1059. Concurrent Glossopharyngeal Neuralgia and Hemi-Laryngopharyngeal Spasm (HELPS)

Authors: Christopher R. Honey, MD; Murray Morrison, MD (Vancouver, Canada)

Introduction: Glossopharyngeal neuralgia (GPN) is a rare but well recognized condition presenting with episodic severe pain in the distribution of the IX th cranial nerve often due to a vascular compression of that nerve. Hemi-laryngopharyngeal spasm (HELPS) is a newly describe syndrome with episodic throat contractions and cough due to a vascular compression of the X th cranial nerve. With the close proximity of these two nerves and the similar etiology of these two conditions, one would expect these two clinical conditions to occasionally present in the same patient. Methods: We present the clinical presentations, diagnostic features, operative findings and surgical outcomes of two patients with concurrent GPN and HELPS. We then reviewed the literature for all papers describing the clinical features of GPN to see if any had additional features compatible with HELPS. Results: Both patients presented with a combination of clinical features in keeping with GPN and HELPS. The GPN was described as a progressive, episodic, severe, unilateral pain from the ear to the throat triggered by swallowing. The HELPS was described as i) progressively severe episodic throat contractions, and ii) progressively severe episodic coughing triggered by a 'tickling sensation' behind the xiphisternum. Both patients underwent MVD with rapid resolution of their GPN and slow recovery (partial in one) of HELPS. A literature review in the English, French, German, Dutch, and Russian languages found 1200 papers reporting GPN and 120 with enough description to determine if the patients also suffered with any symptoms compatible with HELPS. Multiple examples were found describing patients with classic GPN as well as additional 'unexplained' or 'unusual' symptoms of coughing, choking, difficulty breathing or vocal changes. Conclusion: We believe patients with HELPS have been previously described but not recognized in the literature. We present our diagnostic and surgical protocol for these patients.