Diagnostic Test of Choice for HELPS Syndrome

Microvascular Decompression for HELPS Syndrome: What is the Best Diagnostic Test?

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.

Study Overview

• Status: Completed
• Conditions: HELPS Syndrome
• Intervention / Treatment: Diagnostic test: Pre-Operative CISS-MRI Sequences; Diagnostic test: Pre-Operative Interictal Laryngoscopy; Diagnostic test: Pre-Operative Unilateral and Contralateral Botox

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 Type: Observational
• Enrollment (Actual): 6

Contacts and Locations

Study Locations

• Canada
  • British Columbia
    • Vancouver, British Columbia, Canada, V5Z 1M9
      • Vancouver General Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 14 years to 61 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

HELPS (Hemi-Laryngo-Pharyngeal Spasm) Syndrome

-Subgroup of patients who are unable to localize lateralization of throat contractions

Description

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

Exclusion Criteria:

- Unable to provide informed consent

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort

Intervention / Treatment

HELPS Syndrome unable to lateralize contractions; 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).

Diagnostic test: Pre-Operative CISS-MRI Sequences; Compare pre-operative MRI diagnosis to intra-operative findings on if both are in agreement; Diagnostic test: Pre-Operative Interictal Laryngoscopy; Compare pre-operative laryngoscopy diagnosis to intra-operative findings on if both are in agreement; Diagnostic test: Pre-Operative Unilateral and Contralateral Botox; Compare pre-operative diagnosis using Botox to intra-operative findings on if both are in agreement; Other Names: Injections spaced 3-months apart

What is the study measuring?

Primary Outcome Measures

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

Collaborators and Investigators

• Sponsor: University of British Columbia
• Investigators: Principal Investigator: Christopher R Honey, MD, DPhil, FRCSC, University of British Columbia

Publications and helpful links

General Publications

• Honey CR, Gooderham P, Morrison M, Ivanishvili Z. Episodic hemilaryngopharyngeal spasm (HELPS) syndrome: case report of a surgically treatable novel neuropathy. J Neurosurg. 2017 May;126(5):1653-1656. doi: 10.3171/2016.5.JNS16308. Epub 2016 Jul 8.

Study record dates

Study Major Dates

• Study Start (Actual): April 30, 2017
• Primary Completion (Actual): April 30, 2018
• Study Completion (Actual): October 30, 2018

Study Registration Dates

• First Submitted: April 10, 2017
• First Submitted That Met QC Criteria: April 21, 2017
• First Posted (Actual): April 25, 2017

Study Record Updates

• Last Update Posted (Actual): November 5, 2020
• Last Update Submitted That Met QC Criteria: November 3, 2020
• Last Verified: November 1, 2020

More Information

Terms related to this study

• Keywords: microvascular decompression, Xth nerve
• Additional Relevant MeSH Terms: Pathologic Processes; Disease; Syndrome
• Other Study ID Numbers: H17-00953

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: No

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No