An unusual presentation of unilateral vagoglossopharyngeal neuralgia accompanied by unilateral accessory nerve palsy

Abstract Background Vago-glossopharyngeal neuralgia (VGN) is a rare condition characterized by neuropathic pain in the areas innervated by the glossopharyngeal and vagus nerve. Diagnosis can be challenging due to its rarity and the need to differentiate it from other sources of pain. Neurovascular conflicts involving the glossopharyngeal nerve and surrounding vasculature are often implicated in the development of VGN. Surgical interventions, such as microvascular decompression, have shown promise in relieving symptoms, but long-term outcomes remain poorly understood. This case report presents a unique manifestation of VGN accompanied by unilateral accessory nerve palsy and syncope, shedding light on the complexities involved in diagnosis and management. Case presentation The presented case involves a 42-year-old patient without comorbidities with a 1-year history of syncope, dysphonia, cough, oropharyngeal pain, dysphagia, right periaricular pain, and weakness of the right sternocleidomastoid and trapezius muscle. After an initial evaluation, compression of the right cranial nerves IX, X, and XI by the posterior inferior cerebellar artery (PICA) was identified as the cause. The patient underwent microvascular decompression, which provided 3 years of symptom relief. However, symptoms recurred with additional manifestations, including left periauricular pain and increased syncopal episodes of cardiogenic origin. A follow-up brain MRI revealed compression of the left cranial nerves IX and X. A second surgical intervention targeting the left side resulted in partial improvement but symptom reappearance after 1 year. Finally, a third surgery involving the left side was performed, and along with isolating the affected cranial nerves, two superior rootlets of the vagus nerve were rhizotomized. Postoperative recovery was successful, with complete resolution of symptoms and no recurring issues during follow-up examinations. Conclusion This case underscores the complexity of diagnosing and managing neurovascular conflicts associated with VGN pain syndrome. While initial microvascular decompression provided relief, the recurrence of symptoms and emergence of new manifestations highlight the challenges in managing this condition. Precise identification of nerve compression and targeted surgical interventions, such as sectioning the superior roots of the vagus nerve, offer potential efficacy in refractory cases. Further studies and long-term follow-up are necessary to gain comprehensive insights into the progression and outcomes of these interventions.