title: Episodic hemilaryngopharyngeal spasm (HELPS) syndrome: case report of a surgically treatable novel neuropathy.
abstract: 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.
authors.affiliation: Divisions of 1 Neurosurgery and.
authors.forename: Christopher R
authors.initials: CR
authors.lastname: Honey
authors.affiliation: Divisions of 1 Neurosurgery and.
authors.forename: Peter
authors.initials: P
authors.lastname: Gooderham
authors.affiliation: Otolaryngology, University of British Columbia, Vancouver, British Columbia, Canada.
authors.forename: Murray
authors.initials: M
authors.lastname: Morrison
authors.affiliation: Divisions of 1 Neurosurgery and.
authors.forename: Zurab
authors.initials: Z
authors.lastname: Ivanishvili
mesh_terms: D005260:Female; D005930:Glossopharyngeal Nerve; D006801:Humans; D007826:Laryngismus / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery*; D061145:Microvascular Decompression Surgery; D008875:Middle Aged; D009408:Nerve Compression Syndromes / Q000175:diagnosis* / Q000601:surgery*; D013577:Syndrome; D014630: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