Journal of Autism and Developmental Disorders, Vol. 29, No.

6, 1999

Childhood Disintegrative Disorder

Savita Malhotra1,2 and Nitin Gupta1

Childhood disintegrative disorder (CDD) is a clinical syndrome characterized by disintegration of mental functions and regression of acquired language and intellectual functions after a period of normal development typically of 3 to 4 years. Although recognized for many years, research on this condition is less advanced than that in autism. Epidemiological data are limited but the condition is much less common than autism. The relationship of this condition to autism remains the topic of debate. Neuropathological and other medical conditions are sometimes associated with the disorder but contrary to earlier belief this is not typical. Collaborative research would facilitate our understanding of this condition.
KEY WORDS: Childhood disintegrative disorder; autism.

INTRODUCTION Childhood disintegrative disorder (CDD) is a clinical syndrome characterized by disintegration of mental functions and regression of acquired language and intellectual functions after a period of normal development up to 3-4 years in children. Occurrence of this condition, though with a different name, was described as long back as in 1930 (Heller, 1930) even before Infantile Autism, a related disorder; first described by Kanner in 1943. However, the research and development in CDD subsequently has not kept pace with that in autism, one of the most extensively studied disorder in child psychiatry today. CDD is currently classified under the broad rubric of Pervasive Developmental Disorders (PDD) which includes autism and autisticlike conditions in the major diagnostic systems operational nowadays, namely, ICD-10 (World Health Organization [WHO], 1992) and DSM-IV (American Psychiatric Association [APA], 1994). CDD is considered to be an autistic-like condition; which although recognized clinically, remains in need of better understanding (Rutter & Schopler, 1992). Although there are
Department of Psychiatry, Postgraduate Institute of Medical Education and Research, Chandigarh-160012, India; e-mail: medinst@pgi.chd.nic.in 2 To whom correspondence should be addressed.
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several reasons for continued interest, understanding of the reasons for paucity of research in this condition is equally important. The issues of nosology, relationship with or distinctiveness from other PDDs, and the nature of the disorder are open and still unresolved. The best-known aspect of CDD is its clinical description. Actual rarity of the disorder has been one of the major factors for the meager research data on CDD. In the following review, attempt is made to highlight these issues in the background of the available knowledge on CDD and other PDDs, in order to help form a perspective on CDD. EVOLUTION OF CONCEPT Conditions similar to Kanner's original concept of autism were reported in the early part of 20th century. Heller (1930) described six children who, after normal development to 3 or 4 years of age, showed mood changes, loss of speech, complete regression with limited recovery and termed it as "dementia infantalis." Later on, Kanner (1943) postulated that dementia infantalis and infantile autism were separate. Impetus to identification of such cases was given by the work of Kolvin (1971) who identified a group, intermediate (in age of onset) between infantile psychosis and late-onset psychosis. Following that, numerous case reports and

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case series appeared based on different classificatory systems, yet having commonalties in presentation and course. Over the years, numerous criteria/definitions have been proposed for CDD, reflecting the conceptual shifts in the understanding of CDD. Heller (1969) proposed the following guidelines, namely, (a) onset between 3 and 4 years of age, (b) progressive intellectual and behavioral deterioration with loss/marked impairment of speech noted at the time of onset, (c) associated behavioral/affective symptoms (fear, overactivity) and possible hallucinations, (d) an absence of obvious signs of neurological dysfunction or apparent "organicity" (e.g., normal facial appearance). Rutter et al. (1969) used the term "disintegrative psychosis of childhood" and defined it as (a) normal or near normal development for first few years of life, (b) onset after 2/4 years, (c) severe disintegration involving a severe disorder of emotions, behavior, and relationships and often a loss of speech and other functions. This concept was based on the premise that CDD is a type of childhood psychosis that could occur at varying ages (Kolvin, 1971). By definition, children with autism and childhood schizophrenia were excluded from this category. However, these conditions were included in the ICD-9 (WHO, 1978) under the category "psychosis with origin specific to childhood" (code-299.1). Using the same criteria as in ICD-9, Corbett, Harris, Taylor, and Trimble (1977) suggested a minor modification of the term to "progressive disintegrative psychosis of childhood" in order to distinguish such cases from psychiatric illnesses following nonprogressive encephalopathy. This recommendation was based on the evidence that many such children had the presence of organic brain disease. However ICD-9, and its modifications, did not prove to be very influential in guiding the thinking of clinicians and researchers (Volkmar, Klin, Marans, & Cohen 1997). In contrast, DSM-III primarily emphasized the organic basis and presumed association of this disorder with some progressive neurological processes (APA, 1980) and hence did not accord a formal diagnostic status to CDD and treated it as a prototype of dementia. Instead, in DSM-III, a new category termed "Childhood Onset PDD" (COPDD) came up which included children with an autistic-like condition that developed after 30 months of age. Distinction between COPDD and autism was based only on age at onset; this concept could not be validated and age at onset as a distinguishing feature was dropped in the DSM-III-R (APA, 1987). Autism and COPDD were clubbed together as

