Acta Neurochir (2015) 157:311–321 DOI 10.1007/s00701-014-2302-7
REVIEW ARTICLE - FUNCTIONAL
Vago-glossopharyngeal neuralgia: a literature review of neurosurgical experience Jianqing Chen & Marc Sindou
Received: 1 October 2014 / Accepted: 1 December 2014 / Published online: 21 December 2014 # Springer-Verlag Wien 2014
Abstract Glossopharyngeal neuralgia (GPN), or better named vago-glossopharyngeal neuralgia (VGPN), is a rare disorder amounting to 1 % of the incidence of trigeminal neuralgia (TN). Pain is paroxysmal, of the electrical shooting type, and mainly provoked by stimulation of the pharynx or deep throat, especially during swallowing. Due to its rarity, VGPN is often misdiagnosed. The front line of medical treatment is based on anticonvulsants. Surgery should be considered when the pain is refractory to medications. In most patients, the cause is neurovascular conflict on root entry zone (REZ) or midcistern portion, of the IXth and/or Xth cranial nerves. Compressive vessels can be evidenced by means of a high sensibility and a high specificity resolution MR imaging in most centers. Present consensus is that the first option of neurosurgical treatment be microvascular decompression. In patients with precarious general conditions, stereotactic radiosurgery may be considered. Also, thermo-rhizotomy at the pars nervosa of foramen jugularis or tractotomy-nucleotomy at brainstem may be alternatives, but these methods entail a significant risk of deficits. In this article, the authors reviewed the main literature series on neurosurgical treatments of this disease. Keywords Glossopharyngeal neuralgia . Glossopharyngeal nerve . Vagus nerve . Microvascular decompression . J. Chen Department of Neurosurgery, Hopital Renji, University of Jiaotong Shanghai, Building 7, 160 Pujian Road, 200127 Shanghai, China M. Sindou (*) Department of Neurosurgery A, Hopital neurologique Pierre Wertheimer, University of Lyon I, GHE, 59, Boulevard Pinel, 69003 Lyon, France e-mail: [email protected] M. Sindou e-mail: [email protected]
Thermo-rhizotomy . Trigeminal tractotomy-nucleotomy . Stereotactic radiosurgery . Neurovascular conflicts
Introduction Glossopharyngeal neuralgia (GPN) should be better named vago-glossopharyngeal neuralgia (VGPN), since the pain frequently affects not only the sensory territory corresponding to the glossopharyngeal nerve but also the one tributary of the sensory vagus nerve. VGPN consists of paroxysmal, transient, severe, sharp pain in the back of the throat, the base of the tongue, the tonsillar fossa, the depth of the ear canal, and the area beneath the angle of the jaw. It usually lasts seconds to minutes and is often precipitated by chewing, coughing, yawning, talking, and swallowing. VGPN may also be associated with cardiovascular manifestations [25, 31], and because of its association with cardiac dysrhythmias, some may be fatal. VGPN was first described by Weisenburg et al. in 1910 in a patient with a cerebellopontine angle tumor [88]. Sicard and Robineau reported the painful syndrome in the absence of defined pathology in 1920 and named it as essential velopharyngeal pain [74]. Harris introduced the term “glossopharyngeal neuralgia” in 1921 [27]. White and Sweet advocated naming this neuralgia vago-glossopharyngeal neuralgia due to the fact that the Xth nerve is often involved [89].
Epidemiology VGPN is a relatively rare disease; it represents about 0.2– 1.3 % of all facial pain syndromes [9]. Incidence is estimated at 1 % compared to the one of trigeminal neuralgia (TN). Because of the rarity of the disease, most reports are isolated,
1 1 1 1 1 1 4 3 2 129 2 3 3 2 12
2 1 12 6 3 3 8
Jefferson 1931 [34] Keith 1932 [40] Reichert 1933 [67]
Lillie et al. 1936 [48] Cuneo 1943 [12] Svien et al. 1957 [80] Bohm et al. 1962 [5] Laha et al. 1977 [44] Jannetta 1980 [33]
Rushton et al. 1981 [70] King 1987 [42] Fraioli et al. 1989 [20] Sindou et al. 1991 [77] Ferrante et al. 1995 [18] Taha et al. 1995 [81]
Ceylan et al. 1997 [8] Ozenci et al. 2003 [62] Lou et al. 2008 [49] Kandan et al. 2010 [35] Ma et al. 2010 [50] Martínez-González et al. 2011 [51] Zhang et al. 2014 [95]
NR not reported, T transient, P permanent
No of patients
55.5 51 NR 52.5 61.5 60 48.2
NR 29 62.3 66 63.5 42.7
15 33 37 48 59.3 30-69
34 46 31
Mean age (years)
0 0 0 0 0 0 0
7 0 0 0 0 0
0 0 0 0 0 0
0 0 0
Deaths (%)
2 NR 15 months 4 2 NR 9–39 months
NR 1.6 NR 3.5 6.5 10
6 weeks NR 0.5 5 3 NR
1.25 10 days 0.33
Mean follow-up (years)
Literature series with rhizotomy of IXth and/or Xth cranial nerves, from 1931 to 2014
Authors and year
Table 1
2 (100) 1 11 5 (83.3) 2 3 7
110 (85.3) 1 3 (100) 3 2 12 (100)
1 1 1 2 3 (100) 2 (100)
1 1 1
Total relief (%)
0 0 0
0 0
0 0
0 1 1 (16.7) 1 0 1
0 0
1
0 0 0 0
0 0 0
No relief (%)
13 (10) 0
0 0 0 2
0 0 0
Partial relief (%)
0
0
T. dysphagia 2 (16.7) T. hoarseness 1 (8.3) P. dysphagia 1 (8.3) T. cough 3 (25) Diminished gag reflux 2 (100) 0
0
0 Decreased palatal and gag reflex, 2 (100 %)
0
Complications (%)
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Acta Neurochir (2015) 157:311–321 Table 2
313
Literature series of percutaneous radiofrequency thermocoagulation of IXth or Xth from 1979 to 1991
Authors and year
No. of patients
Mean age (years)
Deaths (%)
Mean follow-up (years)
Total relief (%)
Lazorthes et al. 1979 [45] Isamat et al. 1981 [30] Salar et al. 1983 [71] Oris et al. 1983 [61]
1 3 1 1
45 57 NR 34
0 0 0 0
NR 1.6 0.6 NR
1 3 1 1
Giorgi et al. 1984 [24] Arias 1986 [2] Sindou et al. 1991 [77]
9 2 3
0 0 0
NR 1.5 3.5
6 2 2
54.7 60.5 66
Partial relief (%)
No relief (%)
Complications (%)
0
0
3
P. dysphagia 1 (11.1 %) 0 0
1
NR not reported, P permanent
and there is actually no available population data, therefore it is difficult to estimate the overall incidence of VGPN in the population. Katusic et al. reported a 39-year retrospective study from 1945 to 1984 on the population of Rochester, Minnesota, USA; it was found that the incidence of VGPN in this population was 0.7/100,000 population/year, 0.9 and 0.5 in men and in women, respectively [38]. VGPN is more common on the left side (left:right ratio of 3:2), TN being more common on the right (right:left ratio 5:3) [69].
