Covee and Doughnut Maculopathy: a Cause of Acute Central Ring Scotomas

158 Br J Ophthalmol 2000;84:158–164

CoVee and doughnut maculopathy: a cause of Br J Ophthalmol: first published as 10.1136/bjo.84.2.158 on 1 February 2000. Downloaded from acute central ring scotomas

John B Kerrison, Stephen C Pollock, Valerie Biousse, Nancy J Newman

Abstract both retinal3–6 and optic nerve diseases.7 Disor- Aims—To report the clinical features of ders associated with central ring scotomas have five patients with non-progressive central not been well characterised. This report ring scotomas of acute onset associated describes five patients who had the acute onset with excellent retained visual acuity. of ring scotomas within the central 10 degrees Methods—Complete neuro-ophthalmo- with retention of good visual acuity. logical examinations were performed. Visual fields were performed by tangent Methods screen, Goldmann, or Humphrey perim- Patients underwent complete neuro-ophthal- etry. In some cases further testing was mological examination. Colour vision was as- carried out including fundus photogra- sessed using the 14 numbered Ishihara plates phy, fluorescein angiography, ERG, VEP, (after the control plate) or using the first 10 and neuroimaging. Hardy-Rand-Ritter (HRR) plates. Pupils were Results—The patients were three women assessed using a transilluminator light, and rela- and two men whose ages ranged from 25 to tive aVerent pupillary defects were quantified 57 years. Four patients were heavy caVeine with neutral density filters. Static Humphrey consumers while the fifth patient experi- perimetry was performed using the 24-2 and enced an episode of hypotension. Vision 10-2 programs. Tangent screen testing was per- loss was acute in all cases. The onset of formed in two cases and kinetic Goldmann per- vision loss was bilateral/simultaneous in imetry in one case. Fluorescein angiography was three cases, bilateral/sequential in one obtained in all patients. Full field electroretino- case, and unilateral in one case. All grams (ERGs) were recorded using the Inter- aVected eyes retained visual acuities of national Society for Clinical Electrophysiology 20/25 or better. Colour vision was subnor- of Vision standard techniques under scotopic mal in three of four cases. Visual field and photopic conditions. defects were characterised by a central ring scotoma having an outer diameter less than 10 degrees. Fundus examination Results (Table 1; Fig 1) Vision loss was acute in all patients. The onset

demonstrated temporal optic nerve pallor http://bjo.bmj.com/ in three patients (five of 10 aVected eyes) of vision loss was bilateral/simultaneous in and reddish, petaloid macular lesions in three cases, bilateral/sequential in one case, and unilateral in one case. Four patients were Department of one patient. Good visual acuity was main- Ophthalmology, tained for the duration of follow up in all heavy caVeine consumers, drinking on the Emory University five patients. order of one litre of caVeinated soft drinks or School of Medicine, Conclusion—Central ring scotomas with 20 cups of coVee a day. A fifth patient had an Atlanta, Georgia, USA excellent retained visual acuity may episode of hypotension during epidural anaes-

J B Kerrison on September 27, 2021 by guest. Protected copyright. present as an acute, bilateral syndrome in thesia before vision loss. All aVected eyes V Biousse retained visual acuities of 20/25 or better. Col- N J Newman patients who are heavy caVeine consumers. The configuration of visual field loss our vision was abnormal in three of four cases Department of and its location, combined with the pres- as assessed by Ishihara or HRR testing. In an Neurology ence of temporal pallor in five eyes, additional case, the patient could not see the V Biousse suggest that the defect localises to the Ishihara control plate. On Amsler grids, N J Newman inner layers of the macula. While these patients described a circular defect surrounding the centre dot, sparing the central 1-2 Department of cases could be considered an expansion of Neurological Surgery the clinical spectrum of acute macular degrees. The outer diameter of the defect was N J Newman neuroretinopathy, some may represent a less than 10 degrees in each case. The annular distinct entity. configuration was particularly evident on Department of (Br J Ophthalmol 2000;84:158–164) tangent screen testing (two patients). On auto- Ophthalmology, Duke mated static perimetry using the 10-2 Hum- University School of phrey paradigm (three patients), the defects Medicine, Durham, North Carolina, USA Ring scotomas are annular visual field defects appeared as small central scotomas. Fundus S C Pollock centred on fixation. The ring may be mid- examination was remarkable for mild temporal peripheral, in which case the annulus passes pallor of the optic nerve in three patients (five Correspondence to: between 30 and 60 degrees; pericentral, in eyes) and bilateral, reddish, wedge-shaped Nancy J Newman, MD, Neuro-ophthalmology Unit, which case the ring incorporates the physio- macular lesions in one patient. The retinal ves- Emory Eye Center, 1365-B logical blind spot; or central. The central ring sels were unremarkable in all eyes. Fluorescein Clifton Rd NE, Atlanta, GA scotomas generally reside within the central 10 angiography was normal in four patients but 30322, USA degrees. Mid-peripheral ring scotomas are a demonstrated an area of choroidal hypofluo- 1 Accepted for publication feature of retinitis pigmentosa and aphakic rescence in one eye of the patient who had 9 September 1999 correction.2 Pericentral ring scotomas occur in been hypotensive. Full field ERG was normal 159 Kerrison, Pollock, Biousse, et al

