Idiopathic intracranial hypertension (IIH) is a syndrome characterized by elevated intracranial pressure, without evidence of intracranial mass lesion or venous thrombosis on brain imaging. The syndrome mainly occurs in young, fertile and overweight women, but may emerge in any age group, even in young children or individuals over 45 years of age. The incidence of the disease in Israel is similar to that of other developed countries, approximately 1:100,000. This syndrome's most prominent symptom is headaches, which are reported by approximately 90% of the patients. Other symptoms are transient visual obscurations, tinnitus, or diplopia. Some patients may be asymptomatic, and only diagnosed after having undergone routine fundus examination; however, this is more common in children. Treatment is based on weight loss. The medical therapy prescribed is carbonic anhydrase inhibitors, especially: acetazolamide, and in severe cases, surgery may be indicated. The syndrome is paroxysmal by nature, and an attack may develop even after periods of remission; therefore, a long continuous follow-up is needed to monitor disease progression, and to intervene in time. The etiology of the disease is unknown. Nevertheless, new data has emerged in past years, and the goal of this review is to describe the syndrome and present new recently published information.

T-cell acute lymphoblastic leukemia is a hematologic malignancy with propensity to involve extramedullary organs including the eyes. Optic nerve infiltration is relatively rare. This is the case study of a 25-year-old- man who was in full remission following treatment for T-cell acute lymphoblastic leukemia and presented with bilateral leukemic optic nerve infiltration as the first manifestation of extramedullary relapse. The patient was treated with urgent radiotherapy and systemic dexamethasone. Over the following period, gradual resolution of optic disc swelling was noted in both eyes with marked improvement in vision in the right eye. Unfortunately, a few weeks later, a full blown hematological relapse was diagnosed. Salvage chemotherapy was instituted but was complicated by tumor lysis syndrome and septicemia that proved to be fatal. Ophthalmic assessment is essential in patients with hematological malignancies in order to diagnose ocular involvement as a result of malignant infiltration, hematological disturbances or as a complication of systemic therapy.

Multiple myeloma is a disease caused by neoplastic plasma cells. Initial symptoms in most cases described in the literature include chest and lumbar pain, paresthesia, paraplegia, general weakness and renal failure. We report a case of isolated sixth nerve palsy causing diplopia as the presenting sign of multiple myeloma. Awareness of such a rare clinical presentation - especially when the existence of multiple myeloma is ignored after visualization of a cranial mass with magnetic resonance imaging (MRI) and computerized tomography (CT)--can prevent unnecessary surgical intervention and delay the appropriate treatment.

Carotid-cavernous fistulae may present clinically in different ways, depending on the diameter and flow of the artery involved. In a direct fistula between the internal carotid artery and the cavernous sinus (high - flow fistula) the clinical presentation is usualLy dramatic and the signs and symptoms are severe. Indirect fistulae, in contrast, result from the connection of branches of the internal carotid, external carotid, or both to the cavernous sinus (low - flow fistula) and often present in a moderate and/or indolent manner. Patients with Ehlers-Danlos type IV suffer from vascular wall fragility resulting in a high frequency of aneurysms, dissections and fistulae. We describe a patient with Ehlers-Danlos vascular type IV who presented with mild signs and symptoms, suggesting the existence of a low-flow fistula. After two weeks, the patient's condition rapidly deteriorated. Endovascular catheterization demonstrated direct carotid cavernous fistula which was successfully treated. This case demonstrates the importance of early consultation with an endo-vascular surgeon in every patient with Ehlers-Danlos suspected of harboring a carotid cavernous fistula.

Inflammatory demyelinative diseases of the central nervous system are mostly idiopathic and represent the major cause of neurological disability in young adults. These diseases differ in terms of clinical symptoms, severity, pathological characteristics and epidemiology. However, there are also significant similarities between these diseases, which sometimes bring to a misleading diagnosis. Neuromyelitis optica (NMO) is a demyelinative disease in which the optic nerve and the spinal cord are predominantly affected. The detection of specific antibodies to aquaporin-4 (NMO-IgG) led to a modification of the diagnostic criteria for NMO.We performed a retrospective study on NMO-IgG positive patients referred to the Department of Neurology MS Center (2006-2011) with suspected NMO. Based on the presenting symptomatology of the patients, we identified the cases with optic neuritis and various parameters that may differentiate between NMO and MS. NMO-IgG were evaluated by ELISA.A total of 50% of the 107 patients with NMO-IgG fulfilled the revised criteria of NMO; 38 patients had a single attack of optic neuritis or long lesion in the spinal cord and 15 patients presented with an opticospinal type of MS. The visual acuity following a single attack of optic neuritis remained significantly lower in NMO patients as compared to MS patients. Most of the NMO patients with NMO-IgG had additional attacks of optic neuritis within a short time from the initial event.The finding of NMO-IgG in patients with optic neuritis foreshadows a bad prognosis and relapses. These patients are at high risk of experiencing a second event in the central nervous system and fulfilling the clinical criteria for NMO. Due to the difference in the severity of inflammation of the optic nerve between NMO and MS, it is highly recommended to seek a laboratory check-up for NMO-IgG in serum, immediately after the first event, in order to determine the necessity and the kind of treatment for the patient.

