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Blepharospasm as a masquerade of glaucomatous visual field defects
CASE REPORT Blepharospasm as a masquerade of glaucomatous visual field defects Glaucoma is one of the leading causes of visual loss worldwide. Primary open-angle glaucoma, the most common type, is estimated to rise in global incidence from 57.5 million individuals in 2015 to 65.5 by 2020,1 with 5.9 million having bilateral blindness from the disease.2 With a growing population of patients with glaucoma, it is imperative to properly monitor for the disease and its progression and escalate therapy when appropriate. Alongside careful clinical examination, automated perimetry is an established, quintessential tool in glaucoma evaluation. Detecting true glaucomatous field progression from secondary defects may often be challenging. A multitude of factors causing inauthentic perimetry defects have been described, including pupil size,3 refractive error,4 lens rim artifact,5 blepharoptosis, cataract,6–8 multifocal intraocular lens implants,9 and patient learning or fatigue.10 Here, we present a case of intermittent blepharospasm yielding glaucomatous-like changes on automated perimetry.
CASE REPORT A 72-year-old Thai-American male patient was referred to a glaucoma specialist for evaluation and treatment of low-tension glaucoma. He was diagnosed with glaucoma 2 years before referral, with baseline intraocular pressures (IOPs) in the high teens. With a nightly prostaglandin eye drop, IOPs were maintained in the midteens. Further history revealed mild myopic astigmatism, mild nuclear sclerotic cataracts, and non-neovascular age-related macular degeneration treated with ocular vitamins. His medical history included well-controlled hypertension for the past 15 years, sleep apnea treated with nightly continuous positive airway pressure ventilation, hypercholesterolemia, and diet-controlled diabetes mellitus.
On initial evaluation, the patient noted intermittent blurring of vision with dryness and burning sensation in both eyes. Examination was notable for a best-corrected Snellen visual acuity of 20/20 OD and 20/25 OS, pressures of 16 mm Hg OD and 14 OS via Goldman applanation tonometry, 1þ inferior corneal punctate epithelial erosions, open angles on gonioscopy, and moderate to large optic nerve head cups with inferior notches in both neuroretinal rims (Fig. 1). A 24-2 Humphrey automated visual field perimetry (Humphrey Field Analyzer Model 740i; Carl Zeiss Meditec Inc, Dublin, Calif.) revealed superior arcuate changes in both eyes (left eye represented in Fig. 2), correlating with structural nerve changes. Repeat examination 3 months later revealed IOP of 15 mm Hg OU, a stable arcuate defect in the right eye, and improved, scattered superior defects of the left eye (Fig. 3). Examinations every 3 months over the next year were stable for mild, inferior corneal epithelial erosions and IOPs in the midteens to low teens; furthermore, 24-2 visual field examinations every 3 months were also stable, until development of a superior arcuate change in the left eye approximately 1 year after presentation (Fig. 4). Dilated fundus examination at this visit did not reveal any retinal pathology corresponding to observed visual field defects. At this time, however, the patient noted tightness around the left upper and lower eyelid, which may have been sporadically occurring over the past few years. Upon voluntary, forceful contraction of the orbicularis oculi and oris, hemifacial spasm was elicited. The patient was referred for brain magnetic resonance imaging, which was negative for brainstem pathology or compression of the 7th cranial nerve. He was then referred to an oculoplastics specialist, who treated him with botox therapy to the left upper and lower orbicularis oculi, twice in a 3-month period. At glaucoma clinic visits after 3 and 6 months after botox, the patient noted stable resolution of eyelid
Fig. 1 — Fundus photographs of the right and left optic nerves showing inferior notches OU. CAN J OPHTHALMOL — VOL. ], NO. ], ] 2016
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Case Report
Fig. 2 — Initial Humphrey visual field with a superior arcuate defect (left eye).
Fig. 3 — Humphrey visual field 3 months after initial evaluation, with improvement of arcuate defect (left eye).
tightness. Visual field examinations at both visits showed resolution of the superior arcuate defects (6-month visit represented in Fig. 5).
