Year : 2018 | Volume
: 2 | Issue : 1 | Page : 61--62
Childhood disintegrative disorder
Krunal Hitendrabhai Patel, Mukesh Jyantilal Samani
Department of Psychiatry, PDU Government Medical College and Hospital, Rajkot, Gujarat, India
Krunal Hitendrabhai Patel
A/603, New P.G. Hostel, Near Ruda Building, Jamnagar Road, Rajkot - 360 001, Gujarat
Childhood disintegrative disorder (CDD), a rare clinical syndrome with a prevalence of 1 in 100,000 children, was first described by an educator, Theodore Heller in 1908. He originally termed the condition dementia infantilis; subsequently, it has also been termed disintegrative psychosis or Heller's syndrome. CDD is characterized by marked regression in multiple areas of functioning following a period of at least 2 years of apparently normal development. We are presenting a case of an 8-year-old girl who presented with normal development until 6 years of age followed by gradual developmental deterioration in previously acquired language, social skills, and intellectual functions suggestive of CDD.
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Patel KH, Samani MJ. Childhood disintegrative disorder.Ann Indian Psychiatry 2018;2:61-62
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Patel KH, Samani MJ. Childhood disintegrative disorder. Ann Indian Psychiatry [serial online] 2018 [cited 2020 Jul 13 ];2:61-62
Available from: http://www.anip.co.in/text.asp?2018/2/1/61/232046
Childhood disintegrative disorder (CDD) was first described by Theodore Heller. It is a rare condition with a prevalence of 1 in 100,000 children. He originally termed the condition dementia infantilis; subsequently, it has also been termed disintegrative psychosis or Heller's syndrome. It is a complex disorder that affects many different areas of child's development. CDD was included first time in the fourth edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) in 1994. This condition is characterized by an apparently normal development for at least the first 2 years after birth, as manifested by the presence of age-appropriate verbal and nonverbal communication, social relationships, play, and adaptive behavior. Onset takes place at 3–4 years of age, but it can happen anytime up to age 10. The following case is an 8-year-old female child diagnosed with CDD. The limited number of CDD case presentations in the literature , makes any new case report important and useful in advancing the knowledge about this rare pervasive developmental disorder (PDD).
An 8-year-old girl child was brought by her mother to General Hospital with complaints of irritable behavior, passing urine and stools in clothes, not able to speak, and behavior disturbance. She was admitted in psychiatric ward for further evaluation and management. The patient was absolutely normal till age of 6 years. She was born of nonconsanguineous marriage with full-term normal vaginal delivery with no perinatal complications and had normal developmental milestones and acquired age-appropriate social and communication skills until the age of 6 years. She was toilet trained and was able to control her bowel and bladder. She was also used to be able to take care of herself in terms of maintaining personal hygiene. At the age of 6 years, she gradually stopped performing tasks that she used to be able to do such as asking for food and taking personal hygiene. Her speech deteriorated markedly over a period of 1½ years with total loss of speech since 6 months. Gradually, she decreased relating to family members and other persons in surroundings. She used to remain in her own world doing some repetitive purposeless activities such as continuously walking around and grasping stuff. Then, she lost toilet training and used to pass urine and stools in clothes. She was frequently found to be engaging in self-harming behavior such as banging head on wall, pulling her own hair, screaming, disrobing, and eating noneatables such as sand, soap, and insects. There was marked deterioration of motor skills such as she was not able to hold a glass of water properly.
On mental state examination, she looked appropriate to her age with moderate built and nourishment. Her eye contact was poor. She would continue to go on doing aimless activities without showing any interest in her surroundings. She was barely speaking monosyllables. She exhibited self-stimulatory behavior in the form of walking around and grasping things with her palm. Her gross motor skills were below normal and her balance was not so good.
Her vitals were within normal range. There were no any problems found on systematic examination including neurological examination. All the investigations such as complete blood count, liver function tests, renal function tests, and thyroid functions were within normal range. MRI brain showed possibility of mild demyelination at bilateral frontal-parietal periventricular region, diffuse thinning of corpus callosum with prominent all four ventricles.
As there was marked behavior disturbance present, behavior therapy or sensory integration therapy was given adjuvantly with the main focus on pharmacotherapy. It is difficult to comment on isolated effect of behavior intervention.
The unique feature of this case is very severe behavioral disturbance which caused marked distress to caregiver. Hence, patient was requiring aggressive pharmacological interventions. The patient was started on tablet risperidone 3 mg gradually increased up to 6 mg daily, but there was minimal improvement. Hence, tablet sodium valproate 600 mg was added. There was same status with this pharmacological line of treatment at the end of 3 weeks. Hence, patient was shifted on tablet clozapine which was gradually increased to 300 mg daily. With this treatment, there was full improvement in sleep, moderate improvement in irritability, temper tantrums, purposeless repetitive activities, and self-harming behavior but no improvement in bladder and bowel control, speech, and motor skills at the end of 6 weeks. She was still requiring assistance in feeding, personal hygiene, and toileting. The child is scheduled for clinical interview for every 1 month with above medication to assess the improvement. On subsequent follow-ups for next 1 year, there is same status. The caregiver's burden has been relieved by psychoeducation, counseling, and supportive psychotherapy.
As there was no history of any delusions, hallucinations, and disorganized speech, there was least chance of childhood schizophrenia. CDD, a part of a larger category called autism spectrum disorder, is a neuropsychiatric neurodevelopmental disorder. Unlike autism, children with CDD show severe regression after several years of normal development and more dramatic loss of skills than a child with autism does.
According to DSM-IV TR, to be diagnosed with CDD, a child must show marked regression of age-appropriate development after first 2 years of normal development.
The regression usually occurs in more than two of these areas:
Communication skillsSocial skillsPlayMotor skillsBowel or bladder control.
In addition, the patient must show abnormal functioning in at least two of the following areas:
Impairment in social interactionImpairment in communication orRestricted repetitive and stereotyped patterns of behavior, interests, and activities, including motor stereotypes and mannerisms.
None of the impairments should be better explained by schizophrenia or other PDDs.
In this case, normal developmental milestones till the age of 6 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 favor of a diagnosis of CDD. Pharmacotherapy with atypical antipsychotics has shown varying degrees of effectiveness in controlling behavioral problems in PDD patient; therefore, they are used in patients with CDD as well. However, in this case, pharmacological interventions have not lead to fairly positive changes in her clinical condition as it is not uncommon for therapeutic intervention to fail in PDD patient.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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