Malhotra and Gupta Autistic Disorders, broadly considered to be disorders of development. Thus, "Disintegrative Psychosis" of ICD-9 was equivalent to "Autistic Disorder-onset in childhood" in DSM-III-R. The shift in thinking was evident at this stage. The concept of psychosis of childhood was replaced by the concept of disintegration of mental functions; a concept that was similar to dementia in adults. A further shift was apparent, where the ideas about disintegration of mental functions being acquired due to a possible neurological disorder at some stage were replaced by being developmental in origin. Even though the diagnostic framework of DSMIII-R formed the basis of most of the case reports, controversy was generated about putting cases of CDD under the broad category of developmental disorders of childhood. This was so as CDD diagnosis required an apparently normal development till the stage of regression and progressive course, both of which were basically against the concept of PDD. Burd, Fisher, and Kerbashian (1988) provided some clarity to the basic concept by proposing the following diagnostic criteria, namely, (a) period of relatively normal development from birth past 30 months of age; (b) rapid regression lasting 1-3 years leading to (i) gross and sustained impairment in reciprocal social interaction, (ii) almost total lack of verbal and nonverbal communication, (iii) profound mental retardation with corresponding functioning in adaptive behavior; (c) almost no measurable growth in cognitive, linguistic, social, or adaptive functioning following the period of regression; (d) maintenance of good fine and gross motor skills; (e) the presence of compulsive and/or stereotypic behaviors; (f) not due to storage disease, organomegaly, or diagnosable neurological disease.They labeled such cases Pervasive Disintegrative Disorder. Malhotra and Singh (1993), used these diagnostic criteria in their cases and added to the validity of this concept. Although the diagnostic approaches of ICD-9, DSM-III, and DSM-III-R systems remained highly divergent, there was agreement on certain key features that characterized CDD, that is, (a) onset beyond the age of 2 years of normal development, (b) presence of autistic-like features, and (c) absence of organic or neurological illness. Efforts were made to bring in greater convergence between ICD-10 (WHO, 1992) and DSM-IV (APA, 1994) by adopting conceptually similar criteria. The term CDD was formally introduced in these systems. DSM-IV criteria were less detailed and operationalized than those in ICD-10 (Volkmar et al., 1997). Though CDD has now been subsumed under PDDs, yet its definition emphasized discontinuity with autism on account

