Background: Malignant melanoma of the ciliary body and choroid of the eye is a tumor that disseminates frequently, and 50% of the diagnosed patients die within 10 years. We investigated the hypothesis that, by histopathologic analysis of the arrangement of microvessels (i.e., small blood vessels) in loops and networks, we might be able to differentiate better those patients with a favorable prognosis from those with a poor prognosis. Methods: We conducted a populationbased, retrospective cohort study of melanoma-specific and all-cause mortality for 167 consecutive patients who had an eye surgically removed because of malignant choroidal or ciliary body melanoma during the period from 1972 through 1981. Microvascular loops and networks were evaluated independently by two pathologists who were unaware of patient outcome.

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SUMMARY In Finland during the 20-year period 1958-78 35 patients with amblyopia lost the vision of the healthy eye. In more than 50% the cause was traumatic. The incidence of the loss of the healthy eye was 1'75+0-30 per thousand. During the same period in Finland the overall blindness rate of children was 0 11 per thousand and of adults aged 15-64 years 0-66 per thousand. For the amblyopic patient the risk of becoming blind is markedly higher than for the general population.It is generally thought that amblyopia affects about 2% of the untreated population. In 2 studies of normal Finnish schoolchildren the frequency of amblyopia ranged between 1-8 and 12 %* 2 In many countries much effort and time have been given to the detection and treatment of amblyopia. The result has been discussions on the significance of the treatment of amblyopia in childhood for the patient's laterlife in general.

A combination of congenital central nervous, ocular and muscular abnormalities is characteristic of muscle-eye-brain disease (MEB), of Fukuyama congenital muscular dystrophy (FCMD), and of Walker-Warburg syndrome (WWS). The nosological relationship of these inherited malformative disorders is still unestablished, although the genetic locus for FCMD has been excluded in MEB. We present the first postmortem neuropathological study of MEB based on 2 male patients. Apart from sharply limited occipital agyric areas, their brains showed coarse gyri with an abnormally nodular surface ("cobblestone cortex"). Both the cerebral and cerebellar cortices showed a total disorganization without horizontal lamination. The haphazardly oriented cortical neurons formed irregular clusters or islands, separated by gliovascular strands extending from the pia. The ocular abnormalities included a pronounced glial preretinal membrane. Although MEB shares the cobblestone cortex-type malformation with FCMD and WWS, the cerebral and ocular manifestations are less severe than in WWS. Furthermore, a consistently weak staining for laminin alpha2 chain (merosin) was found in muscle biopsy specimens from 4 MEB patients, while normal immunoreactivity was observed for the laminin beta2 chain, reported to be severely deficient in WWS. These findings support nosological independence of MEB.

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