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• W2036836223 abstract "Purpose: To assess retinal morphology in acute zonal occult outer retinopathy (AZOOR) without ophthalmoscopically visible fundus changes. Methods: Retrospective case series. Two consecutive patients with bilateral AZOOR with photopsia corresponding to areas of visual field loss and a normal fundus appearance were examined using optical coherence tomography (OCT), automated perimetry and electroretinography (ERG). Results: Both patients demonstrated photoreceptor atrophy corresponding to partial or complete scotomata with reduced or extinct electroretinographic responses. Attenuation or complete loss of all the segments composing the photoreceptor layer was found by OCT. Full-field ERG revealed affection of the 30 Hz flicker responses and subnormal photopic responses in both patients and subnormal scotopic responses in case 1. Multifocal electroretinography (mERG) revealed localized outer retinal dysfunction. The field loss was more extensive than the area of photoreceptor loss. Conclusion: Photoreceptor atrophy can be demonstrated in AZOOR without ophthalmoscopically visible fundus lesions. Acute zonal occult outer retinopathy (AZOOR) is a rare disease characterized by the acute development of scotomata of retinal origin in eyes without biomicroscopic or fluorescein angiographic abnormalities (Gass 1993). Central vision is reportedly spared in 75% of eyes with AZOOR (Gass et al. 2002), visual loss occurring in the form of irregularly distributed scotomata. Photopsia is a prominent symptom. Visible fundus changes may follow in later stages of the disease. Patients often report having experienced flu-like symptoms and fever a few days before the onset of symptoms. A high proportion of patients are young myopic women who are otherwise in good health. Recurrence of symptoms after a period of quiescence is frequent. Bilateral affection is seen in 75% of patients (Gass et al. 2002). The course is often one of abrupt onset, subsequent slow progression over a period of about 6 months and then stability; occasional bouts of recurrence are seen in some patients. After a certain period of time, visible fundus changes may appear in the form of vessel narrowing, perivascular sheathing and mottled pigmentation (Gass et al. 2002). Electroretinography (ERG) features a delayed a-wave response (Jacobson et al. 1995), showing a locus of retinal dysfunction in the outer retina and delayed 30 Hz flicker response (Francis et al. 2005), indicating that the inner retina is also affected in AZOOR. Additionally, a more diffuse affection of the retina is indicated by observations of abnormal electro-oculographic readings (Francis et al. 2005). The aetiology of AZOOR is unknown. The resemblance with the ophthalmoscopically occult cancer-associated retinopathies has prompted a search for pathogenic antiretinal antibodies, but so far the findings have been inconclusive (Jacobson et al. 1995). No therapy is available, and it is noteworthy in relation to the immunogenic theory that no therapeutic benefit appears to result from immunosuppression. The course of disease is mostly tolerable but severe visual loss does occur in some patients. In this article, we describe the use of multifocal electroretinography (mERG) and optical coherence tomography (OCT) to localize dysfunction of the neurosensory retina corresponding to the scotomata and photopsia of two patients with AZOOR and the use of OCT to search for evidence of corresponding structural damage. Two patients diagnosed with AZOOR were examined using colour and red-free greyscale fundus photography, OCT, multifocal and full-field ERG, automated threshold perimetry and methods of routine ophthalmic examination. OCT scans were enhanced by alignment and summation of multiple scans from identical locations of the retina (Sander et al. 2005). The study was a retrospective review of two consecutive cases diagnosed at our institution between 1999 and 2004. The study was carried out under institutional approval and followed the tenets of the Declaration of Helsinki. A 56-year-old male presented with scintillating scotomata, one area being involved in each eye, both being described as banana-shaped and located at about 30° eccentricity in the upper temporal quadrants. The scotomata were enhanced subjectively by bright light. The photopsia had a regularly pulsating and bilaterally synchronous