Etiology VGPN may be idiopathic; in this type no demonstrable lesion can be found. Since the last decade, these cases are mainly attributed to a glossopharyngeal nerve compression caused by a vessel at the root entry zone (REZ) of the brainstem. VGPN may also be secondary, due to tumors or pathologies in the cerebellopontine angle, aneurysms, arachnoiditis, persistent hypoglossal artery or petrositis, etc. For the extracranial causes, there may be tumors in the oropharynx, elongated styloid process, ossification of the styloid ligament, tonsillitis, peritonsillar abscess, trauma, and even vertebral artery dissection [26], etc.
Table 3
Clinical presentation and diagnosis According to the International Headache Society, idiopathic VGPN is defined as a severe transient stabbing pain experienced in the ear, base of the tongue, tonsillar fossa, or beneath the angle of the jaw [28], and it often has a trigger point to induce the pain such as swallowing, eating, chewing, brushing teeth, etc. The diagnosis of VGPN is clinical; criteria are cited as follows [28, 56, 75]: A. At least three attacks of unilateral pain fulfilling criteria B and C B. Pain is located in the posterior part of the tongue, tonsillar fossa, pharynx, beneath the angle of the lower jaw and/or in the ear C. Pain has at least three of the following four characteristics: 1. recurring in paroxysmal attacks lasting from a few seconds to 2 minutes 2. severe intensity 3. shooting, stabbing, or sharp in quality 4. precipitated by swallowing, coughing, talking, or yawning D. No clinically evident neurological deficit E. Not better accounted for by another ICHD-3 diagnosis.
Literature series with trigeminal tractotomy-nucleotomy from 1961 to 1998
Authors and year
No. of patients
Mean age (years)
Deaths (%)
Mean follow-up (years)
Total relief (%)
Bues 1961 [7] Kunc 1965 [43] Kanpolat et al. 1998 [37]
7 6 6
NR 65 50.5
0 0 0
NR NR 49.5 m
7 (100) 6 (100) 6 (100)
NR not reported
Partial relief (%)
No relief (%)
Complications (%)
0 0
314
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Fig. 1 From left to right, high-resolution T2, TOF-angio, and T1 + gadolinium MRI sequences showing neurovascular conflict between IXth nerve (arrowhead) and posterior inferior cerebellar artery [PICA] (arrow)
MR imaging is recommended to rule out the tumor and a DSA angiography can be also associated for the complicated cases.
fails or drug intolerance, allergies, or side effects become a problem, surgical treatment should be taken into consideration.
Treatment
Craniotomy with rhizotomy or neurotomy
Management of VGPN includes medical and surgical treatments. The medical treatment is the same as for trigeminal neuralgia, mainly based on anticonvulsant medications [6, 17, 28, 73, 75]. As for TN, carbamazepine is the first option [4, 75, 82]; if insufficient or not tolerated, other anticonvulsants may be administered, namely diphenylhydantoin, oxcarbazepine, clobazam, sodium valproate, topiramate, gabapentin, pregabalin, lamotrigine, or levetiracetam [3, 10, 64, 86, 94]. Non-antiepileptic drugs [96], association of baclofen [21] may also be useful. When the medical treatment
In 1920, one of the first attempted procedures was the excranial section of the glossopharyngeal nerve; it was performed by Sicard and Robineau [74]. Then, in 1924, Adson et al. published their work on avulsion of the nerve at the jugular foramen via a high cervical approach [1]. However, on account of a high morbidity of these procedures and a high incidence of recurrence of pain, these methods were not propagated. It was Dandy in 1927 who gave the explanation of the low efficacy of the extracranial procedure and popularized the intracranial section of the glossopharyngeal nerve [13]. Table 1 includes the series of patients who underwent rhizotomy of the IXth and/or Xth cranial nerves.
Percutaneous radiofrequency thermocoagulation
Fig. 2 T2 high-resolution MRI sequence showing IXth nerve (arrowhead) compressed by vertebro-basilar artery ventrally (small arrow) and posterior inferior cerebellar artery vascular loop dorsally (large arrow)
Lazorthes and Verdie reported the first percutaneous radiofrequency(RF) thermocoagulation for VGPN in 1974 [45]. Compared to the procedure for trigeminal neuralgia, it is far more difficult to perform it on the glossopharyngeal nerve due to the location and the high risk of lesioning of the adjacent vessels, namely the jugular vein and the internal carotid artery. Because of the high incidence of side effects, such as a diminished gag reflex, dysphagia, and vocal cord paralysis, Tew recommended that this procedure be reserved for patients whose condition is secondary to cancer of the oropharynx or who are unable to tolerate intracranial procedures [83]. Table 2 includes the series of patients who underwent percutaneous RF-thermocoagulation of ninth and/ or tenth cranial nerves. Whereas craniotomy with posterior fossa exploration had a significant mortality rate in some series, with percutaneous RF rhizotomy reported mortality was none; however, complications were frequently noticed.
