Progressive Disintegrative Psychosis of Childhood

Abstract

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.

Practitioner Review: Diagnosis of autism spectrum disorder in 2‐ and 3‐year‐old children

“…The clinical significance of this is unknown. In some but not all cases of late disintegrative disorder, neurological disorders have been found (Corbett, Harris, Taylor, & Trimble, 1977).…”

Psychiatric Morbidity in Disintegrative Psychosis and Infantile Autism:A Long-Term Follow-Up Study

“…Over the years this condition has been described in the literature mostly in the form of case reports [1], and has been variously termed dementia infantilis, Heller's syndrome, progressive disintegrative psychosis [2], disintegrative psychosis (ICD-9) [3], pervasive disintegrative disorder [4] or, more recently (ICD-10) [5], other childhood disintegrative disorder ('other' differentiating it from Rett's syndrome) and childhood disintegrative disorder (DSM-IV) [6]. The ICD-10 and DSM-IV definitions are conceptually the same and the condition is defined as a type of pervasive developmental disorder with autistic symptomatology in which apparently normal development has been documented for at least 2 years, followed by a definite loss of previously acquired skills.…”

Acute onset of autistic features following brain damage in a ten-year-old

“…It has been hoped that improved techniques of this kind will help to elucidate the specific loci where damage is associated with autism (Corbett, Harris, Taylor, & Trimble, 1977;Lancet, 1976). In Peter's case, the CT scan sug-gested that there were multifocal lesions, though damage was possibly greater in the left (dominant) hemisphere.…”