appearance somewhat above the rhythm of the patient’s heartbeat. The onset of visual symptoms occurred 1 week after the patient had recovered from 2 days of malaise and fever. When seen 1 month later, best-corrected visual acuity (BCVA) was 1.0 in both eyes (refraction: +0.5D / +0.5D). The appearance of the anterior segment and the fundus was normal in both eyes, as was fundus fluorescein angiography. Elevated serum IgG herpes simplex antibody titres prompted a 1 week course of systemic acyclovir and subsequently (when the symptoms persisted and the patient was found to also have elevated serum IgG and IgM anti-toxoplasmosis titres) a 4 week course of systemic sulfadiazin and trimethoprim. Cranial magnetic resonance imaging (MRI) was normal and investigation for cutaneous melanoma, lymphoma, blood dyscrasia, pulmonary disease and bone-marrow dyscrasia were negative. The patient’s symptoms persisted throughout 4 years of follow-up, during which the solid angular subtense of his visual field defects increased by about 30% in both eyes and the toxoplasmosis antibody titres remained elevated (both IgG and IgM) in the absence of generalized antibody concentration elevation. The ophthalmoscopic fundus appearance also remained normal. A woman aged 18 years presented with paracentral scotomata and associated photopsia of acute onset in both eyes, the onset of symptoms having occurred shortly after the patient had suffered a few days of fever and malaise. BCVA was 1.0 in both eyes (refraction: −0.75D/−0.5D). The symptoms gradually improved in the right eye, while persisting to some extent in the left eye, which was also affected most severely at presentation. Three years later, the patient complained of new scotomata, this time primarily in the right eye. BCVA was still 1.0 in both eyes. A thorough ophthalmic examination including fundus biomicroscopy and fluorescein angiography was normal. Visual function was unaffected after a 2 week course of oral prednisolone 1 mg/kg/day. Autofluorescence fundus photography was normal in both eyes. In case 1, manual tangent screen examination and automated threshold perimetry demonstrated heteronymous absolute scotomata, confined largely to the inferotemporal quadrants (Fig. 1). In case 2, manual tangent screen perimetry revealed one small paracentral relative scotoma in the right eye and two in the left eye (Fig. 2). When the patient complained of new scotomata 3 years later, automated threshold perimetry revealed paracentral relative scotomata in both eyes corresponding to the patient’s complaints and a generalized sensitivity reduction in the left eye. Automated threshold perimetry (30–2) of both eyes and multifocal electroretinography (mERG) from the left eye of a male patient with acute zonal occult outer retinopathy 3 months after the onset of symptoms. Several areas of absolute scotomata can be observed. In the left eye, the larger area of absolute scotomata correlates with the reduced first-order amplitude potentials found on the mERG. Patient drawing as manual tangent field screening from both eyes of a female patient with acute zonal occult outer retinopathy at the time of onset of her symptoms. The patient has drawn around the areas where her vision is blurred because of relative scotomata. One affected area is seen in the right eye and two in the left eye. In case 1, full-field ERG showed subnormal scotopic and photopic function, including reduced and delayed scotopic and photopic 30 Hz flicker responses. Reduced first-order amplitude potentials were found by mERG corresponding to the visual field defects (Fig. 1). Electro-oculography demonstrated a light rise within the lower normal range. In case 2, full-field ERG demonstrated abnormal rod responses in both eyes and reduced 30 Hz flicker amplitude in the left eye. mERG confirmed the presence of localized outer retinal dysfunction in the macula in both eyes, most prominently in the left eye. Inner retinal signals were more diffusely delayed and of reduced amplitude in both eyes. In case 1, OCT performed 2 years after the onset of symptoms (OCT Stratus; Zeiss-Humphrey, Dublin, California, USA) and analysed using a new method of image enhancement (Sander et al. 2005) demonstrated attenuation of the photoreceptor layer in both eyes, including (but not limited to) the areas of retinal dysfunction (Fig. 3). In the most severely affected regions of the visual field, retinal atrophy included the outer