Introduction: We describe a novel cranial neuropathy manifesting with life-threatening episodic hemi-laryngopharyngeal spasm (HELPS).
Methods: We studied a case of a 50-year-old woman presenting with a four-year history of intermittent throat contractions, escalating to life-threatening respiratory distress. We provide the details of her diagnostic workup and the treatment strategies, including Botulinum toxin and surgery.
Results: Botulinum toxin injections into her right pharyngeal muscles and 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 (MVD) of the upper rootlets of the vagal nerve was performed, which eliminated her HELPS without complication. She remains free of spasmodic episodes at 1 year follow up.
Conclusions: We propose a mechanism of episodic hemi-laryngopharyngeal spasm implicating isolated involvement of the upper motor rootlets of the vagus nerve. HELPS syndrome can be treated with MVD.
Patient Care: Episodic hemi-laryngopharyngeal syndrome has not been described before. We believe that this condition is underrecognized and undertreated. By raising awareness about the HELPS syndrome, patients with this condition can be cured surgically, as in the case of our patient.
Learning Objectives: By the conclusion of this session participants should be able to 1) recognize HELPS syndrome, 2) differentiate HELPS syndrome from the other paroxysmal laryngospasm conditions, 3) identify MVD as the treatment of choice for patients with HELPS syndrome.
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