in three out of four patients. The single abnor- Br J Ophthalmol: first published as 10.1136/bjo.84.2.158 on 1 February 2000. Downloaded from mal ERG exhibited mild prolongation of the implicit time on 30 Hz ﬂicker in each eye and a small reduction in the dim white scotopic response from the right eye. Neuroimaging was

Onset to most recent exam normal in three cases and demonstrated non-speciﬁc periventricular foci of increased T2 signal on the MRI of one patient. Good visual acuity was maintained for the duration of follow up in all patients (range 3 months to 4 years).

Case reports CASE 1 A 57 year old white man awoke one morning noting spots in the central vision of each eye. This impaired his reading by causing him to skip lines. The page appeared to him as if the “pen was running out of ink.” On occasion, he saw “spinning fans” in the central vision of each eye. He had hypertension, treated with Lotril (amiodipine and benazepril hydrochlo- ride, Ciba-Geigy). He consumed 10 caVein- ated beverages a day. He was seen emergently by his local ophthalmologist who found a visual acuity of 20/25 in each eye, central ring Fundus RE Fundus LE FA ERG MRI/CT macular lesions, scotomas on Amsler grid testing in each eye, normal pupils, and a normal fundus examination in each eye. A ﬂuorescein angiogram showed normal macular perfusion without late leakage. An extensive laboratory evaluation was unremarkable. Magnetic resonance imaging (MRI) of the brain with contrast demon- VF RE VF LE (type of perimetry) HVF 10-2 tangent screen HVF 10-2 tangent screen GVF HVF 10-2 reddish petaloid strated mild, non-speciﬁc periventricular foci of increased T2 signal that did not enhance with contrast. Neuro-ophthalmological examination 4 months later revealed a visual acuity of 20/20−2 in the right eye and 20/25−2 in the

Amsler RE Amsler LE left eye. During visual acuity testing, the patient was only able to find and identify one http://bjo.bmj.com/ letter at a time. He could not see the control Ishihara testing plate with either eye. On Amsler grid testing, he described a scotomatous ring surrounding fixation in each eye (Fig 1). Humphrey visual fields were performed using a 10-2 protocol and demonstrated bilat-

eral, incongruous central defects (Fig 1). The on September 27, 2021 by guest. Protected copyright. pupillary light reactions were normal without a relative aVerent pupillary defect. Biomicros- copy was normal except for bilateral Kruken- berg spindles and iris transillumination defects

CV RE CV LE Pupils consistent with pigment dispersion syndrome. Intraocular pressures were normal. Dilated fundus examination was normal except for bilateral posterior vitreous detachments and 20/25 no control20/15 ISH 10/10 HRR20/20 8/14 ISH20/20 ring scotoma 0/14 ISH central defect ring scotoma ring normal scotoma ring scotoma central defect temporal pallor temporal pallor central defect macular lesions, 20/2020/20 2.5/10 HRR 0/10 HRR normal ring scotoma ring scotoma ring scotoma temporal ring pallor scotoma normal temporal pallor 4 months VA RE/LE mild pigmentary stippling in the maculae. An ERG was normal except for a mild prolongation of the implicit time on 30 Hz ﬂicker in each eye and a small reduction in the dim white scotopic response from the right eye.

