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Optic nerve dysfunction occurred several weeks after traumatic carotid-cavernous fistula developed in a 21-year-old man. Vision was completely restored after the fistula was closed with an intra-arterial detachable balloon. By carefully monitoring visual function in patients with traumatic carotid-cavernous fistulas, delayed optic neuropathy can be recognized and treated successfully.

• Ocular involvement with acute Epstein-Barr virus infection is usually limited to a transient follicular conjunctivitis, although other lesions have been reported. Chronic Epstein-Barr virus infection has recently gained attention, but ocular manifestations have not been emphasized. We describe three patients with chronic infection with prominent ocular involvement. Bilateral uveitis was noted in all patients, ranging from an anterior uveitis that was responsive to steroids to a severe panuveitis with vitritis, cataract, optic disc swelling, and macular edema. In one patient, topical acyclovir ointment resulted in a substantial decrease in the inflammatory reaction when added to systemic acyclovir therapy. Another patient displayed a keratitis that resolved with topical steroid therapy. Cataract and vitreous surgery were also beneficial in the management of these patients.

• One hundred patients with ocular occlusive vascular disease were studied by intravenous digital subtraction angiodraphy (IV DSA). In 17 patients with transient monocular blindness, 65% showed ipsilateral carotid atheroma. The degree of carotid stenosis was greater than 70% in eight of the 17 patients. Severe unilateral or bilateral stenosis was demonstrated in 70% of ten patients with chronic retinal artery obstruction, ie, venous stasis retinopathy or orbital hypoxia. An ipsilateral carotid artery source of emboli or substantial degree of stenosis was documented in 50% of 48 patients with acute retinal artery obstruction. Little or no carotid disease was found in 72% of 25 patients with retinal vein obstruction. The greatest value of IV DSA is in the management of patients with transient monocular blindness.

• The clinical course of patients with signs and symptoms suggestive of temporal arteritis but with negative temporal artery biopsy specimens was evaluated. Ninety-one patients undergoing a biopsy formed the basis of this study. Of these, 63 patients had no evidence of arteritis on biopsy, and 28 patients had biopsy specimens showing granulomatous inflammation. False-negative findings from biopsy specimens occurred in 5% of patients who had the disease. Cancer was the final diagnosis in 21% of patients with negative biopsy specimens v 3% of patients with temporal arteritis. Various chronic systemic inflammatory diseases were found in 16% of patients with negative biopsy specimens, while none of the patients with temporal arteritis had additional systemic inflammatory diseases. Patients with proven arteritis were notably older and had higher ESRs than patients without the disease. However, no laboratory test or frequently observed symptom or sign noted on initial examination, considered alone or in combination with other findings, had diagnostic sensitivity or specificity as high as temporal artery biopsy.

• Monobenzone (the monobenzyl ether of hydroquinone [Benoquin]) is used topically by patients with extensive vitiligo to depigment their remaining normally pigmented skin. A patient who had been applying the drug for one year had an anterior linear deposition of pigment in both corneas. Of 15 additional patients with vitiligo, 11 of whom were using monobenzone, acquired conjunctival melanosis in two patients and pingueculae in three may have been related to monobenzone use. Light and electron microscopy of one corneal epithelial scraping and 12 conjunctival biopsy specimens revealed pleomorphic, single-membrane-limited intracytoplasmic inclusions within the corneal epithelium and within the epithelium, fibrocytes, histiocytes, and vascular endothelium of the conjunctiva. The ultrastructural aspects of these inclusions suggested that they are residual bodies containing lipid and lipofuscin.

Periodic alternating gaze is a rarely reported phenomenon. We have observed two cases that are unique in their early onset at birth and infancy. Multiple congenital defects of the posterior cranial fossa were present on MRI in both cases. A prominent abnormality shared by both was absence of normal structures in the region of the inferior cerebellar vermis. Periodic alternating gaze appears to be associated with pathologic changes in the hindbrain in these and other reported cases.

An image analysis method to measure retinal nerve fiber layer (RNFL) striations from digitized fundus photographs was developed to improve detection and monitoring of progressive diffuse RNFL loss. Striations were measured by comparing the high spatial frequency variability across with the variability along the RNFL. This locally normalized measure of striations compensates for the wide density variations both within individuals and between individuals and RNFL photographs. Five repeated measurements were taken at each of three locations from each retinal image. Measurements from five patients with recorded visual field loss due to optic nerve diseases were compared with five normal subjects and five suspect eyes. Measurements clearly distinguished the three groups when taken at the temporal arcades. Measurements above and below the arcades were also consistent, but did not distinguish normals from suspects. The measure was correlated with graded estimates of RNFL integrity of two trained observers (ρ = −0.57, p <0.001 and ρ = −0.61, p < 0.001).

Ophthalmic complications from moyamoya disease, or multiple progressive intracranial arterial occlusion syndrome, have rarely been reported. This case report describes a patient with this syndrome who developed acute retinal artery occlusion.

INTRODUCTION The aortic arch syndrome represents a chronic disorder of the great arteries which branch from the aortic arch, resulting in diminished caliber and insufficient blood supply to the areas supplied by these vessels. Takayasu-Ohnishi's (pulseless) disease is an example of an aortic arch syndrome characterized by diminished or absent pulsation in the arteries of the head, neck, and upper extremities. Pulseless disease in its classic form is rare and it might be well to consider this as a separate entity or syndrome. Some of the aortic arch syndromes may not even have pulselessness, but when it does not exist in a given case, then it is problematic where carotid and vertebral-basilar insufficiency per se ends and the aortic arch syndromes begin. TERMINOLOGY Takayasu1 in 1908 reported a case which had peculiar, wreath-like anastomoses of the central artery and vein of the retina around the optic disk. Ohnishi, at

INTRODUCTION The ophthalmoscopic manifestations of increased intracranial pressure have stimulated much clinical and experimental investigation during the past century. Türk1 in 1853, was the first to describe retinal venous engorgement in brain tumor. It remained however, for Von Graefe2 to elaborate and describe completely the condition of papilledema. He deserves due credit for emphasizing that the earliest and most reliable manifestation of increased intracranial pressure is distension of the retinal veins. It seemed most logical that this ophthalmoscopic finding was a reflection of venous stasis, and in 1860 he advanced the theory that with increased intracranial pressure there was a concomitant rise in intracranial venous pressure. Increased venous pressure within the dural sinuses would lead to stasis and would result in a rise in venous pressure in the cavernous sinus and, in a retrograde fashion, in the orbital veins and central retinal vein.Sesemann,3 in 1869, demonstrated