The nature and frequency of neovascular age-related macular degeneration.

PURPOSE: This study was designed to evaluate the frequency and nature of neovascularization in age-related macular degeneration (ARMD) utilizing the combination of digital imaging techniques, fluorescein angiography (FA), indocyanine green (ICG) angiography, and optical coherence tomography (OCT). METHODS: A complete clinical examination was performed on 100 eyes of 93 consecutive newly diagnosed patients with neovascular ARMD. Digital fluorescein angiography, ICG angiography, and OCT were also used in evaluating those patients. Comparison of the imaging techniques to determine their value in studying the nature of the lesions. RESULTS: On the basis of existing fluorescein standards, 15 eyes were diagnosed with classic choroidal neovascularization (CNV), 15 with minimally classic CNV, and 70 with occult CNV. ICG angiography was superior for detecting the active vascular component in polypoidal CNV (16 eyes) and retinal angiomatous proliferation (14 eyes). OCT was more sensitive than FA for determining the presence of cystoid macular edema evident in the vast majority of eyes with retinal angiomatous proliferation (RAP). CONCLUSIONS: These results suggest that FA, ICG angiography, and OCT, when used in combination, will assist clinicians in best determining the precise nature of the neovascular process in ARMD.

Late effect of external eye irradiation on choroidal circulation.

PURPOSE: To report two cases of atypical late onset radiation chorioretinopathy occurring 15 and 25 years post exposure and the indocyanine green (ICG) angiographic findings in these patients. METHODS: Clinical examination and imaging including fluorescein and ICG angiography were performed. RESULT: Fundus examination of the first patient revealed microangiopathy with intraretinal hemorrhages, lipid exudation, telangiectatic and aneurysmal capillary changes. Indocyanine green angiography showed an apparent chorioretinal anastomosis and delayed perfusion of the choriocapillaris. Fundus examination of the second patient revealed a pigment epithelial detachment and retinal pigment epithelial changes. Indocyanine green angiography showed atypical, tortuous, dilated, choroidal vessels as well as areas of hypoperfusion. Both patients had multiple dot-like hyperfluorescent spots in the midphase of the ICG angiogram. CONCLUSIONS: External radiation exposure may lead to both retinal and choroidal alterations which may be independent events and which may manifest after a long period of quiescence. Furthermore, ICG angiography appears to be a useful diagnostic tool to study the alterations of the choroid following external eye irradiation.

Clinical and genetic analysis of a large pedigree with juvenile myoclonic epilepsy.

Juvenile myoclonic epilepsy is a common type of idiopathic generalized epilepsy characterized by myoclonic, generalized tonic-clonic, and in 30% of patients, absence seizures. We studied a three-generation pedigree of 33 members, 10 of whom were clinically affected with juvenile myoclonic epilepsy or presented with subclinical electroencephalographic (EEG) 3.5- to 6.0-Hz diffuse polyspike-wave or spike-wave complexes. Juvenile myoclonic epilepsy and the EEG trait segregated as an autosomal dominant trait with 70% penetrance. Linkage analysis using this model showed significant linkage to four microsatellite markers centromeric to human leukocyte antigen (HLA) in chromosome 6p. Maximum lod scores of 3.43 at theta(m=f)=0.00 for D6S272, D6S466, D6S257, and D6S402 were obtained. Recombinant events in 2 affected members defined the gene region to a 43-cM interval flanked by D6S258 (HLA region) and D6S313 (centromere). Our results in this large family provide evidence that a gene responsible for juvenile myoclonic epilepsy and the subclinical, 3.5- to 6.0-Hz, polyspike-wave or spike-wave EEG pattern is located in chromosome 6p.