Acta Ophthalmologica 2014
(A)
(B)
Fig. 1. (A, B) PlGF concentrations in aqueous humour samples obtained from control, DME and PDR group (A), and PDR and NVG group (B). DME, diabetic macular edema; PDR, proliferative diabetic retinopathy; NVG, neovascular glaucoma. * p < 0.05, ** p < 0.01, *** p < 0.001.
cavity to the anterior chamber during DR progression. It has been shown that retinal pigment epithelial (RPE) cells can produce PlGF in response to growth factors such as bone morphogenetic protein-4, known as a participant in ocular angiogenesis (Hollborn et al. 2006). In addition, it was reported that cultured optic nerve head astrocytes secreted PlGF (Kernt et al. 2010). Therefore, it is possible that RPE and glial cells are cellular sources of PlGF in the diabetic retina. Further investigation is necessary to elucidate the mechanism(s) by which PlGF is elevated in the anterior chamber in case with DR. Therefore, PlGF may be an attractive molecular target in the prevention of pathological angiogenesis, and the current data suggest that targeting PlGF may prove beneficial in the treatment of PDR, particularly in case with NVG.
permeability factor. Proc Natl Acad Sci USA 88: 9267–9271. Yamashita H, Eguchi S, Watanabe K et al. (1999): Expression of placenta growth factor (PlGF) in ischaemic retinal diseases. Eye 13: 372–374.
Correspondence: Kousuke Noda, MD, PhD Department of Ophthalmology Hokkaido University Graduate School of Medicine North 15, West 7, Kita-ku Sapporo, Hokkaido 060-8638 Japan Tel: + 81 11 706 5944 Fax: + 81 11 706 5948 Email: [email protected]
A case of cyclic esotropia with menstrual cycle Ju Yeon Lee, Susie Seok and Sei Yeul Oh
References Autiero M, Waltenberger J, Communi D et al. (2003): Role of PlGF in the intra- and intermolecular cross talk between the VEGF receptors Flt1 and Flk1. Nat Med 9: 936–943. Hollborn M, Tenckhoff S, Seifert M et al. (2006): Human retinal epithelium produces and responds to placenta growth factor. Graefes Arch Clin Exp Ophthalmol 244: 732–741. Kernt M, Liegl RG, Rueping J et al. (2010): Sorafenib protects human optic nerve head astrocytes from light-induced overexpression of vascular endothelial growth factor, platelet-derived growth factor, and placenta growth factor. Growth Factors 28: 211–220. Maglione D, Guerriero V, Viglietto G et al. (1991): Isolation of a human placenta cDNA coding for a protein related to the vascular
e246
Department of ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea doi: 10.1111/aos.12306
Editor, yclic esotropia, alternatively called ‘day squint’, is an intermittent disorder. It usually occurs in repeated cycles of every 48 hr (Hutcheson & Lambert 1998). The fusion and binocularity are usually defective on the strabismic days; in contrast, it remains normal during the straight days. Many studies have tried to clarify the pathophysiology of this rare condition, but exact mechanisms remain
C
unknown (Metz & Searl 1984). We present a monthly cycle of these squints with misalignment associated with the days of the menstrual period that has never been reported yet. A 46-year-old female was referred for ocular pain. She had already been diagnosed with orbital pseudotumor at another clinic and took oral steroids for this condition. She was noted to have 45PD esotropia with diplopia. Six months later, her esotropia had improved without diplopia. We could not see her again until 4 years later when she presented to our clinic for a history of a squint, which was worse during her menstrual period. It typically lasted for 2 weeks each month. At that time, her ocular examination showed orthotropia and no diplopia, and visual acuity was 20/20. Two weeks later, she was noticed to have 35PD esotropia, but no change of visual acuity or refraction error was seen (Fig. 1). Her ocular motility was normal, but binocularity did not evaluated. We suggested a self-diary should be started, so she recorded daily whether she had straight eyes or a manifest squint. The diary clearly showed that the manifest deviation did predictably present during her menstrual periods. The cycle started on the first day of menstrual period, and it terminated at the end of menstrual period. Cyclic fluctuation usually occurred over onemonth time frame. For evaluating the recurrence of orbital pseudotumor, she received CT scans, but we did not find any abnormalities on the CT scans (Fig. 1). The cycle had lasted for 4 months, and the strabismic period became more prolonged as time went on, and at last, her esotropia has lasted for 1 month. Finally, the periodic squint became a constant 40PD esotropia. The patient underwent bilateral medial rectus muscle recession (right, 5.5 mm; left, 5.0 mm). Following the surgery, she has remained orthotropic and free of diplopia during her menstrual period. Cyclic esotropia can be idiopathic or consecutive and also occur with defects of the central nervous system (CNS) (Pillai & Dhand 1987). The proposed mechanism of cyclic esotropia is a disruption of the innate biological clock associated with the CNS (Hutcheson & Lambert 1998). Some authors have suggested that a lesion of the suprachiasmatic nucleus of the
Acta Ophthalmologica 2014
(A)
(B)
(D)
(C)
Fig. 1. (A) She had orthotropia without diplopia during straight period. (B) 35 PD esotropia was noticed during menstrual period. (C) CT scan during menstrual period with esotropia. (D) CT scan during orthotrophic period. There was no structural change or remarkable abnormality on the CT scans.
