RESUMO
PURPOSE: To present a case of suspected Leber's hereditary optic neuropathy (LHON) with MRI and OCT findings compatible with pseudotumor cerebri responsive to acetazolamide therapy. CASE REPORT: A five-year-old boy referred to our clinic with optic atrophy and low vision was originally diagnosed with LHON. Laboratory tests were negative for LHON, while OCT and MRI were consistent with pseudotumor cerebri. Acetazolamide therapy resulted in dramatic improvement of visual acuity. CONCLUSION: Some cases of previously labeled hereditary optic neuropathies with clinical signs of idiopathic intracranial hypertension could respond to intracranial pressure lowering treatment.
RESUMO
PURPOSE: To determine the incidence of clinical keratoconus (KCN) in an Iranian population. METHODS: This prospective surveillance study was conducted in Yazd province in central Iran (population 990,818). During 1 year (July 2008 to June 2009), all new patients with KCN were registered and referred. Diagnosis was based on the topographic pattern, and indices were measured and eye examination was done by anterior segment subspecialists. RESULTS: Of the 564 eligible patients referred, 28 did not attend their topography session (95% response rate). Topographic figures showed suspect or definite patterns of KCN in 47.8% of participants (256/536 subjects). Based on topographic images and clinical examination, KCN was confirmed in 221 patients, and in addition, 26 patients were categorized as having suspected KCN and 9 had non-KCN corneal diseases; therefore, the annual incidence rate of KCN was estimated as 22.3 (95% confidence interval, 19.5-25.4) and 24.9 (95% confidence interval, 21.9-28.2) per 100,000 population, excluding and including suspected KCN cases, respectively. CONCLUSION: The incidence of KCN in our context is apparent and comparable with rates in other studies on Asian ethnic populations. This is much higher than the incidence in European Caucasians and warrants further genetic and environmental studies.