1107. Hemi-Laryngopharyngeal Spasm (HELPS): Is this a Newly Recognized Neurovascular Compression Syndrome?

Authors: Christopher R. Honey, MD; Murray Morrison, MD; Amanda Hu, MD; Manraj Heran, MD; Baljinder Dhaliwal, MD (Vancouver, Canada)

Introduction:

We describe a novel condition, hemi-laryngopharyngeal spasm (HELPS), which can cause episodes of severe stridor in association with cough due to unilateral vascular compression of the vagus nerve. We have followed four patients for more than a year after microvascular decompression and present the results of this case series.

Methods:

The clinical presentation, diagnostic features, operative findings and outcomes are presented for four patients with HELPS.

Results:

Each patient presented with a similar clinical pattern of painless episodic choking and coughing that increased in frequency, severity and duration over the years. Individuals were asymptomatic between episodes. The episodes eventually occurred while sleeping and could cause severe stridor with rare loss of consciousness. All were initially misdiagnosed with a psychiatric illness and subjected to multiple intubations and one tracheostomy. Unilateral botulinum toxin injections in the vocal fold eased the severity of the choking but not the cough. MRI showed a looping posterior inferior cerebellar artery juxtaposed to the vagus nerve in each case. Microvascular decompression of that nerve relieved all symptoms. 

Conclusion:

We believe the introduction of this new medical condition may help a small cohort of patients with inducible laryngeal obstructions who have not responded to the current standard treatments. Severe anxiety about the unpredictable symptoms may have contributed to the psychiatric misdiagnoses. MVD for HELPS is more difficult than for trigeminal neuralgia because the nerve is more sensitive to manipulation. We present our recommendations for the diagnostic test of choice and our current hypothesis on the pathophysiology of this condition. Ultimately, the final proof that hemi-laryngopharyngeal spasm is a real and distinct syndrome will require its recognition and successful treatment by our colleagues around the world.