hypothalamus could disrupt the biological clock and lead to cyclic esotropia (Pillai & Dhand 1987). Our case supports this possible mechanism in that the suprachiasmatic nucleus plays an important role in the course of menstruation (Norman et al. 1976). Several authors proposed that patients with cyclic esotropia have a basically strabismic condition with appended ‘bursts of orthophoria’ (Hutcheson & Lambert 1998). After visual maturity, late-onset cyclic esotropia could develop from conditions that disrupt fusion or cause diplopia (Hutcheson & Lambert 1998). The trigger event could be ocular surgery or CNS problems (Pillai & Dhand 1987). In this case, we could propose that constant esotropia by orbital pseudotumor might disrupt fusion. It is hypothesized that underlying this strabismic condition, our patient had entire days except her menstrual period where she experienced an orthotropic burst; therefore, a trigger factor to suppress orthotropic burst condition might also exist. The trigger factor could be menstrual hormones or unknown endogenous CNS transmitters stimulated by menstrual hormones. In conclusion, we present the first unique case of periodic esotropia with
a monthly cycle and onset along with the menstrual period. In our patient, the development of cyclic esotropia could be related to the disruption of an innate biological clock, and the cycle was probably the result of some interacting factors related to the suprachiasmatic nucleus of the hypothalamus.
References Hutcheson KA & Lambert SR (1998): Cyclic esotropia after a traumatic sixth nerve palsy in a child. J AAPOS 2: 376–377. Metz HS & Searl SS (1984): Cyclic vertical deviation. Trans Am Ophthalmol Soc 82: 158–165. Norman RL, Resko JA & Spies HG (1976): The anterior hypothalamus: how it affects gonadotropin secretion in the rhesus monkey. Endocrinology 99: 59–71. Pillai P & Dhand UK (1987): Cyclic esotropia with central nervous system disease: report of two cases. J Pediatr Ophthalmol Strabismus 24: 237–241.
Correspondence: Sei Yeul Oh Department of ophthalmology Samsung Medical Center Sungkyunkwan University School of Medicine 50 Irwon-dong, Gangnam-gu
Seoul 135-710, Korea Tel: + 82-10-3111-5293 Fax: + 82-2-3410-0029 Email: [email protected]
Delayed diagnosis of oculopharyngeal muscular dystrophy in Denmark: from initial ptosis to genetic testing Aurore Mensah,1 Nanna Witting,2 Morten Duno,3 Dan Milea1,4,5 and John Vissing2 1 Department of Ophthalmology, Glostrup University Hospital, Copenhagen, Denmark; 2Neuromuscular Research Unit, Department of Neurology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark; 3 Department of Clinical Genetics, University of Copenhagen, Copenhagen, Denmark; 4Singapore National Eye Centre, Singapore Eye Research Institute, Singapore; 5Duke-NUS Neuroscience and Behavioral Disorders, Singapore
doi: 10.1111/aos.12243
e247
Copyright of Acta Ophthalmologica (1755375X) is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
(A)
(B)
Fig. 1. (A, B) PlGF concentrations in aqueous humour samples obtained from control, DME and PDR group (A), and PDR and NVG group (B). DME, diabetic macular edema; PDR, proliferative diabetic retinopathy; NVG, neovascular glaucoma. * p < 0.05, ** p < 0.01, *** p < 0.001.