No. of patients
3 9 1 1 1 3 16 9 3 40
1 17 1 3 217
47
1 2 31
23
Laha et al. 1977 [44] Jannetta 1980 [33]
Murasawa et al. 1985 [55] Tsuboi et al. 1985 [84] Yoshioka et al. 1985 [93] Michelucci et al. 1986 [53] Wakiya et al. 1989 [85] Sindou et al. 1991 [77] Ferrante et al. 1995 [18]
Resnick et al. 1995 [68]
Platania et al. 1997 [65] Kondo et al. 1998 [41]
Nishikawa et al. 2000 [58] Matsushima et al. 2000 [52] Patel et al. 2002 [63]
Sampson et al. 2004 [72]
Ohyama et al. 2006 [60] Esaki et al. 2007 [16] Ferroli et al. 2009 [19]
Sindou et al. 2009 [76]
NR
61 NR 55.8
56.4
47 59.3 50.2
58 59.3
55
46 39 62 56.9 54.7 66 58.3
44.3 30-69
Mean age (years)
0
0 0 0
0
0 0 3 (5.8)
0 1 (5.9)
2 (5)
0 0 0 0 0 0 0
1 (33.3) 1 (11.1)
Deaths (%)
9
NR NR 7.5
12.7
NR 1.3 4
NR 11.6
4
0.5 1 1 1.8 2 3.5 2.2
0.7 NR
Mean follow-up (years)
Literature series with MVD of IXth and/or Xth cranial nerves from 1977 to 2014
Authors and year
Table 4
21 (91 %)
1 2 28 (90.3)
28 (96.5)
1 3 (100) 29 (58)
1 16 (94.1)
28 (76)
1 1 (100) 1 (100) 3 (100) 15 (93.7) 9 2
1 (50) 6 (75)
Total relief (%)
0
0
12 (24)
9 (18)
0 3 (9.7)
0
0
0
0
3 (8)
0 1
1 (6.3) 0 0 6 (15)
0
0 2 (25)
No relief (%)
0
1 (50)
Partial relief (%)
0 T. dysphonia/dysphagia 3 (9.7) T. hypoacousia 4 (12.9) T. VI/VII palsy 3 (9.6) CSFL 1(3.2) P.CN palsy 2 (8.7)
0 Brainstem infarction2 (0.9) CN palsy15 (6.9) CFL6 (2.8) Dysphagia (0.9) T. hoarseness/dysphagia 13 (28) T. facial paresis 3 (6) P. hoarseness/ dysphagia 4 (6) P. facial paresis 1 (2)
P. mild hoarseness 2 (11.8) T. coughing 2 (11.8)
P. paresis of IX and X 3 (8) T. paresis of IX and X 4 (10) Wound infection1 (2) T. conjunctivitis 1 (2) T. hypertension 2 (5)
0 T. decreased palatal and gag reflexes 2 (22.2) 0 0 0 T. headache 2 (66.6)
Complications (%)
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T. VIII deficit 5 (23.8) T. facial palsy 1 (4.7) 0 0
1 (5.5) NR
Also vasomotor disturbances occurring during the procedure were mentioned, leading to the termination of the procedure in some patients [61]. Literature data are summarized in Table 3.
2 (33)
1 (5.5)
1 (6.7)
0 0 1 (4.7)
4 (66) NR not reported, T transient, P permanent, CSFL cerebral spinal fluid leakage
NR 0 6 Wang et al. 2014 [87]
60.1
21 (100) 3.4 0 21 Xiong et al. 2012 [91]
50.4
4 (100) 16 (88.9) 7 (100) 2 NR NR 0 0 0 4 18 7 Ma et al. 2010 [50] Gaul et al. 2011 [23] Martínez-González et al. 2011 [51]
61.5 54.5 58
14 (93.3) 4 0 15 Kandan et al. 2010 [35]
52.5
1 (100) 20 (95.2) 63 6.5 0 0 1 14 Munch et al. 2009 [54] Kawashima et al. 2010 [39]
63 59.2
No relief (%) Partial relief (%) Total relief (%) Mean follow-up (years) Deaths (%) No. of patients
Mean age (years)
Fig. 3 Landmarks of keyhole (retromastoid, retrosigmoid, infrafloccular) approach for vagal and glossopharyngeal microvascular decompression (right side). M mastoid tip, T transverse and S sigmoid sinus, I skin incision, C keyhole craniectomy
Trigeminal tractotomy-nucleotomy
Authors and year
Table 4 (continued)
NR T. hoarseness/dysphagia 4 (28) P. hoarseness/dysphagia 2 (14) T. hoarseness/dysphagia 4 (28) P. hoarseness/dysphagia 2 (14) 0 T.IX/X CN deficit 6 (33.3)
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Complications (%)
316
Trigeminal tractotomy, defined by the destruction of the descending trigeminal tractus in the medulla, is a procedure first described by Olof Sjöqvist in 1938 [78]. The trigeminal tractus was cut at the inferior olive level via posterior fossa craniectomy. It was used to treat atypical pain syndromes such as glossopharyngeal, vagal or geniculate neuralgias, atypical facial pain, craniofacial cancer pain, postherpetic neuralgias, and atypical forms of trigeminal neuralgia [11, 29, 57]. Latter Kunc et al. applied this method to treat glossopharyngeal neuralgia in six patients in 1954 [43]. He used a simple light mechanical stimulation with a thin needle to localize the target fibers and called it “selective tractotomy”. More recently,
Fig. 4 Schematic drawing of infra- and latero-floccular exposure of the glossopharyngeal (IX) and vagus (X) rootlets in a patient affected with right VGPN due to vertebro-basilar (VB) and posterior inferior cerebellar (PICA) arteries, ventral to the nerves. Ch.Pl choroid plexus
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317
Fig. 5 Patient affected with a right vago-glossopharyngeal neuralgia due to a posterior inferior cerebellar artery (PICA); infra- and latero-floccular microsurgical approach on the right side. a The offending vessel PICA ventral to the root entry zone of the IXth (asterisk) and the Xth (triangle) nerves, note the atrophic and greyish aspect of the IXth, Xth rootlets
testifying of focal demyelination. b The compressive PICA (star), ventrocaudally to the IXth and Xth rootlets. c The dissection and freeing of the rootlets from the PICA loop. d The Teflon felt bundle (T) maintaining artery apart from REZ of IXth and Xth rootlets
Kanpolat et al. developed a CT-guided trigeminal tractotomy with more accuracy and safety to treat intractable facial pain and obtained good results [36, 37]. Literature data are summarized in Table 3.
