblique and is covered by the sple­
amination, tenderness along the course of the GON and LON nius capitis, the longissimus, and the semispinalis muscles. On
can be observed. Sometimes hypoesthesia or dysesthesia can rare occasions, the nerve travels intramuscularly within the in­
occur. The pain is located in the occipital area and may spread ferior oblique muscle. The GON then turns upward and pierces
toward the vertex. Though usually unilateral, it may be bilateral. the semispinalis capitis. Finally, it turns superolaterally to emerge
into the scalp by piercing the aponeurotic fibrous attachment
DIAGNOSTIC METHODS of the trapezius muscle and sternocleidomastoid muscle (SCM)
to the superior nuchal line. At this exit site, the occipital artery
According to the International Classification of Headache Dis­ and GON are closely associated. The GON splits and innervates
order (ICHD-II), ON belongs to the same family as cranial neu­ the occipital skin (medial branches) and the region behind the
ralgias, central and primary facial pain, and other headaches. pinna (lateral branches) immediately below the superior nu­
The diagnostic criteria are as below: chal line (11-14). The emergence point of the GON is at the semi­
A. Paroxysmal stabbing pain, with or without persistent aching spinalis muscle at a point 3 cm below the occipital protuberance
between paroxysms, in the distribution of the greater, lesser, and 1.5 cm lateral to the midline (13,15). Anatomical variations
and/or third occipital nerve of GON are occasionally found, particularly in the vertical axis
B. Tenderness over the affected nerve (16,17). The C2 ramus could be compressed between the pos­
C. Pain is eased temporarily by local anesthetic block of the nerve terior arch of the C1 and the lamina of the C2, but the nerve is
not especially vulnerable at this location (11).
Physical examination   The LON originates from the dorsal ramus of the C2 and, oc­
Along the course of the GON (over the occipital protuberance) casionally, the C3. It ascends toward the occiput along the pos­
and/or the LON (about 3 cm superomedially to the tip of the terior border of the SCM. Near the cranium, it perforates the
mastoid process), tenderness is detected by palpation (8). Tin­ deep fascia and runs upward over the occiput, where it inner­
gling may be evoked by light pressure or percussion on the nerve vates the skin and communicates medially with the GON (18).
(Tinel’s sign). When patients lie on a pillow and hyperextend or Although there is some variability in the anatomy, the nerve
rotate their neck, pain can occur (“pillow sign”). mostly emerges from the posterior border of the SCM superior
to the exit of the great auricular nerve. This point is approximate­
Imaging studies ly 6-7 cm lateral from the midline and 4-6 cm caudal to a line
Magnetic resonance imaging is the most important tool in the connecting the lowest points of the external auditory canals (15).
diagnosis of this disorder as it enables visualization of the sur­
rounding cervical and occipital soft tissues. A simple X-ray is 1) The Cruveilhier plexus: the connection among the C1, C2,
useful to rule out underlying pathologies, such as arthritis and and C3 sensory branches (Fig. 1)
craniocervical instability. A CT scan of the craniocervical junc­ Hollinshead stated that “The upper parts of the back muscles in
tion can reveal neoplastic or degenerative osseous pathology. the cervical region are also supplied by direct twigs from the
Sometimes there may be a discrepancy between the radiologi­ dorsal rami of the second and third cervical nerves, and by bran­
cal findings and the symptoms (9). ches that arise from the so-called posterior cervical plexus, a

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Choi I, et al.  •  Occipital Neuralgia

Splenius capitis muscle Semispinalis capitis muscle


Third occipital nerve
Greater occipital nerve

Rectus capitis minor muscle


Lonqissimus capitis muscle

Superior oblique capitis muscle

Rectus capitis major muscle Vertebral artery

Lesser occipital nerve


Inferior oblique capitis muscle

Cruveilhier plexus

Fig. 1. Anatomy around occipital nerve; Asan medical illustration team modified this figure with permission from Shane Tubbs (91).