Childhood Disintegrative Disorder of a period of normal development. The key defining features of CDD and its relationship with autism are examined further in the following discussion. EPIDEMIOLOGY Community-based epidemiological studies are lacking in this area. The only community-based epidemiological study done so far is by Burd, Fisher, and Kerbeshian (1987), which reported a prevalence rate of 1 per 100,000. A clinic-based study reported CDD in 0.22% of all cases attending a child psychiatric clinic over a 5-year period (Malhotra & Singh, 1993). Various other clinic-based reports of CDDs derived from the samples having broad diagnosis of childhood psychosis, autistic disorders, developmental disorders and so forth reported rates ranging from 1.64% (Volkmar et al, 1994) to 6% (Volkmar & Cohen, 1989). Volkmar reported that approximately 106 cases have been reported in the world to 1997 and a further review to mid-1999 has yielded literature on 20 more cases. However, any considerations on the prevalence of CDD must take into account the fact that this is a rare disorder, perhaps less common than autism. On the other hand, possibility of misdiagnosis, use of different classificatory systems, and the relative lack of familiarity of clinicians in arriving at the diagnosis (Volkmar & Rutter, 1995) could have all contributed to a low frequency of identified cases. Moreover, the data from the developing countries is extremely scarce. Going by the report of Malhotra and Singh (1993), the actual number of cases would be significantly higher considering the population size of these countries. Under the circumstances, pooling of data from different countries could generate meaningful cohorts for extensive and long-term follow-up studies. Males outnumber females in most studies. From the cases identified by Kurita (1988) and Volkmar (1992), male: female ratio is 3:1 (before 1977) whereas it is 5.5:1 thereafter. Overall, males outnumber females by 4:1 (Volkmar et al., 1997). It has been observed that the equal or lower male: female ratio in earlier cases (Rapin, 1965) could be attributed to a misdiagnosis of CDD most commonly as Rett disorder (Hill & Rosenbloom, 1986). ONSET AND PROGRESSION Age at onset (AAO) has been one of the key defining features of the CDD and has been linked with the changing concept and understanding of CDDs. The min-

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imum age at onset was described as 2 1/2 years (Rutter et al., 1969) and 2 years (ICD-9). ICD-10 (WHO, 1992) criteria require a 2-year period of apparently normal development. Volkmar et al., (1997), in their review of 106 cases, had reported a mean AAO of 3.36 years. This, in fact, is in keeping with the age range proposed by Heller (1930), indicating that the cases identified so far appear to resemble the original concept of CDD. But, at a closer look, the age range has varied from 1.2 years (Evans-Jones & Rosenbloom, 1978) to 9 years (Corbett et al., 1977). However, these reports came up at a time when the disorder was construed as disintegrative or psychotic rather than developmental in nature. Onset before 2 years of age requires consideration of other possibilities such as Rett syndrome (in case of females) or the autistic disorders. On the other hand, there is some evidence to suggest that a later AAO is linked with organic or brain dysfunction manifesting with a neurological illness (Corbett et al., 1977; Volkmar 1992; Volkmar et al., 1997). The point to be emphasized here is that one should be cautious in assigning a diagnosis of CDD straightaway if the AAO is towards the extremes (or not in the common age range of 3 to 5 years). A thorough review of the presentation with detailed evaluation and examination is required before arriving at a diagnosis. Some doubts have been expressed on the possibility of the illness starting prior to manifest clinical symptomatology and on the reliability of parental report on the apparently normal development during the first 2-3 years of life in children with CDD. Since AAO is the critical feature differentiating CDD from autistic disorder, this issue has been carefully examined. (Rogers & Dilalla, 1990; Volkmar, Stier, & Cohen, 1985;). It has been observed that children of CDD have a clearly delineated onset and regression, especially for "loss of previously acquired skills" which is absent in autistic disorders. Although some reports have shown that early developments of such children may show some delays or associated abnormalities in pregnancy or labor or delivery (Kurita, 1988; Volkmar, 1992; Volkmar & Cohen, 1989;), these are highly nonspecific and insufficient to account for the severity of developmental deficits. Thus, in CDD, there is characteristically developmental regression followed by continued developmental difficulties, rather than progressive developmental difficulties to start with. Although there is still lack of clarity about the age of onset/recognition, normal development till 2 years of age is an accepted criterion for diagnosis of CDD. Whenever information on early development is lack-