CASE 2 (2 years apart) A 35 year old woman experienced the acute onset of simultaneous bilateral clouding of her central vision. She had a history of hypertension and cigarette smoking. She consumed a litre of caVeinated soft drinks per day. Her medications included Norvasc (amiodipine besylate, Pfizer), Reglan (metoclopramide, 1 572 M 353 acute, bilateral F 354 acute, F bilateral 53 20/205 no acute, M control left ISH eye 20/15 only 25 normal 20/20 acute, sequential, 10/10 HRR F 13.5/14 ISH normal acute, bilateral 0.3 log unit ring left scotoma rapd 20/20 normal central defect 0/14 ISH ring scotoma normal normal ring scotoma normal temporal pallor normal normal normal abnormal normal normal MRI abnormal normal 3 MRI normal months central defect 7 months normal CT reddish petaloid 49 months normal normal CT 20 months Case Age Sex Presentation VA = visual acuity; CVresonance imaging; = CT colour = vision; computed ISH tomography. = Ishihara; HRR = Hardy-Rand-Ritter; VF = visual field; HVF = Humphrey visual field; GVF = Goldmann visual field; FA = fluorescein angiogram; ERG = electroretinogram; MRI = magnetic Table 1 Clinical features of patients with acute central ring scotomas A H Robins), and Monopril (eosinopril 160 Kerrison, Pollock, Biousse, et al Br J Ophthalmol: first published as 10.1136/bjo.84.2.158 on 1 February 2000. Downloaded from Case 1 10-2 Humphrey visual fields Case 1 Amsler grids Left eye Right eye Left eye Right eye

Case 2 Amsler grids Case 3 10-2 Humphrey visual fields Left eye Right eye Left eye Right eye

Case 3 Amsler grids Case 4 Amsler grids Left eye Right eye Left eye Right eye http://bjo.bmj.com/ Case 5 10-2 Humphrey visual fields Left eye Right eye on September 27, 2021 by guest. Protected copyright.

Figure 1 Central visual defects on Amsler grids and static Humphrey visual ﬁelds in patients with acute central ring scotomas.

sodium, Bristol-Myers-Squibb) Initial evalua- Ophthalmoscopy disclosed moderate temporal tions disclosed central visual defects in each pallor of the optic nerve head, reduced nerve eye. An MRI of the brain with contrast fibre layer reflexes in the papillomacular disclosed no abnormalities. A full field ERG bundle, and blunting of the foveal reflex in was normal. Visual evoked potential testing each eye. was consistent with an abnormality of the anterior visual pathways. A neuro- CASE 3 ophthalmological examination performed 7 A 35 year old white woman had a history of months after vision loss disclosed a visual acu- myopia, central serous chorioretinopathy, ity of 20/15 in each eye. Colour vision testing in obesity, hypercholesterolaemia, and toxaemia each eye with HRR plates was normal. Bilateral with her first pregnancy. She drank six to eight ring scotomas were evident on Amsler grids cups of coVee per day. She presented to her (Fig 1) and on tangent screen testing with a red retina specialist with a complaint of a ring test object. The defects were located between 2 around the central dot on Amsler grid testing and 6 degrees from fixation. Pupillary exam- of the left eye. She had a normal appearing ination, biomicroscopy of the anterior seg- retina and fluorescein angiogram. Neuro- ments, and intraocular pressures were normal. ophthalmological examination 5 months later CoVee and doughnut maculopathy 161

