Neuropsychological attention deficits in tuberous sclerosis complex (TSC).

Tuberous sclerosis complex (TSC) (OMIM191100) is a genetic disorder with multi-system involvement including neurodevelopmental manifestations. There is great interest in understanding the pathogenetic mechanisms underlying these neurobehavioral and neurocognitive manifestations. However, there are still significant gaps in knowledge about the exact neuropsychiatric phenotypes observed in TSC. Here we report on the first systematic evaluation of neuropsychological attentional skills in a population-derived sample of children and adolescents with TSC. The study showed that, even when age, gender, IQ, and intra-familial clustering were controlled for, the TSC group had significantly lower scores than their unaffected siblings on a range of neuropsychological attentional tasks, and that they had significantly more neuropsychological attention deficits. Eighteen of the 20 children (90%) showed deficits on one or more attentional tasks, with dual task performance most consistently impaired (85%) and visual selective attention a relative strength. Active seizures and anti-epilepsy medication did not influence attentional profiles. Furthermore, parent rating of attention-related behaviors were not able to identify children at risk of neuropsychological deficits. The findings suggest that clinical neuropsychological evaluation of attentional skills should be performed in children and adolescents with TSC even when they have normal global intellectual abilities, no seizures, and no disruptive behaviors. Results suggest that the mechanisms underlying these deficits may include contributions from structural, seizure-related and molecular factors

The Sussex-Waterloo Scale of hypnotisability (SWASH): measuring capacity or altering conscious experience

The ability to respond to hypnotic suggestibility (hypnotisability) is a stable trait which can be measured in a standardised procedure consisting of a hypnotic induction and a series of hypnotic suggestions. The SWASH is a 10-item adaptation of an established scale, the Waterloo-Stanford Group C Scale of Hypnotic Suggestibility (WSGC). Development of the SWASH was motivated by three distinct aims: to reduce required screening time, to provide an induction which more accurately reflects current theoretical understanding and to supplement the objective scoring with experiential scoring. Screening time was reduced by shortening the induction, removing two suggestions which may cause distress (dream and age regression) and by modifications which allow administration in lecture theatres, so that more participants can be screened simultaneously. Theoretical issues were addressed by removing references to sleep, absorption and eye fixation and closure. Data from 418 participants at the University of Sussex and the Lancaster University are presented, along with data from 66 participants who completed a re-test screening. The subjective and objective scales were highly correlated. The subjective scale showed good reliability and objective scale reliability was comparable to the WSGC. The addition of subjective scale responses to the post-hypnotic suggestion (PHS) item suggested a high probability that responses to PHS are inflated in WSGC screening. The SWASH is an effective measure of hypnotisability, which reflects changes in conscious experience and presents practical and theoretical advantages over existing scales

The First Case of Intraperitoneal Bronchogenic Cyst in Korea Mimicking a Gallbladder Tumor

We present a case of an intraperitoneal bronchogenic cyst located at inferior surface of the liver, next to the gallbladder which clinically mimicked a gallbladder tumor. This is the first case reported in Korea, and we offer reviews of the related literatures. A 48-yr-old woman was admitted to our hospital because of intermittent abdominal pain in right upper quadrant. Computed tomography showed a large mass alongside the gallbladder. During laparotomy, the mass showed an ovoid cystic nature, which was attached to the normal gallbladder and liver bed. Cyst excision with cholecystectomy was performed, and histopathological examination revealed a bronchogenic cyst. Most bronchogenic cysts have a benign nature, but malignant changes have also been reported. Therefore, if a cystic tumor in the abdomen is suspected during preoperative diagnosis, a bronchogenic cyst should be considered in the differential diagnosis

Characterizing the microstructural basis of “unidentified bright objects” in neurofibromatosis type 1:A combined in vivo multicomponent T2 relaxation and multi-shell diffusion MRI analysis

