Reticular tapeto-retinal dystrophy. As a possible late stage of Sjogren's reticular dystrophy.

Abstract

Two patients with a reticular dystrophy of the retina were noted to have fundus changes reminiscent of those reported in patients with Sjogren's reticular dystrophy. Our subjects were older than previously reported cases of Sjogren's dystrophy and manifested more extensive retinal abnormalities involving both the retinal pigment epithelium and photoreceptors. Similarities were noted both ophthalmoscopically and on fluorescein angiography. Reduced central visual acuity, abnormal findings on electroretinography, ophthalmoscopically evident hypopigmentation, and probable atrophy of the retinal pigment epithelium were specific to the more advanced condition. Case reportsCase I, a 72-year-old white man, had a 6-year history of bilateral decrease in central vision and a slowly progressive loss of night vision over the past io years. The patient could not recall having been examined by an ophthalmologist before the onset of these symptoms. His father allegedly had had similar difficulties with both central and night vision which had begun when he was in his forties, although further details of his eye disease were not available. Other family members, including seven children, were said to be not as yet affected. The patient was unaware of either Swedish or Dutch ancestors.When he was seen in September I974, the patient's best corrected vision was 3/200 in the right eye and Io/2oo in the left, with correction + iD sph. in the right eye and + I*25D sph. in the left. His general health was good. External and motility examinations were normal, both pupils reacted normally to direct and consensual light stimuli, slit-lamp examination of the anterior segments was normal, as was the intraocular pressure of 14 mm Hg in the right eye and i2 in the left. There was no increase in cellularity of the vitreous. The patient hadl to 2+ bilateral nuclear sclerotic and anterior cortical changes in both lenses. Fundus examination showed bilateral, midperipheral 360°reticular-appearing lesions (Fig. i) (Fig. 2). Fig. 2 also shows linear or sickle-shaped dark areas of hypofluorescence which correspond to zones of hyperpigmentation. Lesions in the posterior pole appeared more confluent and less hyperpigmented, no longer maintaining a reticular appearance as they approached the optic disc. Within the maculae were accumulations of moderately dense black clumps of retinal pigment epithelium (Fig. 3). The retinal vessels and optic discs were normal. Electroretinographic (ERG) responses were nondetectable in both eyes. Because of poor co-operation and a marked reduction in visual acuity, it was impossible to obtain reproducible visual fields, electro-oculogram (EOG), or dark-adaptation studies. The plasma omithine level was normal (Simmell and Takki, I973).

Sjögren Reticular Dystrophy of the Retinal Pigment Epithelium: A Case Report

“…Sjögre n , in fact, described a pathology involving only the RPE. This observation also confirms that the disease should not be considered a central tapeto-retinal degeneration, as described by Deutman et al (6), nor a late onset tapeto-retinal degeneration, as Fishman et al (8) suggested.…”

“…Reduction of visual acuity has been described only in patients with reticular dystrophy of the RPE with associated eye disease such as high myopia, spherophakia, strabismus, or ingrowth of macular choroidal neovascularization (13). In addition, reduction of visual acuity has been described by Fishman et al (8) in two very old patients; by Kingham et al (2) in a patient who had syphilis at the age of 15; and by Donnel et al (9), Gir a rd et al (10), and Cortin et al (12) in patients with reticular dystrophy of the RPE and extensive re t i n a l pathologies involving both the RPE and the photorec e p t o r s .…”

Long-term follow-up of a case of choroidal neovascularization secondary to reticular pigmentary retinal dystrophy

“…(6) Central vision is usually preserved in Sjögren dystrophy, except in a few cases characterized by geographic atrophy and/or CNV. (7,8) In the case reported, the patient already had these complications in the first examination, which revealed concurrent atrophy in the OD and an active neovascular membrane in the OS, with consequent visual impairment.…”

Pattern dystrophies of the retinal pigment epithelium

“…Pattern dystrophies of the macular pigment epithelium [1][2][3][4][5][6][7][8][9][10] are rare disorders: they are defined as follows: Pattern dystrophies are classically subdivided into several groups: butterflyshaped [11,12], reticular [13][14][15][16][17], macroreticular [18], adult-onset foveomacular vitelliform [1,19,20], fundus pulverulentus [21], benign annular and 'Cogwheel' dystrophy [22]. These subtypes have a more or less different fundus appearance, but were grouped under a common denominator because they share a certain number of characteristics, may be associated within the same pedigree [10,[23][24][25][26] and even within one patient, can show evolution from one subtype to another, but mainly because their pathogenesis is still unsolved.…”