Microvascular decompression Dandy [14] and Gardner [22] raised the theory that vascular compression of the root entry/exit zone of the cranial nerves
318
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might be at the origin of hyperactivity syndromes. In 1977, Jannetta further investigated this mechanism and published the first series of patients with GPN treated with microvascular decompression (MVD) [32]. Since then, MVD gained greater acceptance than the traditional rhizotomy procedure, and a number of series of MVD were published regarding its satisfactory efficacy. Modern MR imaging, associating highresolution 3D T2, 3D-TOF-angio, and 3D-T1+gadolinium with high sensibility and a high specificity to predict presence or not, type, topography, and degree of compression of cranial nerve [46]. Figures 1 and 2 show high-resolution MR imaging of a patient with VGPN. The review of literature includes 28 series and regroups 515 patients. Overall, total relief rate varied from 50 to 100 %. In the more recent series, total relief rate was most often higher than 90 %. Table 4 includes the series of patients who underwent MVD of the IXth and Xth nerve for VGPN. Compared to the percutaneous RF-thermocoagulation procedure, MVD of the vago-glossopharyngeal nerve complex has a lower recurrence rate. In a well-equipped center, a highly efficient team with a well-established neurosurgical tradition and the use of neurophysiological monitoring can achieve excellent results [19]. MVD provides a good outcome with a high rate of pain relief in 80-90 % of patients according to the main series [41, 63, 72, 76]. However, open surgical interventions essentially carry a risk of permanent nerve deficit, and are variable according to the published series as shown in Table 4. Figures 3, 4, and 5 show the MVD procedure.
Stereotactic radiosurgery Stereotactic radiosurgery (SRS) was intended to provide a solution for those who cannot tolerate or who refuse open intervention. In 2009, Dhople reported a study that has one of the longest median follow-up periods. The study shows that the gamma knife surgery (GKS) treatment remains a viable option despite a higher recurrence rate than with MVD (pain relief 84 % for MVD and 64 % for SRS after 1-year follow-
Table 5
up) [15]. The targets can be REZ or cistern segment or pars nervosa of jugular foramen (i.e., vago-glossopharyngeal meatus) [47] with a dose ranging from 60 to 80 Gy [66]. Literature data are summarized in Table 5.
Discussion and conclusions The present work, which aimed at collecting and summarizing the neurosurgical experience for VGP neuralgia, was harvested from the main publications since the early beginning to recent years. Due to the long period covered, comparisons between the various (evolving) methods used and authors’ reports are difficult, and clear-cut conclusions on surgical indications are somewhat hazardous. Further, in some series, the number of patients was small, the effects on pain and side effects not precisely detailed, and follow-ups, when mentioned, were short. However, in spite of many and important discrepancies between reports, we think the following can be admitted. The lesioning techniques, whatever the type: open (namely the sensory rhizotomies) or percutaneous (namely the RFthermocoaculation), produce inevitable sensory deficits responsible for dysphagia and hoarseness. The SRS procedures, which are coming in use for VGPN, might be valuable tools as the lesion-maker if long-term studies confirm lasting efficacy and innocuity. Tracto-nucleotomies are scarcely performed outside desperate situations; they entail serious potential neurological risks due to their location at the brainstem level. Because of its curative and conservative nature, MVD may be proposed as the first neurosurgical option. Consensus among neurosurgeons in most recent publications is that the most frequent cause of VGPN is a neuro-vascular conflict. The vascular compression can be diagnosed with a high sensibility and a high specificity, using high-resolution MR imaging sequences. However, the potentially risky nature of MVD implies that the surgery should be done within the frame of a well-experienced team.
Literature series with stereotactic radiosurgery of IXth and/or Xth cranial nerves, from 2005 to 2013
Authors and year
No. of patients
Mean age (years)
Deaths (%)
Mean follow-up (years)
Total relief (%)
Stieber et al. 2005 [79] Yomo et al. 2009 [92] Williams et al. 2010 [90] Pollock et al. 2011 [66] Leveque et al. 2011 [47] O’Connor et al. 2013 [59]
1 2 1 5 7 1
NR 45 47 61 62 99
0 0 0 0 0 0
6 months NR 11 months NR 1.5 1.3
1 (100) 1 (50) 1 (100) 3 (60) 5 (71) 1(100)
NR not reported
Partial relief (%)
No relief (%)
1 (50) 0 2 (29)
2 (60)
Complications (%)
0 0 0 0 0 0
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Whatever approach, VGPN should be recognized behind frequently atypical and sometimes misleading clinical presentations, as specific treatment can cure the disease.
Conflicts of interest None.
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321
Comments Vago-glossopharyngeal neuralgia is a disabling disease featured by sharp, stabbing, and severe painful attacks affecting the ear, tongue, tonsillar fossa, or beneath the angle of the jaw. Attacks are stereotyped in the individual patient and precipitated by swallowing, chewing, talking, coughing, and/or yawning. Pain can also be associated with hemodynamic instability resulting from reflexive autonomic outflow that can eventually lead to life-threatening syncopal episodes. It may remit and relapse like trigeminal neuralgia with which it is often confused. Following Dandy’s seminal article on the subject [1] published in 1927, the surgical treatment of glossopharyngeal neuralgia has consisted of sectioning the glossopharyngeal nerve proximal to its entrance into the jugular foramen. Fifty years later, Laha and Jannetta [2] advocated microvascular decompression (MVD) for the treatment of this disease. Long-term follow-up studies of MVD demonstrated sustained relief of pain in the majority of patients. Percutaneous thermorhizotomy has been used to treat a limited number of cases with promising results [3]. Radiosurgery is increasingly gaining a role in the treatment of this disabling disease, at least in a subgroup of patients with contraindication for open surgery and should be considered as the alternative treatment to MVD for the satisfactory results and negligible complication rates. This is a concise review article summarizing literature on the treatment of glossopharyngeal neuralgia. The literature is thoroughly reviewed and commented by recognized experts in the field. Alfredo Conti Messina, Italy 1. Dandy W (1927) Glossopharyngeal neuralgia (tic douloureux): its diagnosis and treatment. Arch Surg 15:198–214. 2. Laha RK, Jannetta PJ (1977) Glossopharyngeal neuralgia. J Neurosurg 47:316–20. 3. Giorgi C, Broggi G (1984) Surgical treatment of glossopharyngeal neuralgia and pain from cancer of the nasopharynx. J Neurosurg 61:952–55.
