Faculty Bibliography

Alzheimer's disease (AD), frontotemporal dementia (FTD) and semantic dementia (SD) are neurodegenerative diseases that differ in their socioemotional presentations. Mutual gaze (i.e. when two individuals make eye contact) is a building block of social behavior that may be differentially affected by these diseases. We studied 13 AD patients, 11 FTD patients, 9 SD patients and 22 normal controls as they engaged in conversations with partners about relationship conflicts. Physiological reactivity was monitored during the conversations and trained raters coded mutual gaze from videotaped recordings. Results indicated that mutual gaze was preserved in AD couples. Mutual gaze was diminished in FTD couples while SD couples showed evidence of greater mutual gaze. SD couples also showed lower physiological reactivity compared to controls. Across patient groups, reduced mutual gaze was associated with greater behavioral disturbance as measured by the Neuropsychiatric Inventory, especially on the disinhibition and apathy subscales. These results point to subtle differences between the three types of dementia in the social realm that help to illuminate the nature of the disease process and could aid in differential diagnosis.

OBJECTIVES: Evaluate the benefit of intrathecal baclofen (ITB) therapy on function, quality of life, and progression in patients with multiple system atrophy (MSA). METHODS: We report on three MSA patients at different stages treated with ITB therapy. MSA patients were staged using Watanabe et al. ADL milestones for disease progression and by scales for tone (modified Ashworth scale) ambulation (Hauser ambulation index) and disability (expanded disability status scale) RESULTS: All three patients had an improvement in the modified Ashworth scale and none had progression in their disability or ambulatory outcomes and did not progress as predicted by Watanabe et al. CONCLUSIONS: Our results suggest that ITB can maintain (or improve function) and maintain quality of life in patients with MSA. ITB is currently not indicated for patients with MSA but should be studied further for the quality of life benefits and delay in disease progression it potentially provides.

Verbal dichotic listening performance was examined in 42 right-handed men and women with DSM-IV-defined schizotypal personality disorder (SPD) and 68 right-handed controls. As expected, both male and female control groups showed a right ear advantage on a verbal dichotic listening task. Although SPD subjects in general had lower accuracy scores than comparison subjects, only male SPD subjects showed an abnormal left ear advantage that was specifically due to deficient right ear performance. The results suggest that left hemisphere temporal lobe structures may be particularly involved in male, but not female, SPD.

Behavioral regulation is a hallmark feature of executive functioning (EF). The present study investigated whether commonly used neuropsychological test measures of EF (i.e., working memory, Stroop, trail making, and verbal fluency) were related to ability to downregulate emotion both spontaneously and when instructed to suppress emotional expressions. To ensure a wide range of EF, 24 frontotemporal lobar degeneration patients, 7 Alzheimer's patients, and 17 neurologically normal controls participated. Participants were exposed to an acoustic startle stimulus (single aversive noise burst) under three conditions: (1) unwarned, (2) warned with no instructions (to measure spontaneous emotion downregulation), and (3) warned with instructions to suppress (to measure instructed emotion downregulation). Results indicated that higher verbal fluency scores were related to greater emotion regulation (operationalized as reduction in body movement and emotional facial behavior when warned of the impending startle) in both regulation conditions. No relationships were found between emotion regulation in these conditions and the other EF measures. We conclude that, of four commonly used measures of EF, verbal fluency best indexes the complex processes of monitoring, evaluation, and control necessary for successful emotion regulation, both spontaneously and following instructions to suppress.

A method of fabricating replica figured x-ray optics with integral multilayer coatings is presented. With the intact electroforming multilayer process (IEMP) technique, we sputter multilayers onto a reusable superpolished mandrel, electroform nickel over the multilayers, and remove the multilayer-coated nickel shell intact from the mandrel. This approach offers advantages over more traditional, original, and segmented-replica fabrication techniques, including low cost; compatibility with a wide range of mirror designs, diameters, and focal lengths; simple integration with multilayer sputtering processes; and the ability to produce complete shells of revolution. The fabrication of W/Si multilayer-coated 10-cm-diameter conical x-ray mirrors is described, as are reflectivity measurements at 10 and 30 keV. The measured reflectivity of the IEMP multilayers at the 10-keV primary Bragg peak was 17%. Measurements of multiple points on the cone showed multilayer uniformity to within a few percent around the mirror.