Transcutaneous vagus nerve stimulation (t-VNS): A novel effective treatment for temper outbursts in adults with Prader-Willi Syndrome indicated by results from a non-blind study.

Temper outbursts are a severe problem for people with Prader-Willi Syndrome (PWS). Previous reports indicate that vagus nerve stimulation (VNS) may reduce maladaptive behaviour in neurodevelopmental disorders, including PWS. We systematically investigated the effectiveness of transcutaneous VNS (t-VNS) in PWS. Using a non-blind single case repeat measures modified ABA design, with participants as their own controls, t-VNS was evaluated in five individuals with PWS [three males; age 22-41 (M = 26.8)]. After a baseline phase, participants received four-hours of t-VNS daily for 12 months, followed by one month of daily t-VNS for two-hours. The primary outcome measure was the mean number of behavioural outbursts per day. Secondary outcomes included findings from behavioural questionnaires and both qualitative and goal attainment interviews. Four of the five participants who completed the study exhibited a statistically significant reduction in number and severity of temper outbursts after approximately nine months of daily four-hour t-VNS. Subsequent two-hour daily t-VNS was associated with increased outbursts for all participants, two reaching significance. Questionnaire and interview data supported these findings, the latter indicating potential mechanisms of action. No serious safety issues were reported. t-VNS is an effective, novel and safe intervention for chronic temper outbursts in PWS. We propose these changes are mediated through vagal projections and their effects both centrally and on the functioning of the parasympathetic nervous system. These findings challenge our present biopsychosocial understanding of such behaviours suggesting that there is a single major mechanism that is modifiable using t-VNS. This intervention is potentially generalizable across other clinical groups. Future research should address the lack of a sham condition in this study along with the prevalence of high drop out rates, and the potential effects of different stimulation intensities, frequencies and pulse widths.

Understanding self-reported difficulties in decision-making by people with autism spectrum disorders.

Autobiographical accounts and a limited research literature suggest that adults with autism spectrum disorders can experience difficulties with decision-making. We examined whether some of the difficulties they describe correspond to quantifiable differences in decision-making when compared to adults in the general population. The participants (38 intellectually able adults with autism spectrum disorders and 40 neurotypical adults) were assessed on three tasks of decision-making (Iowa Gambling Task, Cambridge Gamble Task and Information Sampling Task), which quantified, respectively, decision-making performance and relative attention to negative and positive outcomes, speed and flexibility, and information sampling. As a caution, all analyses were repeated with a subset of participants ( nASD = 29 and nneurotypical = 39) who were not taking antidepressant or anxiolytic medication. Compared to the neurotypical participants, participants with autism spectrum disorders demonstrated slower decision-making on the Cambridge Gamble Task, and superior performance on the Iowa Gambling Task. When those taking the medications were excluded, participants with autism spectrum disorders also sampled more information. There were no other differences between the groups. These processing tendencies may contribute to the difficulties self-reported in some contexts; however, the results also highlight strengths in autism spectrum disorders, such as a more logical approach to, and care in, decision-making. The findings lead to recommendations for how adults with autism spectrum disorders may be better supported with decision-making.

Making sense of non-epileptic seizures.

BACKGROUND: Non-epileptic seizures (NES) are a chronic condition that is frequently misdiagnosed. Limited awareness of the condition may contribute to mismanagement and poor outcome. METHODS: Medline and PsycLit review of clinical and laboratory studies. RESULTS: The concept of NES is clarified, common clinical presentations are reviewed and the differential diagnoses considered. A general overview of possible mechanisms and pathological findings is presented. Finally, epidemiological, prognostic, and treatment issues are described. CONCLUSIONS: Clinicians increased awareness of NES, together with new diagnostic techniques will improve diagnosis and outcome of this condition. Further research is needed into the pathophysiology, etiology and maintenance mechanisms of non-epileptic seizures.

A comparative study of mismatch negativity (MMN) in epilepsy and non-epileptic seizures.

This study investigated mismatch negativity (MMN) differences between subjects with non-epileptic seizures (NES), subjects with epilepsy, and healthy controls. Event-related potentials (ERPs) were obtained from 14 patients with NES, 15 patients with epilepsy and 16 healthy control subjects. A conventional MMN procedure was used with a random sequence of 12% deviant tones (922 Hz) and 88% standard tones (1000 Hz). Subjects were instructed to ignore the tones delivered through headphones whilst reading a book. Significant differences in distribution of the mismatch negativity (MMN) in patients with NES compared to controls were obtained (F3, p

Prevalence, associations, and predictors of apathy in adult survivors of infantile (<5 years of age) posterior fossa brain tumors.

BACKGROUND: Apathy is associated with pervasive and disadvantageous effects on daily functioning. It has been observed transiently in some children after surgery for posterior fossa tumors. In this study, our objective was to examine prevalence, associations, and predictors of apathy in adult survivors of an infantile posterior fossa brain tumor (PFT). METHODS: One hundred seventeen adult survivors of a childhood PFT diagnosed before age 5 years and 60 of their siblings were assessed in a cross-sectional study a mean of 32 years (range, 18-53 years) after survivors' initial tumor diagnoses, using the Marin Apathy Evaluation Scale (AES), the Weschler Abbreviated Scale of Intelligence and the Composite International Diagnostic Interview for psychiatric disorders. RESULTS: Marin Apathy Evaluation Scale, the Weschler Abbreviated Scale of Intelligence reached or exceeded a criterion score for clinically significant apathy in 35% of survivors, compared with 18% in a sibling comparison group. In both siblings and survivors, apathy was associated with lower verbal and full-scale IQ and, among survivors, with having undergone partial rather than total tumor resection (independent of irradiation status). Apathy was not related to presence of concurrent International Classification of Diseases, 10(th) Revision, depression. Female sex was associated with late apathy after a PFT, with increased likelihood of women reaching the apathy criterion relative to men if they were survivors. CONCLUSIONS: Clinically significant and potentially treatable apathy occurs relatively commonly in adult survivors of an infantile childhood PFT, particularly women. Clinicians, including those managing posterior fossa pathology in very young children, should be aware of this association, and future research should clarify whether specific treatment-related variables are implicated in increasing this risk of apathy.

Atypical movement performance and sensory integration in Asperger's syndrome.

The aims of this study were to investigate whether individuals with AS have impaired motor abilities and sensorimotor processing and whether these impairments were age-related. Sensorimotor abilities were examined using the Movement Assessment Battery for Children-2, and the Sensory Integration Praxis Test. Fifty boys with AS aged 7-14 years old were compared with typically developing boys. Overall, children with AS showed significant impairment of movement performance as well as proprioceptive and vestibular processing. There were no interaction effects of age and clinical group on level of performance deficit in any of the modalities tested. Increasing our understanding of motor and sensory impairment in AS could have treatment implications for those supporting individuals with AS.

Evidence supporting a cognitive model of depression in Parkinson's disease.

The objective of this study was to explore the possibility that depression in Parkinson disease (PD) arises from an illness-related vulnerability to negative emotional stimuli. The Color-Word and Emotional Stroop tasks were performed by 27 nondepressed patients with PD and 27 healthy volunteers. Patients with PD showed greater interference to the sad words of the Emotional Stroop than did control patients, despite similar performances to the controls on the neutral and Color-Word Stroop tasks. Nondepressed patients with PD may have attentional mechanisms that are vulnerable to negative stimuli. This vulnerability might arise out of a common involvement of anterior cingulate regions in both selective attention and mood regulation, and could in turn predispose to cognitive distortions that may lead to depression.