CHILDHOOD DISINTEGRATIVE DISORDER: A CASE STUDY Sujoy Kumar Makar Audiologist and Speech-Language Pathologist Pamela Samaddar Audiologist and Speech-Language Pathologist Sharon Victoria Rout Internee AYJNIHH (ERC) KOLKATA -90 Abstract:- Introduction CDD is a developmental disorder that resembles autism, characterized by at least two years of normal development, followed by loss of language, social skills, and motor skills before ten years. Synonyms- Heller's syndrome, dementia infantilis. Aim was to study audiological, speech and language characteristics of a case with CDD. Methods- Audiological assessment included BOA, IA, TEOAE. Speech Assessment include REELS, SIRS, Autistic checklist, CARS for attention. Results moderate level of autistic feature is seen in child in form of communication deficit and impairment in reciprocal social interaction. No significant presence of repetitive stereotypic behaviour. CARS indicates moderate level of attention deficits and hyperactivity. Audiological assessment showed normal hearing. Discussion Child shows features of autism, however, characteristics disturbances did not occur before the age of 2 years so it diagnosed as CDD. Conclusion: Since the causes of CDD are unknown, there are no known ways to prevent this disorder. Introduction Childhood disintegrative disorder (CDD), also known as Heller's syndrome and disintegrative psychosis, is a rare condition characterized by late onset (>3 years of age) of developmental delays in language, social function, and motor skills. Thomas Heller, an Austrian educator, first described childhood disintegrative disorder in 1908. It is a complex disorder that affects many different areas of the child's development. It is grouped with the pervasive developmental disorders(PDDs) and is related to the better known and more common disorder of autism. Initially CDD was considered strictly a medical disorder and was believed to have identifiable medical causes. After researchers reviewed the reported cases of CDD, however, no specific medical or neurological cause was found to account for all occurrences of the disorder. For that reason, CDD was included in the fourth edition of the Diagnostic and Statistical Manual of Mental Disorders, or DSM-IV, in 1994. The cause of childhood disintegrative disorder is unknown. Research findings suggest, however, that it may arise in the neurobiology of the brain. About half the children diagnosed with CDD have an abnormal electroencephalogram (EEG). EEGs measure the electrical activity in the brain generated by nerve transmission (brain waves). CDD is also sometimes associated with seizures, another indication that the neurobiology of the brain may be involved. Children with CDD have at least two years of normal development in all areas— language understanding, speech, skill in the use of large and small muscles, and social development. After this period of normal growth, the child begins to lose the skills he or she has acquired. This loss usually takes place between ages three and four, but it can happen any time up to age ten. Studies have not shown much significant association between Childhood Disintegrative Disorder and deafness. Some authors have reported the association with hearing loss to be up to as low as 25%. Methods: A case aged three and a half years came with complaint of speech problem. The child showed slightly delayed development at 3 years of age and social interactions and communication became severely restricted. The problem started with sudden social withdrawal and being in his own world. According to parents report, impaired communication and social interaction, restlessness, disturbed sleep cycle, smiling to self and self- muttering, learning difficulty, and difficulty in writing. No history of convulsion, head injury or any other serious medical illness. Milestones like walking and talking delayed. As far as milestones for speech is concerned; it is reported that the child spoke normally and could say sentences till about 3 years of age, after which communication became restricted to monosyllables or very simple sentences. Audiological assessment included clinical examination and otoscopy. Pure tone audiometry could not be done as the child was too small instead Behavioural Observation Audiometry was done. Behavioural Observation audiometry was done using calibrated instruments drum, jhankara, bell other stimuli like name calling, clapping table banging etc were done. Baby reactometer was used to observe the free field responses.Impedance testing using GSI 38 Impedance Audiometer.Otoacoustic emission and auditory Brainstem Evoked Response Audiometry was done to confirm the diagnosis using the MAICO ERO SCAN and Nicolet MB11 time step standard ABR.The child was not able to follow instructions for play audiometry.Speech assessment was done using REELS Test by Bzoch & League, Speech Intelligibility Rating Scale,Autistic Checklist, Conner’s Abbreviated Rating Scale for attention . His speech and language stimulation were directive type. Oral peripheral examination reveals normal in appearance and vegetative function reported to be normal. Parameters of voice were perceptually normal. His suprasegmental aspects were normal except improper/asynchronous pattern of intonation for e.g., imitates vocalization with low loudness and infrequently varies inflection with high loudness and sometimes abrupt. His imitation skills were fair. Results Audiological evaluation reveals as per Behavioral observation findings and Impedance Audiometry as shown in Table 1 and Table 2. Otoscopic examination reveals of no occurrence of ongoing or intermittent episodes of SOM and clear EAC. CDD is a rare disease, much less common than autism. About 1 in 100,000 children are thought to have CDD. It is possible, however, that the disorder is under-diagnosed. For a long time, it was thought that CDD occurred equally among boys and girls. Newer research suggests that it is about four times more common in boys, and that many girls who were diagnosed with CDD actually had Rett's disorder, a disorder that shares many of the symptoms of CDD but occurs almost always in girls. There have been studies reported by Hagsberg & Gottsmen that CDD is also associated with mental retardation. The Behavioral observation findings as shown in Table 1
