case series study of leber's congenital amaurosis: Clinical description and ERG findings

To evaluate the incidence of Leber's Congenital Amaurosis [LCA] in low vision children referred to electrophysiology ward of Farabi Eye Hospital, and review the clinical features of disease and Electroretiongraphy [ERG] test values to confirm the diagnosis and severity of the disease in Iran. Prospective observational case series. Two-hundred and fifteen cases of low vision infants and young children were referred to electrophysiology ward of Farabi Eye Hospital during 18 months. Clinical LCA diagnosis was made and ERG tests were done and LCA diagnosis was confirmed. The symptoms, signs and the results of eye examination and ERG findings were recorded. The mean age of the patients was 27.43 [range, 1-120 months]. Among low vision patients fourteen percent of patients had LCA. Fifty-four percent of the patients were female. Nystagmus and low vision were the two most common clinical manifestations of these patients. Hyperopia was the main refractive error [54.80%] and mild abnormalities in fundus examinations were found in 67.70% of cases. In nearly 90% of cases consanguinity was found. ERG was flat or unrecordable in more than 90% of cases, but in less than 10% of cases with recordable curves, severe decrease in amplitude of waves was encountered. ERG confirmed LCA diagnosis in 31 out of 37 patients [positive predictive value of 83.7%]. The incidence of LCA in low vision children is similar to other studies. ERG helped in confirmation of presence or absence of overall retinal dysfunction in the majority 31/37 [83.7%] of LCA patients. It can differentiate these cases from other cases with poor vision in infantile age but genetic testing is recommended

Efficacy of memantine in acute non-arteritic ischemic optic neuropathy

Evaluation of efficacy of Memantine [N-Methyl-D-Aspartate Receptor Antagonist] on visual function of patients with acute non-arteritic ischemic optic neuropathy [NAION]. The study was conducted as interventional case series from November 2005 through November 2006 in Farabi Eye Hospital. Twenty-two patients with acute NAION of less than 8 weeks duration entered the study. Memantine was prescribed with a dose of 5 mg per day for the first week and 10 mg per day for the following two weeks. Baseline best corrected visual acuity [BCVA]; visual evoked potential [VEP] and visual field was done for all patients. BCVA recording repeated 3 weeks, 3 and 6 months later. VEP and perimetry repeated 3 months after treatment. After 3 weeks, 3 and 6 months, BCVA improved -0.32 +/- 0.40 LogMAR, -0.51 +/- 0.49 and -0.51 +/- 0.49, respectively [P=0.005, P=0.001 and P=0.001, respectively]. VEP recordings after 3 months, demonstrated -8.61 +/- 14.51 db mean decrease in implicit time [P=0.019]. Amplitude of voltage did not show significant difference with baseline [P=0.10]. Perimetry results after 3 months showed that mean deviation [MD] improved 2.77 +/- 3.94 db [P=0.016]. Memantine resulted in significant improvement of BCVA 3 weeks, 3 and 6 months after treatment of acute NAION. Memantine also resulted in significant decrease of implicit time and significant improvement of mean deviation in VEP and perimetry after 3 months