Childhood disintegrative disorder Clinical features

In this condition an 'autistic-like' clinical pictures develops after a prolonged period of normal development. (l°) More than 100 cases have now been reported; the condition is rare but probably underdiagnosed. (1J.> Essential clinical features include a relatively long period of normal development followed by a marked developmental regression and development of various 'autistic-like' features; onset is typically between the ages of 3 and 4 years.

Onset can be relatively abrupt (days to weeks) or more gradual (weeks to months); a premonitory phase of non-specific anxiety or agitation may be observed. Parents often note associations between the onset of the condition and various psychosocial or medical events. (65,,6,6 and6Z> Such associations are probably correlational rather than causative, in that they are frequently observed at around this age. Factors reported include birth of a sibling or death of a grandparent, admission to hospital for elective surgery, and immunizations/68»

Once the condition is established it resembles autism, i.e. social and communicative skills are markedly impaired and various unusual behaviours and restricted interests are typically observed.(69) Given the behavioural similarity to autism, it might be argued either that the condition does not warrant separate diagnostic recognition or that the condition is inevitably the result of an association with some progressive medical condition. However, the pattern of onset is quite distinctive, the outcome appears to be even worse than in autism, and usually even with very intensive medical evaluations no general medical condition accounting for the regression is identified.

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