Summary

Patients with Urbach±Wiethe disease constitute a unique nature experiment as more than half have bilat-erally symmetrical damage in the amygdaloid region. Ten such patients were studied neuropsychologically and, nine of them, neuroradiologically with static (CT) and functional imaging techniques [single-photon emis-sion computed tomography (SPECT) and PET]. Their principal bilateral amygdala damage was con®rmed. Neuropsychologically, the patients showed cognitively little deviation from normal subjects, while they dif-fered emotionally. This was evident in their judgement of all emotions in facial expressions, in an odour±®gure association test as well as in remembering negative and positive pictures. This suggests that the human amyg-dala in¯uences both negative and positive emotional processing