disclosed a visual acuity of 20/20+3 in the right segments and intraocular pressures were nor- Br J Ophthalmol: first published as 10.1136/bjo.84.2.158 on 1 February 2000. Downloaded from eye and 20/20−2 in the left eye. Colour vision mal. In each eye, dilated fundus examination was 13.5 out of 14 Ishihara plates in the right disclosed a cup to disc ratio of 0.7 with mild eye and 2.5 out of 14 plates in the left eye. temporal pallor, decreased nerve fibre layer Amsler grid testing disclosed a ring scotoma reflexes in the maculopapillary bundle, a around fixation in the left eye only (Fig 1). diminished foveal reflex, and mild attenuation Humphrey visual fields using the 30-2 protocol of the retinal arterioles. disclosed a normal right eye and central depression in the left eye. A Humphrey 10-2 CASE 5 visual field in the left eye showed a central A 25 year old white woman experienced bilat- defect (foveal threshold: 37 dB right eye and 23 eral, painless, acute loss of vision a day after dB left eye) (Fig 1). Pupillary examination dis- spontaneous vaginal childbirth. The delivery closed a 0.3 log unit left relative aVerent pupil- was unremarkable except for a brief episode of lary defect. Biomicroscopy of the anterior seg- hypotension during epidural anaesthesia. She ments and intraocular pressures were normal. underwent computed tomography (CT) of the Dilated fundus examination of the right eye head without contrast, lumbar puncture, visual was normal. In the left eye, mild disc pallor was evoked response (VER), and carotid ultra- present. Further testing included a normal sonography, all of which yielded normal computed tomograph (CT) scan of the orbits, results. On neuro-ophthalmological examina- a normal full field electroretinogram, and a tion 19 months later, she had a visual acuity of normal laboratory evaluation, including eryth- 20/20 in each eye. She was only able to identify rocyte sedimentation rate, rapid plasmin reagin the Ishihara control plate with some diYculty (RPR), antinuclear antibodies (ANA), and in each eye. Humphrey visual fields performed complete blood cell count (CBC). The patient using the 30-2 protocol disclosed bilateral cen- has been followed for 4 years without change in tral defects (foveal threshold: 36 dB right eye visual acuity or visual fields. and 31 dB left eye) (Fig 1). Pupillary examination, biomicroscopy of the anterior segments, CASE 4 and intraocular pressures were normal. Dilated A 53 year old man experienced the sudden fundus examination disclosed bilateral reddish, onset of a spot in the central portion of the petaloid macular lesions. Both optic nerves vision in his left eye. At the centre of this spot appeared healthy, as did the vessels and was a clear area through which he could see. It periphery. A fluorescein angiogram demon- was recommended that he take aspirin. The strated choroidal filling defects nasal to the spot remained unchanged. Two years later, fovea in the right eye. while playing golf, he noted that objects viewed with his right eye had a “misty” appearance but that there remained a small central aperture Discussion through which he could see clearly. He had a Each of our patients experienced the sudden history of classic migraine headaches and took onset of a unilateral or bilateral central ring a multivitamin once a day. He was a 40 pack a scotoma. The visual defect was maximal at http://bjo.bmj.com/ year cigarette smoker and drank 20 cups of onset and did not improve or progress. The coVee a day. He consumed two alcoholic scotomatous ring extended between 1 and 8 drinks three days per week. On initial evalua- degrees from fixation. Because automated tion by a retinal specialist soon afterwards, his static perimetry tests a matrix of fixed points, visual acuity was 20/25 in the right eye and the defect appeared as a central scotoma when 20/50 in the left eye. He had bilateral, central, tested by this modality (Fig 1). However, the

doughnut-shaped scotomas, no relative aVer- ring configuration was evident on Amsler grid, on September 27, 2021 by guest. Protected copyright. ent pupillary defect, and no fundus abnormali- Goldmann perimetry, and tangent screen test- ties. On neuro-ophthalmological examination ing and was attested to by the retention of 3 months later, he had a best corrected visual excellent visual acuity. acuity of 20/20 in both eyes. He correctly iden- The term ring scotoma may be applied to tified 2.5 out of 10 HRR plates in the right eye any visual field defect that encircles fixation. and no plates in the left eye. On Amsler grid Morphologically, such defects can be separated testing, he described a scotomatous ring into three categories: mid-peripheral ring surrounding central fixation in each eye (Fig scotomas, pericentral ring scotomas, and cen- 1). Tangent screen testing at a distance of 2 tral ring scotomas. metres demonstrated bilateral central ring Mid-peripheral ring scotomas are a charac- scotomas. The annulus of the ring passed teristic clinical feature of retinitis pigmentosa.1 between 2 and 7 degrees in the each eye, with In this condition, scotomas initially develop the width being smallest along the 6 o’clock between 40 and 60 degrees and then progres- meridian. Goldmann perimetry in the right eye sively coalesce. They also may gradually disclosed a bilobed defect to the I1e target expand at both their peripheral and central within 5 degrees of fixation. A narrow tongue margins. Mid-peripheral ring scotomas also of preserved sensitivity extended upwards occur as a consequence of aphakic spectacle through the defect. Another scotoma to the I1e correction.2 stimulus was oblong in shape and located 20 Pericentral ring scotomas occupy the zone degrees superotemporal to fixation. In the left between 5 and 30 degrees, thus incorporating eye, a central ring scotoma was present. Pupil- the physiological blind spot. Both retinal and lary examination disclosed no relative aVerent optic nerve disorders may cause pericentral pupillary defect. Biomicroscopy of the anterior scotomas. 162 Kerrison, Pollock, Biousse, et al

