Unusual presentation of a multiple sclerosis case involving central retinal artery occlusion

The term intermediate uveitis [IU] refers to a subgroup of uveitis in which the vitreous is the site of greatest inflammation. Patients with multiple sclerosis [MS] have a greater frequency of IU compared with the general population. The IU associated with MS is characterized by the presence of pars planitis [occasionally accompanied by anterior uveitis] and the presence of peripheral retinal vasculitis in the form of periphlebitis [venous sheathing] in 6-26% of patients. We present a patient with an unusual initial presentation of MS involving central retinal artery occlusion [CRAO] in the right eye [RE]. Although retinal vascular changes are asymptomatic in the majority of MS patients, the spectrum of impairment ranges from simple peripheral retina periphlebitis to the presence of peripheral occlusive retinal vasculitis in 6.5% of patients. This atypical case may represent an extreme of the spectrum of retinal vasculitis associated with demyelinating disease

Clinical variability in hereditary optic neuropathies: two novel mutations in two patients with dominant optic atrophy and Wolfram syndrome

Dominant optic atrophy [DOA] and Wolfram syndrome share a great deal of clinical variability, including an association with hearing loss and the presence of optic atrophy at similar ages. The objective of this paper was to discuss the phenotypic variability of these syndromes with respect to the presentation of two clinical cases. We present two patients, each with either DOA or Wolfram syndrome, and contribute to the research literature through our findings of two novel mutations. The overlapping of several clinical characteristics in hereditary optic neuropathies can complicate the differential diagnosis. Future studies are needed to better determine the genotype-phenotype correlation for these diseases

Macular star formation in diabetic patients with non-arteritic anterior ischemic optic neuropathy [NA-AION]

NA-AION is a condition that exhibits a number of unique characteristics in diabetics compared with the rest of the population. In some diabetic patients with NA-AION, lipid deposits can be observed around the macula forming an incomplete macular star. Methods: We describe 12 case studies of patients with NA-AION observing the development of lipid deposits around the macula forming an incomplete macular star. All our patients developed some level of lipid deposits around the macula in the form of a macular hemistar in the course of their illness. Some authors have suggested that the macular star is formed by transudation from capillaries deep in the optic disk through the intermediary tissue of Kuhnt, which is located between the retina and the anterior portion of the lamina retinalis. However, the development of the macular star is currently understood not as a simple transudation but as a multifactorial process involving the presence of vascular damage around the optic disk, which is considered one of the most important factors leading to its occurrence. Although some studies mention the presence of a macular star in patients with NA-AION, we believe that this phenomenon may be significantly more common than the current literature suggests

Band atrophy of the optic nerve: a report on different anatomical locations in three patients

Lesions of the optic tract are accompanied by various signs that help to distinguish them from hemianopias located posterior to the lateral geniculate body. Band optic nerve atrophy is one of these signs and typically occurs contralateral to the optic tract lesion. We report on three patients with band atrophy in the fundus of the eye. These three patients present examples of how three lesions with different anatomic locations can cause band atrophy of the optic disk in similar ways. In these cases, the presence of relative afferent pupillary defect [RAPD] and band atrophy becomes important in identifying the injury to the optic tract, because when the hemianopia is complete visual fields do not allow distinguishing optic tract lesions from occipital lesions. The RAPD occurs in the eye in which the visual field defect is greater. In this paper we review the different theories about the explanation for RAPD in patients with optic tract lesions. It does not seem as simple as the anatomical differences between the number of fibers that decussate in particular cases, rather, it is associated with the difference between the sensitivity levels of the two functioning hemiretinas

Sequential, non-arteritic anterior ischemic optic neuropathy in patients taking sildenafil: a report of ten cases

To present a summary of 10 cases of non-arteritic anterior ischemic optic neuropathy [NAION] in patients who received phosphodiesterase type 5 [PDE-5] inhibitors. A case series of 10 patients who, after regular intake of Sildenafil, presented with a first episode of NAION in one eye. NAION was diagnosed based on the following criteria: acute, painless, unilateral loss of vision, fundus features consistent with NAION and exclusion of other possible causes. Despite the initial adverse event [first episode of NAION], all of these patients continued to use the medication and developed a second episode of NAION in the contralateral eye. Only one of the 10 patients presented with bilateral simultaneous NAION. This largest case series published to date, reinforces the general consensus that PDE-5 inhibitors are contraindicated in patients with a history of unilateral NAION.

Papilledema and optic disc pit The association of two pathologies in one patient

Histologically, optic disc pit [ODP] is described as a small congenital defect in the cribriform plate where a herniation of the dys-plastic retina extends to the subarachnoid space in the optic nerve, which is surrounded by a layer of collagen-rich tissue. Approximately 25-75% of cases are complicated by optic disc pit maculopathy [ODPM]. The pathogenesis of maculopathies associated with ODP has not been fully elucidated. We present an unusual case of a patient with a history of papilledema secondary to a fronto-temporal meningioma. The patient underwent a successful surgical intervention with resolution of the papilledema 15 years before the detection of an ODP that was associated with retinal detachment in the macular region. Several studies have reported on the importance of intracranial pressure in the development of maculopathies associated with ODP. During the period of intracranial hypertension and papilledema in our patient, the hernia sac that was associated with ODP may have been small, which would have decreased the chances of developing a maculopathy that was associated with OPD