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Brain Advance Access originally published online on August 22, 2003
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Brain, Vol. 126, No. 12, 2627-2637, December 2003
© 2003 Guarantors of Brain
doi: 10.1093/brain/awg271

Amygdala, affect and cognition: evidence from 10 patients with Urbach–Wiethe disease

Michaela Siebert1, Hans J. Markowitsch1 and Peter Bartel2

1 Physiological Psychology, University of Bielefeld, D-33501 Bielefeld, Germany 2 Department of Neurophysiology, University of Pretoria, Pretoria, Republic of South Africa

Correspondence to: Hans J. Markowitsch, Physiological Psychology, University of Bielefeld, PO Box 10 01 31, D-33501 Bielefeld, Germany E-mail: hjmarkowitsch{at}uni-bielefeld.de

Patients with Urbach–Wiethe disease constitute a unique nature experiment as more than half have bilaterally 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 emission computed tomography (SPECT) and PET]. Their principal bilateral amygdala damage was confirmed. Neuropsychologically, the patients showed cognitively little deviation from normal subjects, while they differed emotionally. This was evident in their judgement of all emotions in facial expressions, in an odour–figure association test as well as in remembering negative and positive pictures. This suggests that the human amygdala influences both negative and positive emotional processing.


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