Retinal disorders associated with pericentral level of involvement based on the biomicro- Br J Ophthalmol: first published as 10.1136/bjo.84.2.158 on 1 February 2000. Downloaded from ring scotomas include photoreceptor dystro- scopic appearance of the lesions has varied phies and paraneoplastic/autoimmune disor- considerably.13 15 17 ders. Cone dystrophies that are associated with The cause of AMNR is not known, but both pericentral ring scotomas are typically associ- inflammatory19–21 and vasculopathic12 14 24 ated with abnormalities on full field ERG and mechanisms have been proposed. In support of fundus examination.34 Autoimmune retinal an inflammatory process is the observation that diseases associated with ring scotomas include most cases occur in young women and that cancer associated retinopathy and autoim- many aVected individuals report having had an mune retinopathy.56These disorders are char- antecedent viral illness.15–17 19 20 Other cases acterised by progressive loss of vision, an occurred in the setting of allergic drug abnormal full field ERG, and usually a normal reactions.12 13 The development of lesions char- fundus examination, although the retinal arte- acteristic of multiple evanescent white dot rioles may be attenuated. syndrome (MEWDS), either simultaneously or Optic neuropathies can result in pericentral years after the appearance of macular lesions ring scotomas when superior and inferior arcu- characteristic of AMNR21 has prompted the ate defects coalesce. Paired arcuate defects may inclusion of AMNR among the inflammatory circumferentially encompass fixation in ante- disorders grouped together as acute zonal occult rior ischemic optic neuropathy, glaucoma, and outer retinopathy (AZOOR).24 25 Nevertheless, optic disc drusen.7 The presence of a horizon- some cases have been reported to occur in the tal step, asymmetry of the ring across the hori- setting of severe blood loss and moderately zontal meridian, and visible abnormalities of severe bodily injury, suggesting that focal the nerve head may help to localise the hypoperfusion, adrenergic stimulation, or acute disorder to the optic nerve. In a subgroup of elevation of ocular venous pressure may repre- patients with Leber’s hereditary optic neu- sent contributing factors.12 14 23 ropathy, large central scotomas may fenestrate, One could argue that our patients may resulting in a variant of ring scotoma and a represent a clinical variant of AMNR. Features measurable improvement in visual acuity.8 that our patients share with AMNR patients The patients that are the subject of this include acute onset, bilaterality in some cases, report have central ring scotomas, a form of good visual acuity, and normal fluorescein ring scotomas which has not been well charac- angiography. Furthermore, case 5 had retinal terised in the literature. In 1905, Wisselink lesions typical of AMNR. By contrast, the described an individual who, after a fall, had a remainder of our cases did not have the typical central scotoma associated with a grey appear- AMNR maculopathy. These cases are further ance of the macula. The centre of the defect distinguished by their older age (cases 1, 4), cleared, leaving a small ring.9 In 1933, Fuchs occurrence in men (cases 1, 4), dyschromatop- described two similar cases following trauma sia, and optic disc pallor (cases 2, 3, 4). The and thought that the phenomenon represented last two findings have not been reported in injury to Henle’s nerve fibre layer.10 Of 20 cases AMNR. Finally, while true central ring scoto- described by Stoll in 1950 as having “narrow mas have been described in AMNR, they http://bjo.bmj.com/ ring scotomas,” five patients had visual acuities appear to be infrequent, occurring only in the of 20/25 or better. Two of his cases had experi- setting of intravenous sympathomimetic enced trauma, but in three patients, he administration.12–14 More commonly, the de- attributed the defects to “a failure of adequate fects are paracentral, sometimes coalescing, supply in the small terminal retinal vessels” or but rarely completely encircling fixation. All of “central angiospastic retinopathy,” a term he our patients had central ring scotomas. We 11 attributed to GiVord and Marquardt. therefore believe that the cases selected for on September 27, 2021 by guest. Protected copyright. Central ring scotomas also have been inclusion in this study, with the possible excep- described in the setting of acute macular tion of case 5, comprise a distinct clinical neuroretinopathy (AMNR) where they are group and may result from a common presumed to result from a coalescence of para- pathophysiological mechanism. central scotomas.12–14 AMNR is a disorder The inner layers of the macula represent the characterised by acute, usually bilateral, vision most likely site of visual pathway injury in our loss associated with reddish-brown macular patients. The observation of disc pallor in three lesions.12–23 The disease has a strong predilec- of our patients is indicative of loss of retinal tion for young women. ERG and electro- ganglion cells with anterograde (ascending) oculography are typically normal.15 19 Fluores- degeneration of axons. While temporal disc cein angiography is usually normal but has pallor can be a feature of cone dystrophies, been reported to show subtle dilatation of presumably as a result of transynaptic degen- macular capillaries,15 faint hyperfluorescence eration,26 27 the development of disc pallor soon of the fovea in the early phases,13 and subtle after vision loss along with the generally areas of hypofluorescence corresponding to the normal ERG findings argues against an outer reddish macular lesions at later stages.13 17 The retinal process in our patients. The possibility level at which the injury occurs within the that the temporal disc pallor observed in three retina has not been established. Acutely, the of these patients resulted from injury to the macula appears oedematous, suggesting inner optic nerve is likewise untenable. For an optic retinal involvement.12 13 However, the docu- neuropathy to produce a central ring scotoma, mentation of an abnormality of the early those axons originating from ganglion cells that receptor potential in one case suggested an subserve vision between 2 and 8 degrees would outer retinal process.18 Interpretation of the have to be selectively lost while axons from CoVee and doughnut maculopathy 163

