Glossopharyngeal Neuralgia

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Glossopharyngeal neuralgia (GPN) is a rare craniofacial pain syndrome characterized by paroxysmal, severe, stabbing pain in the distribution of the glossopharyngeal nerve (cranial nerve IX). This pain is often described as electrical shock-like and can be triggered by activities such as swallowing, talking, coughing, or yawning.

Anatomy

The glossopharyngeal nerve originates from the medulla oblongata and exits the skull through the jugular foramen. It innervates the posterior third of the tongue, the pharynx, the tonsils, and the middle ear, and it has both sensory and motor functions.

Pathophysiology

The most common cause of GPN is neurovascular compression at the root entry zone of the glossopharyngeal nerve, often by the posterior inferior cerebellar artery (PICA) or other vessels. Other less common causes include trauma, elongated styloid process (Eagle's syndrome), and neoplasms.

Clinical Features

Patients with GPN typically present with unilateral, lancinating pain in the areas innervated by the glossopharyngeal nerve, including the throat, ear, and base of the tongue. The pain is episodic, lasting from seconds to minutes, and can be severe enough to cause syncope or cardiac arrhythmias in rare cases.

Management

The first-line treatment for GPN is pharmacological, primarily using anticonvulsants such as carbamazepine or gabapentin. If medical management fails, surgical options are considered. Microvascular decompression (MVD) is the preferred surgical treatment, offering high rates of pain relief by alleviating the neurovascular conflict. Other surgical options include rhizotomy and stereotactic radiosurgery (SRS), though these are associated with higher risks of complications such as dysphagia and vocal cord paralysis.