Diagnostic Test of Choice for HELPS Syndrome

HELPS Syndrome Clinical Trial

Official title:

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

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT03126955
• Other study ID #: H17-00953
• Status: Completed
• Start date: April 30, 2017
• Est. completion date: October 30, 2018

Study information

• Verified date: November 2020
• Source: University of British Columbia
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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.

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?"

Recruitment information / eligibility

• Status: Completed
• Enrollment: 6
• Est. completion date: October 30, 2018
• Est. primary completion date: April 30, 2018
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years to 65 Years

Eligibility

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

Related Conditions & MeSH terms

• HELPS Syndrome
• Syndrome

Intervention

Diagnostic Test:
• Pre-Operative CISS-MRI Sequences
Compare pre-operative MRI diagnosis to intra-operative findings on if both are in agreement
• Pre-Operative Interictal Laryngoscopy
Compare pre-operative laryngoscopy diagnosis to intra-operative findings on if both are in agreement
• Pre-Operative Unilateral and Contralateral Botox
Compare pre-operative diagnosis using Botox to intra-operative findings on if both are in agreement

Locations

Country	Name	City	State
Canada	Vancouver General Hospital	Vancouver	British Columbia

Sponsors (1)

Lead Sponsor	Collaborator
University of British Columbia

Country where clinical trial is conducted

Canada, 

References & Publications (1)

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. — View Citation

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	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
Primary	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
Primary	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