Childhood Disintegrative Disorder: Clinical Features and Diagnostic Criteria

Waleed Ahmad

Published online by MRCPsych UK: Tuesday, 03 May 2022

Introduction

There is a loss of skills in several areas of development and deficits in social, communicative, and behavioural functioning that follow normal development in this condition. Often the condition follows a prodromic period during which children develop obscure symptoms; they become restive, irritable, anxious, and overactive. Impoverishment follows this and then loss of speech and language, accompanied by behavioural disintegration. Sometimes the loss of skills is persistently progressive (especially if there is an underlying progressive neurological condition), but more often, the decline over some months and then a slight improvement. The prognosis is usually abysmal, and it leaves most individuals with severe intellectual disability. There is uncertainty about the extent to which this condition differs from autism. Sometimes, the disorder can be because of some associated encephalopathy, but clinicians should make the diagnosis on the behavioural features. If the condition occurs because of an underlying neurological condition, clinicians should record that separately.

Diagnostic Features

Diagnosis requires a healthy development during the first 2 years of life, followed by a loss of skills; qualitatively, abnormal social functioning accompanies this.
A profound regression of language, level of play, social skills, adaptive behaviours, and bowel, or bladder control are common.
Patients also lose interest in the environment and develop stereotypes, mannerisms, and social, and communication deficits.
Unlike dementia, there is no evidence of organic disease, and the lost skills may recover. Thus, the ICD-10 has classified the syndrome as a pervasive developmental disorder, instead of dementia.1

Inclusions and Exclusions

The ICD-10 includes the following under childhood disintegrative disorder:

1. Symbiotic psychosis
2. Heller disease
3. Disintegrative psychosis
4. Dementia infantilis

However, the following are excluded:

1. Selective mutism
2. Schizophrenia
3. Rett disorder
4. Acquired epileptic aphasia

With autistic disorder, Asperger syndrome, and atypical autism, the DSM-5 and ICD-11 classifications have subsumed it under autism spectrum disorders.2-3

About the Author

Waleed Ahmad, consultant psychiatrist at the Department of Psychiatry, Mercy Teaching Hospital, Peshawar, Faculty member at the Department of Psychiatry and Behavioural Sciences, Peshawar Medical College, Peshawar, 25 000, KP, Pakistan. Email: dr.waleed@outlook.com

Copyright

Bibliography

1. The ICD-10 Classification of Mental and Behavioural Disorders: Clinical Descriptions and Diagnostic Guidelines. 10th ed. World Health Organization; 1992.
2. Association AP. Diagnostic and Statistical Manual of Mental Disorders. 5th ed. American Psychiatric Association; 2013.
3. Organization WH. International Classification of Diseases, Eleventh Revision (ICD-11). World Health Organization. Updated February 11, 2022. Accessed April 25, 2022. https://icd.who.int/browse11/l-m/en

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