Diagnostic Test of Choice for HELPS Syndrome

Completed

• Conditions: HELPS Syndrome
• Interventions: Diagnostic Test: Pre-Operative CISS-MRI Sequences; Diagnostic Test: Pre-Operative Interictal Laryngoscopy; Diagnostic Test: Pre-Operative Unilateral and Contralateral Botox

• Registration Number: NCT03126955
• Lead Sponsor: University of British Columbia

Brief Summary

Our team recently described a new medical condition called HELPS (Hemi-Laryngo-Pharyngeal-Spasm) syndrome(1). HELPS syndrome is a condition caused by a blood vessel pinching the nerve rootlets of the Vagus nerve (Xth cranial nerve). It is similar to the well recognized hemifacial spasm syndrome but the nerve involved is the Vagus instead of the Facial nerve. As a result, the symptoms are episodic throat contractions and cough. The throat contractions become stronger and more frequent over the years and can lead to a terrifying inability to breath. Patients may end up intubated in the Emergency Department or with a tracheostomy because of inability to breath during a severe episode. Some but not all of our patients can tell which side of their throat (left or right) contracts during a choking episode. In between these choking episodes, patients feel normal. A surgical cure for these patients is Microvascular Decompression of the Xth nerve.

Detailed Description

The treatment of HELPS syndrome begins with the correct diagnosis. Some patients are able to localize the side of their symptoms in HELPS syndrome while others are unable to. The purpose of this study is to prospectively study which of the following is the diagnostic test of choice for patients who are unable to localize their HELPS: 1. CISS MRI sequences, 2. interictal laryngoscopy, and 3. unilateral and contralateral Botox injections separated 3-months apart.

Both neuroradiologist and otolaryngologist will be blinded to the outcome of surgery and be asked "based on your diagnostic test, which side do you believe we should perform MVD surgery?"

Study Timeline

• Study First Submitted: 2017-04-10
• Study First Submitted QC: 2017-04-21
• Study First Posted: 2017-04-25
• Study Start: 2017-04-30
• Primary Completion: 2018-04-30
• Study Completion: 2018-10-30
• Status Verified: 2020-11
• Last Update Submitted: 2020-11-03
• Last Update Posted: 2020-11-05

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 6

Inclusion Criteria

• Patients diagnosed with HELPS as described in our initial publication (Honey et al. 2016)
• Patients unable to localize the side of their HELPS syndrome

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Exclusion Criteria

• Unable to provide informed consent

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Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
HELPS Syndrome unable to lateralize contractions	Pre-Operative Interictal Laryngoscopy	Each patient will have the following 3 diagnostic pre-operative tests: i) MRI (CISS sequence), ii) video laryngoscopy, and iii) sequential Botox injections in their throat (left side and then 3 months later on the right side).
HELPS Syndrome unable to lateralize contractions	Pre-Operative CISS-MRI Sequences	Each patient will have the following 3 diagnostic pre-operative tests: i) MRI (CISS sequence), ii) video laryngoscopy, and iii) sequential Botox injections in their throat (left side and then 3 months later on the right side).
HELPS Syndrome unable to lateralize contractions	Pre-Operative Unilateral and Contralateral Botox	Each patient will have the following 3 diagnostic pre-operative tests: i) MRI (CISS sequence), ii) video laryngoscopy, and iii) sequential Botox injections in their throat (left side and then 3 months later on the right side).

Primary Outcome Measures

Name	Time	Method
Pre-Operative CISS MRI (One month before surgery) compared to Intraoperative Findings	Pre-Operative and Intra-Operative	Determine if CISS MRI sequence lateralization in concordance with intraoperative findings
Pre-Operative Interictal Laryngoscopy (One month before surgery) compared to Intraoperative Findings	Pre-Operative vs. Intra-Operative	Determine if interictal laryngoscopy in concordance with intraoperative findings
Pre-Operative 3 and 6-month Botox Injections compared to Intraoperative Findings	Pre-operative 3 and 6 months vs. Intra-Operative	Determine if unilateral vs contralateral Botox-induced symptom reduction in concordance with intraoperative findings

Trial Locations

Locations (1)

Vancouver General Hospital; 🇨🇦 Vancouver, British Columbia, Canada