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Show LETTERS TO THE EDITOR Treatment of Nonarteritic Anterior Ischemic Optic Neuropathy With Intravitreal Bevacizumab Vascular endothelial growth factor ( VEGF) results in a rapid and reversible increase in vascular permeability ( 1). Inhibition of VEGF signaling therefore provides an avenue for reducing vasogenic edema after nonarteritic ischemic optic neuropathy ( NAION) and preserving viable but threatened optic nerve tissue. We report the first use of intravitreal bevacizumab ( Genentech, South San Francisco, CA) for the treatment of NAION demonstrating a rapid and substantial reduction in optic nerve head edema and an unanticipated level of visual recovery. An 84- year- old woman with a history of NAION in the right eye presented with a 3- week history of visual loss in the left eye. Visual loss was acute, painless, and nonprogressive. She denied any accompanying neurologic or ophthalmologic complaints including headaches, temporal artery tenderness, jaw claudication, or polymyalgia. The initial ophthalmologic examination revealed optic disc swelling in the left eye with associated nerve fiber layer ( NFL) hemorrhages. Sedimentation rate and C- reactive protein level were normal. Visual acuity was 20/ 400 in the right eye and count fingers ( CF) at 1 foot in the left eye. Confrontation visual fields demonstrated temporal islands in both eyes. Automated perimetry was not reliably performed. The pupils measured 4 mm and reacted sluggishly to light; there was a right afferent pupillary defect. The ocular ductions were full. Ophthalmoscopic examination revealed a diffusely pale, cupless right optic nerve. The left optic nerve had marked edema and peripapillary NFL hemorrhages ( Fig. 1A). The neurologic examination was normal. Optical coherence tomography ( OCT) documented marked swelling of the left optic nerve ( Fig. 2). Fluorescein angiography ( FA) showed capillary microangiopathy and dye leakage in the mid- phase and late- phase ( Fig. 3A- B). An intravitreal injection of 1.25 mg/ 0.05 mL bevacizumab was administered to the left eye. Nine days later, there was marked reduction in the swelling of the left optic nerve ( Figs. IB, 2) with significant resolution of the dye leakage and microangiopathy ( Figs. 3C- D). The patient noticed FIG. left 1. Fundus photographs of the eye at presentation ( A) and 9 days after intravitreal injection of bevacizumab ( B) show a rapid reduction in the degree of nerve fiber layer edema. Microns 300 200- 100- 180 200 INF 240 TEMP FIG. 2 . Ocular coherence tomography ( OCT) of the left optic nerve at presentation, 9 days after injection and 2 months after injection. The plane of imaging is through the horizontal nasotemporal axis. On initial presentation, the level of edema exceeded the available scale provided by the OCT software. Presentation 9 days post- injection 8 weeks post- injection 238 I Neuro- Ophthalmol, Vol. 27, No. 3, 2007 Letters to the Editor JNeuro- Ophthalmol, Vol. 27, No. 3, 2007 FIG. 3. Fluorescein angiogram ( FA) of the left fundus. A. Mid- phase FA at presentation. B. Late- phase FA at presentation. C. Mid- phase FA at 9 days after intravitreal injection. D. Late- phase FA 9 days after intravitreal injection. » £ • * • • • FIG. 4. Automated threshold perimetry ( Humphrey SITA 30- 2) of the left eye 2 weeks after intravitreal injection of bevacizumab. concurrent improvement in visual acuity to 20/ 1002 in the left eye. Ten days later, visual acuity had improved to 20/ 70 in the left eye, and the visual field had improved ( Fig. 4). Two months after injection, there remained modest inferior and superior optic disc swelling ( Fig. 2). Vision has remained stable for more than 24 weeks postinjection. After intravitreal bevacizumab, we observed a rapid and significant resolution of NAION- induced optic disc edema. Although visual recovery after NAION is not uncommon ( 2), the resolution of disc edema and visual recovery typically occur over 8 weeks ( 3). The rapid resolution of disc swelling and prompt improvement in visual acuity after bevacizumab administration suggest that VEGF- induced vascular permeability may play a role in tissue injury in NAION. Interestingly, cabergoline, a dopamine receptor agonist, inhibits VEGF- mediated vascular permeability ( 4). This feature may explain the potential benefit observed with carbidopa- levodopa therapy in recent-onset NAION ( 5). Although local VEGF expression after NAION may promote long- term beneficial angiogenesis, acute expression may result in deleterious edema and secondary injury. Indeed, inhibition of VEGF signaling reduces cerebral edema and tissue injury in a murine stroke model ( 6). Therefore, VEGF inhibition may offer a novel therapeutic approach to limit injury in NAION. 