AbstractIntroductionThe histopathological basis of “unidentified bright objects” (UBOs) (hyperintense regions seen on T2-weighted magnetic resonance (MR) brain scans in neurofibromatosis-1 (NF1)) remains unclear. New in vivo MRI-based techniques (multi-exponential T2 relaxation (MET2) and diffusion MR imaging (dMRI)) provide measures relating to microstructural change. We combined these methods and present previously unreported data on in vivo UBO microstructure in NF1.Methods3-Tesla dMRI data were acquired on 17 NF1 patients, covering 30 white matter UBOs. Diffusion tensor, kurtosis and neurite orientation and dispersion density imaging parameters were calculated within UBO sites and in contralateral normal appearing white matter (cNAWM). Analysis of MET2 parameters was performed on 24 UBO–cNAWM pairs.ResultsNo significant alterations in the myelin water fraction and intra- and extracellular (IE) water fraction were found. Mean T2 time of IE water was significantly higher in UBOs. UBOs furthermore showed increased axial, radial and mean diffusivity, and decreased fractional anisotropy, mean kurtosis and neurite density index compared to cNAWM. Neurite orientation dispersion and isotropic fluid fraction were unaltered.ConclusionOur results suggest that demyelination and axonal degeneration are unlikely to be present in UBOs, which appear to be mainly caused by a shift towards a higher T2-value of the intra- and extracellular water pool. This may arise from altered microstructural compartmentalization, and an increase in ‘extracellular-like’, intracellular water, possibly due to intramyelinic edema. These findings confirm the added value of combining dMRI and MET2 to characterize the microstructural basis of T2 hyperintensities in vivo

Trait phenomenological control predicts experience of mirror synaesthesia and the rubber hand illusion

In hypnotic responding, expectancies arising from imaginative suggestion drive striking experiential changes (e.g., hallucinations) — which are experienced as involuntary — according to a normally distributed and stable trait ability (hypnotisability). Such experiences can be triggered by implicit suggestion and occur outside the hypnotic context. In large sample studies (of 156, 404 and 353 participants), we report substantial relationships between hypnotisability and experimental measures of experiential change in mirror-sensory synaesthesia and the rubber hand illusion comparable to relationships between hypnotisability and individual hypnosis scale items. The control of phenomenology to meet expectancies arising from perceived task requirements can account for experiential change in psychological experiments

Fact or Factitious? A Psychobiological Study of Authentic and Simulated Dissociative Identity States

BACKGROUND: Dissociative identity disorder (DID) is a disputed psychiatric disorder. Research findings and clinical observations suggest that DID involves an authentic mental disorder related to factors such as traumatization and disrupted attachment. A competing view indicates that DID is due to fantasy proneness, suggestibility, suggestion, and role-playing. Here we examine whether dissociative identity state-dependent psychobiological features in DID can be induced in high or low fantasy prone individuals by instructed and motivated role-playing, and suggestion. METHODOLOGY/PRINCIPAL FINDINGS: DID patients, high fantasy prone and low fantasy prone controls were studied in two different types of identity states (neutral and trauma-related) in an autobiographical memory script-driven (neutral or trauma-related) imagery paradigm. The controls were instructed to enact the two DID identity states. Twenty-nine subjects participated in the study: 11 patients with DID, 10 high fantasy prone DID simulating controls, and 8 low fantasy prone DID simulating controls. Autonomic and subjective reactions were obtained. Differences in psychophysiological and neural activation patterns were found between the DID patients and both high and low fantasy prone controls. That is, the identity states in DID were not convincingly enacted by DID simulating controls. Thus, important differences regarding regional cerebral bloodflow and psychophysiological responses for different types of identity states in patients with DID were upheld after controlling for DID simulation. CONCLUSIONS/SIGNIFICANCE: The findings are at odds with the idea that differences among different types of dissociative identity states in DID can be explained by high fantasy proneness, motivated role-enactment, and suggestion. They indicate that DID does not have a sociocultural (e.g., iatrogenic) origin