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Purpose: To report findings of optic disc melanocytoma (ODM) obtained using spectral domain optical coherence tomography (SD OCT), with special reference to the central retinal artery and vein surrounded by hyperreflective dots., Methods: Retrospective review of five eyes of five patients with ODM. Demographic information, ophthalmic examination including best-corrected visual acuity, dilated funduscopic examination, and SD OCT images were evaluated., Results: Dome-shaped, darkly pigmented tumors were seen ophthalmoscopically in the optic discs of all eyes. On OCT, the first branches of the central retinal artery and/or vein were well defined as oblique sections of tubular structures with a perivascular distribution of hyperreflective dots in the elevated retina (nerve fiber layer) over the tumor. The portions where these vessels turn toward the retina were displaced more anteriorly than those of eyes without ODM. Hyperreflective dots of various sizes were also observed in elevated retinas over the tumors, which shadowed and obscured the subjacent tissue in all eyes., Conclusions: SD OCT provides higher definition images of ODM relating to the branches of the central retinal artery/vein, revealing anterior displacement of vessels and perivascular distribution of hyperreflective dots that suggest melanophages and/or tumor cells or proteins and/or lipid deposits.

Glycoconjugates, consisting of O- and N-linked types, are present on the surface of photoreceptor cells and in the interphotoreceptor matrix (IPM). This study was undertaken to facilitate understanding of the metabolic features of O-linked sialoglycoconjugates in comparison with those of N-linked glycoconjugates in the rat retina. We examined the developmental change in distribution of Gal beta 1,3GalNAc alpha 2,3-sialyltransferase mRNA in rat retinas using in situ hybridization histochemistry to detect the synthesis period of O-linked sialoglycans. Positive hybridization signal was observed in the ganglion cells throughout the postnatal days (P) examined, whereas strong signals were detected in the photoreceptor inner segments and the inner nuclear layer exclusively at P16 and P18. At P20 and older, weak or sparse signals were detected in these regions. It is likely that O-linked sialoglycoconjugates, which are detected in the mature IPM, are actively synthesized from P16 to P18 in the rat photoreceptor inner segments, suggesting that the O-linked sialoglycoconjugates in the photoreceptor layer may be stable with litter turnover in the mature retina.

Ultrastructural changes in the distribution of free ribosomes in photoreceptor inner segments in relation to photoreceptor degeneration in Purkinje cell degeneration (pcd) were studied in mutant mice reared under cyclic light and sacrificed at 30 and 120 postnatal days. On postnatal day 30, some photoreceptors appeared to be normal whereas others had degenerated to varying degrees with numerous spherules in the extracellular space surrounding degenerating inner segments. By postnatal day 120, retinal degeneration had progressed with a marked loss of photoreceptors. The distribution of free ribosomes in the mutant inner segments was random, clustered, or uniform. This was distinct from normal control retinas in which random distribution predominated. Markedly degenerated inner segments contained sparse ribosomes which coalesced in small aggregates. These changes in ribosome distribution seemed to be associated with the extent of photoreceptor degeneration. The significance of these changes in ribosome distribution, in particular clustered distribution, is discussed together with our previous findings.

The binding of amaranthin, specific for the Gal beta 1,3 GalNAc and NeuAc alpha 2,3 Gal beta 1,3 GalNAc sequences, to the photoreceptors of the monkey retina was investigated using the avidin-biotinylated peroxidase method. Amaranthin bound to the surfaces of both cone and rod photoreceptors. This and previous lectin histochemical studies show that O-glycoside-linked glycoconjugates are present on the surfaces of both cones and rods: Gal beta 1,3 GalNAc and NeuAc alpha 2,3 Gal beta 1,3 GalNAc are the terminal sugars of the glycoconjugates around cones and rods, respectively.

The developmental changes in the binding of Maackia amurensis lectin, specific for sialic acid alpha 2,3 galactose sequence, to the rat retina was investigated using the avidin-biotinylated peroxidase method. The lectin bound to the surfaces of photoreceptor outer segments from postnatal day 16 (P16), whereas it had bound to the other retinal layers from P14. The intense labelings of the outer segments were interspersed with unstained portions, which may correspond to cone photoreceptors. These results confirm that the sialic acid residues on the terminus of carbohydrate chains increase at P16 and mask the beta-galactose residues around rod outer segments.

Effects of nucleotide phosphates on the sialyltransferase activity in the neurosensory retina of the bovine eye were studied. Enzyme activity was assayed using cytidine monophosphate-[14C]-N acetylneuraminic acid as a substrate and desialylated fetuin as an exogenous acceptor. Cytidine-5'-diphosphate and adenosine triphosphate inhibited the enzyme activity. Uridine diphosphate and guanosine diphosphate increased the enzyme activity at low concentrations and decreased the activity at high concentrations. Cyclic adenosine monophosphate and cyclic guanosine monophosphate increased the enzyme activity at concentrations up to 8 mM. It is thus concluded that sialyltransferase activity of the neural retina may be affected by various nucleotides, its alteration depending on either the type of nucleotides or their concentration.

Ophthalmological evaluations were made of the records of a series of 38 patients with HTLV-I-associated myelopathy, a chronic progressive myelopathy caused by human T-lymphotropic virus type I (HTLV-I). Four patients with no contributory family history showed pigmentary degenerative changes of the retina and choroid. Two of the patients (73-year-old woman, 68-year-old woman) had a progressive visual loss and night blindness with morphologic and functional features of diffuse pigmentary retinal degeneration. The other two patients (59-year-old man, 72-year-old man) complained of recently developed visual loss with sectorial or regional retinochoroidal atrophy. These elderly patients claimed that they had been healthy until a few years before presentation, not only visually but also neurologically. It was concluded, together with an epidemiologic consideration, that the coexistence of pigmentary retinal degeneration and HTLV-I-associated myelopathy is not simply chance but indicates a close association between the two conditions. It is proposed that HTLV-I infection might be a primary causative factor of degenerative changes of the retina and choroid, although the pathogenesis remains to be defined.

Ocular manifestations in patients infected with human T-lymphotropic retrovirus type I (HTLV-I) consisted of a wide range of neoplastic, infectious and noninfectious vascular or inflammatory lesions. These disorders were associated with two distinct HTLV-I-induced systemic diseases, ie, adult T-cell leukemia/lymphoma and HTLV-I-associated myelopathy. Five of the 10 cases of adult T-cell leukemia/lymphoma had inflammatory or opportunistic infectious ocular lesions, including cytomegalovirus retinitis or eyelid tumor as part of generalized lymphomas. Four of the 17 cases of HTLV-I-associated myelopathy showed noninfectious lesions such as isolated, transient cotton-wool spots and granulomatous iridocylitis. Twenty-four (26.9%) of 89 cases with various ocular diseases but no HTLV-I-induced systemic disease had antibodies to HTLV-I in the serum. The aqueous humor antibodies to HTLV-I in the seropositive carriers were negative, except one carrier case who showed isolated cotton-wool spots in one eye and massive vitreous opacities in the other eye.