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ing or there is difficulty in timing the onset of developmental regression, it is better to categorize the condition as atypical autism (Volkmar et al., 1997). Two types of onset have been seen. More commonly, it is insidious onset developing over weeks to months and less common is abrupt or acute onset developing over days to weeks. Occasionally a premonitory phase is evident wherein the child is overactive, restless, anxious, and irritable (Volkmar, 1992). SYMPTOMATOLOGY AND RELATIONSHIP WITH AUTISM Clinical description is the best known aspect of CDD so far. Comprehensive reviews by Kurita (1988), Volkmar (1992), and Volkmar et al., (1997) constitute the most significant sources of reading about the disorder. Most case reports (especially those reported after 1977) have been based on diagnostic concepts similar to those in ICD-9/ICD-10. Also most of the literature is from the West with only two reports from developing countries (five cases by Malhotra & Singh, 1993; and one case by Jaydeokar, Bal, & Shah, 1997). As per the current diagnostic criteria, patients of CDD would manifest with features indistinguishable from autism. However, going by the available data for cases of CDD, it was seen that speech deterioration/loss was universally present followed by social disturbance, stereotypy/resistance to change, overactivity, affective symptoms/anxiety, deterioration of self-help skills in decreasing order of frequency (Volkmar et al., 1997). In fact, apart from the prominent regression in acquired skills, it is the communication disturbance that is the most striking. The loss of speech tends to be so great that it is akin to the marked lack of speech development seen in autism (Volkmar et al., 1997). Associated features reported are aggression, agitation, self-injurious behavior, fecal smearing (Volkmar & Cohen, 1989) with compulsive behavior (Malhotra & Singh, 1993). Deterioration in social and self-help skills is more marked than motor skills (Volkmar, 1992, Volkmar et al., 1997). Although ICD-10 includes a general loss of interest in the environment, it has not been commented/ reported upon in the available literature. Also, it is important to keep in mind that the cases are diagnosed according to certain concept and criteria which pattern the clinical profile of cases. Hence, it is important to pay attention to all the symptoms described in the children with CDD rather than look for just the fulfillment of specified criteria. This exercise could help in validating and delineating clinical boundaries of the disorder. Another exercise that needs to be carried out is to document

Malhotra and Gupta the progression, onset, and severity of various skills that undergo regression which could help in understanding the natural course of development of CDD. Since there is a considerable overlap of clinical features between CDD and autism, consideration can be given to the possibility that CDD is a variant of autism. Going by the later onset of illness in CDD, it can be hypothesized that CDD may be a late-onset variant of autism. There is considerable evidence to suggest that earlier the onset of illness, the more severe is the illness manifestation; especially during the developmental years, for example, childhood onset schizophrenia (Jacobsen & Rapoport, 1998). If such is the case, then autism should manifest with more severe psychopathology than seen in CDD. But evidence available is contrary (Volkmar & Cohen, 1989, Volkmar & Rutter, 1995); CDD is more severe than autism. Additionally, an entity "later-onset autism" has not been found to be similar to CDD (Volkmar & Cohen, 1989) as CDD is more severe and exhibits regression of acquired skills which is not seen in autism. This raises the other possibility that CDD could be a more severe form of autism. Evidence for the same is available from a few studies that compared CDD with autism (Volkmar & Cohen, 1989; Volkmar & Rutter, 1995). However this is a conceptual question as CDD is described to be associated with normal development before onset whereas autism, as per definition, is associated with developmental problems. The relationship between autism and CDD is still not clear and continues to be a subject of debate. Autistic-like features can be seen in several disorders. There are reports that children diagnosed as childhood schizophrenia fulfilled criteria for diagnosis of infantile autism in early infancy in 39% and presented with severe deficits in language and motor development in 72% of the cases (Watkins, Asarnow, & Tanguay, 1988). Children with schizotypal disorder met criteria for PDD in 18 out of 20 children reported by Nagy and Szatmari (1986), and are likely to be diagnosed invariably as PDDNOS (Russel, Bott, & Sammons, 1987). Description of symbiotic psychosis (Mahler, 1952; Mahler & Gosliner, 1955), though based on psychoanalytic concepts, the clinical profile described in the case reports was very similar to that of autism, that is, extreme ambivalence, extreme anxiety, aggressive outbursts, preoccupation with inanimate objects, and marked fantasies. Asperger syndrome although has been considered as a mild or less severe form of autism (Gillberg, 1985; Szatmari, Bartolucci, & Bremner, 1989), clinical and neurobiological characteristics were reported to be similar to infantile autism (Gillberg, 1985, 1987, 1989). All these above conditions, including CDD, have now been included under the broad category of PDDs where the uni-

Childhood Disintegrative Disorder fying features are typically autistic (qualitative abnormalities in reciprocal social interaction and in the patterns of communication; restricted, stereotyped, repetitive repertoire of interests and activities; in a pervasive manner) occurring in early infancy or childhood upto 5 years of age (ICD-10). ICD-10 also mentions that there is disagreement on the subdivision of this overall group of PDDs. Thus the differences between CDD and autism can be considered as operational and not final, which should help facilitate further research for better validation.