REVIEW ARTICLE - FUNCTIONAL
Vago-glossopharyngeal neuralgia: a literature review of neurosurgical experience Jianqing Chen & Marc Sindou
Received: 1 October 2014 / Accepted: 1 December 2014 / Published online: 21 December 2014 # Springer-Verlag Wien 2014
Abstract Glossopharyngeal neuralgia (GPN), or better named vago-glossopharyngeal neuralgia (VGPN), is a rare disorder amounting to 1 % of the incidence of trigeminal neuralgia (TN). Pain is paroxysmal, of the electrical shooting type, and mainly provoked by stimulation of the pharynx or deep throat, especially during swallowing. Due to its rarity, VGPN is often misdiagnosed. The front line of medical treatment is based on anticonvulsants. Surgery should be considered when the pain is refractory to medications. In most patients, the cause is neurovascular conflict on root entry zone (REZ) or midcistern portion, of the IXth and/or Xth cranial nerves. Compressive vessels can be evidenced by means of a high sensibility and a high specificity resolution MR imaging in most centers. Present consensus is that the first option of neurosurgical treatment be microvascular decompression. In patients with precarious general conditions, stereotactic radiosurgery may be considered. Also, thermo-rhizotomy at the pars nervosa of foramen jugularis or tractotomy-nucleotomy at brainstem may be alternatives, but these methods entail a significant risk of deficits. In this article, the authors reviewed the main literature series on neurosurgical treatments of this disease. Keywords Glossopharyngeal neuralgia . Glossopharyngeal nerve . Vagus nerve . Microvascular decompression . J. Chen Department of Neurosurgery, Hopital Renji, University of Jiaotong Shanghai, Building 7, 160 Pujian Road, 200127 Shanghai, China M. Sindou (*) Department of Neurosurgery A, Hopital neurologique Pierre Wertheimer, University of Lyon I, GHE, 59, Boulevard Pinel, 69003 Lyon, France e-mail: [email protected] M. Sindou e-mail: [email protected]
Thermo-rhizotomy . Trigeminal tractotomy-nucleotomy . Stereotactic radiosurgery . Neurovascular conflicts
Introduction Glossopharyngeal neuralgia (GPN) should be better named vago-glossopharyngeal neuralgia (VGPN), since the pain frequently affects not only the sensory territory corresponding to the glossopharyngeal nerve but also the one tributary of the sensory vagus nerve. VGPN consists of paroxysmal, transient, severe, sharp pain in the back of the throat, the base of the tongue, the tonsillar fossa, the depth of the ear canal, and the area beneath the angle of the jaw. It usually lasts seconds to minutes and is often precipitated by chewing, coughing, yawning, talking, and swallowing. VGPN may also be associated with cardiovascular manifestations [25, 31], and because of its association with cardiac dysrhythmias, some may be fatal. VGPN was first described by Weisenburg et al. in 1910 in a patient with a cerebellopontine angle tumor [88]. Sicard and Robineau reported the painful syndrome in the absence of defined pathology in 1920 and named it as essential velopharyngeal pain [74]. Harris introduced the term “glossopharyngeal neuralgia” in 1921 [27]. White and Sweet advocated naming this neuralgia vago-glossopharyngeal neuralgia due to the fact that the Xth nerve is often involved [89].
Epidemiology VGPN is a relatively rare disease; it represents about 0.2– 1.3 % of all facial pain syndromes [9]. Incidence is estimated at 1 % compared to the one of trigeminal neuralgia (TN). Because of the rarity of the disease, most reports are isolated,
1 1 1 1 1 1 4 3 2 129 2 3 3 2 12
2 1 12 6 3 3 8
Jefferson 1931 [34] Keith 1932 [40] Reichert 1933 [67]
Lillie et al. 1936 [48] Cuneo 1943 [12] Svien et al. 1957 [80] Bohm et al. 1962 [5] Laha et al. 1977 [44] Jannetta 1980 [33]
Rushton et al. 1981 [70] King 1987 [42] Fraioli et al. 1989 [20] Sindou et al. 1991 [77] Ferrante et al. 1995 [18] Taha et al. 1995 [81]
Ceylan et al. 1997 [8] Ozenci et al. 2003 [62] Lou et al. 2008 [49] Kandan et al. 2010 [35] Ma et al. 2010 [50] Martínez-González et al. 2011 [51] Zhang et al. 2014 [95]
NR not reported, T transient, P permanent
No of patients
55.5 51 NR 52.5 61.5 60 48.2
NR 29 62.3 66 63.5 42.7
15 33 37 48 59.3 30-69
34 46 31
Mean age (years)
0 0 0 0 0 0 0
7 0 0 0 0 0
0 0 0 0 0 0
0 0 0
Deaths (%)
2 NR 15 months 4 2 NR 9–39 months
NR 1.6 NR 3.5 6.5 10
6 weeks NR 0.5 5 3 NR
1.25 10 days 0.33
Mean follow-up (years)
Literature series with rhizotomy of IXth and/or Xth cranial nerves, from 1931 to 2014
Authors and year
Table 1
2 (100) 1 11 5 (83.3) 2 3 7
110 (85.3) 1 3 (100) 3 2 12 (100)
1 1 1 2 3 (100) 2 (100)
1 1 1
Total relief (%)
0 0 0
0 0
0 0
0 1 1 (16.7) 1 0 1
0 0
1
0 0 0 0
0 0 0
No relief (%)
13 (10) 0
0 0 0 2
0 0 0
Partial relief (%)
0
0
T. dysphagia 2 (16.7) T. hoarseness 1 (8.3) P. dysphagia 1 (8.3) T. cough 3 (25) Diminished gag reflux 2 (100) 0
0
0 Decreased palatal and gag reflex, 2 (100 %)
0
Complications (%)
312 Acta Neurochir (2015) 157:311–321
Acta Neurochir (2015) 157:311–321 Table 2
313
Literature series of percutaneous radiofrequency thermocoagulation of IXth or Xth from 1979 to 1991
Authors and year
No. of patients
Mean age (years)
Deaths (%)
Mean follow-up (years)
Total relief (%)
Lazorthes et al. 1979 [45] Isamat et al. 1981 [30] Salar et al. 1983 [71] Oris et al. 1983 [61]
1 3 1 1
45 57 NR 34
0 0 0 0
NR 1.6 0.6 NR
1 3 1 1
Giorgi et al. 1984 [24] Arias 1986 [2] Sindou et al. 1991 [77]
9 2 3
0 0 0
NR 1.5 3.5
6 2 2
54.7 60.5 66
Partial relief (%)
No relief (%)
Complications (%)
0
0
3
P. dysphagia 1 (11.1 %) 0 0
1
NR not reported, P permanent
and there is actually no available population data, therefore it is difficult to estimate the overall incidence of VGPN in the population. Katusic et al. reported a 39-year retrospective study from 1945 to 1984 on the population of Rochester, Minnesota, USA; it was found that the incidence of VGPN in this population was 0.7/100,000 population/year, 0.9 and 0.5 in men and in women, respectively [38]. VGPN is more common on the left side (left:right ratio of 3:2), TN being more common on the right (right:left ratio 5:3) [69].
