[Alexander disease as an explanation for long-term unexplained psychiatric symptoms in a young girl]. / De ziekte van Alexander als verklaring voor langdurig onbegrepen psychiatrische klachten bij een jong meisje.

Alexander disease is a rare genetic disorder, usually characterized by leukodystrophy, causing progressive degeneration of white matter in the central nervous system. We describe a case of a 7-year-old girl with long term medically unexplained symptoms and suspicion of a psychiatric disorder, who was diagnosed with Alexander disease. Building on this case, a literature search was conducted for psychiatric symptoms..

Adolescents at ultra-high risk for psychosis: long-term outcome of individuals who recover from their at-risk state.

Studies of individuals at ultra-high risk (UHR) for psychosis have mostly reported on long-term outcome of those individuals who develop psychosis compared to those who do not. However, these studies show that a large number of UHR individuals no longer meet criteria for UHR at follow-up. Therefore, this study aimed to investigate functioning at 6-year follow-up in remitted individuals, and to explore the course of their clinical symptoms. Forty-four UHR adolescents completed extensive clinical assessments at baseline and participated in long-term follow-up approximately six years later. UHR adolescents who had either converted to psychosis or who still met UHR criteria (n=26) at follow-up were compared to individuals who had remitted from their UHR status (n=18) on clinical and psychosocial variables. Results show that more than 40% of UHR individuals had fully remitted from their UHR status. At six-year follow-up, remitted individuals had improved clinically on most symptoms. The course of their symptoms showed that the most substantial reduction in positive symptoms occurred within the first two years, while improvements in general, mood and anxiety symptoms occurred at a later stage. Baseline socio-demographic characteristics and clinical symptoms did not distinguish between remitters and non-remitters. Although remitters no longer met criteria for UHR, they did meet diagnostic criteria for a wide range of psychiatric disorders. Our findings suggest that, when related to long-term outcome, UHR criteria capture non-specific psychotic symptoms rather than risk for psychosis per se and relate more to general psychopathology.

Neuroendocrine markers of high risk for psychosis: salivary testosterone in adolescent boys with prodromal symptoms.

BACKGROUND: The peak in age of onset of psychotic disorders such as schizophrenia during puberty and early adulthood suggests a relationship between the expression of psychopathology and the changes in the brain and body that take place during this dynamic maturational period, including a dramatic increase in circulating oestrogens and androgens. This study examined levels of salivary testosterone and oestradiol in adolescents with prepsychotic, prodromal symptoms, as this may mediate risk for psychosis by having an impact on brain development. METHOD: In 21 male adolescents with prodromal symptoms and 21 male non-clinical controls levels of testosterone and oestradiol were measured in saliva. Tanner pubertal stage and prodromal symptoms were also assessed. RESULTS: Levels of testosterone were significantly lower in adolescents with prodromal symptoms as compared with non-clinical controls. No group differences in oestradiol were found. In the total sample, level of testosterone was significantly correlated with age and Tanner pubertal stage. CONCLUSIONS: Our observations are in line with current hypotheses stressing the role of neuroendocrine factors during adolescence in the expression of psychotic symptoms. From a developmental perspective, susceptibility to psychotic disorders increases during adolescence. Our data suggest that testosterone might, in part, mediate this increased vulnerability. Further research is needed to assess the mediating, neural, mechanisms through which testosterone may have an impact on the development of psychotic symptoms. In the search for early risk markers for psychosis, studying neuroendocrine factors might increase our understanding of 'at-risk' developmental pathways.

Misattribution of facial expressions of emotion in adolescents at increased risk of psychosis: the role of inhibitory control.

BACKGROUND: By studying behavior, cognitive abilities and brain functioning in adolescents at high risk for psychosis, we can gain an insight into the vulnerability markers or protective factors in the development of psychotic symptoms. Although many high-risk studies have focused on impairments in neurocognitive functions, such as memory and attention, very few studies have investigated problems in processing social cues such as facial expressions as a possible vulnerability marker for psychosis. METHOD: Thirty-six adolescents at ultra-high risk (UHR) for psychosis and 21 non-clinical controls completed a face recognition test, a facial affect labeling test and an inhibitory control test. Schizotypal traits and schizophrenia symptoms were assessed using a schizotypy questionnaire and the Positive and Negative Syndrome Scale (PANSS). RESULTS: The UHR group showed impairments in labeling facial expressions of others, in addition to a spared ability to recognize facial identity. More specifically, the UHR group made more errors in labeling neutral expressions compared to the controls, and an analysis of error types indicated that neutral faces were misattributed as being angry. The degree of misattribution of neutral-as-angry faces correlated significantly with reduced inhibitory control. CONCLUSIONS: Our findings suggest that misattributing social cues might contribute to vulnerability for psychosis. This social cognitive deficit may be related to problems in inhibitory control, which potentially plays an important role in the selection of appropriate social meaning. These findings may have relevance for understanding the mechanisms underlying prodromal social dysfunction, which should be targeted in future remediation interventions.