very simple series of loops between the first and second and
the second and third dorsal rami, sometimes also with a loop to
the fourth (19).” Articular branches of the C2-3 facet joint aris­ Midbrain
ing from communicating branches between the third occipital
nerve and C2 dorsal ramus have also been found (20). V1
Pons
  Neural interconnections, such as the Cruveilhier plexus, can V2
Trigeminal
affect the therapeutic results of cervical muscular and facet de­ nerve (V)
V3
nervation procedures. Therefore, the anatomical variations in
the craniocervical region, such as the Cruveilhier plexus, may
result in resistant pain after partially destructive surgical proce­
Trigeminothalamic
dures for ON (21). tract
Spinal tract of trigeminal nerve
2) The innervations of the C1, 2, and 3 nerves
Although the C1 nerve is not thought to innervate a cutaneous C1 spinal nerve
tissue, it supplies some sensory innervations to deep somatic
tissues in the suboccipital region, including the short muscles C2 spinal nerve
of the occipital triangle, through its dorsal ramus, and the SCM,
Trigeminocervical
trapezius, and atlanto-occipital joint, through its ventral ramus. nucleus
In addition, the sinuvertebral nerve of C1 innervates the medi­ C3 spinal nerve
an atlantoaxial joint, dura mater, and vertebral artery, in con­
junction with the sinuvertebral nerves of the C2 and C3. The
dorsal ramus of the C2 innervates the splenius capitis and semi­
spinalis capitis and finally becomes the GON. The ventral ra­ Fig. 2. Mechanism of cervicogenic headache: pain referred pain from cervical struc-
tures due to convergence between trigeminal nerve and C1, 2, 3 verves in trigemino-
mus of the C2 supplies articular branches to the lateral C1/2 cervical nucleus.
joint as well as the prevertebral muscles, SCM, and trapezius.
The dorsal ramus of the C3 has three significant branches, the is also known as the third occipital nerve, and innervates the
lateral, deep medial, and superficial medial branches. The lat­ C2-C3 zygapophyseal joint as well as the skin in the suboccipi­
eral branch supplies the splenius capitis, cervicis, and longissi­ tal region (22). Cervicogenic headache, which involves pain re­
mus capitis; the deep medial branch innervates the semispina­ ferral from cervical structures, appears to be produced by con­
lis cervicis and multifidus; and the superficial medial branch vergent excitation that is evoked by stimulation of these nerves
innervates the semispinalis capitis. The superficial medial branch and that results in excitation of second-order neurons in the tri­

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Choi I, et al.  •  Occipital Neuralgia

Greater occipital nerve


External occipital tubercle

Lesser occipital nerve


Superior nuchal line

Inferior nuchal line


Injection point
Mastoid process

2.0-2.5 cm

Location of electrical rod 1.5-2.0 cm

6.0-7.0 cm

Fig. 3. Landmarks for injection of the occipital nerves and electrical stimulation; Rogier Trompert Medical Art, modified with permission from Vanelderen et al. (9).

geminocervical complex (Fig. 2) (23,24). for migraine, cluster headache, tension headache, or hemicra­
nia continua (28). ON must be distinguished from referred pain
Injection point (Fig. 3) from the atlantoaxial or upper zygapophyseal joints or from trig­
The injection points are variable. One of the points is located on ger points in neck muscle or their insertions (cervicogenic head­
a line that connects the middle of the ears, 3.18 cm from the mid­ ache) (23,24,28). The critical differential point is that ON is neu­
line. The other point is situated 3.8 cm lateral to the midline and ralgia from the occipital nerve, whereas cervicogenic headache is
one quarter of the distance along a line connecting the external nociceptive referred pain from cervical structures.
occipital protuberance to the mastoid (or 2 cm lateral and 2 cm
inferior to the external occipital protuberance). Some authors TREATMENT OPTIONS
have described this point as being 1.5 cm lateral and 2 to 2.5 cm
inferior to the external occipital protuberance (15-17,25-27). Since ON was first described in 1821, numerous causes have
Great variability in the course of the GON is described in the lit­ been suggested and a variety of interventions have been used
erature (18,25,27). in its treatment (29). Currently, there is still no clear consensus
on the management of ON. Medication, physical therapy, mini­
Differential diagnoses mal intervention, and aggressive surgery are applied step-by-
Several disorders share certain features with ON, such as pain in step. Evidence for the success of this approach is relatively weak.
the posterior neck and head. It is therefore sometimes difficult In addition, well-designed studies have rarely been reported.
to distinguish these disorders, unless additional features are seen. Treatment methods include medication, nerve blocks and, in
There are two important disease categories that clinicians should refractory cases, neurosurgical procedures. Education, patient
keep in mind. First, tumors, infections, and congenital anoma­ support, and reassurance are also important components of
lies (e.g., Arnold-Chiari malformation) should be distinguished. treatment. Management of neuropathic pain, which can devel­
It is crucial to diagnose these disorders. If they are missed by cli­ op following long-standing ON, should address both the pain
nicians, a devastating situation may result. ON can be mistaken itself and the psychological aspects associated with it.