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an associated neurological condition; (b) progressive and rapid deterioration appears to be a feature of this association (Volkmar et al., 1997). The clinical syndrome of CDD can occur in known neurological conditions, but CDD can also occur without it. These organic disorders, if present, are not part of the exclusion criteria but these conditions are coded on a different axis in ICD-10 or DSM-IV. Occasional association with neurological disease may help in providing an insight into the neuropathological basis of CDD. Clinically, sudden onset of regression after a period of normal development in a child would raise suspicion regarding presence of neurological disorder. As CDD is relatively rare, it would be worthwhile studying the clinical and psychological profile of children presenting with known neurological disorders and to establish presence of CDD in them. This approach would help in ascertaining the degree of association between the two. NEUROBIOLOGY AND GENETICS Despite over 126 cases reported in literature, there is complete absence or paucity of information regarding the neurochemistry, neuropsychology, neuroanatomy, and neurophysiology of CDD (Volkmar et al., 1997). EEG is the only neurophysiological parameter that has been studied in some detail. Additionally, only a single work is available on the neurochemistry of CDD (Gillberg, Terenius, Hagberg, & Witt-Engerstrom, 1990). The authors reported absence of low levels of CSF betaendorphins in two children with CDD compared to children with autism and Rett syndrome. Family history of children with CDD has generally revealed an absence of autism, schizophrenia, or organic illnesses (Burd et al., 1998; Corbett et al., 1977, EvansJones & Rosenbloom, 1978). Additionally, information on the genetic linkage of this condition in extremely limited (Volkmar et al., 1997). There is a need to seriously focus on this issue as it can shed light on its etiology. STRESS AND CHILDHOOD DISINTEGRATIVE DISORDER Presence of both medical or psychosocial events has been associated with the onset of CDD. This was first noted by Evans-Jones and Rosenbloom (1978); a finding found commonly by Volkmar and Cohen (1989) in their study of 10 cases. In details available for 35 cases after 1977, psychosocial events were found in 10 cases. Most commonly, birth of a sibling or a family crisis were noted. Additionally, many cases have been apparently associated with some medical condition (e.g., measles,

ORGANIC BRAIN PATHOLOGY CDD has been associated with certain diagnosable neurological conditions such as neurolipidosis (Malamud, 1959), tuberous sclerosis (Creak, 1963; Mouridsen, Rich, & Isager, 1998) subacute sclerosing panencephalitis (Mouridsen et al., 1998; Rivinus, Jamison, & Graham, 1975;), metachromatic leukodystrophy, Addision-Schilder disease (Corbett et al., 1977), and seizures (Malhotra & Singh, 1993). The association of neurological disorders, however, appears an exception rather than the rule. However, association of seizures or EEG abnormality have been documented in about half the cases of CDD (Volkmar et al., 1997). EEG abnormality is encountered more commonly than actual seizures. Although, the frequency of seizures and EEG abnormality has been reported to be similar in both CDD and autism, a recent report (Mouridsen et al., 1998) found higher rates of symptomatic epilepsy in CDD. Additionally, it should be noted that seizures most commonly develop after the onset of CDD; rarely do seizures herald the onset (Malhotra & Singh, 1993). The degree and frequency of mental retardation (MR) encountered in the CDD children has not been described in as much detail as in autism (Volkmar et al., 1997). On examining the available literature, it was seen that majority of the children function at an IQ level commensurate with severe to profound MR (Evans-Jones & Rosenbloom 1978; Malhotra & Singh, 1993; Volkmar & Cohen, 1989; Volkmar & Rutter, 1995). Although borderline IQ was reported by Corbett et al. (1977) in one of the two cases reported by him, enough attention has not been paid to this aspect of the condition. Autism also has a strong association with MR (Volkmar et al., 1994). In field trials for DSM-IV autistic disorders, nearly 25% of children with a diagnosis of autism had IQ < 40 compared to nearly 60% of patients with CDD indicating a tendency for greater frequency as well as severity of MR in CDD than in autism. Certain important observations can be drawn from the limited literature available, (a) the later the onset of CDD probably higher the chances of

