Hemifacial spasm, neuralgia, and syncope due to cranial nerve compression in a patient with vertebral artery ectasia
- 1Department of Neurology, University of Athens, Athens, Greece
- 2Encepahlos Diagnostic Institute, Athens, Greece
- 3Department of Cardiology, Laikon Hospital, Athens, Greece
- Correspondence to: Dr Konstantinos Spengos Department of Neurology, University of Athens, Vas. Sofias 82, 11528 Athens, Greece; spengoshol.gr
- ectasia
- glossopharyngeus
- hemifacial spasm
- magnetic resonance imaging
- neurovascular compression
- syncope
- vagus
- vertebral artery
A 42 year old woman with a history of an incompletely remitted peripheral left facial nerve palsy that was followed by clusters of hemifacial spasm and paroxysms of lancinating pain located behind the left ear and radiating to the ipsilateral eye, the neck, or shoulder, was admitted for re-evaluation due to additionally manifested short lasting syncope-like episodes of increasing frequency. Beneath a mild residual facial nerve palsy no further focal findings were documented. Extensive cardiologic workup revealed only a positive tilt table test, indicating an intense vagovasal reaction.
Re-evaluation of previously performed imaging examinations focused on an already angiographically described tortuous course of the vetrebrobasilar arteries (panel A), which compressed the left lateral surface of the medulla, pons, and pontomedullar junction, as also demonstrated on MRI (panel B). Based on the documented vagovasal reaction we assumed an affection of the lower cranial nerves and additionally performed ultra thin MRI. In this way we could impressively demonstrate a severe compression of the proximal stem of the cranial nerves IX and X (panel D), and the root entry zone of the facial nerve on the left (panel C). Owing to the escalation of symptoms the patient underwent microvascular decompressive surgery with interposition of a muscle graft. Two years later she remains asymptomatic. A repeated tilt table test was this time negative.
This is an uncommon case of neurovascular compression,1 characterised by known, however previously not in this combination, described features such as hemifacial spasms, variant glossopharyngeal neuralgia, and syncope,2–5 which all ceased after surgery.
(A) Superimposed projection of the digital subtraction angiograms of both vertebral arteries demonstrates impressively the tortuous course of the vertebrobasilar vessels. (B) Axial T1-GRE weighted MRI sequences demonstrating the tortuous course of both vertebral arteries and their compressing effect on the left frontolateral surface of the pontomedullar junction, the pons, and the cerebellar peduncles. (C) Frontal T1 weighted MRI slice showing the compressing effect of both vertebral arteries (white arrow heads) on the root entry zone of the left facial nerve. (D) Ultra thin (0.8 mm) M3D/FSE weighted MRI slice showing the direct contact between the vertebral artery (black arrow head) and the IX/X cranial nerve (grey arrow).
