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David Mcleod - One of the best experts on this subject based on the ideXlab platform.

  • letter to the editor partial central retinal artery occlusion offers a unique insight into the ischemic penumbra
    Clinical Ophthalmology, 2011
    Co-Authors: David Mcleod
    Abstract:

    Kurimoto and colleagues recently reported three cases of unilateral visual loss associated with striking ischemic changes in the posterior retina and impressive visual recovery following treatment.1 The presenting signs included an indistinct foveal Cherry-Red Spot and a circle of cotton-wool Spots (CWSs) surrounding the optic disc. Fundus fluorescein angiography (FFA) revealed a marked delay in the arm-retina circulation time, together with “areas of occlusion of the retinal arterioles” that corresponded to the location of the CWSs. The authors recognized that this was a variant of central retinal artery occlusion (CRAO), but they confessed to being unclear as to the processes underlying this distinctive clinical picture.1 They surmised that the central retinal artery (CRA) had been incompletely obstructed and that multiple arterioles closer to the capillary network had also been occluded. Thus, the polymorphous CWSs were regarded as “retinal microinfarctions” involving the nerve-fiber layer (NFL), a view that is in line with widely received wisdom in this regard. If this was truly the case, however, simultaneous occlusions of a dozen or more arterioles of differing sizes must have contributed to the fundus appearance, which is unlikely. Kurimoto and colleagues will need to set aside some of the current orthodoxy in the field of ocular vascular occlusive disorders to appreciate the pathophysiological processes at play in their patients.

Haci Koc - One of the best experts on this subject based on the ideXlab platform.

  • isolated macular cherry red Spot without systemic disease
    Journal of Clinical & Experimental Ophthalmology, 2016
    Co-Authors: Ibrahim Kocak Faruk Kaya Hakan Baybora, Haci Koc
    Abstract:

    Introduction: Macular Cherry-Red Spot can be a sign of different disorders. It is mostly a sign of retinal artery occlusion in adults, and sphingolipid storage diseases in infants. In the presenting case, parafoveal ganglion cell hypertrophy in an otherwise healthy man presented. Case presentation: A 52 years old man presented with nearsightness. He had no significant past medical history except myopia. Best corrected visual acuity was 10/10 in both eyes. Dilated fundus examination showed bilateral abnormal parafoveal yellow-white light reflex resembling Cherry-Red Spot. Midperipheral retina had tiny pigmentary mottling and pigment epithelium atrophy areas in both eyes. OCT demonstrated bilateral hyperreflective parafoveal thickened ganglion cell layer. Bilateral mottled hyperfluorescent areas of midperipheral retina was seen in fundus fluorescein angiography. Hexosaminidase A, β-galactosidase, neuraminidase, and acidsphingmyelinaze activity were normal. Abdominal ultrasonography and central nervous system imaging had no pathological findings.

Tsunehiko Ikeda - One of the best experts on this subject based on the ideXlab platform.

  • central retinal artery occlusion resembling purtscher like retinopathy
    Clinical Ophthalmology, 2011
    Co-Authors: Takuji Kurimoto, Norio Okamoto, Yuko Kanbara, Tomohiko Etomi, Masahiro Tonari, Tsunehiko Ikeda
    Abstract:

    This paper reports three cases of central retinal artery occlusion (CRAO) with Purtscher-like retinopathy and good recovery of visual function. The three cases of CRAO had similar fundus changes, ie, cotton wool patches surrounding the optic disc and whitening of the retina surrounding the fovea with a cherry red Spot. Fluorescein angiography showed a delay of arm-to-retina circulation time and a partial defect of choroid circulation. Although the three cases were treated by different regimens of steroid pulse therapy and antiplatelet therapy, visual function recovered well and all disturbances of the retinal and choroid circulations resolved. Although eyes with a CRAO normally have a poor visual prognosis, our three cases responded well to the treatments and recovered good visual function. Thus, cases showing fundus changes similar to our three cases may have a pathogenesis different from that of a complete CRAO.

Wuh-liang Hwu - One of the best experts on this subject based on the ideXlab platform.

  • a longitudinal study of taiwanese sialidosis type 1 an insight into the concept of cherry red Spot myoclonus syndrome
    European Journal of Neurology, 2009
    Co-Authors: S.-c. Lai, R.-s. Chen, H.-c. Chang, L.-y. Kao, Ying-zu Huang, Y.-h. Weng, J.-k. Chen, Y Wu H Chou, Wuh-liang Hwu
    Abstract:

    Background and purpose:  Sialidosis type 1 (ST-1) is a neurodegenerative disorder with limited long-term follow-up report. This study is to document the chronological profile of ST-1. Methods:  We perform serial analysis of 17 Taiwanese patients with ST-1 focusing on evolution of clinical features, electrophysiological findings, genetic studies, and neuroimage examinations. Results:  All patients had a mutation at 554AG in exon 3 of the NEU1 gene causing Ser182Gly substitution. Fifteen patients were homozygous. Two patients were heterozygous with novel mutations, 956CT causing Ala319Val in one and 163CT causing Gln55stop codon in the other. The neuraminidase activity was markedly decreased in all 11 available patients. Only three patients (17.6%) manifested the macular Cherry-Red Spot. The majority of patients (82.3%) developed full-blown manifestation of myoclonus, ataxia, and seizures within 5 years. Abnormal somatosensory evoked potentials with giant cortical waves were found in all patients. Prolonged P100 peak latency of the visual evoked potentials (VEPs) were found in 16 patients (94.1%) in the early stage even without visual symptoms. Conclusion:  ST-1 in Taiwanese population illustrates distinct characteristics of phenotype with infrequent Cherry-Red Spot. We suggest to screen the NEU1 mutations in patients presenting action myoclonus with abnormal VEPs, even without macular Cherry-Red Spots.

M Fukuda - One of the best experts on this subject based on the ideXlab platform.

  • Observations on time course changes of the cherry red Spot in a patient with Tay-Sachs disease
    British Journal of Ophthalmology, 2000
    Co-Authors: M Nakaya-onishi, A Suzuki, N Okamoto, M Fukuda
    Abstract:

    Editor,—Tay-Sachs disease is characterised by lesions in the central nervous systems due to the precipitation of GM2 trihexosylceramide in neurocytes.1 Its onset at around 6 months after birth is manifested by mental and emotional retardation together with hypomyotonia and hyperacusis as its typical signs. Subsequently, the patient tends to develop convulsions insidiously and usually dies at 2–4 years of age. A cherry red Spot and optic nerve atrophy are the characteristic ophthalmic signs in this disease. This report deals with observations of a patient with Tay-Sachs disease whose ophthalmic signs were monitored from birth to his death at the age of 5 years 8 months. During this period, a cherry red Spot developed and then diminished in both eyes. ### CASE REPORT The subject was a boy who was born weighing 1600 g at a gestational age of 36 weeks. Two weeks after birth, ophthalmoscopy …