Clinical trial NCT03126955: Diagnostic Test of Choice for HELPS Syndrome
Official title: Microvascular Decompression for HELPS Syndrome: What is the Best Diagnostic Test?
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: HELPS Syndrome
Keywords: microvascular decompression, Xth nerve
Lead sponsor: University of British Columbia
Sponsor class: OTHER
First posted: 2017-04-25
Enrollment: 6
Sex: ALL
Minimum age (years): 18
Maximum age (years): 65
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
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

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?"

Eligibility criteria: 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

Primary outcomes:
measure: Pre-Operative CISS MRI (One month before surgery) compared to Intraoperative Findings
timeFrame: Pre-Operative and Intra-Operative
measure: Pre-Operative Interictal Laryngoscopy (One month before surgery) compared to Intraoperative Findings
timeFrame: Pre-Operative vs. Intra-Operative
measure: Pre-Operative 3 and 6-month Botox Injections compared to Intraoperative Findings
timeFrame: Pre-operative 3 and 6 months vs. Intra-Operative
Secondary outcomes:
Other outcomes:
Locations:
city: Vancouver
country: Canada
facility: Vancouver General Hospital
state: British Columbia
Study officials:
affiliation: University of British Columbia
name: Christopher R Honey, MD, DPhil, FRCSC
role: PRINCIPAL_INVESTIGATOR
References:
citation: 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.