UNASSIGNED: To report on a case of the successful treatment of Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) in a pediatric patient with a prior diagnosis of cerebral vasculitis.
UNASSIGNED: A 16-year-old male with a prior diagnosis of cerebral vasculitis presented without ocular complaints. Visual acuity was 20/20, and color vision remained normal. Fundus examination revealed yellowish-white placoid lesions and retinal pigmented epithelial changes involving the posterior pole. A work-up including a rapid plasma reagin test, complete cell blood count, comprehensive metabolic panel, and urinalysis was within normal limits. A head computed tomography angiography without contrast and a brain magnetic resonance imaging scan were compatible with acute and past episodes of ischemia. Ancillary testing was compatible with an assessment APMPPE. Immunosuppressive and monoclonal antibody therapy resulted in the improvement and remission without residual neurologic deficits and with a BCVA of 20/20.
UNASSIGNED: This case suggests that a diagnosis of cerebral vasculitis should prompt physicians to consider an ophthalmic evaluation that includes a dilated fundus exam, regardless of the presence or absence of ocular symptoms. Ophthalmic findings may affect the diagnostic processes, particularly concerning infectious and non-infectious etiologies, or potentially neoplastic diseases.

UNASSIGNED: To report a case of tuberculosis-related serpiginous-like choroiditis (TB-SLC) in Denmark in a patient with few risk factors.
UNASSIGNED: Single case report.
UNASSIGNED: A 54-year-old Caucasian male with no relevant travel history presented with unilateral light placoid confluent elements in the macula of the right eye with a best-corrected visual acuity of 0.2 Snellen. The left eye was normal. Wide-field Fluorescein and Indocyanine green-angiography were performed, and findings were consistent with acute posterior multifocal placoid pigment epitheliopathy. Since the condition was considered sight-threatening, and the patient had no recognizable risk factors for tuberculosis (TB), he was prescribed 50 mg of oral prednisolone. Blood tests and an X-ray were ordered to exclude infectious causes. The first interferon-ỿ release assay (IGRA) test was inconclusive and a new test was ordered. Over the following weeks new white dots appeared in the retina. After the patient had been treated for seven weeks with prednisolone, the second IGRA came back positive, and he was diagnosed with TB-SLC. Upon repeated questioning two months after baseline, the patient remembered that ten years ago he had been in a workplace with 50 different nationalities, and seven years ago he had been in contact with a friend who was treated for latent TB, thus supporting relevant exposure.
UNASSIGNED: TB-SLC may occur even in a patient with few recognizable risk factors and in a setting that is not TB endemic. It is imperative to continuously reassess differential diagnoses and initiate or repeat paraclinical testing in cases with atypical features.

This review covers the utility of electrophysiological studies relevant to inflammatory diseases of the retina in conditions such as acute posterior multifocal placoid pigment epitheliopathy, acute zonal occult outer retinopathy, Adamantiades-Behçet disease, autoimmune retinopathy and neuro-retinopathy, birdshot chorioretinopathy, multiple evanescent white dot syndrome, and Vogt-Koyanagi-Harada disease. Electrophysiological studies can help with the diagnosis, prognostication, evaluation of treatment effects, and follow-up for these conditions.

