Glossopharyngeal and Vagoglossopharyngeal neuralgia

Ross Hauser, MD

In this article we will discuss diagnosis, symptoms and possible treatments for Glossopharyngeal and Vagoglossopharyngeal neuralgia. In describing the possible symptoms of this disorder, we find it helpful to share some of the experiences of people who have reached out to us. Below are some of their emails. The emails have been edited for clarity.

Pain and voice change after a virus

I’ve been diagnosed with Postviral vagal neuropathy, Glossopharyngeal Neuralgia and muscle tension dysphonia (A change in the sound of voice caused by tension on the vocal chords, voice box, and muscles of the larynx). I’ve seen probably 50 specialists and nobody can really give me a proper diagnosis or tell me where the pain is stemming from. I’ve had so many tests done and they’re all non-conclusive.

Difficulty swallowing

I began to develop a decreased appetite and difficulty swallowing. Then I woke up one morning with nausea and violent vomiting of stomach acid and bile. During the dry heaving and coughing, I felt a sharp electric shock run up my throat and the nape of my neck. In that moment, it also felt like part of the automatic function had turned off. My swallowing muscles now feel weakened and gag reflex diminished.

So far, all of the bloodwork and diagnostic imaging for physical abnormalities have come back normal. I think there could be an emotional/mental component too. I was under a lot of stress and anxiety when this developed. I had a decreased appetite and trouble swallowing. Then there was the vomiting episode, which came a few days after a triggering event. Maybe this was just my body’s way of reacting to these emotional stressors. It’s like my sympathetic nervous system (fight-or-flight) became overly-engaged, to the point where it felt like I physically lost some sensation of these automatic functions (swallowing, gagging, yawning). And now my body has memorized that “feeling”. The muscles in my throat feel both tense and weak when I swallow, not fluid and effortless like before. The muscles in the back of my throat don’t relax when I yawn.

The common factor of symptoms in the vagus nerve.

The caption of the image below reads: The closeness of the Glossopharyngeal Cranial Nerve IX, the Vagus Cranial Nerve X, and the spinal accessory Cranial Nerve XI. The Glossopharyngeal Cranial Nerve IX, the Vagus Cranial Nerve X, and the spinal accessory Cranial Nerve XI run together just in front of the C1 and C2 vertebrae. They can be easily damaged by the excessive motions of these vertebrae caused by upper cervical instability.

In our two stories above, symptoms are described which can lead to a suspicion of Vagoglossopharyngeal neuralgia. The vagus nerve is mostly tied to the glossopharyngeal nerve, which is its traveling buddy as they go down the carotid sheath and innverate the pharynx (the area behind the nose and the mouth) and esophagus. The vagus nerve and  glossopharyngeal nerve are so tied together that when a person has horrific lancinating or lightning-like pharyngeal pain, which can make it hard to swallow and have associated nausea and vomiting, the condition is called is vagoglossopharyngeal neuralgia. The attacks of pain are brought on by swallowing, talking, coughing, sneezing, touching the tragus of the ear, turning the head towards the side of the pain, and rolling towards the side of the pain. It is important to remember, the vagus nerve has sensory functions and everywhere the vagus nerves goes can cause pain.  

Vascular compression as a cause of Vagoglossopharyngeal neuralgia

The caption of this image reads: Kinking of the carotid sheath at the craniocervical; junction. The carotid sheath contents include the internal carotid artery, internal jugular vein, as well as the vagus, glossopharyngeal, and spinal accessory nerves. They can be compressed as they make a sharp turn up to 90 degrees, a right angle around the C1 (atlas) transverse process (the white arrow at the center of the screen) into the brain through the jugular and carotid foramens. This leads to increased intracranial pressure, localized CSF accumulation and resultant cerebral atrophy and increased eye pressure (double arrows).

A case history presented in the Asian journal of neurosurgery

We are going to look at a case history presented in the Asian journal of neurosurgery (1). The doctors here offer and introduction to understanding the problem created by vascular compression.

Vascular compression has been reported to be the most common reason for Vagoglossopharyngeal neuralgia. The treatment may include medications, ganglion blockade with a radiofrequency ablation, and microvascular decompression. A review of the literature reveals that Vagoglossopharyngeal neuralgia may develop due to choroid plexus compression, and the number of reported cases is very limited. Secondary reasons could include neoplasms, vascular malformations, demyelinating diseases (multiple sclerosis), infection, trauma, Chiari malformation, Eagle’s syndrome, and choroid plexus overgrowth.

What are choroid plexus?