cavity to the anterior chamber during DR progression. It has been shown that retinal pigment epithelial (RPE) cells can produce PlGF in response to growth factors such as bone morphogenetic protein-4, known as a participant in ocular angiogenesis (Hollborn et al. 2006). In addition, it was reported that cultured optic nerve head astrocytes secreted PlGF (Kernt et al. 2010). Therefore, it is possible that RPE and glial cells are cellular sources of PlGF in the diabetic retina. Further investigation is necessary to elucidate the mechanism(s) by which PlGF is elevated in the anterior chamber in case with DR. Therefore, PlGF may be an attractive molecular target in the prevention of pathological angiogenesis, and the current data suggest that targeting PlGF may prove beneficial in the treatment of PDR, particularly in case with NVG.
permeability factor. Proc Natl Acad Sci USA 88: 9267–9271. Yamashita H, Eguchi S, Watanabe K et al. (1999): Expression of placenta growth factor (PlGF) in ischaemic retinal diseases. Eye 13: 372–374.
Correspondence: Kousuke Noda, MD, PhD Department of Ophthalmology Hokkaido University Graduate School of Medicine North 15, West 7, Kita-ku Sapporo, Hokkaido 060-8638 Japan Tel: + 81 11 706 5944 Fax: + 81 11 706 5948 Email: [email protected]
A case of cyclic esotropia with menstrual cycle Ju Yeon Lee, Susie Seok and Sei Yeul Oh
References Autiero M, Waltenberger J, Communi D et al. (2003): Role of PlGF in the intra- and intermolecular cross talk between the VEGF receptors Flt1 and Flk1. Nat Med 9: 936–943. Hollborn M, Tenckhoff S, Seifert M et al. (2006): Human retinal epithelium produces and responds to placenta growth factor. Graefes Arch Clin Exp Ophthalmol 244: 732–741. Kernt M, Liegl RG, Rueping J et al. (2010): Sorafenib protects human optic nerve head astrocytes from light-induced overexpression of vascular endothelial growth factor, platelet-derived growth factor, and placenta growth factor. Growth Factors 28: 211–220. Maglione D, Guerriero V, Viglietto G et al. (1991): Isolation of a human placenta cDNA coding for a protein related to the vascular
e246
Department of ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea doi: 10.1111/aos.12306
Editor, yclic esotropia, alternatively called ‘day squint’, is an intermittent disorder. It usually occurs in repeated cycles of every 48 hr (Hutcheson & Lambert 1998). The fusion and binocularity are usually defective on the strabismic days; in contrast, it remains normal during the straight days. Many studies have tried to clarify the pathophysiology of this rare condition, but exact mechanisms remain
C
unknown (Metz & Searl 1984). We present a monthly cycle of these squints with misalignment associated with the days of the menstrual period that has never been reported yet. A 46-year-old female was referred for ocular pain. She had already been diagnosed with orbital pseudotumor at another clinic and took oral steroids for this condition. She was noted to have 45PD esotropia with diplopia. Six months later, her esotropia had improved without diplopia. We could not see her again until 4 years later when she presented to our clinic for a history of a squint, which was worse during her menstrual period. It typically lasted for 2 weeks each month. At that time, her ocular examination showed orthotropia and no diplopia, and visual acuity was 20/20. Two weeks later, she was noticed to have 35PD esotropia, but no change of visual acuity or refraction error was seen (Fig. 1). Her ocular motility was normal, but binocularity did not evaluated. We suggested a self-diary should be started, so she recorded daily whether she had straight eyes or a manifest squint. The diary clearly showed that the manifest deviation did predictably present during her menstrual periods. The cycle started on the first day of menstrual period, and it terminated at the end of menstrual period. Cyclic fluctuation usually occurred over onemonth time frame. For evaluating the recurrence of orbital pseudotumor, she received CT scans, but we did not find any abnormalities on the CT scans (Fig. 1). The cycle had lasted for 4 months, and the strabismic period became more prolonged as time went on, and at last, her esotropia has lasted for 1 month. Finally, the periodic squint became a constant 40PD esotropia. The patient underwent bilateral medial rectus muscle recession (right, 5.5 mm; left, 5.0 mm). Following the surgery, she has remained orthotropic and free of diplopia during her menstrual period. Cyclic esotropia can be idiopathic or consecutive and also occur with defects of the central nervous system (CNS) (Pillai & Dhand 1987). The proposed mechanism of cyclic esotropia is a disruption of the innate biological clock associated with the CNS (Hutcheson & Lambert 1998). Some authors have suggested that a lesion of the suprachiasmatic nucleus of the
Acta Ophthalmologica 2014
(A)
(B)
(D)
(C)
Fig. 1. (A) She had orthotropia without diplopia during straight period. (B) 35 PD esotropia was noticed during menstrual period. (C) CT scan during menstrual period with esotropia. (D) CT scan during orthotrophic period. There was no structural change or remarkable abnormality on the CT scans.