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seizures, accidental injury, elective surgery, or hospital admission). Although the frequency of association of such events with onset of CDD is somewhat striking, to term the same as a precipitating event is not fully justified. In fact, such events (especially psychosocial) are commonly reported in this age group and the association may be by chance only (Rutter, 1985). However, even if these events are taken only as a form of stress associated with the onset of CDD, there is a theoretical possibility that the regression noticed is a consequence of the same. Arnsten (1999) reported that stress can lead to impairment in functioning of the prefrontal context (PFC) manifesting in the form of hyperactivity, disorganized, and impulsive behavior and even cognitive deficits. COURSE AND OUTCOME Information on the course and outcome of CDD has been available in detail from 1977 onwards (Volkmar, 1992). The follow-up period has varied from 1.2 years (see Volkmar, 1992) to 32 years (Mouridsen et al., 1998). Developmental regression leading onto severe to profound MR is seen in more than 75% of all cases (Volkmar et al., 1997). Commonly, the course is one of minimal to mild improvement (Volkmar & Cohen, 1989). However, static course has also been commonly observed (Evans-Jones & Rosenbloom, 1978). It is only in a few cases that the course is one of progressive deterioration (Corbett et al., 1977; Mouridsen et al., 1998). This type of course is especially reported in cases with comorbid neurological illnesses. Life expectancy appears to be normal with some cases even showing worthwhile degree of improvement (Volkmar et al., 1997). However, it has been noted that such patients tend to be more mute and in residential placement as against patients with infantile autism (Volkmar & Rutter, 1995). In a recent study by Mouridsen et al. (1998), CDD was compared with infantile autism on numerous outcome variables. It was seen that CDD patients tended to have lower functioning, were more aloof, and had greater comorbid epilepsy; thereby supporting the notion of a poorer course. Also, 15% of their sample of CDD had died as compared to only 3% in the autism group. ASSESSMENT Despite the onset being clearly demarcated from the premorbid period, CDD requires a detailed assessment for the purposes of proper diagnostic labeling and

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treatment. A careful, proper and thorough history can have no substitute. Details of the pregnancy and neonatal period, early developmental stages, and the general medical and family health problems is necessary. This should, as far as possible, be supplemented with movies, videotapes, and baby books for evaluating closely the early development and using the same as external validating sources of information to parents' reporting (Volkmar et al., 1997). If necessary, another option could be to apply the checklist for Autism in Toddlers (CHAT., Baron-Cohen, Allen, & Gillberg, 1992); a screening instrument for autistic spectrum disorders, thereby reducing the chances of such children diagnosed as CDD. Keeping in mind the unusual presentation of CDD, detailed information on the pattern and AAO is essential. To supplement the historical information, observation of the child's play may be helpful in recording the levels of language, social, and cognitive organization, gross and fine motor skills, and problematic or unusual behaviors. As such children have a high prevalence of mental retardation, appropriate scales should be administered, for example, Reynell Developmental language Scales (Reynell & Gruber, 1990), Vineland Adaptive Behavior Scales (Sparrow, Balla, & Cicchetti, 1984) for documenting the level of communication achieved and adaptive behaviors. This will be helpful in the psychoeducational-cum-rehabilitation program for such children. A very important aspect, not to be overlooked, is to assess the degree of engagement and knowledge of the parents. Also, it is necessary to evaluate the burden of care felt by the caregivers as families of such patients experience tremendous social and emotional burden (Malhotra & Singh, 1993). If this aspect is not taken care of, there can be difficulties in ensuring adequate treatment for the patients. Extensive medical investigations have generally not revealed evidence of abnormality in such cases (Burd et al., 1998; Malhotra & Singh 1993;). Despite this observation, a detailed workup is necessary especially so if (a) onset of CDD is beyond 6 years, (b) deterioration is progressive. Table I lists the possible investigations that can be done depending upon the clinical indication. TREATMENT There are no fixed guidelines or treatment strategies for management of CDD. In the various case reports (Burd et al., 1998; Evans-Jones & Rosenbloom, 1978; Malhotra & Singh, 1993;), management was

