EXPERTOPINION Edited by Randolph W. Evans, MD What’s the Cause of This Mysterious Eyelid Twitch?

Eyelid can signify brainstem disease — or it could be as benign as psychosomatic reaction to stress. Here’s how to tell the difference.

Case history submitted by: ent, the likely diagnosis is benign essen- normal. A second patient developed MS, Randolph W. Evans, MD tial (BEB). If BEB is and a third patient was diagnosed with present but there are also of the Alzheimer’s disease, but in neither of these Expert opinion by: Rudrani Banik, MD perioral area, tongue or neck muscles, cases was the eyelid myokymia directly then consider Meige syndrome.3 linked. Furthermore in the study, neu- Case • What is the etiology? Eyelid myo- roimaging was negative in 13 of 15 A 25-year-old woman presents with a sev- kymia is a subset of facial myokymia. patients (87 percent).8 eral-week history of twitching of the right Facial myokymia tends to be associated Thus, eyelid myokymia tends to be a lower eyelid lasting up to a few minutes with underlying neurologic disease affect- benign condition, different from facial and occurring 5-6 times per day. During ing the facial nerve nucleus in the pons or myokymia. The etiology of the former the last several weeks, she has been under the peripheral nerve. Intrinsic brainstem condition remains unclear. It may be increased stress due to law school finals and disease such as pontine glioma4 or hypothesized that because the process is has had less sleep than usual. She drinks a demyelination from multiple sclerosis5 localized to the eyelid and spares the few cups of coffee a day. Past medical histo- may result in facial myokymia, as may remaining facial muscle groups, the lesion ry is negative. Neurological examination extrinsic disease such as obstructive is quite peripheral; nerve fibers which was normal except for a single episode of hydrocephalus or an extra-axial innervate the orbicularis oculi may be fine contractions of the right lower eyelid compressing the brainstem.3 selectively affected and hyperexcitable.8 lasting about 15 seconds. The common final pathway in facial However, no electrophysiologic studies What is your diagnosis? myokymia is deafferentation of nerve have yet been performed on eyelid myo- fibers—be it supranuclear, perinuclear or kymia patients in order to better under- Expert Opinion infranuclear—which in turn leads to dis- stand the nature of this disorder. This patient’s complaint of new-onset inhibition and hyperexcitability of facial Both ocular surface disorders as well as eyelid twitching occurring intermittently nerve fibers.6,7 Ephaptic transmission psychosocial factors should be investigat- but on a daily basis is known as eyelid with “cross-talk” of fibers or ectopic exci- ed in cases of new onset, isolated eyelid myokymia. Myokymia is a condition in tation may occur in neighboring branch- myokymia. Local eyelid irritation from which involuntary, fine, undulating con- es of the facial nerve, which then mani- such entities as dry eye, ocular allergy, or a tractions occur in select striated muscle fests as myokymia. foreign body may precipitate symptoms. groups, in this case confined to the eyelid. However unlike facial myokymia, Lifestyle factors such as , lack of Electrophysiologically, the contractions when the myokymic symptoms are lim- sleep, physical exertion, stress, smoking, are characterized by rhythmic or semi- ited to the eyelid, no underlying neuro- alcohol, or excess caffeine intake, may also rhythmic bursts of a single motor unit dis- logic condition tends to be associated. A play a role in the onset of eyelid myo- charging several times per second.1 These recent study of 15 patients with chronic kymia.3 myokymic discharges are non-synchro- isolated eyelid myokymia showed that in • Can the upper eyelid ever be nous in different motor units within the long-term follow-up (12-240 months, involved? Eyelid myokymia is usually same muscle,2 thereby producing the fine, with a mean of 91 months), 12 patients unilateral, affecting the lower lid. How- undulating appearance. (80 percent) had no evidence of any type ever, on occasion the upper eyelid may be Eyelid myokymia tends to be unilat- of neurologic disorder.8 Of the remain- involved. If so, this should not raise suspi- eral and localized. If additional lower ing three patients, in only one patient cion of any underlying neurologic lesion. facial muscle groups are involved, con- who developed ipsilateral hemifacial • When is diagnostic testing indicat- sider facial myokymia or hemifacial spasm did the spasms herald progression ed? When the myokymia remains isolated spasm. If bilateral eyelid spasms are pres- of disease, though neuroimaging was to the eyelid, even if chronic, no further

