The Australian Childhood Dementia Study

To estimate the prevalence of childhood dementia, identify aetiological factors, and provide a brief survey of the psychosocial impact of childhood dementia.

The Australian Paediatric Surveillance Unit (APSU) was used to identify children with dementia. A total of 224 cases were notified about the dementia study and 141 (63%) questionnaires were completed and returned. Ascertainment was restricted to those most likely to have progressive neurocognitive decline because of practitioner bias against reporting non-progressive cases as dementia.

Eighty children with dementia were identified. The estimated prevalence for dementia in childhood in Australian was 5.6/100,000. Thirteen cases (16%) were notified before the age of two years. Seventeen (21%) children were reported to have uncertain or unknown aetiology. Impact upon day to day family functioning was reported by clinicians to be 'marked' or 'extreme' in 50 (63%) families. Only four (6%) clinicians judged present services to be 'very adequate', while 16 (20%) clinicians judged current psychological support for the needs of the family to be 'very' or 'moderately inadequate'. Dementia as an overarching diagnostic concept offers integrative possibilities for research, management and service planning and provision. Childhood disintegrative disorder as a concept is criticised.