Childhood Disintegrative Disorder: Distinction From Autistic Disorder and Predictors of Outcome
ABSTRACT Childhood disintegrative disorder, a rare, relentlessly progressive neurologic disorder, first described by Heller in 1908, remains a condition of great interest. It has long been debated whether it is a discrete disorder or simply a late-onset variant of childhood autism. We have studied 6 cases of childhood disintegrative disorder, collected over 8 years, and followed for 2.5 to 22 years (mean 8.6 years). Childhood disintegrative disorder begins later in life than autism, and following a period of entirely normal development; the regression is more global and more severe than in autism; seizures are more frequent than in autism, yet demonstrable organicity in childhood disintegrative disorder is decidedly rare. Lastly, the prognosis is usually much worse than in autism, but in those cases with neither seizures nor epileptiform activity on electroencephalography (EEG), the outcome may be more favorable. Childhood disintegrative disorder should be viewed as a condition distinct from childhood autism.
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ABSTRACT: In humans, brain connectivity implements a system for language and communication that spans from basic pre-linguistic social abilities shared with non-human primates to syntactic and pragmatic functions particular to our species. The arcuate fasciculus is a central connection in this architecture, linking regions devoted to formal aspects of language with regions involved in intentional and social communication. Here, we outline a new anatomical model of communication that incorporates previous neurofunctional accounts of language with recent advances in tractography and neuropragmatics. The model consists of five levels, from the representation of informative actions and communicative intentions, to lexical/semantic processing, syntactic analysis, and pragmatic integration. The structure of the model is hierarchical in relation to developmental and evolutionary trajectories and it may help interpreting clinico-anatomical correlation in communication disorders.Current Opinion in Neurobiology 08/2014; 28C:165-171. DOI:10.1016/j.conb.2014.07.018 · 6.77 Impact Factor