Fragile X Syndrome and Attentional Deficits

This study examined whether boys with Fragile X syndrome have a characteristic behavioural profile associated with the underlying genetic abnormality. Parents of 49 boys with Fragile X syndrome, 45 boys with Down's syndrome and 42 boys with intellectual disabilities of unknown aetiology were interviewed. Measures included the Childhood Behaviour Checklist, parent and teacher versions (CBCL), Parental Account of Childhood Symptoms (PACS) and Vineland social age and social quotient derived from the MRC Schedule of Handicaps, Behaviour and Skills (HBS).     

Knowledge and beliefs about tuberculosis among non-working women in Ravensmead, Cape Town

The results of a community-based survey on knowledge and beliefs about tuberculosis in non-working women are presented. The women in the sample showed a very good knowledge of the important aspects of tuberculosis: 90% were aware that it is a problem in their area; 97% knew that it affects the chest; 94% said that it could be fatal; 85% considered it to be infectious and 88% knew that the local clinic provided treatment. Their knowledge of symptoms was good overall but the study revealed misconceptions about the causes and transmission of tuberculosis; 16% indicated that they would not be keen to associate with people with tuberculosis owing to fear of infection. The design effect of cluster sampling was considered in the analysis. The highest design effects (i.e. the most clustering of responses) were found for responses to questions on the causes of tuberculosis and places where treatment could be obtained, possibly reflecting that these beliefs are influenced by neighbourhood contacts. Future tuberculosis education in this group needs to build on existing knowledge and awareness and should focus on changing attitudes such as misconceptions about transmission and the stigmatisation of the disease. Health workers face the challenge of changing behaviour in this community to ensure that people with symptoms present early for screening and that people diagnosed as having tuberculosis comply with treatment.     

Comparing the validity of clinician-generated diagnosis of conduct disorder to the diagnostic interview schedule for children.

Clinician diagnoses of conduct disorder (CD) were compared to the diagnoses of CD generated by a structured interview against an observed criterion. Participants were 534 youth from a large residential program in the Midwest for delinquent youth. Rates of in-program CD behaviors were gathered from staff observations of the youth over a 9-month time period. Youth diagnosed with CD by the Diagnostic Interview Schedule for Children (DISC) displayed significantly more CD behaviors in the first 6 months of treatment compared to both youth without an externalizing disorder and youth diagnosed with CD by a clinician. Youth diagnosed with CD by a clinician had rates of CD identical to youth without an externalizing disorder. Clinicians may have weighted contextual information more heavily, as this group was significantly more likely to have an arrest record. Results support the use of structured interviews and provide evidence that typical clinician diagnoses may lack adequate validity.     

Pathological laughter and crying in patients with multiple system atrophy-cerebellar type.

In the cerebellar type of multiple system atrophy (MSA-C), the burden of pathological changes involves the cerebellum and its associated brainstem structures in the basis pontis and the inferior olivary nucleus, and as a result, the clinical phenotype is dominated early on by the cerebellar dysfunction. We report our clinical and post mortem findings in a patient with MSA-C who exhibited pathological laughter in the absence of any congruent changes of mood. A review of the clinical notes of 27 other patients with MSA-C revealed a problem with pathological laughter, or crying, or both in 9 more patients. Our finding of about 36% occurrence suggests that the problem of dysregulation of emotional expression is more prevalent in MSA-C than the paucity of reports in the literature suggests. Our findings are consistent with the view that the cerebellum and its interconnected structures may be involved in the regulation of emotional expression.