239 J Neuro- Ophthalmol, Vol. 27, No. 3, 2007 Letters to the Editor A clinical trial of intravitreal bevacizumab in NAION is warranted. Jeffrey L. Bennett, MD, PhD Departments of Neurology and Ophthalmology Rocky Mountain Lions Eye Institute University of Colorado at Denver and Health Sciences Center Denver, Colorado Scott Thomas, MD Jeffrey L. Olson, MD Naresh Mandava, MD Department of Ophthalmology Rocky Mountain Lions Eye Institute University of Colorado at Denver and Health Sciences Center Denver, Colorado naresh. mandava@ uchsc. edu REFERENCES 1. Weis, SM, Cheresh DA. Pathophysiological consequences of VEGF- induced vascular permeability. Nature 2005; 437: 497- 504. 2. The Ischemic Optic Neuropathy Decompression Trial Research Group. Optic nerve decompression surgery for nonarteritic anterior ischemic optic neuropathy ( NAION) is not effective and may be harmful. JAMA 1995; 273: 625- 32. 3. Hayreh SS, Zimmerman MB. Optic disc edema in non- arteritic anterior ischemic optic neuropathy. GraefesArch Clin Exp Ophthalmol 2007; 245: 1107- 21. 4. Gomez R, Gonzalez- Izquierdo M, Zimmermann RC, et al. Low-dose dopamine agonist administration blocks vascular endothelial growth factor ( VEGF)- mediated vascular hyperpermeability without altering VEGF receptor 2- dependent luteal angiogenesis in a rat ovarian hyperstimulation model. Endocrinology 2006; 147: 5400- 11. 5. Johnson LN, Guy ME, Krohel GB, et al. Levodopa may improve vision loss in recent- onset, nonarteritic anterior ischemic optic neuropathy. Ophthalmology 2000; 107: 521- 6. 6. van Bruggen N, Thibodeaux H, Palmer JT, et al. VEGF antagonism reduces edema formation and tissue damage after ischemia/ reperfusion injury in the mouse brain. J Clin Invest 1999; 104: 1613- 20. Central Retinal Vein Occlusion as a Possible Presenting Manifestation of Sneddon Syndrome We report a case of central retinal vein occlusion ( CRVO) and livedo reticularis in a patient in whom Sneddon syndrome was subsequently diagnosed. A 61- year- old man presented with a sudden and painless decline in visual acuity of his right eye. Visual acuities evaluated with Early Treatment Diabetic Retinopathy Study ( ETDRS) measurements were 0.62 ETDRS lines ( 20/ 60 Snellen equivalent) for the right eye and 0.9 ETDRS lines ( 20/ 30 Snellen equivalent) for the left eye, there was no relative afferent pupillary defect, and intraocular pressures were 18 mm Hg in both eyes. Examination of the right fundus revealed retinal edema, optic disc swelling, tortuous and dilated retinal veins, and extensive superficial hemorrhages in all four quadrants. A diagnosis of acute CRVO was made. Pre- fluorescein angiographic red- free fundus photography ( Fig. 1) showed venous stasis but good retinal capillary perfusion, disc edema, tortuosity, and dilation of all branches of the central retinal vein and a few scattered peripheral fundus hemorrhages, consistent with nonischemic CRVO. The periphery of the eye showed no extensive areas of capillary nonperfusion or neovascularization. The patient had no personal or family history of eye disease, systemic hypertension, diabetes mellirus, thrombophilia, or malignancy. Systemic examination revealed a generalized livid discoloration of the skin in a net- like pattern all over the body except on the face ( Fig. 2). The patient had noted the skin discoloration 13 days earlier. Results of ultrasound studies of the heart and extracranial vessels as well as laboratory serology analyses were negative, including antinuclear antibodies ( ANAs), FIG. 1. Red- free fundus photography of the right eye at presentation shows tortuosity and dilation of all branches of the central retinal vein, mild disc edema, a few scattered peripheral fundus hemorrhages, and good retinal capillary perfusion. These are features consistent with nonischemic central retinal vein occlusion. 