72 Practical Neurology July 2005 8 1. Hjorth RJ, Willison RG. The electromyogram in facial myokymia work-up such as neuroimaging is warrant- spontaneously remit at any time. and hemifacial spasm. J Neurol Sci 1973;20:117-126. ed. However, if there is spread to neigh- • What treatment would you recom- 2. Horowitz SH. Hemifacial spasm and facial myokymia: electro- boring ipsilateral facial muscle groups, an mend? In the case of new-onset isolated physiological findings. Muscle and Nerve 1987;10:422-7. 3. Miller NR. Walsh & Hoyt's Clinical Neuro-Ophthalmology, 5th ed. underlying cause for the symptoms should eyelid myokymia, any exacerbating fac- Vol 1. Philadelphia: Williams & Wilkins, 1998;1571. be investigated. As noted previously, facial tors such as eyelid irritation or lifestyle 4. Gutmann L, Hopf HC. Facial myokymia and contraction persisting myokymia tends to be associated with factors must first be identified and 20 years: A case of pontine glioma. Muscle Nerve 1994;17:1461-3. 5. Jacobs L, Kaba S, Pullicino P. The lesion causing continuous intrinsic or extrinsic disease of the brain- addressed. For example, in the case facial myokymia in . Arch Neurol 1994;51:1115-9. stem or peripheral facial nerve; by con- above, the patient should be first advised 6. Merchut MP, Biller, J, Brumlik J, et al. Isolated facial myokymia 8 and facial contracture: computed tomography and magnetic reso- trast, eyelid myokymia is not. to try to reduce her stress level, improve nance imaging correlation. J Clin Neuro-ophthalmol 1985;5:120-3. • What is the prognosis? Eyelid on her sleep, as well as decrease caffeine 7. Oge AE, Boyaciyan A, Sarp A, et al. Facial myokymia: segemental demyelination demonstrated by magnetic stimulation. Muscle and myokymia, whether transient or chronic, intake. Nerve 1996;19:246-9. is a benign entity with a good prognosis If these lifestyle modifications are not 8. Banik R, Miller NR. Chronic myokymia limited to the eyelid is a for spontaneous resolution. When local possible or not effective in eliminating benign condition. J Neuro-Ophthalmol 2004;24:290-2. 9. Ruusuvaara P, Setala K. Long-term treatment of involuntary facial eyelid irritation or psychosocial factors the eyelid myokymia, and symptoms spasms using botulinum toxin. Acta Ophthalm 1990;68:331-8. are at play (as in the case in the patient have persisted continuously for greater 10. Sedano MJ, Trejo JM, Macarron JL, et al. Continuous facial myokymia in multiple sclerosis: treatment with botulinum toxin. Eur described above), the myokymia will like- than three months, then treatment may Neurol 2000;43:137-140. ly be transient. Symptoms commonly be initiated. Botulinum toxin in low 11. Jordan DR, Anderson RL, Thiese SM. Intractable orbicularis regress within several weeks to months, doses to the eyelid is quite effective and, myokymia: treatment alternatives. Ophthal Surg 1989;20:280-3. particularly upon removal of the aggra- in some instances, may even be cura- Rudrani Banik, MD is Assistant Professor in the 8,9,10 vating factor(s). However, even when the tive. Another alternative is surgical Departments of Ophthalmology and Neurological myokymia is persistent, lasting beyond myectomy of the affected orbicularis Surgery and Director of Neuro-Ophthalmology Service at Albert Einstein College of Medicine/Montefiore three months and even up to several oculi muscle, although even with resec- Medical Center in Bronx, New York. decades, the symptoms may occasionally tion symptoms may recur.11 PN