François-Xavier Borruat

Multiple Evanescent White Dot Syndrome After Hepatitis B Vaccine

PURPOSEnHepatitis B vaccine has become an effective means of preventing complications of hepatitis B. However, it occasionally induces serious side effects. We report a case of multiple evanescent white dot syndrome (MEWDS) that occurred following hepatitis B vaccination.nnnMETHODSnA 23-year-old woman with a one-week history of progressive loss of vision in the left eye and bilateral photopsia was referred for examination. Her symptoms appeared 24 hours after a booster intramuscular injection of hepatitis B vaccine.nnnRESULTSnClinical examination, fluorescein angiography, and the course of events were typical of MEWDS.nnnCONCLUSIONSnThis case demonstrates the occasional occurrence of MEWDS after hepatitis B vaccine and suggests that hepatitis B virus immunization may be a risk factor for this retinal condition.

An unusual presentation of herpes zoster ophthalmicus: orbital myositis preceding vesicular eruption.

PURPOSEnTo present a case of orbital myositis associated with herpes zoster ophthalmicus.nnnDESIGNnObservational case report.nnnMETHODSnA 47-year-old woman with acute retrobulbar eye pain and diplopia preceding the vesicular rash of herpes zoster ophthalmicus was evaluated and treated.nnnRESULTSnMagnetic resonance imaging showed enlargement and enhancement of extraocular muscles consistent with an inflammatory myopathy. Following acyclovir and prednisone treatment, all symptoms resolved, and neuralgia did not develop.nnnCONCLUSIONSnHerpes zoster may cause symptoms and signs of orbital myositis before eruption of cutaneous skin lesions and thus should be considered in the differential diagnosis of an acute orbital myositis.

Patterns of rod and cone dysfunction in Bardet-Biedl syndrome.

We studied visual function in 16 patients with the Bardet-Biedl syndrome. Visual acuity, kinetic perimetry, and electroretinography results indicated a severe loss of central and peripheral vision and rod and cone function by the second or third decade of life. Light- and dark-adapted static perimetry in patients 10 to 15 years of age with early involvement showed a parallel and marked loss of rod and cone sensitivity across the visual field. Patients with more advanced disease and no measurable peripheral visual field showed different patterns of central visual dysfunction: an island of only cone function centered in a bulls-eye lesion; patches of rod function surrounding geographic atrophy; or a central island of excellent rod sensitivity but severely impaired cones. In the two least-affected patients, a 13-year-old boy and the asymptomatic 45-year-old sibling of a patient, there were more rod than cone abnormalities as determined by electroretinography and static perimetry.

Radiation optic neuropathy: report of cases, role of hyperbaric oxygen therapy, and literature review

Background: Occlusive vasculitis affecting the optic nerve or chiasm is an infrequent delayed complication of radiation treatment. Hyperbaric oxygen (HBO) has been used to treat such a complication, but its efficacy is controversial. Objectives: To report the outcome of radiation-induced optic neuropathy (RON) treated with HBO, compared against natural course of untreated RON and against other forms of therapy. Methods: We report five patients with RON, four treated with HBO. Cases accrued in the world literature are analyzed for outcome and possible confounding factors. Results: From our treated series, two patients recovered significant visual function, one patients vision remained unchanged, one patient continued to deteriorate. Among 120 similar cases, no instances of spontaneous improvement of vision are documented, nor is recovery with other forms of therapy. Conclusion: Our data suggest that HBO at a minimum pressure of 2.4 atmospheres may be effective in reversing the visual loss induced by radia...

Rhodopsin levels and retinal function in cats during recovery from vitamin A deficiency

Extended vitamin A deficiency in the cat led to an abnormal appearance in the tapetal fundus with the formation of a dark brown streak centered on the area centralis. At this time rod sensitivity, as measured by the b-wave of the electroretinogram, was reduced by more than two log units; the level of rod visual pigment was reduced by about 90% throughout the paracentral retinal region and was essentially absent from the area centralis. Following oral supplementation with vitamin A there was a rapid partial recovery of both rhodopsin levels and rod sensitivity. Further recovery continued over more than 18 days to levels that were not substantially below normal. This recovery was absent from the area centralis, in which measured visual pigment levels remained very low. In supplemented cats, the brown color in the fundus faded but there remained a small hyper-reflective zone at the area centralis. Morphological examination of the central retina in a supplemented cat showed an outer nuclear layer reduced to one or two rows in the small zone with low rhodopsin levels. Cone but not rod photoreceptors were present in this zone and they appeared to lack outer segments. During recovery, the increase in rod sensitivity was approximately linearly related to the recovery of rhodopsin levels. Thus, in these conditions reduction in sensitivity resulting from previous vitamin A deficiency was limited by the ability of the photoreceptors to absorb incident quanta. The time course of the recovery of rhodopsin and sensitivity suggests that at least two processes were involved. The faster of these may be the regeneration of rhodopsin from existing opsin molecules in the outer segments, while the slower may depend on the renewal of the outer segments themselves.

