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Childhood Disintegrative Disorder : A Case Report

Childhood Disintegrative Disorder : Sir, We would like to report a rare case of childhood disintegrative disorder. This disorder was first described by Theodore Heller in 1908. He described 6 children, who after 3 to 4 years of normal development displayed severe developmental regression in language, social skills and cognition. He called it 'Dementia Infantilis.' It was also called as 'Heller's disease'. It is rare disorder with reported prevalence of 0. Master V., a 4 year old boy was brought by his parents with history of gradual loss of speech and altered behaviour in past few months before the consultation. V. was born of a full term normal delivery and had normal milestones till the age of three and half years and had acquired age appropriate social and communication skills. About 6 months prior to consultation, a change in his behaviour was noticed. Initially parents noticed that at times he would appear very fearful, and on enquiry would not be able to give any reason for it. He gradually stopped relating to people as well as to his parents. His speech also deteriorated to a few unintelligible words, and finally he became totally mute. He would not even indicate his toilcl needs or ask for food or other tilings. He would remain occupied in his own world doing some repetitive purposeless activity like running in circle or laughing to self. During a couple of sessions in CGC, V. did not relate to any one or made any attempts to communicate in a meaningful way. He would not maintain eye to eye contact and would continuously go on doing some aimless activity without showing any interest in the surrounding. On examination there was no evidence of any systemic or neurological abnormalities. EEG showed occasional bursts of spike and sharp wave discharges, though clinically there was no evidence of seizure disorder. CT scan or MRI could not be done due to financial restrains. In this case, normal developmental milestones till the age of three and half years, gradual loss of language, cognitive and social skills, marked regression , loss of ability to relate to people and absence of any other neurological deficit were the points in favour of diagnosis of childhood disintegrative disorder. Increased incidence of seizures and EEG abnormalities have been reported in this disorder