240 © 2007 Lippincott Williams & Wilkins Letters to the Editor J Neuro- Ophthalmol, Vol. 27, No. 3, 2007 FIG. 2. Left hip shows livedo reticularis, a reddish netlike discoloration of the skin. antineutrophilic cytoplasmic antibodies ( ANCAs), complement C3 and C4, antiphospholipid antibodies, and lupus anticoagulant, prothrombin time, antithrombin III, fibrinogen, protein C, protein S, and activated protein C resistance. Brain MRI showed an old cerebral infarction in the left basal ganglia. Aortic arch, neck, and brain MRA revealed no evidence of atherosclerosis or vasculitis. A skin and skeletal muscle biopsy revealed endothelial thickening in the small vessels of the subcutis without signs of vasculitis, consistent with Sneddon syndrome. The patient was treated with 400 mg intravenous pentoxifylline daily and 7,500 IU dalteparin sodium subcutaneously for 7 days, followed by 100 mg aspirin per day. On re- examination 3 months later, visual acuity had increased to 0.71 ETDRS lines ( 20/ 50 Snellen equivalent) for the right eye and remained at 0.9 ETDRS lines ( 20/ 30 Snellen equivalent) for the left eye. The right fundus revealed persisting optic disc swelling and retinal hemorrhages but a reduction in retinal edema. Repeated fluorescein angiography showed no signs of ischemia of the right eye. Sneddon syndrome, first described in 1965, is characterized by generalized livedo reticularis and stroke ( 1,2). The etiology of this syndrome is unknown, although there are correlations with the antiphospholipid syndrome, systemic secondary vasculitis, and coagulopathies. It may start as an inflammatory and possibly immunologically mediated disorder and lead to a proliferation of smooth cells of small blood vessels and obstruction of the vessel lumen. Although central retinal artery occlusion ( 3- 5) and peripheral retinal capillary occlusions and neovascularization ( 6) have been reported previously in Sneddon syndrome, CRVO has not. It is unclear whether the CRVO in our patient was directly related to Sneddon syndrome. Tina Aggermann, MD Ludwig Boltzmann Institute for Retinology and Biomicroscopic Laser Surgery Department of Ophthalmology Rudolf Foundation Clinic Vienna, Austria tina. aggermann@ wienkav. at Paulina Haas, MD Ludwig Boltzmann Institute for Retinology and Biomicroscopic Laser Surgery Vienna Austria Susanne Binder, MD Ludwig Boltzmann Institute for Retinology and Biomicroscopic Laser Surgery Department of Ophthalmology Rudolf Foundation Clinic Vienna, Austria REFERENCES Sneddon IB. Cerebrovascular lesions and livedo reticularis. Br J Dermatol 1965; 77: 180- 5. Stockhammer G, Felber SR, Zelger B, et al. Sneddon's syndrome: diagnosis by skin biopsy and MRI in 17 patients. Stroke 1993; 24: 685- 90. Pauranik A, Parwani S, Jain S. Simultaneous bilateral central retinal arterial occlusion in a patient with Sneddon syndrome: case history. Angiology 1987; 38: 158- 63. Jonas J, Kolble K, Volcker HE, et al. Central retinal artery occlusion in Sneddon's disease associated with antiphospholipid antibodies. Am J Ophthalmol 1986; 102: 37^ 10. Shimizu K, Numaga J, Takahashi M, et al. A case of Sneddon syndrome ( in Japanese). Nippon Ganka Gakkai Zasshi 1995; 99: 104- 8. Gobert A. Sneddon's syndrome with bilateral peripheral retinal neovascularization. Bull Soc Beige Ophtalmol 1995; 255: 85- 90. Angiographic Shaded Surface Display Artifact Falsely Suggests Ophthalmic Artery Stenosis We would like to alert our colleagues to an artifact commonly seen on three- dimensional reconstruction catheter angiography and CT angiograms. We evaluated a 41- year- old healthy man with recurrent transient monocular visual loss associated with headaches and periocular pain. He was found to have a branch retinal artery occlusion ( BRAO) on ophthalmoscopic examination. Results of the initial workup with MRI and MRA, transesophageal echocardiography, and laboratory studies were unremarkable. Three- dimensional reconstruction catheter cerebral angiography demonstrated a 3 mm long right ophthalmic artery stenosis ( Fig. 1A). All intracranial and extracranial large arteries, including the aortic arch, were normal. 