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[Authors found the following ophthalmologic, neurologic and EEG abnormalities in an 8 and a half year old boy with hemihypertrophy of the whole right side of the body: Developmental defects of the tissues of the right eye of neroepithelial origin (sphincter, dilator pupillae lesion, peripapillary and macular coloboma, Bergmeister pupil). Segmental skin tumours common in phakomatosis. Imbecility; rare left side facio-brachial clonal epileptic seizures with loss of consciousness. On PEG, the hemihypertrophic side of the lateral ventricle was wider and protruded in the parietal direction. On awake EEG. recordings, sinusoidal delta activity with a constant amplitude of 2.5-3 c/s in the temporal, parietal and occipital areas of the right side was inhibited by eye opening. When evipan sodium was administered, diffusely periodically recurring complete depression phases "electrical silences" developed over the right hemisphere at 0.35-0.38 g evipan sodium, which began to develop in the right side only 0.20 g.times later at 0.55-0.58 g evipan sodium, i.e. the impaired right hemisphere showed an increased barbiturate sensitivity by more than 50%. The ocular developmental abnormalities suggest that hemihypertrophy is a true embryopathy that develops at a very early stage of development. In the present case, developmental damage to the central nervous system was continuous throughout intrauterine life on the hemihypertrophic side. The clinical signs and literature data also point to the role of a hemispherical lesion of the autonomic nervous system. ]
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