UNASSIGNED: To reassess the underlying pathophysiology of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and relentless placoid chorioretinitis (RPC) through comparison with the non-inoculated eye of the von Szily animal model of neurotropic viral retinal infection.
UNASSIGNED: Narrative review.
UNASSIGNED: Literature reports of isolated neurotropic viral entities and rising serological viral titers in APMPPE after presentation support a potential direct infective etiology. In general, viral transport along axons results in mitochondrial stasis and disruption of axoplasmic flow. Clinical manifestations of axoplasmic flow disruption in APMPPE/RPC may signify the passage of virus along the neuronal pathway. From a case series of 11 patients, we demonstrate a timely, spatial, and proportional association of optic disc swelling with APMPPE lesion occurrence. Signs within the inner retina appear to precede outer retinal lesions; and acute areas of outer nuclear layer (ONL) hyperreflectivity appear to be the result of coalescence of multiple hyperreflective foci resembling axonal spheroids (which occur as a consequence of axoplasmic disruption) and follow the Henle fiber layer neurons. Underlying areas of retinal pigment epithelium (RPE) hyper-autofluorescence follow ONL hyperreflectivity and may signify localized infection. Areas of apparent choriocapillaris hypoperfusion mirror areas of RPE/Bruch\'s membrane separation and appear secondary to tractional forces above. Increases in choroidal thickness with lesion occurrence and focal areas of choriocapillaris hypoperfusion are observed in both APMPPE/RPC and the von Szily model.
UNASSIGNED: The neurotrophic infection model provides significant advantages over the existing primary choriocapillaris ischemia hypothesis to account for the range of imaging signs observed in APMPPE and RPC.

UNASSIGNED: The term white dot syndromes has been used to refer conditions that differ in their morphology and prognosis. We report three cases of different pathologies encompassed within the white dot syndromes.
UNASSIGNED: Case 1: A 26-year-old female presented with scotoma in her right eye. Fundus examination revealed multiple white dots that demonstrated early hyperfluorescence with late staining on FA. OCT showed discontinuities in inner segment-outer segment junction associated with columnar-shaped outer retinal hyperreflective bands. AF revealed multiple hyperautofluorescent dots around the posterior pole, compatible with multiple evanescent white dot syndrome. The symptoms improved without treatment. Case 2: A 16-year-old male presented with retinal lesions compatible with punctate inner choroidopathy in his right eye. OCT showed lesion in the outer retinal layer. FAF revealed parafoveal hypoautofluorescent dots with early hyperfluorescence and late staining on FFA. After oral corticotherapy, they progress to atrophic scars. Case 3: A 65-year-old male presented with scotoma and decreased vision in his right eye. OCT showed hyperreflectivity in the outer layer that progresses to a large atrophic plaque with foveal affectation. FAF demonstrated hyperautofluorescent placoid lesion occupying macular area, compatible with acute posterior multifocal placoid pigment epitheliopathy. Retinal lesions improved with systemic corticosteroids.
UNASSIGNED: The FAF pattern helps know the distribution of the lesions. It represents a noninvasive method that has been shown to be useful in the diagnosis and monitoring of white dot syndromes.

BACKGROUND: Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare inflammatory eye disorder that is characterized by the presence of multiple placoid lesions in the posterior pole of the eye. Relentless placoid chorioretinitis (RPC) is an inflammatory chorioretinopathy that combines clinical features of APMPPE and serpiginous chorioretinitis, which is a progressive condition with a high risk of visual disability. Patients with COVID-19 can develop various ocular manifestations, however, there have been limited reports of APMPPE and RPC associated with the infection. We report a case of a patient who developed APMPPE after a COVID-19 infection and subsequently progressed into RPC.
METHODS: A 17-year-old male presented with a one-week history of painless gradual visual loss in both eyes. Two months prior to the visual symptoms, the patient had a SARS CoV-2 infection, confirmed by polymerase chain reaction test. Clinical findings with fundoscopy, optical coherence tomography and fluorescein angiography were consistent with APMPPE. Due to the severely affected vision in both eyes, the patient was started on 50 mg oral prednisolone daily, after which vision began to improve rapidly. Two months after symptom onset during steroid taper, the impression of continued inflammatory activity and new lesions in the retinal periphery of both eyes suggested RPC. Adalimumab 40 mg every other week was initiated with 12.5 mg prednisolone daily followed by slow tapering. Vision improved and five months after the start of the adalimumab treatment, the steroid was discontinued and there were no signs of active inflammation. The patient has been followed for a total of 21 months since presentation, had full visual recovery and good tolerance of the immunosuppressive treatment.
CONCLUSIONS: COVID-19 might cause long-lasting activity of APMPPE. The scarcity of reports compared with the number of confirmed COVID-19 infections worldwide suggests a rare entity. The association of APMPPE with a variety of infections may suggest a common immunological aberrant response that might be triggered by various factors. Further examinations and case reports are needed to understand the role of biological therapy in the treatment of such cases.

