The "happy puppet" syndrome.

In 1965 Angelman described three children whose clinical features were sufficiently similar to warrant the constitution of a new syndrome-so-called 'Puppet Children'. It was immediately apparent to us that two patients whom we had studied for several years conformed to his description. Moreover, they show certain unusual EEG features which, we suggest, constitute another aspect of the syndrome. Angelman's 'Puppet' children were grossly subnormal, had major fits, and frequent infantile spasms or minor fits. The more specific features were: easily provoked and prolonged paroxysms of laughter, ataxic jerky movements like the movements of puppets, and the ability to protrude the tongue to an unusual degree. Physical examination showed brachycephaly, a horizontal occipital depression, certain ocular features, and apparent prognathism with a common facial appearance, difficult to describe but illustrated in his paper. Skull x-ray films showed a vertical inclination of the base, and all cases showed spikes and slow waves in their EEGs at some time. The histories of our two cases are as follows.