hypothalamus could disrupt the biological clock and lead to cyclic esotropia (Pillai & Dhand 1987). Our case supports this possible mechanism in that the suprachiasmatic nucleus plays an important role in the course of menstruation (Norman et al. 1976). Several authors proposed that patients with cyclic esotropia have a basically strabismic condition with appended ‘bursts of orthophoria’ (Hutcheson & Lambert 1998). After visual maturity, late-onset cyclic esotropia could develop from conditions that disrupt fusion or cause diplopia (Hutcheson & Lambert 1998). The trigger event could be ocular surgery or CNS problems (Pillai & Dhand 1987). In this case, we could propose that constant esotropia by orbital pseudotumor might disrupt fusion. It is hypothesized that underlying this strabismic condition, our patient had entire days except her menstrual period where she experienced an orthotropic burst; therefore, a trigger factor to suppress orthotropic burst condition might also exist. The trigger factor could be menstrual hormones or unknown endogenous CNS transmitters stimulated by menstrual hormones. In conclusion, we present the first unique case of periodic esotropia with
a monthly cycle and onset along with the menstrual period. In our patient, the development of cyclic esotropia could be related to the disruption of an innate biological clock, and the cycle was probably the result of some interacting factors related to the suprachiasmatic nucleus of the hypothalamus.
References Hutcheson KA & Lambert SR (1998): Cyclic esotropia after a traumatic sixth nerve palsy in a child. J AAPOS 2: 376–377. Metz HS & Searl SS (1984): Cyclic vertical deviation. Trans Am Ophthalmol Soc 82: 158–165. Norman RL, Resko JA & Spies HG (1976): The anterior hypothalamus: how it affects gonadotropin secretion in the rhesus monkey. Endocrinology 99: 59–71. Pillai P & Dhand UK (1987): Cyclic esotropia with central nervous system disease: report of two cases. J Pediatr Ophthalmol Strabismus 24: 237–241.
Correspondence: Sei Yeul Oh Department of ophthalmology Samsung Medical Center Sungkyunkwan University School of Medicine 50 Irwon-dong, Gangnam-gu
Seoul 135-710, Korea Tel: + 82-10-3111-5293 Fax: + 82-2-3410-0029 Email: [email protected]
Delayed diagnosis of oculopharyngeal muscular dystrophy in Denmark: from initial ptosis to genetic testing Aurore Mensah,1 Nanna Witting,2 Morten Duno,3 Dan Milea1,4,5 and John Vissing2 1 Department of Ophthalmology, Glostrup University Hospital, Copenhagen, Denmark; 2Neuromuscular Research Unit, Department of Neurology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark; 3 Department of Clinical Genetics, University of Copenhagen, Copenhagen, Denmark; 4Singapore National Eye Centre, Singapore Eye Research Institute, Singapore; 5Duke-NUS Neuroscience and Behavioral Disorders, Singapore
doi: 10.1111/aos.12243
e247
Copyright of Acta Ophthalmologica (1755375X) is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
