[A rare and not very studied disorder: childhood-onset schizophrenia. A case report].

UNLABELLED: Childhood-onset schizophrenia is rare: its prevalence is about 50 times lower than the one observed in adulthood. It is also frequently unrecognized, notably because its clinical aspect varies with age. The authors report the case of a prepubertal girl who developed a typical clinical picture of schizophrenia (paranoid subtype) by age 9.

CASE REPORT: The patient was 10 years old when she was hospitalized for a relapse of a suspected childhood-onset schizophrenia. Several significant mental disorders were found in her family history: her mother was treated for mood disorders (including dysthymia and major depression with postpartum onset), while her father and a aunt exhibited schizophrenic disorders. In addition, prenatal and perinatal events (including probable prenatal maternal infection and obstetric complications) were reported by her mother. Demonstrable impairments were already present in her premorbid development: from the age of 3.5, she showed significant manifestations of behavioural inhibition and separation anxiety, severe difficulties in social adaptation, and language abnormalities (qualified by her general practitioner as selective mutism). At the age of 9, when her mother was hospitalized for a diabetes mellitus, she suddenly showed auditory and visual hallucinations associated with delusions. Their content included filiation, somatic, and persecutory themes. Grossly disorganized behaviour (and more particularly catatonic motor behaviours including catatonic rigidity and negativism and bizarre postures) was also observed. Negative symptoms (eg anhedonia, affective flattening, and alogia) were noted. Her IQ scores were 74 in the verbal subtests and 53 in the performance subtests. Because the diagnostic of childhood-onset schizophrenia was suspected, a neuroleptic treatment, haloperidol 3 mg/day, was tried. After a partial remission during a few months period (characterized by a decrease in delusions, anxiety and sleep difficulties), she showed a relapse leading to her hospitalization. At the time of her admission, she showed severe manifestations of separation anxiety including agitation, anger, crying, and insomnia, for which she received a short-lived treatment by lorazepam. When sedation was obtained, the clinical picture proved similar to the one previously observed: hallucinations, delusions, grossly disorganized behaviour, and thought disorder were noted. As soon as the diagnostic of childhood-onset schizophrenia was confirmed, she was administered a new antipsychotic agent, amisulpride, at dose of 600 mg/day. This treatment was going on during several weeks with no significant clinical effect. Because the early onset of the disorder, the family history of schizophrenia, and the lack of effectiveness of the two previously administered antipsychotic agents, a treatment with clozapine was started at the dose of 12,5 mg/day. From the outset of this treatment, clinically significant reductions in hallucinations and disorganized behaviours were noted. Dose was then progressively increased until 200 mg/day, resulting in significant improvement in cognitive and motor functioning. The patient is now in an educational institute. Her adaptation is considered satisfactory, in spite of regular exacerbations of delusions in response to stressful life events. Treatment with clozapine is going on, without any significant undesirable clinical effects.

DISCUSSION: If an abrupt onset is rarely observed in prepubertal children, all the authors report that patients with very early onset schizophrenia show to have demonstrable impairments in their premorbid language as well as in their motor and social development. In addition, several studies suggest that more pronounced early developmental abnormalities are usually associated with a poor outcome in schizophrenia. The clinical picture also agrees with recent studies showing that in children paranoid subtype is as frequent as seen in adult disorders. If genetic factors play a significant role in the pathogenesis of schizophrenia, the notion that such factors may be more salient in very early onset and more severe cases is now usually accepted. However, a number of environmental factors, including prenatal maternal infections and perinatal complications, may also be implicated in the pathogenesis of schizophrenia, in addition to genetic factors. Because a significant relationship between stressful life events and exacerbations in positive symptoms was found in the case reported, the authors examine the role of such stress factors in the pathogenesis of schizophrenia and in the course of illness. A brief review of studies that have examined the effects of antipsychotic agents in children with schizophrenia underscores the paucity of data available to guide clinicians in this area. However, these data suggest that children who receive conventional neuroleptics experience significant adverse effects, primarily sedation and extrapyramidal symptoms. In addition, they suggest that new antipsychotic agents, such as clozapine, may be more effective than conventional neuroleptics, particularly in negative symptoms. Lastly, the authors emphasize the poor outcome usually reported in childhood-onset schizophrenia, highlighting the need of a long-term pharmacological and behavioural treatment.

CONCLUSION: This case report, such as others, supports the hypo-thesis that there is a clinical continuity between early and later onset schizophrenia. It also suggests that very early onset schizophrenia is a more severe form of the disorder and may be secondary to greater familial vulnerability. Consequently, systematic studies of these patients may be particularly informative and may provide important informations for understanding the etiologic processes involved in the pathogenesis of schizophrenia.