DISCUSSION Blepharospasm is an involuntary, episodic contraction of the eyelid muscles that occurs in both primary and secondary forms, as well as part of hemifacial spam. Primary blepharospasm is a dystonia originating in the basal ganglia and manifests as uncontrolled, forceful contracture of eyelid musculature. Secondary blepharospasm
also involves involuntary contracture of eyelid muscles and is implicated in various syndromes and causes of ocular surface irritation, including dry eye syndrome or preservative load from ophthalmic drops. There is a paucity of research examining the relationship between blepharospasm and glaucoma, without conclusive evidence linking the two.11,12 Similarly, there is a lack of research investigating the effects of belpharospasm on automated perimetry. In addition to central nervous system etiologies, psychological and environmental stressors during perimetry testing, including desiccation of an already compromised ocular surface, may also lead to
Fig. 4 — Humphrey visual field 1 year after presentation, with superior arcuate changes after previously normal, stable fields (left eye).
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Case Report
Fig. 5 — Six months after botox therapy—stable, improved superior arcuate changes (left eye).
blepharospasm and fallacious defects on automated perimetry. As this may lead to inappropriate escalation of therapy, we believe that the effects of blepharospasm should be considered in the same vein as other causes of spurious perimetry defects and a low threshold for treatment of blepharospasm be maintained in glaucoma patients.
Disclosure: The authors have no proprietary or commercial interest in any materials discussed in this article.
Mark S. Dikopf, MD, Pete Setabutr, MD, Thasarat S. Vajaranant, MD Illinois Eye and Ear Infirmary, Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, Illinois. Correspondence to: Mark Dikopf, MD: [email protected] REFERENCES 1. Kapetanakis VV, Chan MP, Foster PJ, Cook DG, Owen CG, Rudnicka AR. Global variations and tie trends in the prevalence of primary open angle glaucoma (POAG): A systematic review and meta-analysis. Br J Ophthalmol. 2016;100:86-93. 2. Quigley HA, Broman AT. The number of people with glaucoma worldwide in 2010 and 2020. Br J Ophthalmol. 2006;90:262-7.
3. Mikelberg FS, Drance SM, Schulzer MD, Wijsman K. The effect of miosis on visual field indices. In: Greve EL, Heijl A, eds. Seventh International Visual Field Symposium. Doc Ophthalmol Proc Ser 49. Dordrecht: Martinus Nijhoff/Dr W Junk; 1987:645-9. 4. Weinreb RN, Perlman JP. The effect of refractive correction on automated perimetric thresholds. Am J Ophthalmol. 1986;101: 706-709. 5. Zalta AH. Lens rim artifact in automated threshold perimetry. Ophthalmology. 1989;96:1302-11. 6. Heuer DK, Anderson DR, Knighton RW, Feuer WJ, Gressel MG. The influence of simulated light scattering on automated perimetric threshold measurements. Arch Ophthalmol. 1988;106:1247-51. 7. Lam BL, Alward WL, Kolder HE. Effect of cataract on automated perimetry. Ophthalmology. 1991;98:1066-70. 8. Budenz DL, Feuer WJ, Anderson DR. The effect of simulated cataract on glaucomatous visual field. Ophthalmology. 1993;100: 511-7. 9. Farid M, Chak G, Garg S, Steinert RF. Reduction in mean deviation values in automated perimetry in eyes with multifocal compared to monofocal intraocular lens implants. Am J Ophthalmol. 2014; 158:227-31. 10. Wild JM, Searle AE, Dengler-Harles M, O’Neill EC. Long-term follow-up of baseline learning and fatigue effects in the automated perimetry of glaucoma and ocular hypertensive patients. Acta Ophthalmol (Copenh). 1991;69:210-6. 11. Nicoletti AG, Zacharias LC, Susanna R Jr., Matavoshi S. Patients with essential blepharospasm and glaucoma: Case reports. Arq Bras Oftalmol. 2008;71:747-51. 12. Lee MS, Harrison AR, Grossman DS, Sloan FA. Risk of glaucoma among patients with benign essential blepharospasm. Ophthal Plast Reconstr Surg. 2010;26:434-7. Can J Ophthalmol 2016;]:]]]–]]] 0008-4182/16/$-see front matter & 2016 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2016.11.023
