The neuropsychological and clinical histories of three male siblings affected by pyridoxine‐dependent seizures with known homozygous antiquitin mutations are presented. Neuropsychological evaluation is reported from when the siblings were 11, 9, and 7 years of age. Two of the siblings had received early pyridoxine treatment (antenatal, 2–4wks into pregnancy) and one had received late treatment (2mo postnatal). However, there was no differential effect on cognitive outcome, with all three siblings having moderate to severe learning disability. Unlike previously reported cases that received early postnatal treatment, none of the siblings had relatively preserved non‐verbal cognitive skills. Equally, their intellectual performance over time did not increase above the 1st centile despite high maintenance doses of vitamin B6 (range 16–26mg/kg/d), and mild sensory neuropathy was reported on nerve conduction studies. The findings in these siblings challenge assumptions that early and high dose pyridoxine treatment can benefit cognition in this population and suggest routine electromyography monitoring may be beneficial.

This investigation tested the hypotheses that individuals disposed towards hallucination are deficient in the meta-cognitive skills of reality discrimination and reality monitoring, and that there is a strong correlation between the measures of these skills. Normal subjects scoring high or low on a scale measuring predisposition towards hallucination were tested on an auditory signal detection task. High scorers on the scale were found to differ from low scorers on a measure of perceptual bias but not on a measure of sensitivity. The same groups of subjects were tested on a reality monitoring measure in which they were required to monitor two different types of word: words they were required to listen to, and words they were required to listen to on some trials and imagine on others. On a surprise frequency test it was found that high scorers did not differ from low scorers on their ability to accurately count the memories of words they had heard. It was also found that both high scorers and low scorers overestimated the number of times they had heard words when they had also been required to imagine these words on tests; however, high scorers made this error to a significantly greater extent. A strong correlation was found between reality discrimination and reality monitoring measures for high scorers, but no correlation was found for low scorers.

The short-form will be useful when pressure of time or client fatigue precludes use of a full-length WISC-IV. The accompanying computer programme scores and analyses an individual's performance on the short-form instantaneously and minimizes the chance of clerical error.

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Background: Paranoid delusions are associated with abnormal attributions and abnormal beliefs about the self. Some researchers have also reported an association between paranoid beliefs and abnormal attachment representations. Sampling and Methods: Perceptions of relationships with the family of origin were measured in 14 currently ill paranoid patients, 9 remitted paranoid patients and 15 healthy controls, using two methods: the Parental Bonding Instrument (PBI), and the Relationship with Family of Origin Scale (REFAMOS), an interview-based assessment. Results and Conclusions: On the PBI, both currently ill and remitted patients reported low parental care and overprotectiveness during childhood. Similar negative accounts of relationships with parents during the 16–20 years age period, and also at the time of assessment, were elicited from both the ill and remitted patients on the REFAMOS. These findings might reflect the influence of illness on patients’ perceptions of their parents. However, the fact that the remitted patients did not differ from the currently ill patients raises the possibility that dysfunctional relationships are a common feature of the history of paranoid patients, perhaps contributing to the development of paranoid thinking.