Abstract

Background: Cranial neuralgias are characterized by sharp lancinating pain that occurs on specific regions served by cranial nerves. Glossopharyngeal neuralgia (GPN) and trigeminal neuralgia (TN) are disorders for which etiology, epidemiology, and pain regions differ. On the other hand, the treatment stays the same as microvascular decompression, which serves as a safe approach for these entities.

Methods: We performed a specific search strategy using the following Boolean terms: (Combined hyperactive dysfunction syndrome) OR (“Trigeminal” AND “Glossopharyngeal” AND “neuralgia”) AND (“Concomitant” OR “Combined” OR “Simultaneous” OR “Concurrent” OR “at once” OR synchronously”). Including PubMed and Scopus, we found 73 results, of which 10 were included in the study. Furthermore, we did a manual search for one relevant study. The studies were included only if cases with concomitant TN and GPN were present.

Results: Fifteen cases were retrieved. The mean age of presentation was 66. About 53.3% of cases were left-sided; the most common etiology was posterior inferior cerebellar artery compression (73.3%). Microvascular decompression is this pathology’s most popular management approach (73.3%). Only two cases reported complications (13.3%).

Conclusion: The concomitant appearance of TN and GPN is scarce. The microvascular decompression persists as the most popular approach. There are a few treatment modalities used apart from surgical decompression. More research on these areas should be done to generate interest in their application.

Keywords: Concomitant, Glossopharyngeal, Neuralgia, Trigeminal

INTRODUCTION

Cranial neuralgias are conditions characterized by sudden, sharp pain in regions the cranial nerves serve. The most frequently encountered types are trigeminal and glossopharyngeal neuralgia (GPN), commonly linked to these nerves’ compression by nearby blood vessels. [ 4 ]

Trigeminal and GPNs are painful disorders that tend to appear individually. Trigeminal neuralgia (TN) is much more common in proportion compared with GPN, with only one patient with GPN for every 75 patients with TN. Studies indicate that an offending vessel is responsible for idiopathic TN in 96.9% of cases, while cases without a compressive factor have been observed in a range of 3.1–17%.[ 16 ] TN is associated with compression on the encephalic exit point, commonly with compression by the superior cerebellar artery (SCA). At the same time, the glossopharyngeal nerve (GNv) tends to be compressed by the posterior inferior cerebellar artery (PICA). In rare cases, the choroid plexus could elicit irritation of these nerves.[ 12 , 21 ]

The prevalence of TN is reported to be 4.7/100,000 people. TN is presented as episodes of lancinating, stabbing, or “electric shock” pain in the orofacial region.[ 9 ] Age is considered an important risk factor as occurrence is commonly in the sixth and seventh decades of life.[ 9 ] These symptoms could be triggered by quotidian behaviors such as shaving, brushing, or eating. GPN prevalence is reported as 0.8/100,000 people, much rarer than TN. It presents as pain in the posterior tongue, tonsils, throat, or external ear canal that could be triggered by swallowing, talking, coughing, or pharyngeal movements.[ 12 ] This case presents a concurrent TN and GPN.

The concomitant appearance of TN and GPN is uncommon, with only a few cases reported in the literature. While microvascular decompression (MVD) remains the primary treatment option, alternative therapeutic approaches have not been explored thoroughly. Due to the limited data and lack of a synthesis of existing evidence, conducting a scoping review is essential to systematically map the available research, identify gaps in knowledge, and establish a basis for future high-quality studies.

Illustrative case

A 59-year-old female patient with a medical history of hypertension managed with candesartan and hydrochlorothiazide and no known allergies presented with symptoms that began 10 years ago (2014). She initially experienced right mandibular electric-shock-like paroxysms, rated 4/10 on the numerical rating scale (NRS), lasting <10 s and occurring multiple times daily. The pain radiated to the right anterior auricular and right frontotemporal regions and was exacerbated by eating, brushing teeth, and talking. In addition, the pain was accompanied by phosphenes, tinnitus, and vertigo.

