Schizophrenia is a neurodevelopmental disorder that most commonly emerges in early adulthood. DSM-5 (American Psychiatric Association 2013) criteria for schizophrenia are the same at all ages. At least one of three “positive symptoms”—delusions, hallucinations, and disorganized speech—is required for diagnosis. The DSM-IV subtypes have been eliminated because of poor reliability, lack of stability, and limited utility for treatment or prognosis. When the disorder develops before age 18 years, it is called early-onset schizophrenia (EOS) or, in the rare cases in which it develops before age 13 years, childhood-onset schizophrenia (COS).
Schizophrenia is characterized by positive and negative symptoms. Positive symptoms may include hallucinations, delusions, disorganized speech, and grossly disorganized or catatonic behavior. Negative symptoms include apathy, avolition, and poverty of speech. Cognitive decline, including deficits in attention, memory, processing speed, and executive function, often occurs at the time of illness onset.
EOS is rare, and COS is even more so. Peak age at onset of schizophrenia is between 15 and 25 years in men and between 20 and 30 years in women. Lifetime prevalence is estimated at 0.3%–0.7%.
There is a strong genetic contribution to risk for schizophrenia, with first-degree relatives of an affected person having 5%–20% higher lifetime risk of developing the disorder than the general population. No single gene or genetic locus is implicated; rather, multiple genes and genomic loci are involved, and factors such as epigenetics, somatic mutations, and gene-by-environment interactions likely contribute. Risk factors likely act through these mechanisms and include prenatal infection, obstetric complications, in utero exposure to famine, advanced paternal age, marijuana use, migration, and lack of social support (Kodish and McClellan 2016).
COURSE AND PROGNOSIS
As schizophrenia affects cognitive, emotional, and social functioning and development, EOS often has a severe course and poor prognosis. Schizophrenia is characterized by four phases: prodromal, acute, recovery, and residual (McClellan et al. 2013). The prodromal phase is marked by a decline in function, which in youth may present as social isolation, academic decline, odd preoccupations, and mood symptoms. This is followed, after a brief or extended course, by the acute phase, in which positive symptoms appear. Hallucinations, delusions, and disorganized or dangerous behavior generally bring the child to treatment. Recovery from the acute phase may take 6 months or longer and is often incomplete with persistent deficits. The residual phase is generally marked by chronic impairment in social and educational function. Adolescents with schizophrenia are at increased risk for death from suicide or accidents.
EVALUATION AND DIFFERENTIAL DIAGNOSIS
A comprehensive history is necessary to clarify premorbid functioning and current positive and negative symptoms. Information from multiple sources will increase the likelihood of getting a full assessment of family history, prenatal exposures, exposure to trauma or neglect, and longitudinal academic and social function. A complete physical exam is indicated to rule out any nonpsychiatric medical condition that might cause psychotic symptoms. Use of neurological consultation, laboratory tests, and neuroimaging evaluations should be guided by the history and physical examination.
Urgent medical conditions that must be ruled out or treated in a child presenting with psychosis include delirium; substance intoxication; medication reaction; brain neoplasm; head injury; stroke; autoimmune encephalitis; meningitis; AIDS; fluid, electrolyte, or glucose abnormalities; endocrine abnormalities; Wilson’s disease; and porphyria.
Children and adolescents with mood disorders may present with psychotic symptoms that overlap with those of schizophrenia. A child with psychotic depression may have affective flattening and mood congruent or incongruent hallucinations. Adolescents with mania may present with hallucinations, delusions, and disorganized behavior. Longitudinal observation while treating the mood symptoms should clarify the diagnosis over time.
Children and adolescents who have been victims of trauma may report psychotic-like symptoms, including reexperiencing in the form of hallucinations, as well as restricted and repetitive play. The intrusive thoughts and compulsive behaviors of obsessive-compulsive disorder (OCD) may suggest impaired reality testing without the presence of true psychosis. The symptoms of autism spectrum disorder (ASD), including deficits in communication and social aloofness, may mimic the negative symptoms of schizophrenia, but symptoms of ASD are generally earlier in onset, more pervasive, and more stable over time.
Normative experiences can be mistaken for psychotic symptoms. Children may have vivid fantasy play and magical thinking beyond the typical age. They may confuse hallucinations with dreams or the hallucinations that can occur with falling asleep (hypnagogic) and waking (hypnopompic). They may report apparent hallucinations or delusions that actually reflect shared familial, religious, or cultural beliefs. Young children with fever may be prone to acute hallucinations, unrelated to a psychiatric disorder.
Treatment of schizophrenia in youth requires a comprehensive approach that involves the patient, family, and community and incorporates medication as well as psychosocial and educational interventions.
The cornerstone of psychopharmacological treatment for EOS is antipsychotic medication (see Chapter 17, “Psychopharmacology”). Controlled trials have confirmed the effectiveness of antipsychotics in improving psychotic symptoms and overall functioning. Trials have not established clear superiority of second-generation over first-generation antipsychotics in terms of efficacy or tolerability. The U.S. Food and Drug Administration has approved indications for risperidone, olanzapine, paliperidone, quetiapine, aripiprazole, and lurasidone for treatment of youth age 13 years and older with schizophrenia, but controlled trials have also supported haloperidol, loxapine, thioridazine, and thiothixene. Clozapine may be considered in refractory cases (Schneider et al. 2015).
Antipsychotic treatment in youth requires ongoing monitoring for side effects. Both first- and second-generation antipsychotics can produce extrapyramidal symptoms, including dystonia, akathisia, tardive dyskinesia, and neuroleptic malignant syndrome. The second-generation agents are more strongly associated with weight gain and adverse effects on glucose and lipid metabolism. Monitoring should be accompanied by education on diet, exercise, and sleep.
Psychosocial interventions include cognitive-behavioral therapy to address deficits in thought processes, skills training for improved role functioning and self-efficacy, and family-based therapies. There is growing evidence for cognitive remediation in improving verbal memory and executive function in youth with schizophrenia (Armando et al. 2015). Family education and support are crucial to help families with the complex emotional, social, and financial burden of a child with schizophrenia, and to help promote a stable environment for the child. The NIMH RAISE project for first-episode psychosis has demonstrated the superiority of an integrated approach, including supportive education and employment, in improving length of time in treatment, quality of life, participation in work and school, and symptoms (Kane et al. 2016).
American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, 5th Edition. Arlington, VA, American Psychiatric Association, 2013
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