Responses with instrument baby reactometer as per Table 1b
Swp 60 present 0.5 60 present 1.5 50 present 3 50
Mode of response were localization, eye movement, head turning and localization
Table 2b. OAE and ABR : both ears pass Diagnosis: The child was diagnosed as normal hearing sensitivity. Speech and language Assessment:
REELS Test reveals RLA to be 2yrs and ELA to be one and half year.Speech intelligibility
was rated as 3. He was in exploratory play stage. His attention level was rated 3 (i.e., single channelled but still difficult to control). According to autistic checklist moderate level of autistic feature is seen in the child in form of communication deficit and impairment in reciprocal social interaction. However, no significant presence of repetitive stereotypic behaviour was noticed. Conner’s Abbreviated Rating Scale indicates moderate level of attention deficits and hyperactivity in the child. On the basis of above mentioned features the case was diagnosed as childhood disintegrative disorder. In differential diagnosis it is to be remembered that although the child does have some features of autism, the characteristics disturbances did not occur before the age of 2 years. Psychological Assessment: Child was diagnosed as mild developmental deficiency. Discussion CDD is most commonly diagnosed when the parents of the affected child consult the pediatrician about the child's loss of previously acquired skills. The doctor will first give the child a medical examination to rule out epilepsy or other medical conditions such as head trauma or a brain tumor. Following the medical examinations and tests, the child will be referred to a psychiatrist who specializes in treating children and adolescents. The psychiatrist will then make the differential diagnosis of CDD.
To be diagnosed with CDD, a child must show loss or regression in at least two of the areas
listed below. Usually regression occurs in more than two areas. These are: •
receptive language skills (language understanding)
expressive language skills (spoken language)
bowel or bladder control, if previously established
In this case study the receptive and expressive language age were delayed also the social and motor skills were also affected.Children with CDD are unable to start conversations with other people and often do not communicate with nonverbal signals (smiles, gestures, nodding the head, etc.) either. They also lose interest in playing games and in relationships with other people. They may engage in strange repetitive behavior, such as bobbing the head up and down, or other repeated movements. These changes must not be caused by a general medical condition or another diagnosed mental disorder. Treatment for CDD is very similar to treatment for autism. The emphasis falls on early and intense educational interventions. Most treatment is behavior-based and highly structured. Educating the parents is also emphasized in overall treatment plan. Speech and language therapy, occupational therapy, social skills development, and sensory integration therapy may all be used according to the needs of the individual child. Conclusion CDD must be differentiated from autism and such other specific pervasive developmental disorders as Rett's disease. It also must be differentiated from schizophrenia. One of the differences between CDD and other PDDs is that to be diagnosed with CDD, a child must develop normally for at least two years before loss of skills occurs, and the loss must occur before age ten. Most adults with CDD remain dependent on full-time caregivers or are institutionalized. Since the causes of CDD are unknown, there are no known ways to prevent this disorder. Further research is requered to rehabilitate these types of cases. REFERENCE American Psychiatric Association Diagnostic and Statistical Manual of Mental Disorders.4th ed. text revised. Washington DC: American Psychiatric Association, 2000. ental Disorder: Childhood Disintegration Disorder 1999;23(2):185-8. Belichenko PV, Hagberg B, Dahlström A. Morphological studyneocortical areas in Rett syndrome. Acta Neuropathol (Berl).1997;93(1):50-61. Fombonne E. Prevalence of childhood disintegrative disorder. Autism.2002;6(2):149-57 Gottesman II, Gould TD. The endophenotype concept in psychiatry:etymology and strategic intentions. Am J Psychiatry.2003;160(4):636-45. Hagberg B, Aicardi J, Dias K, Ramos O. A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett’s syndrome: report of 35 cases. Ann Neurol. 1983;14(4):471-9. Hagberg B, Witt-Engerström J. Rett syndrome: a suggested staging system for describing impairment profile with increasing age towards adolescence. Am J Med Genet. 1986;1:47-59. Risperidone treatment of children and adolescents with pervasive developmental disorders: a prospective open-label study. J Am Acad Child Adolesc Psychiatry 1997 May;36(5):685-93.[abstract] Paper was presented in ISAM 2008, Bhubaneswar
Facelifts As part of the aging process which happens to al of us sooner or late, our skin progressively loses its elasticity and our muscles tendto slacken. The stresses of daily life, effects of gravity and exposure to sun can be seen on our faces. The folds and smile linesdeepen, the corners of the mouth droop, the jaw line sags and the skin of the neck becomes slack. Around the eyes, t
[C’è un uomo di spalle, in piedi. Indossa l’impermeabile e il cappello. Ha appena spento la luce della sua stanza d’ufficio. Con la mano destra reg- ge una borsa marrone di pelle, voluminosa; la sinistra è appoggiata alla maniglia interna della porta che sta per aprire. (Uscito, la richiuderà dietro di sé, si avvierà verso l’ascensore, saluterà il portiere nell’atrio con un «buon