The patient received medical treatment, including pregabalin, initially 75 mg every 24 h, then increasing to 75 mg every 12 h, then 150 mg every 12 h, and eventually 150 mg every 8 h (as of 2023). Before seeking further medical attention, she consulted a dentist, suspecting dental pain, particularly from the lower alveolus. Despite undergoing endodontic treatment, her symptoms persisted, prompting a referral to our clinic. Two months before planning MVD surgery, the patient reported a new pain pattern. This included increasing episodes of the right neck and mandibular discomfort, along with constant right deep ear pain radiating to the right pharynx, rated 4/10 on the NRS. These symptoms were aggravated by eating, talking, and swallowing. There were no signs of bradycardia, syncopal episodes, or convulsions suggestive of vasovagal attacks.

A magnetic resonance imaging (MRI) scan using three dimensional fast imaging employing a steady-state acquisition sequence revealed compression of the trigeminal nerve (TNv) by the SCA and compression of the GNv [ Figure 1 ]. No additional abnormalities were noted in cranial nerve examinations, and there were no signs of Eagle’s syndrome. These clinical and imaging findings were consistent with concomitant trigeminal and GPN.

At this point, the patient was taking pregabalin (150 mg every 8 h), oxcarbazepine, buprenorphine patches, tramadol, and ketorolac, with no significant improvement. Subsequently, morphine was added, providing partial symptom relief.

Figure 1:

(a) Axial three dimensional fast imaging employing steady-state acquisition (3D FIESTA) magnetic resonance imaging (MRI) image reveals the right glossopharyngeal nerve (arrow) in contact with a loop formed by right posterior inferior cerebellar artery at supraolivary fossette. No abnormal vessel is seen near the left glossopharyngeal nerve. (b) Axial 3D FIESTA MRI demonstrates a close association between the right trigeminal nerve (indicated by the arrow) and the superior cerebellar artery at the nerve’s root entry zone.

On office evaluation, there were no remarkable findings on physical examination, and the oral cavity and larynx appeared normal. Given the chronic nature of her symptoms, limited response to conservative management, and MRI findings, neurosurgical decompression was planned as the definitive treatment [ Figure 2 ].

Figure 2:

(a) Intraoperative view showing the trigeminal nerve in direct contact with the arterial loop at the root entry zone, demonstrating the vascular compression responsible for trigeminal neuralgia. The nerve appears slightly distorted due to the close relationship with the vessel. (b) Post-decompression view showing a piece of Gore-Tex material carefully placed between the trigeminal nerve and the arterial vessel. The Gore-Tex acts as a protective barrier, effectively eliminating the vascular compression and restoring the nerve’s normal configuration. (c) Intraoperative view illustrating the glossopharyngeal nerve in close proximity to the arachnoid layer and posterior inferior cerebellar artery previously shown on the magnetic resonance imaging. The relationship highlights the potential for neurovascular conflict contributing to symptoms. (d) Post-decompression view demonstrating the careful separation of the arachnoid membrane from the glossopharyngeal nerve, effectively alleviating the neurovascular conflict. This precise dissection restores a clear anatomical distinction between the nerve and the vessel.

The procedure was performed without complications. Postoperatively, the patient reported immediate clinical improvement and was discharged after a 2-day hospital stay.

MATERIALS AND METHODS

We performed a specific search strategy using the following Boolean terms: (Combined hyperactive dysfunction syndrome) OR (“Trigeminal” AND “Glossopharyngeal” AND “neuralgia”) AND (“Concomitant” OR “Combined” OR “Simultaneous” OR “Concurrent” OR “at once” OR synchronously”). Including PubMed and Scopus, we found 73 results, of which 9 were included. Furthermore, we did a manual search in which we found 1 relevant study. The studies were included only if concomitant TN and GPN cases were present. Cases that additionally included hemifacial spasms were added.

The Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews (PRISMAScR) flow diagram was also followed in this process.[ 18 ] A duplicate screening was conducted, and when discrepancies arose between the reviewers, a third reviewer was involved to resolve the differences. This approach ensured a more rigorous and transparent selection process, minimizing bias and improving the reliability of the review’s findings. By following the PRISMA-ScR guidelines, the methodology adhered to established best practices, ensuring the systematic inclusion of relevant studies and maintaining the integrity of the review process [ Figure 3 ].

