VGN is a rare and challenging-to-diagnose condition characterized by neuropathic pain in the regions innervated by the glossopharyngeal and vagus nerves, including the larynx, pharynx, and inner ear. It presents a difficulty in distinguishing between pain originating from the ninth cranial nerve and the tenth pair [1]. Accessory nerve palsy, on the other hand, involves weakness or spasms of the sternocleidomastoid or trapezius muscles. Pain in VGN typically occurs after activities such as chewing, coughing, yawning, or speaking, with durations ranging from a few seconds to minutes. Atypical symptoms may include cardiac arrhythmias and syncopal episodes [6].

VGN and accessory nerve palsy can be either primary, without a known anatomical cause, or secondary to factors such as masses in the cerebellopontine angle, oropharyngeal tumors, ossification of the stylohyoid ligament, multiple sclerosis, or vascular malformations. The primary cause usually involves a conflict between the ninth and tenth cranial nerves and the PICA and/or the vertebral artery [7].

The best way to characterize the neurovascular conflict is through a brain MRI or CT scan [8]. MRI is considered the ideal test since it allows localization and characterization of the lesion, as well as assessment and surgical planning for the best treatment of patients [9].

For the management of VGN and accessory nerve palsy, anticonvulsant drugs like carbamazepine, oxcarbazepine, gabapentin, or pregabalin are considered the first line of treatment [10]. In cases resistant to medical management, microvascular decompression through craniotomy is preferred, although percutaneous procedures such as radiofrequency and rhizotomy, as well as Gamma Knife radiosurgery, have also been described [5].

When opting for microvascular decompression, it is essential to address the challenge of distinguishing between the ninth and tenth cranial nerves as the origin of symptoms. Some authors recommend partial removal of the glossopharyngeal nerve and upper rootlets of the vagus nerve (approximately 1–3) to achieve better therapeutic outcomes. However, this segment of the vagus nerve also provides fibers to the superior and recurrent laryngeal nerves, which can lead to complications like dysarthria or dysphagia, making intervention in these segments risky [11].

Recent studies by Krüger et al. have suggested that rhizotomy of the upper rootlets of the vagus nerve yields higher rates of symptom relief. They classified two types of rootlets: Type A (sensory) and Type B (motor) and used intraoperative neurophysiological monitoring to reduce the chances of motor complications such as dysphagia or dysphonia. The study concluded that when the upper rootlets were predominantly sensory fibers, patients who underwent rhizotomy experienced better symptom relief with no associated complications. In cases where the rootlets were predominantly Type B, intervention in the vagus nerve was not recommended, with a complication rate of approximately 19%. The anatomical and physiological basis for this lies in the close relationship between the vagus and glossopharyngeal nerves in their origin in the solitary tract and their trajectory, although this relationship is not yet fully understood [12].

Currently, there are no reported cases in the literature of unilateral VGN combined with unilateral accessory nerve palsy and syncope. However, authors such as Ganaha et al. [13] describe a case of a patient in her early twenties who had previously undergone microvascular decompression of the right glossopharyngeal nerve. She presented with intractable left hemifacial pain initially associated with speech, chewing, or mouth opening. Initial treatment with pharmacotherapy and steroid infiltration did not provide sufficient relief. Magnetic resonance imaging and cranial tomography did not reveal any abnormalities. Subsequently, the patient underwent a left suboccipital surgery where the left glossopharyngeal nerve and the upper rootlets of the vagus nerve were sectioned, with intraoperative monitoring of nerves VII, VIII, X, and XI. This procedure had previously been successfully performed on the right side. In the postoperative period, the patient experienced a prompt recovery and complete resolution of symptoms. The authors suggest that the sectioning of the ninth and upper tenth roots can be safe if preoperative functional tests indicate a minimal risk of postoperative oropharyngeal and cardiac complications.

In our present case, due to the refractory nature of the symptoms to medical and surgical management, we decided to intervene on two upper rootlets of the vagus nerve during the most recent surgical procedure. We believe that sectioning the upper rootlets of the vagus nerve may be a suitable option in complex cases where symptomatology does not improve despite surgical management. This decision involves carefully weighing the risk–benefit ratio and the potential for injury to the recurrent or superior laryngeal nerve. Additionally, in patients with vasovagal syncope of unknown cause, resistant to fluid therapy and medications, it is advisable to consider a possible neurovascular conflict involving the ninth and tenth cranial nerves as a potential underlying cause of the symptoms.

It is important to note that this study is based on a single case, and further research involving a larger number of patients is needed to establish a correlation between VGN neuralgia and syncope. Moreover, the efficacy and outcomes of rhizotomy of the superior rootlets of the vagus nerve remain a topic of debate, and future studies are required to provide more insights into the postoperative effects of this technique.