Hemifacial spasm is characterized by unilateral, involuntary contractions of the eyelid and facial muscles and is frequently related to compression of the facial nerve by aberrant vascular loops. Central mechanisms involving the brainstem, however, may also contribute.1, 2, 3 “Gaze‐evoked” eyelid and hemifacial spasm has rarely been reported but is not a well recognized entity.
Case Report
A 72‐year‐old man presented with right periorbital pain. There had been a previous shrapnel injury to the right forehead 11 years prior. Following this he developed constant boring periorbital pain, with acute exacerbations triggered by touch or visual tasks. There were no autonomic features. He had undergone numerous procedures, including supraorbital nerve ablation, removal of a frontal nerve neuroma, and balloon compression of trigeminal nerve V1, without relief. He also complained of unilateral, right eyelid spasm, with an onset 5 years after the onset of pain. His right eyelid would lower on right lateral gaze. He had discovered alleviating “tricks” of touching the ipsilateral face or wearing a hat. The gaze‐evoked spasms would not occur if supine, although occur if his neck were flexed. Occasional spontaneous right eyelid spasms could also occur but did not develop until sometime after the initial gaze‐evoked spasms.
Examination revealed right orbicularis oculi contraction with voluntary right lateral gaze (Video 1). This did not occur with passive eye movements. Placing a finger on the forehead or wearing a hat prevented the spasm. The spasms would not occur if he looked to the right when lying supine but would occur if his neck were flexed. Occasional spontaneous spasms were also observed. With spasm there is subtle elevation of the right eyebrow (the Babinski sign). Extra‐ocular movements were otherwise normal. There was reduction in sensation over the right V1, but systemic neurological examination was otherwise unremarkable.
Video 1.
Right facial scarring from previous surgery. Right eyebrow is higher than the left at rest. Occasional spontaneous spasm of the orbicularis oculi. There is marked spasm on right lateral gaze. This is not present on passive head movement and is relieved with sensory trick of touching the forehead and wearing a hat. There is spasm on lying supine, which is then relieved on flexing the neck. There is subtle elevation of the eyebrow with spasm. There is no facial droop. Not shown is normal hearing and head impulse test.
Repeated MRIs of the brain were normal, and specifically there were no brainstem lesions. A blink reflex showed an isolated absent R1 response on right sided stimulation, consistent with right pontine pathology, though this was the side of supraorbital neurectomy. Brainstem auditory evoked potentials were normal on the right, though all waveforms were absent on the left, consistent with known left sensorineural hearing loss. The patient declined electromyography (EMG) analysis. Blood tests and cerebrospinal fluid including oligoclonal bands were negative.
The patient was not troubled by his eyelid symptoms and declined botulinum toxin.
Discussion
The phenomenology is most reminiscent of right hemifacial spasm, albeit with atypical features of being “gaze‐evoked” and associated with ipsilateral pain. There are also features which overlap with unilateral blepharospasm. The distinction can be difficult. Hemifacial spasm often begins in periocular muscles, with subsequent spread to other facial muscles, though may remain around the eye. Unilateral blepharospasm is rare (17%), with most cases becoming bilateral after a mean interval of 5.8 years. Sensory tricks may be common to both. Pain is exceedingly uncommon in blepharospasm (0.8–5.3%),4 but possibly more common in hemifacial spasm. The Babinski sign is thought to be more specific to hemifacial spasm (seen in no cases of blepharospasm in one series).5
The clinical features in our patient might superficially mimic Duane syndrome, in which aberrant innervation of the 6th and 3rd nerves causes globe retraction on attempted horizontal gaze that produces ptosis. However, unlike Duane syndrome, our patient had normal eye movements, no globe retraction, the presence of sensory tricks and pain.
Hemifacial spasm is a well‐known clinical entity. The presence of “gaze‐evoked” hemifacial spasm, however, is more difficult to explain. It is possible that activation of lateral horizontal saccades and the right sixth nerve nucleus could result in ephaptic transmission and activation of the right facial nerve. Ephaptic transmission has been reported in many conditions, including hemifacial spasm.6 Stimulation of the pontine tegmentum 2 mm lateral to the midline, an area adjacent to the paramedian pontine reticular formation (PPRF) area (concerned with lateral gaze) has been found to produce selective unilateral eyelid closure.7
Patients with similar phenomenology to our patient have been described. Kim et al.8 report the coexistence of ocular neuromyotonia and hemifacial spasm. Carmichael and Critchley in 1925 noticed “facial nystagmus” with lateral gaze movements in patients with MS, which included repetitive contractions involving the ear, frontalis, and corner of the mouth.7 Keane7 described patients with gaze‐evoked blepharoclonus, though this was bilateral. Sethi et al.9 illustrate other various phenomena, including vertigo, tinnitus, blepharoclonus, eyelid nystagmus, facial nystagmus, involuntary laughter, and seizures. They also suggest that the mechanism may relate to ephaptic transmission.
The contribution from the original peripheral sensory pathology is also uncertain. Of interest, trigeminal autonomic cephalalgias have been linked to activation within the trigeminal nucleus caudalis, an area immediately adjacent to the facial nucleus and the paramedian pontine area.10
We present this case for its intriguing phenomenology and in order to alert readers to rare cases with overlapping clinical features reported in the literature.
Disclosures
Ethical Compliance Statement
The authors confirm that the approval of an institutional review board was not required for this work. Written patient consent was obtained and is available on request. 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.
Funding Sources and Conflicts of Interest
There are no funding sources to declare for this manuscript. There are no conflicts of interest to declare for this manuscript.
Funding Disclosure for the Previous 12 Months
Dr Fung receives a salary from NSW Health, has received unrestricted research grants from the Michael J Fox Foundation, Abbvie and Merz, is on Advisory Boards and/or has received travel grants from Abbvie, Allergan, Cavion, Ipsen, Merz, Praxis, Seqirus, Stada, Teva and UCB, and receives royalties from Health Press Ltd.
Dr Martin, Dr Ong and Dr Babu have no funding sources for the past 12 months to declare.
Acknowledgments
We wish to thank the patient.
References
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