Figure 3:

Preferred reporting items for systematic reviews and meta-analyses extension for scoping reviews flow diagram.

RESULTS

This study identified 15 reported cases of concomitant trigeminal and GPN presentation. The gender distribution showed a slight female predominance, with 7 men (46.6%) versus 8 women (53.3%), with an average age of 66 years (range 56–82 years). Six (40%) cases defined as Hyperactive Dysfunction Syndrome with TN and GPN were included. Regarding laterality, 7 cases (46.6%) were right-sided, and 8 (53.3%) were left-sided [ Table 1 ].

Table 1:

Revised cases of concomitant trigeminal and glossopharyngeal neuralgia: Clinical characteristics, treatment modalities and outcomes.

The etiology of GPN was predominantly due to vascular compression by the PICA in 11 cases (73.3%), while 3 cases (20%) did not specify the vascular cause. One case (6.7%) was secondary to medullary infarction. On the other hand, TN presented a more varied distribution of compressive causes: six cases due to SCA compression (40%), two cases involving AICA (1 case simultaneous with vertebrobasilar system, and another of isolated AICA, both representing 13.3%), one case due to petrosal vein compression (6%), one by isolated vertebrobasilar system (6%), one by medullary infarction (6%, same case as the compression in the previously mentioned GN case), and 4 cases (26.6%) with other or unspecified causes.

The most frequently used treatment was MVD in 11 cases (73.3%, plus one case with decompression and radiosurgery for recurrence in the same patient), followed by 1 case of Gamma Knife radiosurgery only (6.6%), one case managed with clonazepam (6%), another case managed with acupuncture (6%), and another with glycerol hemolysis (6%). The results showed complete resolution in 12 cases (80%), significant improvement in 1 case (6.6%), and only 2 cases (13.3%) were refractory to treatment.

Two cases (13.3%) reported complications, consisting of lingual pain controlled with gabapentin and the sensation of deja vu on the side of the previous compression (corresponding to the case treated with acupuncture). Only two instances presented recurrence (13.3%).

DISCUSSION

Regarding the incidence of TN, American literature reports average rates of 4.5/100,000 people/year, contrasting with a higher incidence reported in European studies of 12.6– 27/100,000 people/year.[ 1 ]

We presented a rare case of concurrent TN and GPN. As we described, our case derived from vascular nerve compression and arachnoiditis [ Figure 1 ]. The TNv was decompressed and detached from the SCA, and GoreTex was placed between the two without complications. GNv has decompressed alone and detached from arachnoidal fibers.

This entity is infrequent, as only 0.3–0.5% of TN cases are presented concurrently. TN is predominant in females (3:2).[ 19 ]

Wang et al. suggest a subtle distinction between Hyperactive Dysfunction Syndrome (HDS) and the co-occurrence of trigeminal neuralgia (TN) and glossopharyngeal neuralgia (GPN), proposing that HDS typically shares a common etiology, often involving a tortuous vertebrobasilar artery.[ 19 ] In contrast, Cao et al. argue that this distinction lacks clinical significance, as TN and GPN may arise independently, implying that their coexistence does not necessarily indicate a shared pathophysiology.[ 2 ] Nonetheless, we included studies involving patients diagnosed with HDS, as this was not considered a limiting factor in the scope of our review on TN and GPN.

Diagnostic clinical criteria

GPN is a condition marked by a sudden, unilateral stabbing pain that starts and ends abruptly, affecting the GNv distribution and (in some cases) the auricular and pharyngeal branches of the vagus nerve. The pain occurs in the ear, at the base of the tongue, in the tonsillar fossa, or under the jaw angle. It is often triggered by swallowing, talking, or coughing. It can exhibit remissions and relapses like TN, as in our case, which lasted with remissions for about 10 years. The pain must be characterized as recurring paroxysmal attacks of unilateral pain in the distribution of the GNv lasting from a few seconds to 2 min of severe intensity and described as electric shock-like, shooting, stabbing, or sharp in quality. It must be precipitated by swallowing, coughing, talking, or yawning.[ 5 ]