241 J Neuro- Ophthalmol, Vol. 27, No. 3, 2007 Letters to the Editor FIG. 1 Catheter cerebral angiography. A. Lateral view shaded surface display demonstrates a 3 mm localized 75% stenosis of the ophthalmic artery ( arrow). B. Same view on the planar digital subtraction images shows a normal- appearing ophthalmic artery. The patient was diagnosed with isolated ophthalmic artery stenosis as the cause of recurrent visual loss and BRAO and treated with antiplatelet agents. The episodes ceased. Isolated ophthalmic stenosis has been reported in patients with recurrent transient monocular visual loss and demonstrated mostly on digital subtraction angiography ( 1- 3). However, further review of our patient's angiogram demonstrated a normal- appearing ophthalmic artery on the planar digital subtraction images ( Fig. IB). The area of apparent stenosis ( pseudo- stenosis) seen on the three-dimensional reconstruction images represented a shaded surface display ( SSD) artifact ( 4). This artifact is seen relatively commonly on three- dimensional reconstruction of catheter angiograms ( 5) and even CT angiograms ( 4), suggesting that correlation of these reconstruction images with planar digital subtraction images is very important. In our patient, this pseudo- stenosis was probably a result of a dense planum sphenoidale that projected over the proximal segment of the ophthalmic artery. Potential explanations for this artifact include thresholding errors, geometric differences in x- ray absorption for contrast material- filled structures, and incomplete filling of vessels due to hemodynamic factors ( 5). Maria Woodward, MD Department of Ophthalmology Emory University Atlanta, Georgia Nancy J. Newman, MD Departments of Ophthalmology Neurology, and Neurological Surgery Emory University Atlanta, Georgia Valerie Biousse, MD Departments of Ophthalmology and Neurology Emory University Atlanta, Georgia vbiouss@ emory. edu REFERENCES 1. Weinberger J, Bender AN, Yang WC. Amaurosis fugax associated with ophthalmic artery stenosis: clinical simulation of carotid artery disease. Stroke 1980; 11: 290- 3. 2. Braat AE, Hoogland PH, de Vries AC, et al. Amaurosis fugax and stenosis of the ophthalmic artery- a case report. Vase Surg 2001; 3 5: 141- 3. 3. Gupta M, Puri P, Rundle PA, et al. Retinal infarction following lipoma excision in a patient with secondary ophthalmic artery stenosis. Eye 2004; 18: 436- 7. 4. Takahashi M, Ashtari M, Papp Z, et al. CT angiography of carotid bifurcation: artifacts and pitfalls in shaded- surface display. AJR Am J Roentgenol 1997; 168: 813- 7. 5. Hirai T, Korogi Y, Ono K, et al. Pseudostenosis phenomenon at volume- rendered three- dimensional digital angiography of intracranial arteries: frequency, location, and effect on image evaluation. Radiology 2004; 232: 882- 7. Retinal Migraine The central thesis of Hill et al ( 1) in their recent publication in this journal proposing that " definite retinal migraine, as denned by International Headache Society ( IHS) criteria, is an exceedingly rare cause of transient monocular visual loss" conforms to my clinical experience. What factors can account for the persistent and unjustified inclusion of the concept of " retinal migraine" in the clinician's diagnostic toolbox? I suggest two factors: 1) the legitimacy conferred by the IHS classification ( 2) and 2) the rare but incontrovertible identification of the apparently true existence of monocular migraine ( 3). The most facile explanation for the perception that migraine is monocular is the frequent misperception that homonymous visual phenomena are coming from only one eye. This quandary was plainly stated in 1865 by G. B. Airy, the Astronomer Royal, who sketched and described his own migrainous fortification spectra and averred that " I have never been able to decide with certainty whether the disease really affects both eyes. The first impression on the mind is that only one eye is affected" ( 4). 