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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.
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?"
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| 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). |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Pre-Operative CISS-MRI Sequences | Diagnostic Test | Compare pre-operative MRI diagnosis to intra-operative findings on if both are in agreement |
|
| 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 |
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Inclusion Criteria:
Exclusion Criteria:
- Unable to provide informed consent
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HELPS (Hemi-Laryngo-Pharyngeal Spasm) Syndrome
-Subgroup of patients who are unable to localize lateralization of throat contractions
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| Name | Affiliation | Role |
|---|---|---|
| Christopher R Honey, MD, DPhil, FRCSC | University of British Columbia | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Vancouver General Hospital | Vancouver | British Columbia | V5Z 1M9 | Canada |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 27392269 | Background | 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. |
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| Pre-Operative Interictal Laryngoscopy | Diagnostic Test | Compare pre-operative laryngoscopy diagnosis to intra-operative findings on if both are in agreement |
|
| Pre-Operative Unilateral and Contralateral Botox | Diagnostic Test | Compare pre-operative diagnosis using Botox to intra-operative findings on if both are in agreement |
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