Issue: May/June 2020
Disclosures: Semes, Skorin and Tran report no relevant financial disclosures.
June 22, 2020
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Patient experiences unilateral eyelid spasms

Issue: May/June 2020
Disclosures: Semes, Skorin and Tran report no relevant financial disclosures.
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A 42-year-old white female was referred to our clinic by her optometrist for an evaluation of spasms of her right upper and lower eyelids. The patient reported that these spasms began within the last 2 years. They occur every day and are continuous throughout the day. The spasm would sometimes involve the ipsilateral cheek area. The patient did not recall any specific cause for the spasms, but the symptoms seemed to get worse throughout the day and with caffeine intake. She was uncertain whether the spasms persisted while asleep. She also reported increased light sensitivity. The patient denied any reduced hearing, vertigo or balance issues.

The patient’s ocular history was unremarkable. There was no significant family ocular history. Her medical history included multiple cesarean sections and a laparoscopic appendectomy. The patient reported no known allergies and was not currently taking any medications. She denied any illicit drug use and was a nonsmoker.

Christina Tran
Leonid Skorin Jr.

Uncorrected distance visual acuities were 20/20 OD and 20/20 OS. Her pupils were equal, round and reactive to light with no relative afferent pupillary defect. Extraocular motilities were full without restrictions. Confrontation visual fields were full to finger count in both eyes. IOPs using the Icare tonometer (Icare Finland) were 14 mm Hg OD and 12 mm Hg OS. During the exam, spasms of the right upper and lower eyelids were observed. The spasms appeared to be localized to the palpebral portion of the orbicularis oculi muscle. During the spasms, the eyelids never closed completely.

Review of the exam findings from the patient’s comprehensive eye examination by the referring optometrist 11 weeks earlier were consistent with the results found at our exam. The patient previously tried reducing her caffeine intake and has tried using over-the-counter allergy eye drops and artificial tears. None of these treatments showed any improvement in her spasm activity.

Series of axial MRIs shows tortuous course of left vertebral artery indicated by the red arrow. Scans (left to right) progress superiorly from the level of the medulla to the base of the pons.

Source: Leonid Skorin Jr., OD, DO, MS, FAAO, FAOCO

Based on the patient’s history and exam findings, neuroimaging was ordered to evaluate for possible intracranial pathology. Magnetic resonance imaging (MRI) of the brain with and without contrast was ordered and performed the following day. The MRI showed no midline shift, mass effect or abnormal extra-axial fluid collection. There were no abnormal foci of restricted diffusion or contrast enhancement. The MRI revealed an anatomical variant of the left vertebral artery V4 segment. The artery took a tortuous course by crossing over the midline of the brainstem before joining the right vertebral artery and becoming the basilar artery.

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What’s your diagnosis?

See answer below.

Differential diagnoses for patients with spasms of the eyelid muscles include benign essential blepharospasm, Meige syndrome, Breughel syndrome and hemifacial spasm. It is important for eye care clinicians to recognize the differences in etiology and presentation for these conditions and to know when an MRI scan of the brain is warranted.

Benign essential blepharospasm

Benign essential blepharospasm (BEB) is an idiopathic condition characterized by bilateral contractions of the orbicularis oculi, procerus and corrugator muscles resulting in closure of the eyelids. BEB is three times more prevalent in women than men, and patients are typically 50 to 70 years old (MacIntosh). Patients will experience an increase in spontaneous blinking frequency and duration lasting from seconds to minutes. They may progress to having contractions of other muscles of facial expression and muscles of the neck. In contrast to hemifacial spasm, BEB is actually relieved by sleep and almost always presents bilaterally. Neuroimaging is required only if spasms are unilateral or related to other neurologic symptoms. Once a diagnosis of BEB is established, neuroimaging is not required.

The pathophysiology is explained by overactivity of the blinking control center. Conditions such as dry eye and conjunctivitis are known to exacerbate eyelid spasms by causing secondary blinking. BEB can result in inflammation and worsen dry eye symptoms as well. One study determined that 40% to 60% of patients reported symptoms of dry eye before or at the onset of BEB (MacIntosh). This study also found that treating BEB could actually improve dry eye symptoms. Another common finding in patients with BEB is an increase in light sensitivity. Photophobia occurs in approximately 80% of patients with BEB and is known to trigger eyelid spasms (MacIntosh).