ganglion cells whose receptive fields subserve is an inhibitor of adenosine, the latter being a Br J Ophthalmol: first published as 10.1136/bjo.84.2.158 on 1 February 2000. Downloaded from the central one to two degrees of vision survive. potent vasodilator of the retinal vasculature. Given the compact relation of these two Therefore, large amounts of caVeine can be subpopulations of axons within the optic nerve expected to cause retinal vasoconstriction. At a and the fact that they intermingle with one dose of 200 mg (equivalent to approximately another, it seems unlikely that a pathological one cup of coVee), caVeine has been shown to process at this location could be characterised modestly decrease the mean velocity of leuco- by this degree of selectivity. Furthermore, the cytes within macular capillaries, and presum- fact that superotemporal and inferotemporal ably, macular blood flow.29 The eVect of large macular projections are separated in space doses on macular blood flow is not known. within the optic nerve28 means that for a hypo- Also unknown is the extent to which the thetical neuropathic process to be associated vasoactive eVects of caVeine on the retinal cir- with a central ring scotoma having continuous culation are additive to other causes of boundaries, the area of neural injury would vasoconstriction (for example, nasal decon- have to be perfectly symmetrical across the gestants, smoking, hypertension, Raynaud’s horizontal midline of the disc. phenomenon, migraine, etc). We believe that the pathophysiological We describe five patients who acutely devel- mechanism of macular injury in our patients is oped central ring scotomas associated with transient ischaemia, based on the acute onset excellent visual acuity. Four of the patients were of symptoms, the stability of the visual deficits heavy caVeine consumers, and the fifth patient following onset, and the normal results of fluo- had suVered an episode of hypotension. The rescein angiography in all cases but one. The configuration of the visual field defect in all area of the macula corresponding to the central cases and the observation of optic nerve pallor in ring scotomas is characterised by a high three cases suggest a process localised to the concentration of ganglion cells and metabolic inner retina. Transient ischaemia of the macula, activity, features which may make this portion possibly related to vasoconstriction, may be the of the macula particularly vulnerable to the responsible pathophysiological mechanism. eVects of ischaemia and hypoxia. This combination of clinical features leads us to The dual circulation of the macula provides suggest the term “coVee and doughnut macu- a plausible explanation for the spared aperture lopathy” to describe this condition. in patients with central ring scotomas due to vascular compromise. The outer retina and We thank R Mitchell Newman Jr, MD for referring patient 1. central portion of the fovea are supplied by the This work was supported by a grant from the Heed Founda- tion (JBK) and as part of an unrestricted grant to Emory Eye choroidal circulation via the choriocapillaris Center, Emory University School of Medicine, from Research while the remainder of the inner retina is sup- to Prevent Blindness, Inc, New York, and National Institute of Health CORE grant No P30-EYO 6360. plied by the retinal vessels. In patients with ischemic macular damage from reduced blood 1 Weleber RG. Retinitis pigmentosa and allied disorders. In: flow in the retinal microvasculature, the Ryan SJ, Ogden TE, Schachat AP, Murphy RP, Glaser BM, choroidal circulation would spare not only eds. Retina. 2nd ed. St Louis: Mosby, 1994:335–466.