In the July 2021 update of the medical publication STATPearls (2) housed at the National Library of Medicine they vital roles of the choroid plexus is given. The choroid plexus is a network of blood vessels called capillaries. One of the primary functions is to produce cerebrospinal fluid (CSF). Among problems reported with a deficiency of cerebrospinal fluid production are the symptoms of: Sleep disturbance, head buzzing or noise, tinnitus, nausea or vomiting, dizziness, anxiety or depression, vertigo, and others. The challenges of diagnosis are seen in the many overlapping symptoms that can be caused by a compression caused cervical spine instability. For a detailed discussion, please see my article: Symptoms and conditions of Craniocervical and Cervical Instability.

Returning to the case history in the Asian journal of neurosurgery:

“A 64-year-old female patient had been diagnosed with left Vagoglossopharyngeal neuralgia four years before her admission to (the study) clinic and had been treated with carbamazepine, and gabapentin, which are effective on neuropathic pain. Ganglion blockade with radiofrequency ablation (RFA) had been used twice; as a result, the patient had benefited from these procedures for a short time. However, on her admission, she had an electricity-shock-like intolerable pain beginning from the left ear and extending to the larynx. She stated that talking and chewing triggered the pain.”

A point the researchers stressed was “no other characteristics of pain caused by choroid plexus compression were described by our patient compared to typical pain described by Vagoglossopharyngeal patients.” In other words, there was no clue that choroid plexus compression should be considered the primary diagnosis. Finally, her cranial magnetic resonance imaging (MRI) revealed a suspected compression. The patient underwent an microvascular decompression operation and her pain relieved in postoperative period.”

Cardiac-like and Autonomic symptoms

When Glossopharyngeal neuralgia is suspected and the patient also presents cardiac-like and autonomic manifestations – a condition termed vagoglossopharyngeal neuralgia is used as a diagnosis.

First let’s look at autonomic manifestations. In my article Neurologic, digestive, cardiac, and bladder disorders: Some of the symptoms of Autonomic nervous system dysfunction and treatment options, I explain some of these symptom manifestations. I will briefly review them here.

Autonomic manifestations are among the same symptoms described above but perhaps caused by other problems. Some people will have a diagnosis of dysautonomia because of symptoms of blood pressure and heart rate (See my article Can cervical spine instability cause cardiovascular-like attacks, heart palpitations and blood pressure problems? Some will have a concurrent diagnosis of Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorders. Some will also have Lyme Disease and/or thyroid problems. Others will tell us that they pass out for no reason or that their heart starts beating uncontrollably. Food gets stuck in their throat. The list of symptoms some people display seemingly goes on and on. Burning mouth syndrome, digestive disorders, Chronic Fatigue Syndrome, headaches, and migraines.

Treatment options

In December 2020, (3) doctors at the Department of Neurosurgery, University of Pennsylvania and the Department of Neurosurgery, Rowan School of Osteopathic Medicine presented two case histories of surgical relief of vagoglossopharyngeal neuralgia symptoms.

They write: Conservative treatment for vagoglossopharyngeal neuralgia includes antiepileptic medications. Surgical treatments include trigeminal tractotomy-nucleotomy, Gamma Knife® stereotactic radiosurgery, radiofrequency thermocoagulation, rhizotomy, and, as shown in this study, endoscopic microvascular decompression.

The doctors of this article present surgical two cases.

Case 1 demonstrates a 53-year-old male with right-sided Glossopharyngeal neuralgia symptoms that began to experience fainting or passing out episodes 10-years after the initial presentation.

Case 2 presents a 61-year-old female with a history of Ehlers-Danlos syndrome, and the malignant vasovagal syndrome that became associated with painful, shooting left anterior neck spasms consistent with Glossopharyngeal neuralgia.

Both patients underwent endoscopic microvascular decompression, leading to complete relief of neuralgia and cardiac symptoms.

Long-term complications occurred much more frequently following vagoglossopharyngeal rhizotomy

A December 2020 report in the journal Neurosurgery (4) compared surgical techniques for glossopharyngeal neuralgia. They note that Microvascular decompression and vagoglossopharyngeal rhizotomy are effective treatment for glossopharyngeal neuralgia. However, “surgical choice is controversial due to the need to maximize pain relief and reduce complications.”

In this study:

• Of 87 patients with glossopharyngeal neuralgia, 63 underwent Microvascular decompression alone, 20 underwent vagoglossopharyngeal rhizotomy alone, and 4 underwent vagoglossopharyngeal rhizotomy following a failed Microvascular decompression. The long-term rate of pain relief was slightly, but not significantly, lower following Microvascular decompression than vagoglossopharyngeal rhizotomy. However, long-term complications occurred much more frequently following vagoglossopharyngeal rhizotomy. The  Brief Pain Inventory-Facial (BPI-Facial) questionnaire, which evaluates pain and complications, showed that Microvascular decompression had better postoperative quality of life than vagoglossopharyngeal rhizotomy. However, 91.7% of patients who underwent vagoglossopharyngeal rhizotomy experienced no or mild complications. There was no significant difference in the overall satisfaction rates between the groups (83.3% vs 83.6%).