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Table I. Investigation Profile for CDD Urine examination Complete haemogram Liver function test S. Calcium/phosphorous Serum lactic acid Serum ammonia VDRL for syphilis Lactate dehydrogenese Urinalysis Porphobilinogen Aryl sulphatase A activity Hormonal Thyroid function tests Synacthen test CSF analysis Culture Electrophoresis Neurophysiological EEG (sleep, awake) Nerve conduction studies Evoked potentials (visual, auditory) Neuropsychological IQ assessment Neuroimaging CT Scan (plain, contrast)

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the patients of CDD as the level of burden of care by parents/caregivers is enormous (Malhotra & Singh, 1993; Volkmar et al., 1997). Psychoeducation, supportive therapy, and induction into support groups are helpful in dealing with their distress and in channeling efforts towards effective management of their wards. SUMMARY AND CONCLUSIONS CDD is one of the rare and severe neuropsychiatric disorder of childhood occuring during early developmental years and has a poor prognosis. Although there is still controversy about the true nature of the disorder, partial similarity of its clinical picture with autistic disorders, developmental regression, and occasional association with known neurological disorders point towards a possible biological basis for the disorder. Apart from its clinical description, the disorder has not been studied in any detail. Inclusion of CDD in the official classifications, better understanding and delineation of autism, advancements in the investigative techniques would and should facilitate further research into this disorder. Considering the rarity of the disorder, which could largely be an artefact of misdiagnosis, further research by international collaboration and by pooling the cases across centers could serve as a more useful strategy. It is very important to undertake studies on neurochemistry, neurophysiology, neuropathology, genetics, and so forth on the same pattern as for autism. This would help in the better understanding of not just CDD but also of the larger processes involved in the pathogenesis of other related PDDs. REFERENCES
American Psychiatric Association. (1980). Diagnostic and statistical manual of mental disorders (3rd ed.). Washington, DC: Author. American Psychiatric Association. (1987). Diagnostic and statistical manual of mental disorders (3rd ed/., Rev.). Washington, DC: Author. American Psychiatric Association. (1994). Diagnostic and statistical manual of mental disorders (4th ed.). Washington, DC: Author. Arnsten, A. F. T. (1999). Development of the cerebral cortex: XIV. Stress impairs prefrontal cortical function. Journal of American Academy of Child and Adolescent Psychiatry, 38, 220-222. Baron-Cohen, S., Allen., J., & Gillberg, C. (1992). Can autism be detected at 18 months? The needle, the haystack, and the CHAT. British Journal of Psychiatry, 161, 839-843. Burd, L., Fisher, W., & Kerbeshian, J. (1987). A prevalence study of pervasive developmental disorders in North Dakota. Journal of American Academy of Child and Adolescent Psychiatry, 26, 700-703. Burd, L., Fisher, W., & Kerbeshian, J. (1988). Childhood onset pervasive developmental disorder. Journal of Child Psychology and Psychiatry, 29, 155-163.

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Others (SPECT, PET, fMRI) Miscellaneous Heavy metal screening Galactosemia assay Phenlyketonuria assay Brain biopsy (in selected cases)

more symptom-based with numerous psychotropics being tried out. For symptoms of hyperactivity, aggression, and overall behavioral control, antipsychotics (e.g., haloperidol) have been tried with mild improvement only. Even benzodiazepenes (Evans-Jones & Rosenbloom, 1978), antidepressants, and lithium (Burd et al., 1998) have been employed but with disappointing results. Only recently, clozapine has been tried in three children with CDD with some effectiveness but leucopenia was observed in one case (Gelly, Chambon, & Marie-Cardine, 1997). Seizures are a common cooccurrence in CDD, treatment for the same is done with antiepileptics; most commonly carbamazepine. Apart from the pharmacological measures employed, nonpharmacological techniques such as behavior modification and special education to help encourage the reacquisition of the basic adaptive skills (Volkmar et al., 1997) can be applied. Children can be kept at home by their parents or in the residential settings. The treating clinician should pay attention to the caregivers of

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