Etiology VGPN may be idiopathic; in this type no demonstrable lesion can be found. Since the last decade, these cases are mainly attributed to a glossopharyngeal nerve compression caused by a vessel at the root entry zone (REZ) of the brainstem. VGPN may also be secondary, due to tumors or pathologies in the cerebellopontine angle, aneurysms, arachnoiditis, persistent hypoglossal artery or petrositis, etc. For the extracranial causes, there may be tumors in the oropharynx, elongated styloid process, ossification of the styloid ligament, tonsillitis, peritonsillar abscess, trauma, and even vertebral artery dissection [26], etc.
Table 3
Clinical presentation and diagnosis According to the International Headache Society, idiopathic VGPN is defined as a severe transient stabbing pain experienced in the ear, base of the tongue, tonsillar fossa, or beneath the angle of the jaw [28], and it often has a trigger point to induce the pain such as swallowing, eating, chewing, brushing teeth, etc. The diagnosis of VGPN is clinical; criteria are cited as follows [28, 56, 75]: A. At least three attacks of unilateral pain fulfilling criteria B and C B. Pain is located in the posterior part of the tongue, tonsillar fossa, pharynx, beneath the angle of the lower jaw and/or in the ear C. Pain has at least three of the following four characteristics: 1. recurring in paroxysmal attacks lasting from a few seconds to 2 minutes 2. severe intensity 3. shooting, stabbing, or sharp in quality 4. precipitated by swallowing, coughing, talking, or yawning D. No clinically evident neurological deficit E. Not better accounted for by another ICHD-3 diagnosis.
Literature series with trigeminal tractotomy-nucleotomy from 1961 to 1998
Authors and year
No. of patients
Mean age (years)
Deaths (%)
Mean follow-up (years)
Total relief (%)
Bues 1961 [7] Kunc 1965 [43] Kanpolat et al. 1998 [37]
7 6 6
NR 65 50.5
0 0 0
NR NR 49.5 m
7 (100) 6 (100) 6 (100)
NR not reported
Partial relief (%)
No relief (%)
Complications (%)
0 0
314
Acta Neurochir (2015) 157:311–321
Fig. 1 From left to right, high-resolution T2, TOF-angio, and T1 + gadolinium MRI sequences showing neurovascular conflict between IXth nerve (arrowhead) and posterior inferior cerebellar artery [PICA] (arrow)
MR imaging is recommended to rule out the tumor and a DSA angiography can be also associated for the complicated cases.
fails or drug intolerance, allergies, or side effects become a problem, surgical treatment should be taken into consideration.
Treatment
Craniotomy with rhizotomy or neurotomy
Management of VGPN includes medical and surgical treatments. The medical treatment is the same as for trigeminal neuralgia, mainly based on anticonvulsant medications [6, 17, 28, 73, 75]. As for TN, carbamazepine is the first option [4, 75, 82]; if insufficient or not tolerated, other anticonvulsants may be administered, namely diphenylhydantoin, oxcarbazepine, clobazam, sodium valproate, topiramate, gabapentin, pregabalin, lamotrigine, or levetiracetam [3, 10, 64, 86, 94]. Non-antiepileptic drugs [96], association of baclofen [21] may also be useful. When the medical treatment
In 1920, one of the first attempted procedures was the excranial section of the glossopharyngeal nerve; it was performed by Sicard and Robineau [74]. Then, in 1924, Adson et al. published their work on avulsion of the nerve at the jugular foramen via a high cervical approach [1]. However, on account of a high morbidity of these procedures and a high incidence of recurrence of pain, these methods were not propagated. It was Dandy in 1927 who gave the explanation of the low efficacy of the extracranial procedure and popularized the intracranial section of the glossopharyngeal nerve [13]. Table 1 includes the series of patients who underwent rhizotomy of the IXth and/or Xth cranial nerves.
Percutaneous radiofrequency thermocoagulation
Fig. 2 T2 high-resolution MRI sequence showing IXth nerve (arrowhead) compressed by vertebro-basilar artery ventrally (small arrow) and posterior inferior cerebellar artery vascular loop dorsally (large arrow)
Lazorthes and Verdie reported the first percutaneous radiofrequency(RF) thermocoagulation for VGPN in 1974 [45]. Compared to the procedure for trigeminal neuralgia, it is far more difficult to perform it on the glossopharyngeal nerve due to the location and the high risk of lesioning of the adjacent vessels, namely the jugular vein and the internal carotid artery. Because of the high incidence of side effects, such as a diminished gag reflex, dysphagia, and vocal cord paralysis, Tew recommended that this procedure be reserved for patients whose condition is secondary to cancer of the oropharynx or who are unable to tolerate intracranial procedures [83]. Table 2 includes the series of patients who underwent percutaneous RF-thermocoagulation of ninth and/ or tenth cranial nerves. Whereas craniotomy with posterior fossa exploration had a significant mortality rate in some series, with percutaneous RF rhizotomy reported mortality was none; however, complications were frequently noticed.