CAN J OPHTHALMOL — VOL. ], NO. ], ] 2016
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CASE REPORT A 72-year-old Thai-American male patient was referred to a glaucoma specialist for evaluation and treatment of low-tension glaucoma. He was diagnosed with glaucoma 2 years before referral, with baseline intraocular pressures (IOPs) in the high teens. With a nightly prostaglandin eye drop, IOPs were maintained in the midteens. Further history revealed mild myopic astigmatism, mild nuclear sclerotic cataracts, and non-neovascular age-related macular degeneration treated with ocular vitamins. His medical history included well-controlled hypertension for the past 15 years, sleep apnea treated with nightly continuous positive airway pressure ventilation, hypercholesterolemia, and diet-controlled diabetes mellitus.
On initial evaluation, the patient noted intermittent blurring of vision with dryness and burning sensation in both eyes. Examination was notable for a best-corrected Snellen visual acuity of 20/20 OD and 20/25 OS, pressures of 16 mm Hg OD and 14 OS via Goldman applanation tonometry, 1þ inferior corneal punctate epithelial erosions, open angles on gonioscopy, and moderate to large optic nerve head cups with inferior notches in both neuroretinal rims (Fig. 1). A 24-2 Humphrey automated visual field perimetry (Humphrey Field Analyzer Model 740i; Carl Zeiss Meditec Inc, Dublin, Calif.) revealed superior arcuate changes in both eyes (left eye represented in Fig. 2), correlating with structural nerve changes. Repeat examination 3 months later revealed IOP of 15 mm Hg OU, a stable arcuate defect in the right eye, and improved, scattered superior defects of the left eye (Fig. 3). Examinations every 3 months over the next year were stable for mild, inferior corneal epithelial erosions and IOPs in the midteens to low teens; furthermore, 24-2 visual field examinations every 3 months were also stable, until development of a superior arcuate change in the left eye approximately 1 year after presentation (Fig. 4). Dilated fundus examination at this visit did not reveal any retinal pathology corresponding to observed visual field defects. At this time, however, the patient noted tightness around the left upper and lower eyelid, which may have been sporadically occurring over the past few years. Upon voluntary, forceful contraction of the orbicularis oculi and oris, hemifacial spasm was elicited. The patient was referred for brain magnetic resonance imaging, which was negative for brainstem pathology or compression of the 7th cranial nerve. He was then referred to an oculoplastics specialist, who treated him with botox therapy to the left upper and lower orbicularis oculi, twice in a 3-month period. At glaucoma clinic visits after 3 and 6 months after botox, the patient noted stable resolution of eyelid
Fig. 1 — Fundus photographs of the right and left optic nerves showing inferior notches OU. CAN J OPHTHALMOL — VOL. ], NO. ], ] 2016
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Case Report
Fig. 2 — Initial Humphrey visual field with a superior arcuate defect (left eye).
Fig. 3 — Humphrey visual field 3 months after initial evaluation, with improvement of arcuate defect (left eye).
tightness. Visual field examinations at both visits showed resolution of the superior arcuate defects (6-month visit represented in Fig. 5).