242 © 2007 Lippincott Williams & Wilkins Letters to the Editor J Neuro- Ophthalmol, Vol. 27, No. 3, 2007 A second explanation for the perception that migraine is monocular comes from the possibility that spreading depression ( SD) is confined to the most anterior portion of the primary visual cortex, giving rise to teichopsia limited to the temporal crescent of the visual field in one eye ( 5). Until we have accurate functional neuroimaging during monocular migraine, SD limited to the approximately 10% of the calcarine cortex subserving the monocular temporal crescent remains plausible but unconfirmed. Rigorous adherence to IHS criteria aids immeasurably in limiting the diagnosis of retinal migraine ( IHS 1.4), but there is considerable ambiguity in descriptors of " positive visual phenomena" ( criterion B). Do " scintillations" identify retinal phenomena and fortification spectra identify cortical phenomena? On the basis of Richards' speculation in 1971 that migrainous fortifications arise from the columnar organization of visual cortex neurons specializing in " detection of lines of a particular length and orientation" ( 6), the " columnless" retina is judged as an unlikely site of origin for teichopsia. The distinction between the appearance of migrainous positive visual phenomena arising from cortex versus retina is blurred by reports of " simple" phosphenes heralding the cortical dysfunction of classic migraine ( 7). Must we require that all retinal migraines be accompanied by headache ( IHS criterion C)? The IHS concedes that " some cases without headache have been reported" ( 2). We should allow for the existence of " acephalgic retinal migraine" given that we allow for acephalgic migraine or " typical aura without headache" ( IHS 1.2.3) in referring to painless visual disturbances of migraine. An accurate estimate of the frequency of retinal migraine in the differential diagnosis of transient monocular visual disturbance will require more than conformation to " strict IHS criteria" ( 1). A revised and more precise semiology of the visual world of migraineurs is needed. For example, distinctions between the appearance ( and complexity) of scintillations, photopsias, sparks, and phosphenes remain ill- defined and impede accurate classification of positive migrainous visual symptoms. Although greater precision in the description of migrainous visual phenomena will improve the nosology of migraine, accurate localization and a thorough grasp of the neurophysiology of migraine aura remain elusive. Although Penfield and Rasmussen were unable to reproduce " zigzag outlines of migraine images" with then-bipolar electrodes ( 8) during cortical stimulation, functional MRI mapping of blood oxygenation level- dependent ( BOLD) events during migraine aura has demonstrated retinotopic progression at a characteristic SD velocity of 3.5 =/- 1.1 mm/ min ( 9). Surprisingly, BOLD imaging in a single migraineur revealed that the initial part of the visual aura correlated with changes in the extrastriate cortex ( area V3A), not in the primary visual cortex. The BOLD signal changes in migraine aura are posited to be a surrogate for SD, lending credence to Milner's insightful theory that the scintillating scotoma corresponds to SD ( 10). The contemporary theory of migraine as a primarily neuronal disorder is built on the compelling consonance of functional neuroimaging, SD, and Lashley's measurement of the " march" of his own scintillating scotoma ( 11). This " spreading depression theory" ( 12) remains ascendant despite the inability to record SD in uninjured human neocortex or retina in vivo. ( 1) As neuroimaging and neurophysiologic tests for migraine evolve from the investigational stage, our contemporary clinical predicament is evocative of the approach to epilepsy before electroencephalography. Lacking a comprehensive atlas of the migraine aura, the lessons of functional neuroimaging and the solicitation of more precise descriptors of patients' visual symptoms will go a long way toward refining the diagnosis of migraine and distinguishing retinal migraine from other monocular disturbances of vision. Frederick E. Lepore, MD Departments of Neurology and Ophthalmology UMDNJ/ Robert Wood Johnson Medical School New Brunswick, New Jersey lepore fe@ umdnj . edu REFERENCES 1. Hill DL, Daroff RB, Ducros A, et al. Most cases labeled as " retinal migraine" are not migraine. J Neuroophthalmol 2007; 27: 3- 8. 