No. of patients
3 9 1 1 1 3 16 9 3 40
1 17 1 3 217
47
1 2 31
23
Laha et al. 1977 [44] Jannetta 1980 [33]
Murasawa et al. 1985 [55] Tsuboi et al. 1985 [84] Yoshioka et al. 1985 [93] Michelucci et al. 1986 [53] Wakiya et al. 1989 [85] Sindou et al. 1991 [77] Ferrante et al. 1995 [18]
Resnick et al. 1995 [68]
Platania et al. 1997 [65] Kondo et al. 1998 [41]
Nishikawa et al. 2000 [58] Matsushima et al. 2000 [52] Patel et al. 2002 [63]
Sampson et al. 2004 [72]
Ohyama et al. 2006 [60] Esaki et al. 2007 [16] Ferroli et al. 2009 [19]
Sindou et al. 2009 [76]
NR
61 NR 55.8
56.4
47 59.3 50.2
58 59.3
55
46 39 62 56.9 54.7 66 58.3
44.3 30-69
Mean age (years)
0
0 0 0
0
0 0 3 (5.8)
0 1 (5.9)
2 (5)
0 0 0 0 0 0 0
1 (33.3) 1 (11.1)
Deaths (%)
9
NR NR 7.5
12.7
NR 1.3 4
NR 11.6
4
0.5 1 1 1.8 2 3.5 2.2
0.7 NR
Mean follow-up (years)
Literature series with MVD of IXth and/or Xth cranial nerves from 1977 to 2014
Authors and year
Table 4
21 (91 %)
1 2 28 (90.3)
28 (96.5)
1 3 (100) 29 (58)
1 16 (94.1)
28 (76)
1 1 (100) 1 (100) 3 (100) 15 (93.7) 9 2
1 (50) 6 (75)
Total relief (%)
0
0
12 (24)
9 (18)
0 3 (9.7)
0
0
0
0
3 (8)
0 1
1 (6.3) 0 0 6 (15)
0
0 2 (25)
No relief (%)
0
1 (50)
Partial relief (%)
0 T. dysphonia/dysphagia 3 (9.7) T. hypoacousia 4 (12.9) T. VI/VII palsy 3 (9.6) CSFL 1(3.2) P.CN palsy 2 (8.7)
0 Brainstem infarction2 (0.9) CN palsy15 (6.9) CFL6 (2.8) Dysphagia (0.9) T. hoarseness/dysphagia 13 (28) T. facial paresis 3 (6) P. hoarseness/ dysphagia 4 (6) P. facial paresis 1 (2)
P. mild hoarseness 2 (11.8) T. coughing 2 (11.8)
P. paresis of IX and X 3 (8) T. paresis of IX and X 4 (10) Wound infection1 (2) T. conjunctivitis 1 (2) T. hypertension 2 (5)
0 T. decreased palatal and gag reflexes 2 (22.2) 0 0 0 T. headache 2 (66.6)
Complications (%)
Acta Neurochir (2015) 157:311–321 315
T. VIII deficit 5 (23.8) T. facial palsy 1 (4.7) 0 0
1 (5.5) NR
Also vasomotor disturbances occurring during the procedure were mentioned, leading to the termination of the procedure in some patients [61]. Literature data are summarized in Table 3.
2 (33)
1 (5.5)
1 (6.7)
0 0 1 (4.7)
4 (66) NR not reported, T transient, P permanent, CSFL cerebral spinal fluid leakage
NR 0 6 Wang et al. 2014 [87]
60.1
21 (100) 3.4 0 21 Xiong et al. 2012 [91]
50.4
4 (100) 16 (88.9) 7 (100) 2 NR NR 0 0 0 4 18 7 Ma et al. 2010 [50] Gaul et al. 2011 [23] Martínez-González et al. 2011 [51]
61.5 54.5 58
14 (93.3) 4 0 15 Kandan et al. 2010 [35]
52.5
1 (100) 20 (95.2) 63 6.5 0 0 1 14 Munch et al. 2009 [54] Kawashima et al. 2010 [39]
63 59.2
No relief (%) Partial relief (%) Total relief (%) Mean follow-up (years) Deaths (%) No. of patients
Mean age (years)
Fig. 3 Landmarks of keyhole (retromastoid, retrosigmoid, infrafloccular) approach for vagal and glossopharyngeal microvascular decompression (right side). M mastoid tip, T transverse and S sigmoid sinus, I skin incision, C keyhole craniectomy
Trigeminal tractotomy-nucleotomy
Authors and year
Table 4 (continued)
NR T. hoarseness/dysphagia 4 (28) P. hoarseness/dysphagia 2 (14) T. hoarseness/dysphagia 4 (28) P. hoarseness/dysphagia 2 (14) 0 T.IX/X CN deficit 6 (33.3)
Acta Neurochir (2015) 157:311–321
Complications (%)
316
Trigeminal tractotomy, defined by the destruction of the descending trigeminal tractus in the medulla, is a procedure first described by Olof Sjöqvist in 1938 [78]. The trigeminal tractus was cut at the inferior olive level via posterior fossa craniectomy. It was used to treat atypical pain syndromes such as glossopharyngeal, vagal or geniculate neuralgias, atypical facial pain, craniofacial cancer pain, postherpetic neuralgias, and atypical forms of trigeminal neuralgia [11, 29, 57]. Latter Kunc et al. applied this method to treat glossopharyngeal neuralgia in six patients in 1954 [43]. He used a simple light mechanical stimulation with a thin needle to localize the target fibers and called it “selective tractotomy”. More recently,
Fig. 4 Schematic drawing of infra- and latero-floccular exposure of the glossopharyngeal (IX) and vagus (X) rootlets in a patient affected with right VGPN due to vertebro-basilar (VB) and posterior inferior cerebellar (PICA) arteries, ventral to the nerves. Ch.Pl choroid plexus
Acta Neurochir (2015) 157:311–321
317
Fig. 5 Patient affected with a right vago-glossopharyngeal neuralgia due to a posterior inferior cerebellar artery (PICA); infra- and latero-floccular microsurgical approach on the right side. a The offending vessel PICA ventral to the root entry zone of the IXth (asterisk) and the Xth (triangle) nerves, note the atrophic and greyish aspect of the IXth, Xth rootlets
testifying of focal demyelination. b The compressive PICA (star), ventrocaudally to the IXth and Xth rootlets. c The dissection and freeing of the rootlets from the PICA loop. d The Teflon felt bundle (T) maintaining artery apart from REZ of IXth and Xth rootlets
Kanpolat et al. developed a CT-guided trigeminal tractotomy with more accuracy and safety to treat intractable facial pain and obtained good results [36, 37]. Literature data are summarized in Table 3.