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Choi I, et al.  •  Occipital Neuralgia

Conservative management been successfully used to treat several different types of head­
Conservative treatment includes posture correction and reduc­ aches, including tension-type headaches (39), cervicogenic
ing the neuralgic and muscle pain. Pharmacological treatment headaches (40), migraine (41), and chronic daily headaches
may include tricyclic antidepressants, serotonin reuptake in­ (39). BoNT-A injection can relieve the sharp, shooting pain as­
hibitors, anticonvulsants (e.g., carbamazepine, oxycarbamaze­ sociated with ON, though not dull, aching pain, and improve
pine, gabapentin, pregabalin), and opioids. Nonsteroidal anti- quality of life for several months (42,43). The studies conducted
inflammatory drugs and paracetamol tend to have transient ef­ with Botulinum toxin are listed in Table 2.
fects. The use of ergot derivatives is controversial. Infliximab
has shown some benefit (9,30-32). Pulsed radiofrequency treatment
Pulsed radiofrequency (PRF) treatment is known to reduce pain,
Interventional management primarily by the induction of a low-intensity electrical field aro­
Local anesthetic agent injection with or without steroid und sensory nerves that results in depressed conduction and
Local anesthetic agent injection with steroid may be used for inhibition of long-term activation in the lightly myelinated A-
therapeutic purposes. This method usually has a transient ef­ delta fibers and the small unmyelinated C fibers (44). In animal
fect, but in some cases (15%-36%) the pain subsidence may be studies, PRF-mediated pain relief is suggested to be due to mod­
maintained for several months (2,7,33). ulation of the descending noradrenergic and serotonergic path­
ways (45).
Botulinum toxin infiltrations   To date, a few reports have been published concerning PRF
Several studies have shown an analgesic effect of Botulinum treatment of ON (Table 2). All reports were observational co­
toxin A (BoNT-A) that outlasted the duration of its muscle relax­ hort studies without controls. The treatment in these ON stud­
ant effect (1,34). A few theories have been proposed to explain ies showed short-term to intermediate-term pain control and
this analgesic effect of the toxin (35-38). For instance, BoNT-A’s the parameters used were: 40-60 V voltage output; 2 Hz frequen­
inhibitory effects on sensory nerve mediators like substance-P cy; 20-ms pulses in a 1-second cycle, 120 seconds/cycle; 150-
(36), calcitonin gene-related peptide (37), and glutamate (38) 500 W impedance range; and 42°C plateau temperature. The
may be involved in pain relief. Botulinum toxin may directly in­ authors advise that careful attention to selection criteria and
hibit local neurogenic inflammation and indirectly inhibit cen­ treatment parameters may further improve treatment outcomes
tral sensitization by significantly decreasing the activity of wide (46-49).
dynamic range neurons (38). Clinically, Botulinum toxin has
Table 2. Publications on the treatment of ON with Botulinum toxin injection, pulsed radiofrequency (PRF), and nerve neurolysis
Case
Study Study design Follow-up duration Outcome measure method Results
No.
The treatment of ON with Botulinum toxin injection
Taylor et al. (42) 2008 Retrospective 6 12 wk VPAM Sharp/shooting pain significantly
improved; Dull aching pain not
significantly improved
Kapural et al. (43) 2007 Case series 6 4 wk VAS VAS 8.5 → 1
PDI PDI 56 → 17.5
Volcy et al. (87) 2006 A case report 1 N/A N/A Improved temporarily
The treatment of ON with PRF
Huang et al. (48) 2012 Retrospective, 102 At least 3 mon ≥ 50% pain relief for 51% positive result
multicenter at least 3 mon
Vanelderen et al. (49) 2010 Prospective 19 1, 2, and 6 mon VAS 52.6% significant improvement
Likert scale at 6 mon
Choi et al. (47) 2012 Retrospective 10 6-10 mon VAS, TPI All patients improved
The treatment of ON with nerve neurolysis
Ducic et al. (54) 2008 Retrospective 206 Minimal, 12 mon ≥ 50% pain relief 80.5% positive result
Gille et al. (52) 2004 Retrospective 10 Mean, 37 mon 1) VAS 1) 80/100 → 20/100
(33-43) 2) Consumption of analgesics 2) Decrease in all
3) Patient satisfaction 3) Satisfaction in all
Magnússon et al. (5) 1996 Retrospective 18 Mean, 28.7 mon NRS relief 88.9% better than fair
(12-38) > 75%: excellent
50%-74%: good
25%-49%: fair
< 24%: poor
ON, occipital neuralgia; No., number; VPAM, visual analog pain and medication use diary; VAS, visual analog scale; PDI, pain disability index; N/A, not available; PRF, pulsed ra-
diofrequency; TPI, total pain index; NRS, numerical rating scale.