OBJECTIVE: The study analyzes the patterns of pathological changes in the retina and choroid in acute posterior multifocal placoid pigmented epitheliopathy (APMPPE).
METHODS: The results of the examination of two patients with bilateral APMPPE were analyzed retrospectively. The examination had included visometry, tonometry, static perimetry, autofluorescence, optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA).
RESULTS: The analysis revealed signs of the inflammatory nature of the choriocapillary vascular wall lesion with the development of their local obstruction and, consequently, local ischemia of the retinal pigment epithelium (RPE) and the outer layers of the retina in the acute period. At the same time, partial destruction of the ellipsoid zone, uneven hyperreflectivity of the RPE with local areas of its elevation and impaired connection with photoreceptors were revealed. The subsequent restoration of choriocapillaris perfusion was accompanied by significant restoration of the functional and structural state of the RPE over a larger area, as well as partial restoration of the ellipsoid zone of the retina. Two years after the onset of the disease, separate areas of defects of the RPE and the outer retina were observed, coinciding in localization with zones of severe circulatory deficiency at the level of choriocapillaris in the acute stage.
CONCLUSIONS: According to the results of multimodal imaging, choriocapillaritis is the initial link in the pathogenesis of APMPPE. The change in RPE and the outer retina is secondary to the local ischemic zones due to choriocapillaris nonperfusion. The nature of RPE change over a larger area manifests as a functional structural disorder, with the potential for recovery in case of choriocapillaris reperfusion.
UNASSIGNED: Проанализировать закономерности патологических изменений сетчатки и хориоидеи при острой задней мультифокальной плакоидной пигментной эпителиопатии (ОЗМППЭ).
UNASSIGNED: Ретроспективно проанализированы результаты обследования двух пациентов с двусторонней ОЗМППЭ. Обследование включало визометрию, тонометрию, статическую периметрию, аутофлюоресценцию, оптическую когерентную томографию и оптическую когерентную томографию-ангиографию.
UNASSIGNED: В остром периоде выявлены признаки воспалительного характера поражения сосудистой стенки хориокапилляров с развитием их локальной обструкции и вследствие этого локальной ишемии ретинального пигментного эпителия (РПЭ) и наружных слоев сетчатки. При этом были выявлены частичная деструкция эллипсоидной зоны, неравномерная гиперрефлективность РПЭ с локальными участками его элевации и нарушением соединения с фоторецепторами. Последующее восстановление перфузии хориокапилляров сопровождалось существенным восполнением функционально-структурного состояния РПЭ на большей его площади и частичным восстановлением эллипсоидной зоны сетчатки. Через 2 года от начала заболевания зафиксированы отдельные участки дефектов РПЭ и наружной сетчатки, совпадающие по локализации с зонами тяжелого дефицита кровообращения на уровне хориокапилляров в острой стадии.
UNASSIGNED: По результатам мультимодальной визуализации, хориокапиллярит является первичным звеном патогенеза ОЗМППЭ. Изменение РПЭ и наружной сетчатки является вторичным по отношению к локальным зонам ишемии вследствие хориокапиллярной неперфузии. Характер изменения РПЭ на большей площади поражения проявляется как функциональное нарушение структуры, с потенциалом восстановления при хориокапиллярной реперфузии.

Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) and serpiginous choroiditis are two diseases classified as \"white spot syndromes.\" Both are inflammatory/autoimmune diseases with suspected primary involvement of the choriocapillaris. The former usually has an excellent prognosis, while the latter can rapidly induce legal blindness. Whereas these diseases are well defined and well known, other entities (such as persistent placoid maculopathy or ampiginous choroiditis) with features of both APMPPE and serpiginous choroiditis have been described more recently. This review aims to describe demographic characteristics and multimodal imaging features to help differentiate between these four diseases.