2. Headache Classification Subcommittee of the International Headache Society. The International Classification of Headache Disorders, 2nd edition. Cephalalgia 2004; 24( Suppl 1): 9- 160. 3. Daroff RB. Retinal migraine. J Neuroophthalmol 2007; 27: 83. 4. Airy GB. On hemiopsy. London Edinburgh Dublin Philos MagJSci 1865; 30: 19- 21. 5. Lepore FE. The preserved temporal crescent- the clinical implications of an " endangered" finding. Neurology 2001; 57: 1918- 21. 6. Richards W. The fortification illusions of migraines. Sci Am 1971; 224: 88- 96. 7. Sacks O. Migraine. Berkeley: University of California Press; 1992. 8. Penfield W, Rasmussen T. The Cerebral Cortex of Man. New York: Macmillan; 1955: 145. 9. Hadjikhani N, Sanchez Del Rio M, Wu O, et al. Mechanisms of migraine aura revealed by functional MRI in human visual cortex. Proc Natl Acad Sci USA 2001; 98: 4687- 92. 10. Milner PM. Note on a possible correspondence between the scotomas of migraine and spreading depression of Leao. Electroencephalogr Clin Neurophysiol Suppl 1958; 10: 705. 11. Lashley KS. Patterns of cerebral integration indicated by the scotomas of migraine. Arch Neurol Psychiatry 1941; 46: 331- 9. 12. Lauritzen M. Pathophysiology of the migraine aura- the spreading depression theory. Brain 1994; 117: 199- 210. Retinal Migraine Based on our recently reported series of patients with retinal migraine and our literature review ( 1), we would like 243 J Neuro- Ophthalmol, Vol. 27, No. 3, 2007 Letters to the Editor to comment on the recent article and editorial that were published in the Journal of Neuro- Ophthalmology ( 2,3). First is the editorial, " Retinal Migraine Is an Oxymoron." The label " retinal migraine" is not a contradiction in terms any more than is the term " hemiplegic migraine." Both conditions describe rare types of migraine denned by unusual focal symptoms. The pathophysiology of monocular visual loss in migraine is unknown. Both spreading depression ( SD) and ischemia have been proposed. Dr. Winterkorn stated that, " The pathophysiology of migraine ( spreading depression of neurons) would not explain monocular visual loss." Although the aura of migraine is usually due to SD of the occipital cortex, SD may affect neurons in any part of the brain. There is no physiological reason to assume that retinal neurons are immune to SD. Dr. Winterkorn dismisses SD as a mechanism for retinal migraine because SD in the retina has not been reported in mammals. But it has been reported in the retinas of frogs and chicks. For almost half a century, SD of cortical neurons was dismissed as a pathophysiologic mechanism of aura because it was not observed in humans. Neurosurgeons have not seen SD in the human cortex. But the absence of evidence is not evidence of absence. Over the past decade, neuroimaging during the aura has revealed changes most consistent with SD ( 4). That is not to say that vasospasm never occurs in migraine auras. It may contribute to migrainous cerebral infarction and the rare cases of retinal migraine that in time develop permanent monocular defects. Then, in the article by Hill et al ( 3), the authors stated that most reported cases of retinal migraine do not meet the criteria of the International Classification of Headache Disorders ( ICHD) for retinal migraine. This issue reflects problems with the diagnostic criteria. For example, Carroll ( 5), who introduced the term " retinal migraine," described patients with monocular visual loss not associated with headache. Hill et al ( 3) found only 5 cases of definite retinal migraine and 11 others of probable or possible retinal migraine using strict ICHD- 2 criteria. These criteria were based more on opinion than on a detailed review of the literature. The ICHD- 2 criteria excluded cases with permanent visual loss and cases of migraine with conventional ( cortical) aura. In our review ( 1), we included such cases. Because most reported cases were described before publication of the ICHD- 2 criteria, we used our judgment in assessing whether or not the reports of monocular visual loss were associated