A clinical and neuroradiological study was carried out to clarify the relationship among cerebral dysgenesis, psychomotor retardation and neonatal epilepsy. Twenty-one cases with epilepsy developed within four weeks after birth were selected among 770 in-patients with childhood epilepsy. They consisted of one benign familial neonatal convulsion, nine of early myoclonic encephalopathy, four of Ohtahara syndrome, six of symptomatic localizationrelated epilepsy, and one of undetermined epilepsy. Seven cases with non specific types of epilepsies were investigated in detail. They developed into four of frontal lobe epilepsy, one of temporal lobe epilepsy and one severe epilepsy with multiple independent spike foci. Psychomotor retardation was observed in six of seven cases, and cerebral dysgenesis was considered to be important as an etiological factor. Proton MR spectroscopy revealed the decrease in the ratio of N-acetylaspartate/choline in the area with heterotopic gray matter and in the side of hemimegalencephaly, contrasted with a contralateral area without lesions. These findings can be inferred to reflect the decreased numbers of neuronal cell population or reduced metabolism in the lesions.

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The purpose of the present study is to clarify the changes of choroidal circulation developing in the selective injuries of the outer layer of the retina. One, 2, 3 weeks and 3 months after intravenous administration of Monoiodoacetic acid in the rabbits, choroidal vessels were observed using indocyanine green angiography (ICG). The diameter of retinal arterioles decreased significantly at 3 weeks and 3 months after the treatment and hypofluorescence in the background was observed at 3 months. Histological examination revealed significant decrease in choriocapillaris at 3 weeks, a thickening of the endothelial cytoplasma and a disappearance of fenestration in choriocapillaris after 3 months. These histological findings were considered to be a cause of hypofluorescence of background in ICG. The results suggest that the selective damage of the outer layer of the retina induce changes in the choroidal circulation.

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We observed the morphologic changes of tissue fragments of retinal pigment epithelium (RPE) of chick embryos in floating culture under the presence of bacic fibroblast growth factor (bFGF). At higher concentration (10 and 200ng/ml) of bFGF, rolled up RPE sheets grew to develop non-pigmented lumps with the structure like onions, while dissociated RPE cells did not form such structure. Histologically, the onion-like structure consisted of lens-fiber-like cells, positive for anti-crystallin antibody and anti-bFGF-receptor antibody. This lentoid structure was not formed at 1ng/ml or lower concentration of bFGF. The trans differentiation from RPE cells to lens epithelial cells was dependent upon the concentration of bFGF as well as the proper interaction among RPE cells.

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In order to know frequency of choroidal circulatory disturbance, 126 eyes with central serous chorioretinopathy followed-up between Jan. 1985 and Dec. 1990 were retrospectively investigated by fundus fluorescein angiography. Appearance of choroidal circulatory disturbance was divided into two types. The type 1 showed choroidal filling delay at late retinal venous phase. The type 2 showed mosaic pattern of choroidal background at late retinal venous phase. Among the 126 eyes, 3 eyes were type 1 (2.4%) and 57 eyes were type 2 (45.2%), totaling 60 eyes (47.6%). Sixty-three eyes with normal part of branch retinal vein occlusion matching in sex and age were selected as controls. In the controls, no eye was type 1 (0%) and 8 eyes were type 2 (12.7%), totaling 8 eyes (12.7%). Distinctly, proportion of the type 2 was higher in central serous chorioretinopathy than in branch retinal vein occlusion. In conclusion, the pathogenesis of central serous chorioretinopathy is related to choroidal circulatory disturbance and especially mosaic pattern (type 2) was found to be a new type of circulatory disturbance.

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