Diagnostic Test of Choice for HELPS Syndrome

University of British Columbia

Status: Completed

Conditions: HELPS Syndrome

Treatments: Diagnostic Test: Pre-Operative Interictal Laryngoscopy; Diagnostic Test: Pre-Operative Unilateral and Contralateral Botox; Diagnostic Test: Pre-Operative CISS-MRI Sequences

Study type: Observational

Funder types: Other

Identifiers

NCT03126955

H17-00953

Details and patient eligibility

About

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.

Full 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?"

Enrollment: 6 patients

Sex: All

Ages: 18 to 65 years old

Volunteers: No Healthy Volunteers

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

Trial design

6 participants in 1 patient group

HELPS Syndrome unable to lateralize contractions

Description:

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).

Treatment:

Diagnostic Test: Pre-Operative Unilateral and Contralateral Botox

Diagnostic Test: Pre-Operative CISS-MRI Sequences

Diagnostic Test: Pre-Operative Interictal Laryngoscopy