Follow-up report on the 'Poland anomaly/primary microcephaly association'.
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We report a Brazilian boy, born to consanguineous parents. On the left arm there was a proximal 'ring shaped' constriction, regional aplasia cutis, and a short hand with markedly hypoplastic fingers and nails 2-3. He also had a bilateral cleft lip/palate, preaxial polydactyly involving the distal phalanx of the left index finger, and a supernumerary nipple on the right. The differential diagnosis is discussed.
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Of 41 term infants surviving to seven years of age with head circumferences (HC) - 3 SD, 21 were mentally retarded (IQ less than or equal to 70) at seven years. HC - 2 SD was associated with mental retardation in 11 per cent of children. Socio-economic status did not affect mental outcome of children with HC - 3 SD, but higher socio-economic level was associated with better outcome for those with HC - 2 SD or who were normocephalic. Mental retardation in children with HC - 3 SD was more common in children with additional pathology. Of 25 children with no other pathology, one-quarter were mentally retarded at seven years; this was more common in children of normal birthweight. Disproportionate head-to-height ratio did not significantly affect outcome, although short stature was associated with less favourable outcome for those with HC - 2 SD or who were normocephalic.
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Neonatal rats were exposed to several different doses of ethanol during the first part of the brain growth spurt (postnatal days 5, 6, 7 and 8) and examined for decreased brain weights at 18 days of age. The occurrence of reduced brain weight was found to require a dose of at least 6.0 g ethanol/kg body weight/day. Above this dose, the extent of brain weight reduction was related to increased dose, provided death from overdose did not occur. Measured blood ethanol levels suggested that blood levels below 100 mg/100 ml failed to result in significant brain weight reductions. The possibility that blood ethanol levels below this point may impair brain growth in more subtle ways remains to be determined.
Fischer rats injected with methylazoxymethanol late in pregnancy produce young with considerably reduced cerebral hemispheres. They appear normal otherwise. As adults these animals make many more errors in the Hebb-Williams maze than do control animals.