A brief review and update

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Glossopharyngeal neuralgia is a rare but painful condition that is best managed with a team approach, according to the authors of a recent review.

“Overall, the glossopharyngeal nerve is a very small nerve that runs deep into the neck, and it is sometimes accidentally resected during open-neck dissections,” wrote Rutvij J. Shah, MD, interventional physician at the pain at Louisiana State University Health, Shreveport, and Devang Padalia, MD, anesthesiologist in private practice in Tampa, FL.¹

The glossopharyngeal or ninth cranial nerve “is often called ‘the neglected cranial nerve’. Any infectious, inflammatory, or compressive etiology along the glossopharyngeal nerve’s course from end organs to the brainstem can lead to hyperexcitability of the nerve and produce pain,” wrote Drs. Shah and Padalia.¹

In continuous training update published online in StatPearlsthe two physicians reviewed the diagnostic criteria, pathophysiology, and management options for people with glossopharyngeal neuralgia.

Glossopharyngeal neuralgia is rare but distinct

Symptoms

The International Classification of Headaches describe glossopharyngeal neuralgia (GN) as a pain disorder characterized by “brief episodic unilateral pain, of a sharp, throbbing character, with sudden onset and cessation, in the distribution of the glossopharyngeal nerve (angle of the jaw, ear, tonsillar fossa and of the base of the tongue).” Pain in GN follows a relapsing, remitting pattern, and is most often triggered by coughing, talking, or swallowing.

Glossopharyngeal neuralgia accounts for approximately 1% (0.2% to 1.3%) of all cranial neuralgia. Although rare, “the pattern of pain in an individual with glossopharyngeal neuralgia is remarkably uniform.”¹

Diagnostic criteria

Diagnosis of glossopharyngeal neuralgia should be based on patient reports of recurrent paroxysmal pain in the glossopharyngeal nerve distribution that meets the following criteria: Severe pain, lasting a few seconds to approximately 2 minutes, sharp, throbbing, throbbing or transmitting sensations like electric shocks.

Previous research shows that most people with GN report no pain between attacks, but some report residual sharp pain. In general, the onset and disappearance of pain occur abruptly and attacks may occur without warning.

Differential diagnoses: trigeminal neuralgia, Jacobson’s disease, TMD disorders

Several key factors set GN apart from its closest competitors in a differential diagnosis. Trigeminal neuralgia is a cranial neuralgia with a similar pathophysiology; however, GN pain occurs in the throat and tonsil region, while trigeminal neuralgia pain occurs on the face.

The location also distinguishes GN from Jacobson’s or intermediate neuralgia, a condition involving pain and sensory loss in the ear, although otic variation of GN is possible.

Temporal arteritis and temporomandibular joint dysfunction may be part of the differential diagnosis of GN because pain occurs in the same distribution as GN. However, GN rarely involves the headaches and vision problems that help define temporal arteritis.

Evaluation and treatment of glossopharyngeal neuralgia

Physical examination and tests

The evaluation of a person with GN is primarily clinical, according to Drs. Shah and Padalia. A thorough ENT examination, including throat examination and neck palpation, is essential for diagnosis. Prospective patients with GN should undergo basic laboratory evaluations, including complete blood count, baseline metabolic panel and erythrocyte sedimentation rate, and anti-nuclear antibodies; this information can help rule out an underlying infection, inflammation, malignancy or temporal arteritis, the authors pointed out. Additionally, CT scan, X-ray, or magnetic resonance imaging can be used to identify possible causes of nerve compression at the base of the skull.

Pharmacotherapy

Previous research shows that many people with GN respond to drug therapy, particularly carbamazepine or oxcarbazepine, the authors noted. However, the International Association for the Study of Pain (IASP) also recommends gabapentin, duloxetine, valproic acid, clonazepam, lamotrigine, baclofen, phenytoin, pregabalin, and topiramate to treat neuralgia glossopharyngeal.¹ The general recommendation for these medications is to start at low doses and increase as needed. Non-pharmacological treatments with varying histories of success for GN symptom relief include cold and hot compresses, physical therapy and psychological counseling, the reviewers said.

Speeches

More drastic treatments include nerve blocks and surgery to relieve nerve compression. Microvascular decompression (MVD) is the most common surgical procedure to relieve vascular nerve compression, but resection of the glossopharyngeal nerve, alone or with branches of the vagus nerve, is an alternative for GN patients.

Misdiagnosis and medication management pose challenges

“An updated review on glossopharyngeal neuralgia and its treatments is needed because it is a very rare condition with few treatments,” said Seena Patel, DMD, MPH, associate professor and director of oral medicine at AT Still University, Mesa, Arizona. PPM.

Glossopharyngeal neuralgia can also be easily misdiagnosed, Dr. Patel said. “Having this up-to-date knowledge of diagnosis, differential diagnosis and treatment helps clinicians better understand the disease.”

The current update by Drs. Shah and Padalia provide an in-depth review of the nature of glossopharyngeal neuralgia, with a brief historical overview, diagnostic criteria, tests and treatments, Dr. Patel said. “Unfortunately, there have been no new breakthroughs in treatment,” she noted.

Accurate diagnosis

For clinicians, “I think the biggest challenge with this condition is diagnosing it correctly. Due to the area of ​​innervation of the glossopharyngeal nerve, it can present with so many other painful conditions,” Dr. Patel pointed out.

To aid in the diagnosis of GN, clinicians should pay particular attention to painful features, Dr. Patel added. Glossopharyngeal neuralgia “is extremely painful, rendering the patient inactive during the painful episode,” she said. In particular, “Pain is often triggered by an innocuous stimulus. Immediately after the painful episode, there is a refractory period where the pain can no longer be triggered. All of these features are crucial to understanding nerve pain and should raise red flags for this type of diagnosis,” she explained.

Barriers to treatment

For patients, misdiagnosis is the biggest hurdle to treating GN, Dr. Patel said. “Many patients with glossopharyngeal neuralgia see multiple clinicians and undergo numerous diagnostic tests and treatments before getting the correct diagnosis, which prevents early intervention.” “Another significant hurdle relates to medication management,” Dr. Patel added.

“Pharmacophobia and adverse drug reactions limit appropriate treatment. In some cases, patients are afraid to take the drugs needed to treat the pain, while others find it difficult to tolerate the drugs,” she noted. “Furthermore, given the rarity of the disease, there are no large trials establishing the effectiveness of most treatments, making it challenging for both clinicians and patients.” Key areas for further research include surgical treatments and nerve block, she said.


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