The authors describe a novel cranial neuropathy manifesting with life-threatening episodic hemilaryngopharyngeal spasm (HELPS). A 50-year-old woman presented with a 4-year history of intermittent throat contractions, escalating to life-threatening respiratory distress. Botulinum toxin injections into her right vocal cord reduced the severity of her spasms, but the episodes continued to occur. MRI demonstrated a possible neurovascular conflict involving the cranial nerve IX-X complex and the posterior inferior cerebellar artery. Microvascular decompression of the upper rootlets of the vagal nerve eliminated her HELPS without complication. The authors propose a mechanism of HELPS implicating isolated involvement of the upper motor rootlets of the vagus nerve.
Keywords: CN = cranial nerve; GFN = glossopharyngeal neuralgia; HELPS = hemilaryngopharyngeal spasm; HELPS syndrome; HFS = hemifacial spasm; MVD = microvascular decompression; PICA = posterior inferior cerebellar artery; episodic laryngospasm; functional neurosurgery; hemilaryngopharyngeal spasm; microvascular decompression; neurovascular conflict; vocal cord dysfunction.