2 Dabezies OH Jr. Defects of vision through aphakic spectacle http://bjo.bmj.com/ photoreceptors but also the ganglion cell bod- lenses. Ophthalmology 1979;86:352–79. 3 Ten Hove MW, Siatkowski RM, Smith JL. Foveal cone dys- ies on the bank of the fovea that subserve the function syndrome. J Neuro-Ophthalmol 1998;18:9–14. central one to two degrees of vision. Of course, 4 Szlyk JP, Fishman GA, Alexander KR, et al. Clinical subtypes of cone-rod dystrophy. Arch Ophthalmol 1993; axons of those surviving ganglion cells would 111:781–8. still have to traverse the surrounding ischaemic 5 Ohnishi Y, Ohara S, Sakamoto T, et al. Cancer associated retinopathy with retinal phlebitis. Br J Ophthalmol 1993;77: zone as part of the retinal nerve ﬁbre layer. 795–8. However, as has been hypothesised by Rizzo 6 Mizener JB, Kimura AE, Adamus G, et al. Autoimmune retinopathy in the absence of cancer. Am J Ophthalmol (Rizzo JF III, unpublished data, 1993), gan- 1997;123:607–18. on September 27, 2021 by guest. Protected copyright. glion cell axons may be more resistant to 7 Miller NR, Newman NJ. Topical diagnosis of lesions in the visual sensory pathway. In: Miller NR, Newman NJ, eds. ischaemia than ganglion cell bodies, allowing Walsh and Hoyt’s clinical neuro-ophthalmology. 5th ed. the axons of the more central foveal ganglion Baltimore: Williams and Wilkins, 1998:249–52. 8 Stone EM , Newman NJ, Miller NR, et al. Visual recovery in cells to remain intact and functional despite patients with Leber’s hereditary optic neuropathy and the exposure to a degree of ischaemia suYcient to 11778 mutation. J Clin Neuro-Ophthalmol 1992;12:10–14. 9 Wisselink GW. Ein Fall von traumatischer Erkrankung der destroy surrounding ganglion cells. Macula lutea. Klin Monatsbl Augenheilkd 1905;43:385–91. A discrete precipitating event was identiﬁed 10 Fuchs A. Uber kleinste dauernde Ringskotome nach Verke- hrsunsfällen und parazentrales Skotom nach elektrischer in only one of our patients, a young woman Ohrprüfung. Klin Monatsbl Augenheilkd 1933;91:20–30. who experienced an episode of postpartum 11 GiVord SR, Marquardt G. Central angiospastic retinopathy. Arch Ophthalmol 1939;21:211–28. hypotension shortly before the onset of visual 12 O’Brien DM, Farmer SG, Kalina RE, et al. Acute macular symptoms (case 5). This case also diVered neuroretinopathy following intravenous sympathomimet- ics. Retina 1989;9:281–6. from the other four in that AMNR-like macu- 13 Guzak SV, Kalina RE, Chenworth RG. Acute macular neu- lar lesions were present and were associated roretinopathy following adverse reaction to intravenous contrast media. Retina 1983; 3:312–17. with angiographic evidence of focal choroidal 14 Leys M, Van Slycken S, Koller J, et al. Acute macular hypoperfusion. It may be that a drop in blood neuroretinopathy after shock. Bull Soc Belge d’ Ophtalmol 1991;241:95–104. pressure produced a global decrease in ocular 15 Bos PJM, Deutman AF. Acute macular neuroretinopathy. perfusion that involved both retinal and Am J Ophthalmol 1978;80:573 16 Rush JA. Acute macular neuroretinopathy. Am J Ophthalmol choroidal circulations. If this were so, the 1977;83:490 visible macular lesions may have been related 17 Priluck IA, Buettner H, Robertson DM. Acute macular to outer retinal ischaemia. neuroretinopathy. Am J Ophthalmol 1978; 86:775. 18 Sieving PA, Fishman GA, Salzano T, et al. Acute macular Of note, the other four patients were heavy neuroretinopathy: early receptor potentials change suggests caVeine consumers. Indeed, case 4 consumed photoreceptor pathology. Br J Ophthalmol 1984:68:229–34. 19 Miller MH, Spalton DJ, Fitzke FW, et al. Acute macular approximately 20 cups of coVeeaday!CaVeine neuroretinopathy. Ophthalmology 1989;96:265–9. 164 Kerrison, Pollock, Biousse, et al

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