with " true" migraine. We were surprised that permanent visual defects were found in almost 50% of patients who had had transient monocular visual loss. The ophthalmologic examinations in these 21 patients revealed several different conditions. In some, the defects appeared to be proximal to the retina. For that reason, the term " migraine with monocular visual symptoms," rather than " retinal migraine," would be more appropriate. To take these factors into account, we proposed new criteria and terminology for what is now termed " retinal migraine." Perhaps some of the cases we cited were not truly retinal migraine. But retinal migraine may be underdiagnosed. When a patient reports blurring or blindness in one eye as the aura of migraine, we believe most doctors assume that the patient is misinterpreting a homonymous visual field defect. Doctors usually do not instruct patients to cover one eye and then the other during subsequent attacks of migraine with aura. It may turn out that retinal migraine is a frequent occurrence. That would be an oxymoron. Seymour Solomon, MD Brian M. Grosberg, MD Department of Neurology Montefiore Medical Center and Albert Einstein College of Medicine Bronx, New York ssolomon@ montefiore. org Deborah I. Friedman, MD Departments of Ophthalmology and Neurology University of Rochester School of Medicine and Dentistry Rochester, New York Richard B. Lipton, MD Departments of Neurology and Epidemiology and Population Health Montefiore Medical Center and Albert Einstein College of Medicine Bronx, New York REFERENCES 1. Grosberg BM, Solomon S, Friedman DI, et al. Retinal migraine reappraised. Cephalalgia 2006; 26: 1275- 86. 2. Winterkorn JM. " Retinal migraine" is an oxymoron. J Neuro- Ophthalmol 2007; 27: 1- 2. 3. Hill DL, Daroff RB, Ducros A, et al. Most cases labeled as ' retinal migraine' are not migraine. JNeuroophthalmol 2007; 27: 3- 8. 4. Woods RP, Iacoboni M, Mazziotta JC. Bilateral spreading cerebral hypoperfusion during spontaneous migraine headache. N EnglJ Med 1994; 331: 1689- 92. 5. Carroll D. Retinal migraine. Headache 1970; 10: 9- 13. Reply: We are not surprised to read that Solomon et al disagree with Dr. Winterkorn's editorial ( 1) and our recently published article ( 2) on retinal migraine. This is a highly controversial topic, and our suggestion that " most cases labeled as retinal migraine are not migraine" has most certainly sparked a number of lively discussions. 244 © 2007 Lippincott Williams & Wilkins Letters to the Editor J Neuro- Ophthalmol, Vol. 27, No. 3, 2007 However, none of the points raised by Solomon et al in their letter demonstrate that retinal migraine is a real entity. We do not know whether " migraine" can affect the retina or not, and our conclusion was simply that " if it does exist, retinal migraine must be exceedingly rare" ( 2). We also emphasized that " migraine" is by definition a benign disorder that should not result in permanent visual loss. For example, Solomon et al refer to hemiplegic migraine, a rare form of migraine still classified within the spectrum of migrainous disorders specifically because its motor deficit and confusion are always completely spontaneously reversible. We strongly disagree with Solomon et al's statement that retinal migraine can cause permanent visual loss. Indeed, the patients included in these authors' review ( 3) harbored various retinal and optic nerve disorders, including retinal artery occlusions, vein occlusions, and ischemic optic neuropathies. The wide variety of pathophysiologic conditions required to explain all these cases as retinal migraine suggests that these cases instead reflect a heterogeneous group of disorders in which there was coincidental cranial or ocular pain. Valerie Biousse, MD Nancy J. Newman, MD Emory University School of Medicine Atlanta, Georgia vbiouss@ emory. edu REFERENCES 1. Winterkorn JM. " Retinal migraine" is an oxymoron. J Neuro-ophthalmol 2007; 27: 1- 2. 2. Hill DL, Daroff RB, Ducros A, et al. Most cases labeled as ' retinal migraine' are not migraine. J Neuroophthalmol 2007; 27: 3- 8. 3. Grosberg BM, Solomon S, Friedman DI, et al. Retinal migraine reappraised. 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