Background Cerebral palsy is a heterogeneous group of neurodevelopmental brain disorders resulting in motor and posture impairments often associated with cognitive, sensorial, and behavioural disturbances. Hypoxic–ischaemic injury, long considered the most frequent causative factor, accounts for fewer than 10% of cases, whereas a growing body of evidence suggests that diverse genetic abnormalities likely play a major role. Methods and results This report describes an autosomal recessive form of spastic tetraplegic cerebral palsy with profound intellectual disability, microcephaly, epilepsy and white matter loss in a consanguineous family resulting from a homozygous deletion involving AP4E1, one of the four subunits of the adaptor protein complex-4 (AP-4), identified by chromosomal microarray analysis. Conclusion These findings, along with previous reports of human and mouse mutations in other members of the complex, indicate that disruption of any one of the four subunits of AP-4 causes dysfunction of the entire complex, leading to a distinct ‘AP-4 deficiency syndrome’.

Meaningful living is a central focus of several humanistic theories and therapies. Measurement of life meaning meets many obstacles, including pragmatic concerns, such as measuring subjective experiences, and theoretical objections often offered by humanistic psychologists. The purpose of this article is to summarize empirical efforts related to logotherapy, a humanistic-existential paradigm, to illustrate the utility of assessment within the larger context of humanistic psychology. An overview of five logotherapeutic measures of meaning is provided. These measures include the Purpose in Life test (PIL), the Life Purpose Questionnaire (LPQ), the Seeking of Noetic Goals test (SONG), the Meaning in Suffering Test (MIST), and the Life Attitude Profile Revised (LAP-R). Directions for use of such measures in future research are also offered.

Logotherapy, Meaning, Purpose in life, PIL, Confirmatory factor analysis,

Logotherapy, developed by Viktor Frankl, posits that when one lacks meaning in life, boredom can result. Thus, the two constructs should be inversely related. To examine this relationship, 279 students (M = 19.8 yr., SD = 2.6; 179 women, 98 men) from a university in the southern United States were administered the Purpose of Life test and the Boredom Proneness Scale. As expected, a statistically significant negative correlation was found between the scores on the two scales (r = -.71). Directions for research are offered.

Researchers have extensively investigated the factor structure of the Boredom Proneness Scale (BPS; R. Farmer & N. D. Sundberg, 1986) using exploratory factor analyses (EFAs), with inconsistent methodology and results. Thus, the purpose of the present study was to perform confirmatory factor analyses on the BPS to better determine the nature and extent of its underlying factor structure. Participants were 279 undergraduate students majoring in psychology at a university in the southern United States. None of the tested models derived from earlier studies was a reasonable fit to the present data; therefore, the present authors performed an EFA. Although 2 factors were revealed, these results, coupled with markedly different results from previous factor analytic studies of the BPS, suggest it is unlikely that the present factor structure is replicable. The BPS does not appear to have a replicable factor structure.