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Progressive disintegrative psychosis of childhood.

Summary 
 
The literature concerning dementia and disintegrative psychosis in childhood is reviewed. Two cases with different neurodegenerative disorders presenting with late juvenile onset psychiatric symptoms arc described. There appears little point in retaining the eponymous syndromes of de Sanctis and Heller. The diagnostic category of Progressive Disintegrative Psychosis is appropriate to describe the clinical psychiatric picture in these children. Because of the rarity of such conditions further collaboration between child psychiatrists, paediatiric neurologists and neuropathologists is needed to delineate further the psychiatric syndromes associated with the neurodegenerative disorders.