Conservative treatments for photophobia include sunglasses or the use of FL-41 tint. This tint has been shown to slow the average blink rate and the force of eyelid contraction by filtering out various wavelengths of blue and green light. The most effective management option of BEB is botulinum toxin injections to minimize the muscle spasms. Some oral medications for the management of BEB include muscle relaxants such as baclofen, anticonvulsants such as carbamazepine and gabapentin, and antidopaminergics such as levodopa. These medications have shown inconsistent results and have a higher risk for adverse systemic effects compared with botulinum toxin injections. For patients who may not have had success with either of the previously mentioned management options, surgical treatments include eyelid protractor myectomy, frontalis suspension and differential section of the facial nerve branches.

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Meige, Breughel syndromes

Meige syndrome and Breughel syndrome are idiopathic conditions characterized by blepharospasm combined with dystonia of muscles in the lower half of the face. Meige syndrome presents with orofacial dystonia such as lip pursing, tongue protrusion, lockjaw and difficulty speaking. Breughel syndrome presents with oromandibular dystonia such as contraction of the jaw muscles and a wide-open mouth. Patients with these conditions are most commonly middle-aged to elderly women.

The pathophysiology is known to be related to hyperactivity of dopamine or dysfunction of the basal ganglia. The muscle spasms are exacerbated by stress but relieved by sleep.

Medications include muscle relaxants such as baclofen, anticholinergic agents such as trihexyphenidyl, or antidopaminergics such as levodopa. Some patients may benefit from facial nerve block as well (Marschall).

Hemifacial spasm

Hemifacial spasm (HFS) is characterized by unilateral contractions of the muscles innervated by the facial nerve. Painless, intermittent spasms of the orbicularis oculi muscle are a common initial presentation, and then more inferior muscles of facial expression can become involved over time. Atypical HFS starts in the orbicularis oris, which is a muscle found around the mouth, and progresses superiorly to affect the orbicularis oculi muscle later. Spasms can increase in intensity and frequency with the potential of becoming a sustained tonic contraction and negatively impact a patient’s quality of life.

The pathophysiology of HFS is explained by mechanical compression or irritation of the facial nerve at its root exit zone in primary HFS or anywhere else along the facial nerve in secondary HFS. Sources of compression or irritation may include vasculature such as the anterior inferior cerebellar artery, posterior inferior cerebellar artery, basilar artery, vertebral artery, arteriovenous malformations or aberrant veins. Other causes may also include acoustic schwannomas, meningiomas, brainstem lesions, structural malformations, infections, Bell palsy and parotid gland tumors. Therefore, MRI scans of the brain in all newly diagnosed cases of HFS are necessary to look for intracranial pathology to determine the appropriate course of treatment and management.

The patient was diagnosed with hemifacial spasm secondary to an aberrant left vertebral artery near the root exit zone of the facial nerve at the base of the pons.

Treatment, management

Treatments for HFS include injections of botulinum toxin, medications and surgery. The most popular choice is botulinum toxin injections although oral pharmacotherapy and microsurgical decompression may be beneficial for certain patients.

The botulinum toxin is injected directly into the activated muscles. The patient will need repeat injections every 3 to 6 months. Spasms may also be managed with medications including muscle relaxants such as baclofen, anticonvulsants such as carbamazepine, anticholinergic agents such as trihexyphenidyl or antidopaminergics such as levodopa. Microvascular decompression surgery is the only option that can totally resolve HFS, whereas botulinum toxin therapy and oral medications help manage the symptoms temporarily.

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The surgery, known as the Jannetta procedure, aims to eliminate compression or irritation of the facial nerve. The surgeon exposes the facial nerve and then inserts a small sponge between the facial nerve and the vessel that is compressing or irritating the facial nerve. The percentage of patients with successful relief of hemifacial spasm after microvascular decompression surgery is estimated to be between 85% and 90% (Sindou et al). It is important to note that it is intracranial surgery, so it is primarily reserved for patients who do not respond to botulinum toxin injections or need a permanent cure for HFS.

Patient outcome

The patient was educated about the options for treating her hemifacial spasm. Botulinum toxin injections were presented as the least invasive and most effective choice of treatment. Specifically, botulinum toxin would be injected into the right upper and lower eyelids. Injections would need to be repeated approximately every 3 to 6 months depending on how long the effects of the medication last. Due to the benign nature of her spasm, the patient has decided to not pursue treatment at this time.