Conversely, TN is a condition defined by repeated, brief, electric shock-like pains on one side, with sudden onset and end, confined to one or more branches of the TNv and triggered by harmless stimuli. It can arise without an apparent cause or as a secondary effect of another diagnosed condition; our case described how a possible secondary effect was examined by a dentist with no success. There may also be concurrent moderate continuous pain within the affected nerve branch distributions. The pain must be characterized by recurrent paroxysms of unilateral facial pain in the distributions of one or more divisions of the TNv, with no radiation beyond; it should last from seconds to 2 min, be severe in intensity, and be described as “shock-like,” shooting, stabbing, or sharp in quality. It also must be precipitated by innocuous stimuli within the affected trigeminal distribution.[ 6 ]

We must insist on following these diagnostic criteria due to the clinical nature of these disorders. It is imperative to be specific when performing a physical examination on a patient with suspected TN or GPN.

Sensory anatomy

The GNv sensory fibers arise within and below the jugular fossa. It is important to denote that these fibers do not decussate. The rootlets of the GNv exit the brainstem just cephalic to the rootlets of the vagus nerve along the upper one-third of the post-olivary sulcus. The nerve bundle transverses the cerebellopontine angle cistern and enters the jugular foramen through the glossopharyngeal meatus.[ 3 ]

The TNv is considered a motor and sensory nerve. These roots pass anteriorly through the high cerebellopontine angle cistern between the petrous bone and the middle cerebellar peduncle. Then, they cross the petrous apex and pass beneath the petroclinoid ligament to enter Meckel’s cave.[ 3 ]

Treatment

The management of TN and GPN is a clinical challenge due to the diagnosis’s complexity and treatment response variability. We describe a series of cases treated with various treatment modalities, which thrived in most cases. The first therapeutic option for both conditions is pharmacological, with carbamazepine being the preferred drug; other pharmacological regimens have also been described with oxcarbazepine, lamotrigine, baclofen, phenytoin, topiramate, sumatriptan, gabapentin, and pregabalin. However, many patients do not respond adequately to medical treatment, requiring surgical interventions.[ 15 ] When this fails, surgical modalities such as rhizotomy MVD are the preferred treatment modalities.

These interventions can be grouped into two main types: (1) destructive methods, such as percutaneous ablative techniques and radiosurgery, and (2) non-destructive approaches, including MVD.[ 15 ]

Three commonly employed percutaneous ablative techniques for managing medically refractory TN include radiofrequency rhizotomy, percutaneous retrogasserian glycerol rhizotomy, and balloon microcompression (BMC).[ 11 , 15 ] Wang et al. suggest a subtle distinction between hyperactive dysfunction syndrome (HDS) and the co-occurrence of trigeminal neuralgia (TN) and glossopharyngeal neuralgia (GPN), proposing that HDS typically shares a common etiology, often involving a tortuous vertebrobasilar artery.[ 19 ] In contrast, Cao et al. argue that this distinction lacks clinical significance, as TN and GPN may arise independently, implying that their coexistence does not necessarily indicate a shared pathophysiology.[ 2 ] Nonetheless, we included studies involving patients diagnosed with HDS, as this was not considered a limiting factor in the scope of our review on TN and GPN.

MVD is highly effective, especially in TN;[ 15 ] the success rate of MVD in achieving immediate relief is reported to exceed 80%, while recurrence rates vary, with estimates ranging from 3% to 30%.[ 16 ] GPN, being less common, has been less investigated, but surgical interventions, including MVD, have also shown promising results, with a high success rate and low incidence of complications.[ 15 ]

CONCLUSION

The concomitant appearance of GPN and TN is sporadic. Nevertheless, management tends to be straightforward, as microvascular retrosigmoid decompression is the go-to approach. New treatment options are available, but they are not as popular and well-studied as surgical management.

Ethical approval:

The Institutional Review Board approval is not required.

Declaration of patient consent:

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship:

Nil.

Conflicts of interest:

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation:

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

Acknowledgments

The authors would like to express their gratitude, specially to Dr. Rogelio Revuelta-Gutiérrez, M.D.; Jose Carlos Herrera-Castro, M.D.; Jesus Antonio Jacquez-Graff, M.D.; and Carlos Mauricio Antillón-Elias M.D.; for their valuable contributions to this work. Their insights, support, and dedication were instrumental in the development of this study.

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