AM but not BG was impaired in figural learning and memory, as shown in the Complex Figure Test (Osterrieth, 1944) and the DCS (Weidlich & Lamberti, 2001). In behavioural experiments, BG was impaired in free verbal recognition of fearful faces, and in startle potentiation by threat-related scenes, and had a reduced Metformin order social network compared to control participants, while all these functions were intact
in AM (Becker et al., 2012). Further, both twins showed reduced anterograde and retrograde interference of emotional pictures on memory (Hurlemann et al., 2007). On the other hand, the aforementioned neuropsychological assessment (Talmi et al., 2010) revealed average intelligence (L-P-S Leistungsprüfsystem) (Horn, 1983) and intact verbal learning and memory (Rey Auditory Verbal Learning test) (Helmstedter, Lendt, & Lux, 1981) as well as executive
RAD001 ic50 function measured with the Trail Making Test (Reitan, 1955), Wisconsin Card Sorting Test (Kongs, Thompson, Iversion, & Heaton, 2000), Stroop test (Bäumler, 1985), and semantic fluency (Aschenbrenner et al., 2000). The twins show neither depression nor anxiety (Hamilton, 1959 and Hamilton, 1960). Further, both twins were unimpaired in rapid detection of negative-arousing words (Bach, Talmi, Hurlemann, Patin, & Dolan, 2011), forced-choice recognition of emotional expression in prosody (Bach, Hurlemann, & Dolan, 2013), and framing effects on economic gambles (Talmi et al., 2010). Given the amygdala damage in AM and BG, and the posited function of the amygdala in prioritising threat information, we hypothesised a reduced angry face advantage in the FITC task in AM and BG, compared to healthy individuals. The task followed a 3 (set size: 1/6/12 items) × 2 (target emotion: angry/happy) × 2
(target absent/present) factorial design with RT as dependent variable. Some previous studies have only analysed slopes of a serial search model. Here, because we did not know whether Urbach–Wiethe patients use a serial search strategy, we analyse both raw RTs and search slopes Rutecarpine as dependent variables. AM (previously also labelled patient 1) and BG (patient 2) (Becker et al., 2012), aged 35 years at the time of the present experiment, are monozygous twins with congenital Urbach–Wiethe syndrome due to a de novo mutation (Becker et al., 2012). The calcified volumes on high-resolution computer assisted tomography images included the whole basolateral amygdala and most other amygdala nuclei, only sparing anterior amygdaloid and ventral cortical amygdaloid parts at an anterior level, as well as lateral and medial parts of the central amygdaloid nucleus and the amygdalo-hippocampal area at posterior levels. Control participants were included if they were females between the age of 29 and 41 years, and the final sample comprised 16 healthy females with an age of 33.6 ± 3.4 years.