Diagnostic Test of Choice for HELPS Syndrome

NCT ID: NCT03126955

Last Updated: 2020-11-05

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Total Enrollment

6 participants

Study Classification

OBSERVATIONAL

Study Start Date

2017-04-30

Study Completion Date

2018-10-30

Brief Summary

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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

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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?"

Conditions

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HELPS Syndrome

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

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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).

Pre-Operative CISS-MRI Sequences

Intervention Type DIAGNOSTIC_TEST

Compare pre-operative MRI diagnosis to intra-operative findings on if both are in agreement

Pre-Operative Interictal Laryngoscopy

Intervention Type DIAGNOSTIC_TEST

Compare pre-operative laryngoscopy diagnosis to intra-operative findings on if both are in agreement

Pre-Operative Unilateral and Contralateral Botox

Intervention Type DIAGNOSTIC_TEST

Compare pre-operative diagnosis using Botox to intra-operative findings on if both are in agreement

Interventions

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Pre-Operative CISS-MRI Sequences

Compare pre-operative MRI diagnosis to intra-operative findings on if both are in agreement

Intervention Type DIAGNOSTIC_TEST

Pre-Operative Interictal Laryngoscopy

Compare pre-operative laryngoscopy diagnosis to intra-operative findings on if both are in agreement

Intervention Type DIAGNOSTIC_TEST

Pre-Operative Unilateral and Contralateral Botox

Compare pre-operative diagnosis using Botox to intra-operative findings on if both are in agreement

Intervention Type DIAGNOSTIC_TEST

Other Intervention Names

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Injections spaced 3-months apart

Eligibility Criteria

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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
Minimum Eligible Age

18 Years

Maximum Eligible Age

65 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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University of British Columbia

OTHER

Sponsor Role lead

Responsible Party

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Christopher Honey

Professor & Neurosurgeon

Responsibility Role PRINCIPAL_INVESTIGATOR

Principal Investigators

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Christopher R Honey, MD, DPhil, FRCSC

Role: PRINCIPAL_INVESTIGATOR

University of British Columbia

Locations

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Vancouver General Hospital

Vancouver, British Columbia, Canada

Site Status

Countries

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Canada

References

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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.

Reference Type BACKGROUND
PMID: 27392269 (View on PubMed)

Other Identifiers

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H17-00953

Identifier Type: -

Identifier Source: org_study_id