White Spot Syndromes and Related Diseases

Mirza, Rukhsana G.; Jampol, Lee M.

doi:10.1016/b978-1-4557-0737-9.00076-x

Cited by 10 publications

(15 citation statements)

References 343 publications

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“…Previous studies demonstrated that α-syn triggers neuroinflammation, and that, in turn, inflammation increases α-syn phosphorylation and pathology in synucleinopathies ( Lee et al, 2010 ; Tansey and Goldberg, 2010 ; Ramirez et al, 2017 ; Ferreira and Romero-Ramos, 2018 ). Furthermore, retinal inflammation has been linked to both swelling of the outer retina and ERG deviations, and may therefore underlie -at least in part- the OCT and ERG abnormalities that we observed in the Thy1-h[A30P]α-syn mice ( Mirza and Jampol, 2013 ; Petzold, 2016 ; Pisa et al, 2021 ; Xia et al, 2021 ). Hence, we next investigated macroglia and microglia reactivity and water homeostasis in the retina.…”

Section: Resultsmentioning

confidence: 94%

“…Outer retinal thickening has also been observed to co-occur with supernormal ERG measurements in the retina of the 3×Tg-AD Alzheimer’s ( Chiquita et al, 2019a ). Furthermore, both supernormal scotopic ERG measurements and PL layer thickening have been related to a mild inflammatory phenotype in the early stages of retinal pathology linked to multiple sclerosis ( Mirza and Jampol, 2013 ; Petzold, 2016 ). Yet, with the measurements used in this study, no abnormalities in AQP4 water channels and no inflammatory response of the macro- and microglia was detected.…”

Section: Discussionmentioning

confidence: 99%

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Characterizing the Retinal Phenotype of the Thy1-h[A30P]α-syn Mouse Model of Parkinson’s Disease

et al. 2021

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Despite decades of research, disease-modifying treatments of Parkinson’s disease (PD), the second most common neurodegenerative disease worldwide, remain out of reach. One of the reasons for this treatment gap is the incomplete understanding of how misfolded alpha-synuclein (α-syn) contributes to PD pathology. The retina, as an integral part of the central nervous system, recapitulates the PD disease processes that are typically seen in the brain, and retinal manifestations have emerged as prodromal symptoms of the disease. The timeline of PD manifestations in the visual system, however, is not fully elucidated and the underlying mechanisms are obscure. This highlights the need for new studies investigating retinal pathology, in order to propel its use as PD biomarker, and to develop validated research models to investigate PD pathogenesis. The present study pioneers in characterizing the retina of the Thy1-h[A30P]α-syn PD transgenic mouse model. We demonstrate widespread α-syn accumulation in the inner retina of these mice, of which a proportion is phosphorylated yet not aggregated. This α-syn expression coincides with inner retinal atrophy due to postsynaptic degeneration. We also reveal abnormal retinal electrophysiological responses. Absence of selective loss of melanopsin retinal ganglion cells or dopaminergic amacrine cells and inflammation indicates that the retinal manifestations in these transgenic mice diverge from their brain phenotype, and questions the specific cellular or molecular alterations that underlie retinal pathology in this PD mouse model. Nevertheless, the observed α-syn accumulation, synapse loss and functional deficits suggest that the Thy1-h[A30P]α-syn retina mimics some of the features of prodromal PD, and thus may provide a window to monitor and study the preclinical/prodromal stages of PD, PD-associated retinal disease processes, as well as aid in retinal biomarker discovery and validation.

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Section: Resultsmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 99%

Characterizing the Retinal Phenotype of the Thy1-h[A30P]α-syn Mouse Model of Parkinson’s Disease

et al. 2021

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“…3, 4 The etiology of this inflammation remains unknown; however, an immune driven reaction preceding viral illness or vaccination has been suggested. 5 …”

Section: Introductionmentioning

confidence: 99%

Acute Posterior Multifocal Placoid Pigment Epitheliopathy on Optical Coherence Tomography Angiography

Heiferman

Rahmani

Jampol

et al. 2017

Retina

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Purpose To investigate choroidal involvement in acute posterior multifocal placoid pigment epitheliopathy (APMPPE). Methods A retrospective observational case series using multimodal imaging including optical coherence tomography angiography (OCTA). Results Five patients with APMPPE were included. In most acute lesions, OCTA revealed outer retinal and retinal pigment epithelium (RPE) hyper-reflective lesions with attenuated OCT signal in the underlying choroid, but careful examination allowed us to identify a single lesion with decreased choriocapillaris flow outside the signal attenuation. OCTA obtained after healing of lesions revealed areas of hypo-intense circular flow voids clustered in groups surrounded by either iso- or hyper-intense signal background. Point-by-point evaluation revealed these flow voids did not correspond to areas of RPE thickening or focal pigmentary changes. Larger hypo-intense lesions were observed and did correlate with pigmentary changes. Conclusions Our case series demonstrates choriocapillaris flow abnormalities in acute APMPPE extending beyond the OCT lesions, and distinct residual vascular abnormalities in healed APMPPE lesions on OCTA. Our findings support a primary ischemic insult to the photoreceptors and RPE, but choriocapillaris flow abnormalities could be secondary to (OCT invisible) retinal and RPE involvement. The lack of understanding of the etiology along with the inability to visualize most of the choroid in acute lesions preclude definite conclusions about the true pathogenesis of APMPPE.

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“…The acute phase of APMPPE generally resolves over the course of weeks to months [ 1 , 2 ]. Smaller lesions may resolve spontaneously with little evidence of prior pathology on ophthalmoscopy.…”

Section: Acute Posterior Multifocal Placoid Pigmentary Epitheliopathymentioning

confidence: 99%

“…The white dot syndromes (WDS) comprise a group of diseases characterized by lesions of the outer retina, retinal pigment epithelium, choriocapillaris, choroid or a combination of these sites [ 1 , 2 ]. The WDS are hypothesized to have an inflammatory etiology, and this is more evident for certain conditions, such as birdshot chorioretinopathy (BCR), in which vitreous inflammation and retinal vasculitis often accompany the choroidal birdshot lesions.…”

Section: Introductionmentioning

confidence: 99%

Multimodal Imaging of the White Dot Syndromes and Related Diseases

Knickelbein¹,

Sen²

2016

J Clin Exp Ophthalmol

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The white dot syndromes encompass a group of rare posterior uveitis conditions that are characterized by outer retinal and/or choroidal hypopigmented lesions that are thought to be inflammatory in nature. The size, shape, and location of lesions in the fundus aid in differentiating these conditions. Multimodal imaging, including modalities such as fundus autofluorescence, optical coherence tomography, fluorescein angiography, and indocyanine green angiography, among others, has become integral in diagnosing and monitoring many of the white dot syndromes. Furthermore, multimodal imaging modalities have provided insights into the pathogenesis and exact sites within the retina and choroid affected by white dot syndromes.

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White Spot Syndromes and Related Diseases

Cited by 10 publications

References 343 publications

Characterizing the Retinal Phenotype of the Thy1-h[A30P]α-syn Mouse Model of Parkinson’s Disease

Characterizing the Retinal Phenotype of the Thy1-h[A30P]α-syn Mouse Model of Parkinson’s Disease

Acute Posterior Multifocal Placoid Pigment Epitheliopathy on Optical Coherence Tomography Angiography

Multimodal Imaging of the White Dot Syndromes and Related Diseases

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