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Biomedical subjects

C Cunniff

Publications and source records attributed to C Cunniff.

40 records · Page 3Linked to original sources

Autosomal dominant benign neonatal seizures.

Eight individuals in two generations of a family experienced seizures neonatally or in early infancy. Evaluation in two of these infants documented an EEG pattern suggestive of seizure activity without discernible pathogenesis of their seizures. Subsequently, affected individuals had a normal neurodevelopmental outcome, although one person had later epilepsy. The benign course of this disorder and its association with adult epilepsy agree with previously reported cases from the literature.

Electroencephalography↗

Laterality defects in conjoined twins: implications for normal asymmetry in human embryogenesis.

We evaluated six pairs of conjoined twins: four pairs were dicephalus, and two were of the ischiopagus type. In three of the four dicephalus pairs, the right twin had an abnormality of laterality that included a right aortic arch, reversed great vessel orientation, bilateral right-sided isomerism of the lungs, asplenia, and situs inversus of the viscera. The left twin had normal great vessel orientation and situs solitus in each case. The finding that was unique in these three dicephalus twin pairs was their fused hearts, which were similar in orientation and configuration. The fourth dicephalus twin pair had one normally rotated heart, which was located in the midline and had normally placed chambers and great vessels. Each twin of this pair had normal visceral situs. In the two pairs of ischiopagus twins, each pair had two separate hearts, with normal cardiac structure and great vessel relationships. The viscera expressed normal laterality. Documentation of a defect in laterality in the right twin in three conjoined twin pairs with fusion of the hearts, combined with the presence of normal laterality in three pairs without cardiac fusion, has implications regarding the mechanisms leading to laterality of the human embryo. We suggest that rotation of the heart initiates the embryo's process of lateralization and that the laterality defects of the viscera seen in the right twin are a result of their abnormal cardiac rotation.

Abnormalities, Multiple↗

How automatic are social judgments?

Do people infer personality dispositions automatically when they encode behavior? Tulving's encoding-specificity paradigm was adapted to test three operational indicants of automatism: absence of intention, of interference from other mental activity, and of awareness. Recruited for a digit-recall study, subjects read sentences describing actions during the retention interval of either an easy or a difficult digit recall task. Later, sentence recall was cued by (a) disposition cues, (b) strong semantic associates to the sentence actor, or (c) words representing the gist of the sentence, or (d) sentence recall was not cued. Awareness was measured immediately after the last sentence was read. Disposition-cued recall was higher than (b) or (d) and was unaffected by digit recall difficulty. Awareness of making dispositional inferences was only weakly correlated with disposition-cued recall. Results suggest that disposition inferences occurred at encoding, without intention, without interference by differential drain on processing capacity, and with little awareness. Thus, making dispositional inferences seems to be largely, but not entirely, automatic.

Awareness↗

Aicardi syndrome: more than meets the eye.

An eight-month-old girl with infantile spasms and apparent blindness had electroencephalographic findings compatible with Aicardi syndrome. In addition to optic nerve hypoplasia, there were multiple congenital retinal malformations in the right eye, including chorioretinal lacunae, anomalous retinal vessels, posterior scleral ectasia, and a peripheral fibrous ridge. Magnetic resonance imaging demonstrated agenesis of the corpus callosum, absence of the septum pellucidum, optic nerve and chiasmal hypoplasia, pachygyria, cortical heterotopias, colpocephaly, and hypoplasia of the cerebellar vermis. This patient illustrates the broad spectrum of cerebroretinal malformations now known to characterize Aicardi syndrome.

Abnormalities, Multiple↗