BACKGROUND: Vaccination against the worldwide pandemic coronavirus disease 2019 (COVID-19) is underway; however, some cases of new onset uveitis after vaccination have been reported. We report a case of bilateral acute posterior multifocal placoid pigment epitheliopathy-like (AMPPE-like) panuveitis after COVID-19 vaccination in which the patient\'s pathological condition was evaluated using multimodal imaging.
METHODS: A 31-year-old woman experienced bilateral hyperemia and blurred vision starting 6 days after her second inoculation of the COVID-19 vaccination. At her first visit, her visual acuity was decreased bilaterally, and severe bilateral anterior chamber inflammation and bilateral scattering of cream-white placoid lesions on the fundus were detected. Optical coherence tomography (OCT) showed serous retinal detachment (SRD) and choroidal thickening in both eyes (OU). Fluorescein angiography (FA) revealed hypofluorescence in the early phase and hyperfluorescence in the late phase corresponding to the placoid legions. Indocyanine green angiography (ICGA) showed sharply marginated hypofluorescent dots of various sizes throughout the mid-venous and late phases OU. The patient was diagnosed with APMPPE and was observed without any medications. Three days later, her SRD disappeared spontaneously. However, her anterior chamber inflammation continued, and oral prednisolone (PSL) was given to her. Seven days after the patient\'s first visit, the hyperfluorescent lesions on FA and hypofluorescent dots on ICGA partially improved; however, the patient\'s best corrected visual acuity (BCVA) recovered only to 0.7 OD and 0.6 OS, and the impairment of the outer retinal layer was broadly detected as hyperautofluorescent lesions on fundus autofluorescence (FAF) examination and as irregularity in or disappearance of the ellipsoid and interdigitation zones on OCT, which were quite atypical for the findings of APMPPE. Steroid pulse therapy was performed. Five days later, the hyperfluorescence on FAF had disappeared, and the outer retinal layer improved on OCT. Moreover, the patient\'s BCVA recovered to 1.0 OU. Twelve months after the end of treatment, the patient did not show any recurrences.
CONCLUSIONS: We observed a case of APMPPE-like panuveitis after COVID-19 vaccination featuring some atypical findings for APMPPE. COVID-19 vaccination may induce not only known uveitis but also atypical uveitis, and appropriate treatment is required for each case.

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is classified as a part of the spectrum of the white dot syndromes affecting the inner choroid and the outer retina. It is usually bilateral and affects young patients between the second and fourth decades. The authors report an unusual case of unilateral APMPPE mimicking Vogt-Koyanagi-Harada (VKH) disease where the fundus fluorescein angiography was instrumental in confirming the diagnosis.
UNASSIGNED: A 35-year-old male presented with decreased visual acuity in the right eye for 3 days. Fundus examination revealed minimal vitritis, disc edema, and multifocal yellowish placoid lesions. Optical coherence tomography (OCT) showed the accumulation of subretinal fluid with subretinal septations closely mimicking VKH. Fundus fluorescein angiography depicted features of early hypofluorescence and late staining of the placoid lesions, suggesting APMPPE. Subretinal fluid partly resolved within a week, and visual acuity improved to 6/9(20/30) in the affected eye after the use of oral NSAIDS. Complete resolution of subretinal fluid was seen after 6 weeks.
UNASSIGNED: The most distinguishing feature in this case is the unilateral presentation and macular serous retinal detachment with subretinal septa on OCT imaging, which are not the typical features in APMPPE but quite similar to the characteristic features in acute VKH disease.
UNASSIGNED: APMPPE and acute VKH disease may share some overlapping clinical manifestations and imaging findings on OCT. APMPPE is a self-resolving disease, unlike VKH, and early diagnosis can avoid unnecessary administration of steroids and related side effects.