DISCUSSION Blepharospasm is an involuntary, episodic contraction of the eyelid muscles that occurs in both primary and secondary forms, as well as part of hemifacial spam. Primary blepharospasm is a dystonia originating in the basal ganglia and manifests as uncontrolled, forceful contracture of eyelid musculature. Secondary blepharospasm
also involves involuntary contracture of eyelid muscles and is implicated in various syndromes and causes of ocular surface irritation, including dry eye syndrome or preservative load from ophthalmic drops. There is a paucity of research examining the relationship between blepharospasm and glaucoma, without conclusive evidence linking the two.11,12 Similarly, there is a lack of research investigating the effects of belpharospasm on automated perimetry. In addition to central nervous system etiologies, psychological and environmental stressors during perimetry testing, including desiccation of an already compromised ocular surface, may also lead to
Fig. 4 — Humphrey visual field 1 year after presentation, with superior arcuate changes after previously normal, stable fields (left eye).
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Case Report
Fig. 5 — Six months after botox therapy—stable, improved superior arcuate changes (left eye).
blepharospasm and fallacious defects on automated perimetry. As this may lead to inappropriate escalation of therapy, we believe that the effects of blepharospasm should be considered in the same vein as other causes of spurious perimetry defects and a low threshold for treatment of blepharospasm be maintained in glaucoma patients.
Disclosure: The authors have no proprietary or commercial interest in any materials discussed in this article.
Mark S. Dikopf, MD, Pete Setabutr, MD, Thasarat S. Vajaranant, MD Illinois Eye and Ear Infirmary, Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, Illinois. Correspondence to: Mark Dikopf, MD: [email protected] REFERENCES 1. Kapetanakis VV, Chan MP, Foster PJ, Cook DG, Owen CG, Rudnicka AR. Global variations and tie trends in the prevalence of primary open angle glaucoma (POAG): A systematic review and meta-analysis. Br J Ophthalmol. 2016;100:86-93. 2. Quigley HA, Broman AT. The number of people with glaucoma worldwide in 2010 and 2020. Br J Ophthalmol. 2006;90:262-7.
3. Mikelberg FS, Drance SM, Schulzer MD, Wijsman K. The effect of miosis on visual field indices. In: Greve EL, Heijl A, eds. Seventh International Visual Field Symposium. Doc Ophthalmol Proc Ser 49. Dordrecht: Martinus Nijhoff/Dr W Junk; 1987:645-9. 4. Weinreb RN, Perlman JP. The effect of refractive correction on automated perimetric thresholds. Am J Ophthalmol. 1986;101: 706-709. 5. Zalta AH. Lens rim artifact in automated threshold perimetry. Ophthalmology. 1989;96:1302-11. 6. Heuer DK, Anderson DR, Knighton RW, Feuer WJ, Gressel MG. The influence of simulated light scattering on automated perimetric threshold measurements. Arch Ophthalmol. 1988;106:1247-51. 7. Lam BL, Alward WL, Kolder HE. Effect of cataract on automated perimetry. Ophthalmology. 1991;98:1066-70. 8. Budenz DL, Feuer WJ, Anderson DR. The effect of simulated cataract on glaucomatous visual field. Ophthalmology. 1993;100: 511-7. 9. Farid M, Chak G, Garg S, Steinert RF. Reduction in mean deviation values in automated perimetry in eyes with multifocal compared to monofocal intraocular lens implants. Am J Ophthalmol. 2014; 158:227-31. 10. Wild JM, Searle AE, Dengler-Harles M, O’Neill EC. Long-term follow-up of baseline learning and fatigue effects in the automated perimetry of glaucoma and ocular hypertensive patients. Acta Ophthalmol (Copenh). 1991;69:210-6. 11. Nicoletti AG, Zacharias LC, Susanna R Jr., Matavoshi S. Patients with essential blepharospasm and glaucoma: Case reports. Arq Bras Oftalmol. 2008;71:747-51. 12. Lee MS, Harrison AR, Grossman DS, Sloan FA. Risk of glaucoma among patients with benign essential blepharospasm. Ophthal Plast Reconstr Surg. 2010;26:434-7. Can J Ophthalmol 2016;]:]]]–]]] 0008-4182/16/$-see front matter & 2016 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2016.11.023
CAN J OPHTHALMOL — VOL. ], NO. ], ] 2016
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