Pathways to psychosis: a comparison of the pervasive developmental disorder subtype Multiple Complex Developmental Disorder and the "At Risk Mental State".

BACKGROUND: The comparison of high-risk populations with different developmental pathways to psychosis may lend more insight into the heterogeneity of the manifestation of the psychotic syndrome, and possible differing etiological pathways. AIM: To compare high-risk traits and symptoms in two populations at risk for psychosis, i.e. (1) help-seeking adolescents presenting with prodromal symptoms meeting the criteria for At Risk Mental State (ARMS), and (2) adolescents with Multiple Complex Developmental Disorder (MCDD), a PDD-NOS subtype characterized by severe, early childhood-onset deficits in affect regulation, anxieties, disturbed social relationships, and thought disorder. METHOD: 80 ARMS- and 32 MCDD-adolescents (12-18 years) were compared on prodromal symptoms (Structured Interview of Prodromal Symptoms, and Bonn Scale for the Assessment of Basic Symptoms-Prediction list), and autism traits (Social Communication Questionnaire). In addition, both high-risk groups were compared with 82 healthy controls on schizotypal traits (Schizotypal Personality Questionnaire-Revised). RESULTS: Although the high-risk groups clearly differed in early developmental and treatment histories as well as autism traits, they did not differ with regard to schizotypal traits and basic symptoms, as well as disorganized and general prodromal symptoms. There were, however, group differences in positive and negative prodromal symptoms. Interestingly, 78% of the adolescents with MCDD met criteria for ARMS. CONCLUSION: These findings suggest that children diagnosed with MCDD are at high risk for developing psychosis later in life, and support the notion that there are different developmental pathways to psychosis. Follow-up research is needed to compare the rates of transition to psychosis in both high-risk groups.

Psychiatric disorders and MND in non-handicapped preterm children. Prevalence and stability from school age into adolescence.

In preterm children (N = 66) without major physical and/or mental handicaps the prevalence of psychiatric disorders and minor neurological dysfunction (MND) was assessed at school age (8-10 years). In adolescence (15-17 years) 43 children were reassessed. The study sample was drawn from a cohort of non-handicapped preterm children (N = 218) hospitalised in a Neonatal Intensive Care Unit because of serious neonatal complications. The findings in the preterm group were compared with two control groups (N = 20 and N = 20) matched for age and sex ratio. The association between psychiatric disorders on the one hand and group status (preterm versus control), MND, IQ and family adversity on the other was explored. At both ages the preterm children exhibited more psychiatric disorders and MND than controls. The very preterm and/or very low birth weight children contributed to the differential psychopathological findings between the preterm and control groups. Besides preterm birth, the prevalence of psychiatric disorders was positively associated with MND and negatively associated with VIQ and family adversity. In the preterm group there was a shift from school age into adolescence into a predominance of anxious and depressive disorders. No significant changes with age were found with respect to the prevalence of MND and psychiatric disorders. Thus, very preterm and/or very low birth weight children are at increased risk of persistent psychiatric disorders, especially anxious and depressive disorders. In preterm children the development of psychopathology seems to be mediated by MND, decreased verbal abilities and family adversity.

Long-term behavioral sequelae of prematurity.

OBJECTIVE: Longitudinal evaluation of the competence and the prevalence of behavior problems in preterm children with serious neonatal complications. METHOD: Prospective follow-up of nonhandicapped preterm children who had been hospitalized in a neonatal intensive care unit (n = 177). The follow-up extended from early school age to early adolescence and was conducted with the help of the Child Behavior Checklist (CBCL) (parent form). RESULTS: The preterm children had lower scores on the Social and School Competence scales than did controls (n = 276). They also were more likely to attend special schools than were children in the general population. With regard to behavior problems, the preterm children had more social problems. The very preterm children and the children who were small for gestational age (SGA) were the ones who contributed to the significant findings. The children who were appropriate for gestational age did not differ from controls. No differential changes in CBCL ratings were found between the preterm and control children. The stability with regard to internalizing problems, attention problems, and social problems was high among the very preterm children and SGA children. CONCLUSION: Very preterm and preterm SGA children are at increased risk of problems in social functioning and functioning at school. These problems persist with age.