Rhodopsin topography and rod-mediated function in cats with the retinal degeneration of taurine deficiency.

Cats on a taurine-deficient diet were studied with imaging fundus reflectometry and full-field electroretinography. The pattern of rhodopsin loss and the natural history of the disease were determined from maps of the rhodopsin distribution in the central and nasal retina of cats with different degrees of severity of the retinopathy. Rhodopsin loss is first detectable in a focal region of the central retina. Subsequently, there are decreases in rhodopsin in the paracentral and nasal midperipheral retina. The horizontal streak of high rhodopsin levels is preferentially reduced in this retinopathy. The b-wave amplitude of the rod-dominated ERG is markedly reduced in cats with only mildly decreased levels of rhodopsin in the peripheral retina. In an affected cat with moderate rhodopsin loss in the central retina but minimal loss nasally, a light-microscopic study of the retina showed that there was disorganization and shortening of rod outer segments and loss of rod photoreceptor cells in the areas of reduced rhodopsin levels.

Rod or cone dysfunction: uniocular presentations

Two patients presented with unusual uniocular electroretinographic (ERG) phenomena. One patient showed the ERG characteristics of stationary night blindness in one eye only and the other patient presented with ERG evidence of uniocular cone dysfunction. In both patients, however, further electrophysiological and psychophysical studies and serial testing provided evidence for dysfunction of both receptor systems and relatively subtle involvement of the fellow eyes.

Oculopalatal Tremor: Variations on a Theme by Guillain and Mollaret

Background: Oculopalatal tremor (OPT) is a delayed complication of a brainstem lesion, characterized by involuntary contractions of the soft palate associated with a synchronized ocular pendular nystagmus. MRI reveals inferior olivary nucleus hypersignal/hypertrophy (IONH). Our objective was to refine the clinical profile of patients with OPT and to report a few oddities in both presentation and evolution. Methods: We performed a retrospective study of patients diagnosed with OPT and a literature search. Results: From our database, we retrieved 5 men and 3 women with a diagnosis of OPT. Eighty-two patients with OPT were retrieved from the literature and were compiled with our series. The average age was 54 years and there was a male predominance. Brainstem vascular lesion was the most common etiology (80%). Prominent vertical pendular nystagmus was found in 90%. Dissociated nystagmus was mostly associated to unilateral contralateral IONH on MRI, while bilateral symmetrical nystagmus was due to a bilateral IONH in the majority of cases. Three oddities were found amongst our 8 patients: prominent nystagmus ipsilateral to IONH; disappearance of IONH on MRI despite persisting nystagmus, and asymptomatic OPT. Conclusion: The clinical profile of OPT is rather stereotyped. Rarely do patients deviate from the classical description of OPT.

Unusual Paraneoplastic Cause of Vision Loss: Combined Paraneoplastic Cone Dystrophy and Optic Neuropathy

Comment. Toxoplasmosis is believed to be the most common cause of posterior uveitis. Scleritis in association with toxoplasmic retinochoroiditis is an uncommon entity. Accordingly, in a review of 243 patients with scleritis, no patient was reported to have toxoplasmic infection as a cause; furthermore, 37% had a systemic rheumatologic disorder, only 7% had an infection, and 44% had an associated medical disorder. Herpes zoster virus was the most commonly reported infectious cause and rheumatoid arthritis was the most common rheumatic disease. In cases of toxoplasmic scleritis, the inflammatory response is believed to extend from the active retinochoroiditis to involve the overlying sclera. Accordingly, pathologic specimens from eyes that were enucleated secondary to severe toxoplasmic scleritis displayed granulomatous inflammation of the retina, uvea, and episclera with associated retinal thickening. In many cases, the entire sclera extending outward from the retinitis was inflamed; however, in some cases, a region of uninflamed sclera separated the active scleritis from the underlying retinitis. Isolated toxoplasmic retinochoroiditis can rapidly spread and lead to severe permanent vision loss when treated with steroids alone. In our patient with scleritis, a dilated fundus examination revealed an area of typical toxoplasmic retinochoroiditis, allowing for prompt diagnosis and treatment with appropriate antibiotic therapy. The patient improved and maintained excellent visual acuity. This patient’s course underscores the importance of a complete examination in cases of scleritis, including a dilated fundus examination, to rule out an infectious retinochoroiditis in association with the scleritis.

Visualizing the skip lesions of giant cell arteritis with CT arteriography.

A 77-year-old man developed headaches, jaw claudication and diplopia. Giant cell arteritis was suspected [1] . CT angiography of the extracranial arteries showed focal areas of luminal narrowing and mural thickening consistent with inflammatory ‘skip lesions’ along the right superficial temporal artery ( fig. 1 ). The diagnosis was confirmed with biopsy. Received: December 8, 2008 Accepted after revision: December 15, 2008 Published online: March 31, 2009