BotuLinum toxin A (Botox, Allegan) is a potent neurotoxin that blocks the release of acetylcholine at the neuromuscular junction of cholinergic nerves. Botulinum toxin was introduced to clinical medicine in 1980. Since then it has become a major therapeutic drug in many medical sub-specialties and its use for facial rejuvenation has become increasingly popular. Diplopia after botulinum toxin injection for facial rejuvenation is a rare and transient complication which is related to chemodenervation of adjacent muscle groups. We would like to report 3 cases of double vision related to extra-ocular muscle paresis after an injection of botulinum toxin for facial rejuvenation and blepharospasm. In all 3 cases recovery occurred, without any treatment, over 3 to 4 months (apparently from regeneration of inactivated proteins necessary for degranulation of acetylcholine vesicles). The clinicians engaged in botulinum toxin injections for facial rejuvenation or blepharospasm, should be aware of the possible complications, and inform the patients about the risk of developing double vision. The clinicians should take into account and ask about Botox when treating patients complaining of diplopia.

To report cases of cavernous hemangioma (CH) of the anterior visual pathways (AVP) and analyze the cLinical and radiographic presentation, as well as the surgical approach of this very rare entity.The authors report two cases of patients presenting with headache and visual loss due to anterior visual pathways hemangioma, and conducted a PubMed search for previously reported cases. Each case was analyzed for clinical presentation, imaging characteristics, location of the lesions, treatment methods and visual outcomes.Loss of vision was present in all reported cases, followed by headache and periorbital pain in 60% of the patients. The onset of symptoms was apoplectic in 66% of patients. Sixteen patients suffered from previous episodes of vision loss. MRI was the most sensitive and specific imaging modality, typically demonstrating heterogeneous lesion with mixed signals suggestive of blood of different ages. The gradient echo sequence is the most sensitive to hemorrhagic components. Most patients were surgically treated (93.2%). In 82% of cases visual stabilization or improvement was achieved. Two patients were treated conservatively resulting in complete blindness in one and spontaneous recovery in the other. The two patients reported by the authors were followed-up with spontaneous visual recovery.Vascular lesions of the AVP are very rare, but should always be considered in the differential diagnosis of acute painful visual loss. Resection of these lesions can be associated with favorable visual outcomes; therefore, rapid diagnosis is warranted to prevent permanent damage to visual pathways.

Carotid-cavernous fistulas (CCFs) are abnormal arteriovenous communications in the cavernous sinus. In many cases of CCF's the primary signs are ocular manifestations, which include: pulsatile proptosis, orbital bruit, chemosis and conjunctival injection, elevated intraocular pressure, venous stasis retinopathy, and cranial nerve pareses. Patients in whom the fistula causes arterial drainage into the cerebral veins and sinuses are at risk for intracranial hemorrhage. The most common treatment for CCF's is endovascular occlusion of the lesion. The goal of this procedure is to occlude the fistula but preserve the patency of the internal carotid artery. The CCF itself, as well as its treatment, can be sight- and even life-threatening. We describe 3 case reports of patients with CCF, in order to demonstrate the cooperation between the neuro-opthalmologist and the invasive neuro-radiologist, in the follow-up of the patient and in the treatment timing decision.

Erectile dysfunction medications such as sildenafil citrate (Viagra) or tadalafil (Cialis) are commonly prescribed worldwide. They are selective phosphodiesterase-5 inhibitor and partial phosphodiesterase-6 inhibitors causing smooth muscle relaxation in the corpus cavernosum, allowing penile vasodilatation and erection in response to sexual stimuli. Over the years, there have been an increasing number of case reports concerning patients who developed ischemic optic neuropathy soon after the ingestion of these drugs. Although a cause and effect relationship between usage of the drugs and the development of ischemic optic neuropathy is difficult to prove, it is common nowadays to advise patients, especially those suffering from diabetes, hypertension, and ischemic heart disease, regarding the potential risk of visual loss due to ischemic optic neuropathy and treatment with erectile dysfunction drugs. Patients who were diagnosed with ischemic optic neuropathy soon after the ingestion of these erectile dysfunction drugs should be warned about a similar event in their fellow eye and should be advised regarding drug discontinuation.

AIM:

To report a rare case of swollen optic disc with hard exudates at the center of the retina (macula) with an unknown etiology (idiopathic).

CASE REPORT:

A 49 years old male complained of blurred vision in his left eye. He reported that ten years earlier a similar episode occurred in the right eye. Upon resolution of the macular edema we recognized hard exudates with a macular star pattern. After six months his central vision returned to normal with mild to moderate defects in his lower visual field. IN

SUMMARY:

The literature describes various etiologies that have been reported due to the rare presentation of the macular star In our case we did not find any clear etiology, therefore, the diagnosis of Leber's idiopathic stellate neuroretinitis can be presumed.