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Choi I, et al.  •  Occipital Neuralgia

Surgery ON (Table 2). Factors correlated with a positive outcome in­


Surgical treatment of ON can be considered when a patient does clude tenderness over the GON, a positive response to GON
not respond adequately to medical therapies, such as repeated block or Botox, a history of direct occipital trauma, and preop­
injections, or minimally invasive procedures, such as PRF treat­ eratively being under the care of a neurologist or pain specialist
ment. Neurolysis of the occipital nerve (with or without section­ (54). Sectioning of the inferior oblique muscle is reported to be
ing of the inferior oblique muscle), C2 gangliotomy, C2 ganglio­ effective when occipital pain is exacerbated or triggered by flex­
nectomy, C2 to C3 rhizotomy, C2 to C3 root decompression, ion of the cervical spine (52). However, cervicogenic headache
and neurectomy were historically introduced for medically re­ is a contraindication of neurolysis (51). Careful preoperative di­
fractory patients (5,50-57). However, the results were variable. agnosis is needed.
Recently, there have been a few positive reports on peripheral
nerve stimulation, a less invasive surgery, of the GON or LON. Occipital nerve stimulation
Of these approaches, both occipital neurolysis and occipital Recently, successful results with ONS have been reported in the
nerve stimulation (ONS) have been used commonly in the clini­ management of intractable headaches (Table 3), including cer­
cal field, recently. In selective cases, these methods have shown vicogenic headache, ON, transformed migraine, hemicrania
good outcomes, but a well-designed randomization study with continua, and cluster headaches. This stimulation technique
a long-term observation is not yet available. Clinicians should involves the subcutaneous insertion of electrodes in the C1-C2
bear in mind the risk that destructive procedures carry, which region of the posterior cervical spine (Fig. 3). Convergence of
include the possibility of the development of painful neuroma afferents from C1-C3 cervical nerves with trigeminal afferents
or causalgia, conditions that may be even harder to control than is explained as the cause of the cervicogenic headache that re­
the original complaint (58). sults from activation of these nerves (59). The pain control mech­
anism of ONS is similar to that of gait control theory, consid­
Neurolysis ered in other peripheral nerve stimulation (58). As opposed to
Entrapment of the GON in its peripheral course is a significant destructive surgery, ONS is fully reversible. If the patient does
pathology in ON. Five potential sources of entrapment of the not want to use stimulation, it is easy to stop the stimulation and
GON are suggested: C2 nerve root (rare), inferior oblique mus­ the device can be removed with a simple procedure.
cle (rare), within the semispinalis capitis muscle, within the tra­
pezius muscle/aponeurotic tendon, and angiolymphatics (oc­ Other destructive surgeries
cipital artery/vein crosses the GON; lymph node presence, with­ The approach to patients with ON must initially be conserva­
in or distal to the trapezial tunnel) (54). tive. Although C2 gangliotomy, C2 ganglionectomy, C2 to C3
  Neurolysis of the GON appears to provide safe, durable pain rhizotomy, C2 to C3 root decompression, and distal neurecto­
relief in selected patients with chronic headaches caused by my have been tried historically for intractable cases, these kinds

Table 3. Summary of noteworthy articles on ONS for ON


Study Study design No. Follow-up duration Outcome measure method Results Lead type used
Picaza et al. (88) 1977 Retrospective 6 12-46 mon N/A 3/6, good to excellent N/A
Weiner et al. (64) 1999 Retrospective 13 (1.5-6 yr) > 50% pain relief 92% positive result Cylinder type
Oh et al. (66) 2004 (paddle) Retrospective 20 (10 ON, 1-6 mon > 50% pain relief 1 mon 100% (20/20) Paddle type
10 migraine) 6 mon 94% (17/18)
Kapural et al. (67) 2005 Retrospective 6 3 mon VAS VAS 8 → 2 Paddle type
PDI PDI 48 → 14
Rodrigo-Royo et al. (89) 2005 Retrospective 4 4-16 mon 50% pain reduction Improved in all Cylinder type
Slavin et al. (58) 2006 Retrospective 14 Mean 22 mon (5-32 mon) VAS 50% reduction 70% positive Cylinder type
Johnstone and Sundaraj (65) 2006 Retrospective 7 Mean 25 mon (6-47 mon) VAS 50% reduction 5/7 (71%) positive Paddle type
Opioid doses Reduction in all cases
ONS, occipital nerve stimulation; ON, occipital neuralgia; No., number; N/A, not available; VAS, visual analog scale; PDI, pain disability index.

