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In a patient with juvenile nephronophthisis, sector retinitis pigmentosa was found as an extrarenal manifestation, establishing a hitherto undescribed variety of retinal degeneration occurring in this disorder. The retinal function in this case was identical with that in the classic type of sector retinitis pigmentosa, namely, subnormal ERG amplitudes but(More)
Three patients with cystic fibrosis of the pancreas initially were seen because of optic neuropathy caused by a toxic reaction to chloramphenicol. The ocular symptoms were characterized by loss of visual acuity, central scotomas, red-green dyschromatopsia, and fundus changes. An increase in the latency of the occipital evoked potentials paralleled the(More)
Two patients with post-operative adhesions between the iris and cornea are presented. The term of anterior pupillary block is suggested, and its clinical picture is described. The irido-corneal adherences appeared to be the trigger mechanism of the intraocular pressure rise and deepening of the posterior chamber. The tension was fully controlled following(More)
A 10-month-old girl presented with visual loss in the right eye associated with bilateral optic atrophy. The suggestive clinical diagnosis was an optic nerve glioma. The computerized tomographic findings were unusual in that symmetric bilateral involvement of the anterior visual pathways were present. The radiological diagnosis of an optic glioma was(More)
A 27-year-old woman suffered from multiple congenital defects, including transsphenoidal encephalocele. Recent progressive visual loss was at first attributed to this encephalocele, but was later proved to be caused by a suprasellar epidermoid cyst. Its removal was followed by improvement of vision. To the best of our knowledge, the association of basal(More)
An incomplete form of rod monochromatism is described in a young man with normal visual acuity and absence of nystagmus or photophobia. ERG showed normal threshold sensitivity in white and blue lights but virtual absence of the first portion of response in red light. The sensory threshold curve lacked the typical rod-cone discontinuity, whereas the curve(More)
The familial occurrence of retinal dysplasia in five affected male children suggested X-chromosome-linked recessive inheritance. The clinical features were childhood onset, severe visual impairment, head posture, nystagmus, and strabismus. The ophthalmoscopic findings varied in shape and extension; they ranged from retinal folds to dysplastic tissue(More)
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