Concurrent glossopharyngeal neuralgia and hemi-laryngopharyngeal spasm (helps): A case report and a review of the literature

Abstract

BACKGROUND AND IMPORTANCE: Hemi-laryngopharyngeal spasm (HeLPS) has been recently described but is not yet widely recognized. Patients describe intermittent coughing and choking and can be cured following microvascular decompression of their Xth cranial nerve. This case report and literature review highlight that HeLPS can co-occur with glossopharyngeal neuralgia (GN) and has been previously described (but not recognized) in the neurosurgical literature. CLINICAL PRESENTATION: A patient with GN and additional symptoms compatible with HeLPS is presented. The patient reported left-sided, intermittent, swallow-induced, severe electrical pain radiating from her ear to her throat (GN). She also reported intermittent severe coughing, throat contractions causing a sense of suffocation, and dysphonia (HeLPS). All her symptoms resolved following a left microvascular decompression of a loop of the posterior inferior cerebellar artery that was pulsating against both the IXth and Xth cranial nerves. A review of the senior author's database revealed another patient with this combination of symptoms. An international literature review found 27 patients have been previously described with symptoms of GN and the additional (but not recognized at the time) symptoms of HeLPS. CONCLUSION: This review highlights that patients with symptoms compatible with HeLPS have been reported since 1926 in at least 4 languages. This additional evidence supports the growing recognition that HeLPS is another neurovascular compression syndrome. Patients with HeLPS continue to be misdiagnosed as conversion disorder. The increased recognition of this new medical condition will require neurosurgical treatment and should alleviate the suffering of these patients.