Microvascular decompression Dandy [14] and Gardner [22] raised the theory that vascular compression of the root entry/exit zone of the cranial nerves
318
Acta Neurochir (2015) 157:311–321
might be at the origin of hyperactivity syndromes. In 1977, Jannetta further investigated this mechanism and published the first series of patients with GPN treated with microvascular decompression (MVD) [32]. Since then, MVD gained greater acceptance than the traditional rhizotomy procedure, and a number of series of MVD were published regarding its satisfactory efficacy. Modern MR imaging, associating highresolution 3D T2, 3D-TOF-angio, and 3D-T1+gadolinium with high sensibility and a high specificity to predict presence or not, type, topography, and degree of compression of cranial nerve [46]. Figures 1 and 2 show high-resolution MR imaging of a patient with VGPN. The review of literature includes 28 series and regroups 515 patients. Overall, total relief rate varied from 50 to 100 %. In the more recent series, total relief rate was most often higher than 90 %. Table 4 includes the series of patients who underwent MVD of the IXth and Xth nerve for VGPN. Compared to the percutaneous RF-thermocoagulation procedure, MVD of the vago-glossopharyngeal nerve complex has a lower recurrence rate. In a well-equipped center, a highly efficient team with a well-established neurosurgical tradition and the use of neurophysiological monitoring can achieve excellent results [19]. MVD provides a good outcome with a high rate of pain relief in 80-90 % of patients according to the main series [41, 63, 72, 76]. However, open surgical interventions essentially carry a risk of permanent nerve deficit, and are variable according to the published series as shown in Table 4. Figures 3, 4, and 5 show the MVD procedure.
Stereotactic radiosurgery Stereotactic radiosurgery (SRS) was intended to provide a solution for those who cannot tolerate or who refuse open intervention. In 2009, Dhople reported a study that has one of the longest median follow-up periods. The study shows that the gamma knife surgery (GKS) treatment remains a viable option despite a higher recurrence rate than with MVD (pain relief 84 % for MVD and 64 % for SRS after 1-year follow-
Table 5
up) [15]. The targets can be REZ or cistern segment or pars nervosa of jugular foramen (i.e., vago-glossopharyngeal meatus) [47] with a dose ranging from 60 to 80 Gy [66]. Literature data are summarized in Table 5.
Discussion and conclusions The present work, which aimed at collecting and summarizing the neurosurgical experience for VGP neuralgia, was harvested from the main publications since the early beginning to recent years. Due to the long period covered, comparisons between the various (evolving) methods used and authors’ reports are difficult, and clear-cut conclusions on surgical indications are somewhat hazardous. Further, in some series, the number of patients was small, the effects on pain and side effects not precisely detailed, and follow-ups, when mentioned, were short. However, in spite of many and important discrepancies between reports, we think the following can be admitted. The lesioning techniques, whatever the type: open (namely the sensory rhizotomies) or percutaneous (namely the RFthermocoaculation), produce inevitable sensory deficits responsible for dysphagia and hoarseness. The SRS procedures, which are coming in use for VGPN, might be valuable tools as the lesion-maker if long-term studies confirm lasting efficacy and innocuity. Tracto-nucleotomies are scarcely performed outside desperate situations; they entail serious potential neurological risks due to their location at the brainstem level. Because of its curative and conservative nature, MVD may be proposed as the first neurosurgical option. Consensus among neurosurgeons in most recent publications is that the most frequent cause of VGPN is a neuro-vascular conflict. The vascular compression can be diagnosed with a high sensibility and a high specificity, using high-resolution MR imaging sequences. However, the potentially risky nature of MVD implies that the surgery should be done within the frame of a well-experienced team.
Literature series with stereotactic radiosurgery of IXth and/or Xth cranial nerves, from 2005 to 2013
Authors and year
No. of patients
Mean age (years)
Deaths (%)
Mean follow-up (years)
Total relief (%)
Stieber et al. 2005 [79] Yomo et al. 2009 [92] Williams et al. 2010 [90] Pollock et al. 2011 [66] Leveque et al. 2011 [47] O’Connor et al. 2013 [59]
1 2 1 5 7 1
NR 45 47 61 62 99
0 0 0 0 0 0
6 months NR 11 months NR 1.5 1.3
1 (100) 1 (50) 1 (100) 3 (60) 5 (71) 1(100)
NR not reported
Partial relief (%)
No relief (%)
1 (50) 0 2 (29)
2 (60)
Complications (%)
0 0 0 0 0 0
Acta Neurochir (2015) 157:311–321
Whatever approach, VGPN should be recognized behind frequently atypical and sometimes misleading clinical presentations, as specific treatment can cure the disease.
Conflicts of interest None.
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Comments Vago-glossopharyngeal neuralgia is a disabling disease featured by sharp, stabbing, and severe painful attacks affecting the ear, tongue, tonsillar fossa, or beneath the angle of the jaw. Attacks are stereotyped in the individual patient and precipitated by swallowing, chewing, talking, coughing, and/or yawning. Pain can also be associated with hemodynamic instability resulting from reflexive autonomic outflow that can eventually lead to life-threatening syncopal episodes. It may remit and relapse like trigeminal neuralgia with which it is often confused. Following Dandy’s seminal article on the subject [1] published in 1927, the surgical treatment of glossopharyngeal neuralgia has consisted of sectioning the glossopharyngeal nerve proximal to its entrance into the jugular foramen. Fifty years later, Laha and Jannetta [2] advocated microvascular decompression (MVD) for the treatment of this disease. Long-term follow-up studies of MVD demonstrated sustained relief of pain in the majority of patients. Percutaneous thermorhizotomy has been used to treat a limited number of cases with promising results [3]. Radiosurgery is increasingly gaining a role in the treatment of this disabling disease, at least in a subgroup of patients with contraindication for open surgery and should be considered as the alternative treatment to MVD for the satisfactory results and negligible complication rates. This is a concise review article summarizing literature on the treatment of glossopharyngeal neuralgia. The literature is thoroughly reviewed and commented by recognized experts in the field. Alfredo Conti Messina, Italy 1. Dandy W (1927) Glossopharyngeal neuralgia (tic douloureux): its diagnosis and treatment. Arch Surg 15:198–214. 2. Laha RK, Jannetta PJ (1977) Glossopharyngeal neuralgia. J Neurosurg 47:316–20. 3. Giorgi C, Broggi G (1984) Surgical treatment of glossopharyngeal neuralgia and pain from cancer of the nasopharynx. J Neurosurg 61:952–55.
