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J Neurosurg. 2017 May;126(5):1653-1656. doi: 10.3171/2016.5.JNS16308. Epub 2016 Jul 8.

Episodic hemilaryngopharyngeal spasm (HELPS) syndrome: case report of a surgically treatable novel neuropathy.

Author information

1
Divisions of 1 Neurosurgery and.
2
Otolaryngology, University of British Columbia, Vancouver, British Columbia, Canada.

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.

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

PMID:
27392269
DOI:
10.3171/2016.5.JNS16308

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