The concept and nosology of Heller's syndrome: review of articles and report of two cases

Abstract

Based on a review of 60 historical cases of dementia infantilis and its synonyms (i.e., Heller's disease, Heller's dementia, Heller's syndrome and disintegrative psychosis) and a report of 2 new cases, the concept and nosology of Heller's syndrome, the most appropriate generic name for such conditions, were studied. In the age of onset, sex ratio, symptoms and a favorable prognosis in terms of life, a group of etiologically diverse conditions called Heller's syndrome differs from adult dementias and appears classifiable in pervasive developmental disorders according to DSM-III or DSM-III-R, even though its validity as a clinical syndrome remains to be studied.