A case of cardiovascular, respiratory and digestive systems symptoms

French physicians published a case history to demonstrate the glossopharyngea and vagus nerve component of  digestive symptoms in vagoglossopharyngeal neuralgia. They write:

Vagoglossopharyngeal neuralgia is a rare pathology whose atypical forms, dominated by syncopal (fainting and passing out) manifestations, are still rarer. Although the territory of the vagus nerve involves, beyond the cardiovascular system, the respiratory and the digestive systems, there is no report in literature of atypical forms other than syncopal. (The cardiovascular aspect).

What can be implied here is that IF Vagoglossopharyngeal neuralgia can be suspected from cardiovascular like problems, why then don’t digestive problems lead to the same conclusion? Why is this connection not made?

In this case history, the doctors reported the case of a patient whose vagoglossopharyngeal neuralgia was predominantly revealed by digestive symptoms.

A 58-year-old patient presented with stereotypical severe digestive disturbances including nausea, vomiting and diarrhea. High definition cranial MRI showed a neurovascular conflict (compression) between the posterior inferior cerebellar artery and the glossopharyngeal nerve and the vagus nerve, on the right side. A microsurgical decompression was carried out which confirmed the vascular compression and successful transposition (moving) of the artery (from the nerves) One year after the surgery, the patient was free from all painful and digestive symptoms. . . The hypothesis was that the revealing digestive symptoms are linked to a similar parasympathetic mechanism, implying the compression of the vagus nerve.

Upper cervical instability, by causing a decrease in neurologic function of the lower part of the brainstem can affect the nerves.

(Video at 2:23 ) A connection to constant nausea

• Interesting too is that the nausea center is right in the lower part of the brainstem.  It is amazing how many people feel nauseated all the time and nobody can tell what’s causing it. These patients have a history of seeing gastroenterologists and various gastrointestinal doctors and they would have no idea that the cause of this chronic nausea can be upper cervical instability.

(Video at 2:45 ) Heart palpitations and arrhythmias

• The lower part of the brainstem is what controls heart rate and blood pressure.

(Video at 3:35) Respiratory rate

• The lower part of the brainstem also affects the respiratory rate. There are a lot of people that all of a sudden feel panicky, on the verge of panic attacks.  They start breathing really hard or they have shortness of breath and they don’t know why. It could be that upper cervical instability is decreasing the function of the brainstem.

The Curve of the Cervical Spine

(Video at 4:05) The cervical curve

Treatment Prolotherapy

The clinical signs of upper cervical instability can vary from no symptoms or relatively diffuse complaints to signs and symptoms of extreme importance or seriousness. When clinical symptoms are present, assessing the degree of instability by objective means including digital motion x-ray helps in determining the treatment course. Destabilization of C0-C1 or C1-C2 joints can lead to extremes of instability with severe life-threatening neurologic sequelae that necessitate surgical consult.

We have published dozens of papers on Prolotherapy injections as a treatment in difficult to treat musculoskeletal disorders. Prolotherapy is an injection technique utilizing simple sugar or dextrose. For more information please see my article Ross Hauser, MD Reviews Cervical Spine Instability and Potential Effects on Brain Physiology.

We hope you found this article informative and it helped answer many of the questions you may have surrounding your symptoms.

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References

1 Akay A, Rükşen M, İşlekel S. A case of vago-glossopharyngeal neuralgia caused by choroid plexus. Asian Journal of Neurosurgery. 2018 Apr;13(2):525. [Google Scholar]
2 Javed K, Reddy V, Lui F. Neuroanatomy, choroid plexus. InStatPearls [Internet] 2021 Jul 31. StatPearls Publishing. [Google Scholar]
3 Blue R, Spadola M, McAree M, Kvint S, Lee JY. Endoscopic Microvascular Decompression for Vagoglossopharyngeal Neuralgia. Cureus. 2020 Dec;12(12). [Google Scholar]
4 Du T, Ni B, Shu W, Hu Y, Zhu H, Li Y. Neurosurgical Choice for Glossopharyngeal Neuralgia: A Benefit–Harm Assessment of Long-Term Quality of Life. Neurosurgery. 2021 Jan;88(1):131-9. [Google Scholar]
5 Hauser RA, Steilen D, Gordin K. The Biology of Prolotherapy and Its Application in Clinical Cervical Spine Instability and Chronic Neck Pain: A Retrospective Study. European Journal of Preventive Medicine. 2015;3(4):85-102. [Google Scholar]

• Surgery
• NSAIDs
• Cortisone Shots
• R.I.C.E.