nuclear layer as well as the inner and outer photoreceptor segments (Fig. 3; arrow with asterisk). Atrophy in the photoreceptor layer was also visualized by the lack of a clear junction between photoreceptor outer segments and the retinal pigment epithelium (RPE); the normal structures of the retinal layers were distorted. In case 2, OCT demonstrated moderate photoreceptor layer attenuation corresponding to the dysfunctional retina, where a relatively thick outer plexiform layer was also found (Fig. 3). These abnormalities were most pronounced in the left eye. Optical coherence tomography (OCT) scans in colour and greyscale. To the left, transfoveal OCT scans from a male patient (case 1) with acute zonal occult outer retinopathy (AZOOR) in both eyes (nasal inferior quadrant to the left, temporal superior quadrant to the right). Photoreceptor atrophy was visible in the nasal inferior quadrant. The most prominent change was seen in the photoreceptor layer, with affection of both the outer nuclear layer (ONL) and the inner and outer segments of the photoreceptors (IS/OS). The two arrows pointing out the ONL demonstrate the gradual thinning of this layer until it disappears in the nasal inferior quadrant. An increase in the apparent thickness of the outer plexiform layer (OPL) was also observed. The arrow with asterisk points at the most severely affected area of the retina. To the right, vertical transfoveal OCT scans of the retina of a female patient (case 2) with AZOOR in both eyes (inferior to the left, superior to the right). Above the fovea the hyper-reflective characteristic of the OPL extends outward in a part of the retina where the ONL is attenuated prominently. The location of this abnormality corresponded to a relative scotomata in the left eye. In the two cases described in this article, the focal diagnostic of outer retinal dysfunction was made using mERG. OCT of the dysfunctional retina identified an abnormally thin photoreceptor layer; some degree of abnormality was also visible in the adjacent layers of the retina. The abnormalities ranged from subtle loss of photoreceptor sublayer reflectivity to complete photoreceptor layer atrophy. The reduction in function, which ranged from subtle relative scotomata to absolute scotomata on perimetry and tangent screen testing, was in general agreement with the degree of structural abnormality, although pronounced photoreceptor layer attenuation could be seen outside the absolute scotomata. Electroretinographic abnormalities included low full-field amplitudes and long latency in the most affected eyes, suggesting that an element of diffuse retinal dysfunction may be present. Our findings are compatible with photoreceptors being dysfunctional but preserved in early stages of AZOOR, whereas photoreceptors are lost in the later stages of AZOOR. This sequence of events also appears to be seen in inherited retinal degenerations (Gass et al. 2002; Francis et al. 2005). In AZOOR, a delay in the cone system-derived 30 Hz flicker response of the full-field ERG and a reduction of the light rise of the electro-oculogram have been demonstrated, indicating that the cones are more dysfunctional than the rods (Arai et al. 1998; Francis et al. 2005) and that retinal dysfunction is more widespread than the focal visual field loss would suggest (Francis et al. 2005). Our two cases had definite affection of the 30 Hz flicker response of the full-field ERG. This article is the first to document photoreceptor attenuation and/or loss in AZOOR. It is remarkable that this can occur in the absence of ophthalmoscopic abnormalities. The explanation may be that photoreceptors are reduced in size and/or number, while remaining present to an extent that prevents atrophy of the RPE. This distinction is of importance for the understanding of fundus pathology and its potential for therapeutic restitution. An abstract of parts of the study has previously been presented as a poster at the Nordic Congress of Ophthalmology, Copenhagen, June 17–20, 2006. The study was supported by The Danish Eye Health Society, Copenhagen and by a Patient-oriented Diabetes Research Career Award from the Juvenile Diabetes Research Foundation to Dr Larsen (grant no. 8-2002-130)." @default.
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• W2036836223 title "Photoreceptor atrophy in acute zonal occult outer retinopathy" @default.
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