Table 4. Summary of noteworthy articles on destructive surgery for ON


Study Study design Surgical method No. Follow-up duration Outcome measure method Results
Sharma et al. (90) 2005 Retrospective Neurectomy 22 6 wk Pain relief Relief in 90%
18 mon Relief in 70%
Dubuisson (21) 1995 Retrospective Rhizotomy at C1-3 14 33 mon (3-66) > 50% pain reduction 71% positive result
Wang and Levi (56) 2002 A case report Ganglionectomy of C2 1 N/A N/A Pain relief (+)
ON, occipital neuralgia; No., number; N/A, not available.

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Choi I, et al.  •  Occipital Neuralgia

of destructive surgeries risk the development of painful neuro­ related allergic reactions (3%, 1/37) can occur, though rarely
ma, causalgia, or intractable neuropathic pain, and thus their (58,64-68).
use is decreasing. The studies conducted with destructive sur­
geries are listed in Table 4. CONCLUSION

POTENTIAL COMPLICATIONS AND RED FLAGS Occipital neuralgia is defined by the IHS as paroxysmal shoot­
ing or stabbing pain in the dermatomes of the GON or LON (1).
Complications of interventional management The GON is the medial branch of the dorsal ramus of the C2
Infection or bleeding may result from any percutaneous tech­ spinal nerve. The GON initially courses in a downward, lateral
nique, though these are usually minor problems. A case of sud­ direction, makes the bend along the inferior oblique and is cov­
den unconsciousness due to inadvertent subarachnoid injection ered by the splenius capitis, the longissimus, and the semispi­
in a patient with a craniotomy defect has been reported (60). nalis muscles. The GON then turns upward and pierces the
Temporary dizziness, injection site soreness, focal alopecia, and semispinalis capitis. Finally, it turns superolaterally to emerge
paresthesia due to nerve injury should be anticipated (61-63). into the scalp by piercing the aponeurotic fibrous attachment
of the trapezius muscle and SCM to the superior nuchal line
Complications of ONS (11-13). On diagnosis, tumors, infections, vascular problems
Poor outcomes after electrode implantation are a common prob­ (e.g., posterior circulation hemorrhage or infarction), and con­
lem that has been reported following up to 30% of implanta­ genital anomalies (e.g., Arnold-Chiari malformation) should be
tions. Therefore, a test stimulation before permanent implanta­ ruled out first. Various treatment methods exist, from medical
tion is recommended in many studies. Permanent implanta­ treatment to open surgical procedures.
tion is usually recommended when 50% pain reduction occurs   Clinicians should always remember that destructive proce­
on test stimulation. dures carry grave risks: once an anatomic structure is destroyed,
  Lead migration (4%, 2/54) and postsurgical infection (12%, it cannot be easily recovered, if at all, and with any destructive
6/50) are relatively common and well-known complications. procedure there is always the risk of the development of painful
Fracture and disconnection of the lead (2%, 1/50) and implant- neuroma or causalgia, conditions that may be even harder to
control than the original complaint (58). The treatment of ON
should be performed through adequate algorithm (Fig. 4).

Diagnosis of ON: ACKNOWLEDGMENT


Tenderness (+) on GON or LON
Diagnostic block effect (+)
Rule out other causes of headache: The authors thank Dr. Young hee Baek for helpful advice on this
Tumor, vascular and congenital anomalies article, and Il Hee So, Eui Soo Shin, Yeon Mi Kim, and Eugene
Jung of Asan Medical Library for their efforts in data collection.

DISCLOSURE
Medical treatment, physical therapy, bio-feedback therapy,
The authors have no potential conflicts of interest to disclose.
or lifestyle modification

AUTHOR CONTRIBUTION

Conception and design: Jeon SR, Choi I. Acquisition of data:


Choi I. Analysis and interpretation of data: Choi I. Drafting the
Consider intervention therapy for medical refractory cases:
Corticosteroid or Botulinum toxin injection or PRF treatment article: Choi I. Critically revising the article: Jeon SR, Choi I. Re­
viewed submitted version of manuscript: Jeon SR, Choi I. Sta­
tistical analysis: Choi I. Administrative/technical/material sup­
port: Jeon SR. Study supervision: Jeon SR. Approval of the final
manuscript on behalf of all authors: Jeon SR.
Consider surgical treatment for intractable cases: occipital nerve
stimulation, neurolysis, or other destructive surgeries ORCID

Fig. 4. Treatment algorithm for ON. Il Choi  http://orcid.org/0000-0003-0120-6564

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