Hemi-laryngopharyngeal Spasm (HeLPS): Defining a New Clinical Entity

Abstract

Objective: Hemi-laryngopharyngeal spasm (HeLPS) has recently been described in the neurosurgical literature as a cause of intermittent laryngopharyngeal spasm and cough due to vascular compression of the vagus nerve at the cerebellopontine angle. We present the diagnostic criteria for this syndrome. Methods: A retrospective chart review of six patients with HeLPS and three patients misdiagnosed with this condition are presented. All patients were diagnosed and treated at a tertiary care academic centre from July 2013 to July 2017. Results: Patients with HeLPS had five defining characteristics: 1) All patients had symptoms of episodic laryngopharyngeal spasm and coughing. Patients were asymptomatic between episodes and were refractory to speech therapy and reflux management. 2) Laryngoscopy showed hyperactive twitching of the ipsilateral vocal fold in two of the six patients. No other inter-episodic abnormalities were seen. 3) Botulinum toxin A injections into the thyroarytenoid muscle on the affected ipsilateral side reduced laryngopharyngeal spasms. Botulinum toxin injection in the contralateral thyroarytenoid muscle did not improve laryngopharyngeal spasm. 4) Magnetic resonance imaging revealed ipsilateral neurovascular compression of the vagus nerve rootlets by the posterior inferior cerebellar artery. 5) Microvascular decompression (MVD) surgery of the ipsilateral vagus nerve resolved all symptoms (follow-up 2-4 years). Conclusion: The diagnostic criteria for hemi-laryngopharyngeal spasm (HeLPS) are proposed. Otolaryngology recognition of this new clinical entity may lead to a surgical cure and avoid the unnecessary therapies associated with misdiagnosis. Level of Evidence: 4

Microvascular Decompression for Concurrent Trigeminal Neuralgia and Hemi-Laryngopharyngeal Spasm: A Case Report

“…Previous cases of HeLPS treated using neurosurgery are listed in Table . Only 6 such cases were treated with MVD; symptoms were relieved in all cases although transient vagus nerve palsy occurred in 3 cases. [4][5][6][7][8] Preoperatively, 4 of the 6 reported patients were taken to the emergency department and 2 were admitted to the intensive care unit. One patient was intubated for respiratory distress and stridor; the other required a tracheostomy.…”

“…Only 6 such cases were treated with MVD; symptoms were relieved in all cases although transient vagus nerve palsy occurred in 3 cases. 4-8…”

“…Therefore, offering MVD for HeLPS is challenging, and the decision must be well-considered. 8 We recommend simultaneous MVD for HeLPS when it is concurrent with another neurovascular compression syndrome.…”

“…5 It may be treated with MVD of the vagus nerve, but there is limited experience. [5][6][7][8] We performed MVD on a patient with concurrent TN and HeLPS who had immediate and complete resolution of symptoms without recurrence during the 18-month follow-up. In this article, we report the clinical characteristics, preoperative 3-dimensional (3D) fusion imaging, and intraoperative findings.…”

Supraglottic Botulinum Toxin Improves Symptoms in Patients with Laryngeal Sensory Dysfunction Manifesting as Abnormal Throat Sensation and/or Chronic Refractory Cough

“…Honey et al proposed neurovascular compression of the vagus nerve rootlets identified on magnetic resonance imaging [ 21 ] as a potential cause of vagal dysfunction presenting in the larynx with sensory symptoms of abnormal throat sensations [ 22 ] associated with motor symptoms of laryngospasm/choking, neurogenic cough or intermittent stridor [ 23 ].…”