We tested the hypothesis that extent of severe hypometabolism measured by glucose PET has a U-shaped (non-linear) relationship to IQ in children with unilateral Sturge-Weber Syndrome. Thirty-five consecutive children (age range, 30 to 153 months) with SWS and unilateral brain involvement were enrolled in the study. Participants underwent cognitive assessment and interictal glucose PET scans. Regression analyses tested whether a quadratic model best accounted for the relationship between extent of severe cortical hypometabolism and IQ, controlling for seizure variables. A significant quadratic relationship was found between IQ and extent of severe (but not total) hypometabolism. Seizure variables also contributed significant variance to cognitive functions. Results suggest that intermediate size of severe hemispheric hypometabolism is associated with the worst, small or absent lesions the best, cognitive outcomes. Children with very large extent of the hemisphere severely affected are likely to have relatively preserved cognitive function.

Angelman Syndrome is a genetic disorder characterized by pervasive developmental disability with failure to develop speech. We examined the basis for severe language delay in Angelman Syndrome patients using diffusion tensor imaging. Magnetic Resonance Imaging/diffusion tensor imaging was performed in seven genetically confirmed Angelman Syndrome children (age:70±26 months, five males) and four age-matched controls to investigate the microstructural integrity of arcuate fasciculus and other major association tracts. Six of seven Angelman Syndrome children had unidentifiable left arcuate fasciculus while all controls had identifiable arcuate fasciculus. The right arcuate fasciculus was absent in six of seven Angelman Syndrome children and one of four controls. Diffusion tensor imaging color map suggested aberrant morphology of the arcuate fasciculus region. Other association tracts, including uncinate fasciculus, inferior-fronto-occipital fasciculus, inferior-longitudinal fasciculus, and corticospinal tract, were identifiable but showed decreased fractional anisotropy in Angelman Syndrome children. Increased apparent diffusion coefficient was seen in all tracts except uncinate fasciculus when compared to controls. Angelman Syndrome patients have global impairment of white matter integrity in association tracts, particularly, the arcuate fasciculus which shows severe morphological changes. This could be due to a potential problem with axon guidance during brain development possibly due to loss of UBE3A gene expression.

Background-Sturge-Weber syndrome (SWS) with unilateral hemispheric involvement is a clinical model of early-onset, chronic, often progressive hemispheric injury, resulting in variable neuro-cognitive impairment.Aims-To evaluate if abnormal diffusion and metabolism of the thalamus, a central relay station with extensive cortical connections, may serve as a simple imaging marker of neuro-cognitive dysfunction in SWS.Methods-We obtained both diffusion tensor imaging and FDG PET in 20 children (11 girls; age range: 3 years to 12.4 years) with unilateral SWS. Diffusion parameters as well as FDG uptake were measured in thalami, compared to normal control values, and correlated with the extent of cortical hypometabolism, deep venous abnormalities and cognitive (IQ) as well as fine motor functions.Results-Children with SWS had significantly higher thalamic glucose metabolic asymmetry than controls (p=0.001). Thalamic metabolic asymmetries correlated positively with the asymmetry of thalamic diffusivity (p=0.001) and also with the extent of cortical hypometabolism (p<0.001). Severe thalamic asymmetries of glucose metabolism and diffusion were strong predictors of low IQ (metabolism: p=0.002; diffusivity: p=0.01), even after controlling for age and extent of cortical glucose hypometabolism in children with left hemispheric involvement. Ipsilateral thalamic glucose hypometabolism was also associated with impairment of fine motor functions (p=0.002).Conclusions-Both diffusion and glucose metabolic abnormalities of the thalamus are closely related to cognitive functions, independent of age and cortical metabolic abnormalities, in children

Carefully listening to the patient is of paramount importance for psychoanalysis and psychoanalytic psychotherapy. The present study explored whether patient vocalization as well as the gender of the analyst play significant roles in clinical listening. Fifty-one psychoanalysts and psychoanalytic therapists were randomly assigned to listen to one of two dramatized psychoanalytic sessions. The content of the sessions was the same for both versions, but the sessions were dramatized differently. Some differences emerged between the versions, especially on ratings of reality testing, impulse control, pressured speech, patient was confusing, and awareness of imagery. Furthermore, differences emerged between male and female analysts in terms of ratings of intervention strategies and countertransference reactions to the patient material. Session version and gender affect different ratings. Implications of the findings are discussed as is the utility of using more ecologically valid material in conducting empirical research into clinical judgment.