Hemifacial spasm (HFS) induced by a change in head position has rarely been reported. Here we present such a case accompanied by a video for the first time.
A 48 year‐old non‐white Hispanic woman visited us 3 years after developing position related paroxysmal right eyelid blinking and squinting and facial twitching. The symptoms were involuntary and occurred one second after she extended her head back. Her symptoms consistently resolved completely within minutes after moving her head back to neutral upright position or slightly leaning forwards. Her symptoms persisted when she lay down or during sleep, which resolved in minutes when she sat up or leaned forward. These symptoms started about a month after she had a car accident suffering “whiplash” and “quadriplegia” for several hours, with a full recovery through rehabilitation. She had been seen by neurologists and ear‐nose‐throat specialists but without a confirmed diagnosis. Her workups on electroencephalogram, c‐spine MRI, and brain MRI/MRA without contrast were unremarkable.
On our exam, she had normal pain sensation, hearing and facial symmetry. One second of backward head extension provoked sustained symptoms as described above, which were consistent with HFS, with mild dizziness during the spell. Moving her head back to a neutral upright position or slightly leaning forwards led to complete symptom resolution within 5 minutes (Video 1).
Video 1.
Video clip: Appearance and relief of eye blinking and facial twitching movement with head position changes. The clip shows abnormal inducible movement as described in the text when she leans her head backwards, which is completely recovered soon after she holds her head back to normal position.
MRI/MRA of the brain and internal auditory canals with contrast demonstrated compression of the right cranial nerve (CN) 7 and 8 bundle anteriorly or ventrally at the root exit zone (REZ) by her right vertebral artery (Fig. 1). We believe that gravity dependent compression of the ventrally laid right vertebral artery on the pons at the REZ with head extension or laying down could explain her position‐related symptoms and their reversal when she sat up or leaned forward. She subsequently received botulinum toxin injection, with good response on HFS. She declined a microvascular decompression surgery due to concerns with complications. 1
FIG. 1.
Compression of the CN7 and CN 8 bundle by the right vertebral artery. 3D FIESTA T2 weighted MRA oriented along the length of the 7th cranial nerve (black arrowheads) demonstrate the close proximity of the right vertebral artery (white arrowhead) to the adjacent facial nerve (black arrowheads) at the root entry zone.
In a case series, Ho et al report that 80% of patients with HFS have associated compression on the REZ of the anterior CN7 on MRI/MRA, but with unclear mechanism. 2 Associated vascular compression identified in our case could potentially explain this observation and the position‐related HFS as well reported in other 2 cases decades ago, with one occurring when lying down and another occurring when leaning towards the contralateral side. 3 , 4
In a different case series, more than 90% of patient with HFS were found to be due to the compression of the REZ of the CN7 during decompression surgery, 1 with anterior inferior cerebellar artery, vertebral artery, posterior inferior cerebellar artery, basilar artery and veins commonly involved, followed by mass, demyelinating disease, lacunar stroke and facial nerve injury as other causes. 1 A similar mechanism also explains other microvascular compression syndromes involving CN 5, 8 and 11, 5 causing trigeminal neuralgia, vestibulocochlear neuralgia and vestibular paroxysmia, and glossopharyngeal neuralgia. Similar position‐related variability was also reported, particularly in vestibular paroxysmia. One study found that 60% of the patients with vestibular paroxysmia reported head turn as a provoking factor, particularly the backward/forward head movement, to elicit the vertigo attack. 6
The car accident is less likely to have directly caused her symptoms given the delayed onset by 1 month and unremarkable imaging and expert evaluations, although migration or loosening of the involved artery as a result of the accident could not be ruled out. This case with a video would help us to better recognize and understand HFS.
Author Roles
(1) Research project: A. Conception, B. Organization, C. Execution; (2) Statistical Analysis: A. Design, B. Execution, C. Review and Critique; (3) Manuscript: A. Writing of the first draft, B. Review and Critique.
T.X.: 1A, 1B, 1C, 3A, 3B
G.A.C.: 1C, 3B
Disclosure
Ethical Compliance Statement
Patient was informed and consented. The approval of an institutional review board was not required for this case report. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this work is consistent with those guidelines.
Finding Sources and Conflict of Interest
No specific funding was received for this work. The authors declare that there are no conflicts of interest relevant to this work.
Financial Disclosures for the preceding 12 months
TX receives funding from Parkinson's Foundation and Michael J. Fox Foundation for Parkinson's Research. GAC has no additional disclosure to report.
Acknowledgments
We thank the patient for supporting us to publish this observation.
Data Availability Statement
All the data is available under reasonable request.
References
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Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Data Availability Statement
All the data is available under reasonable request.