Urbach-Wiethe disease in a young patient without apparent amygdala calcification
Markowitsch HJ, Staniloiu A, Wahl-Kordon A (2023)
Neuropsychologia 183: 108505.
Zeitschriftenaufsatz
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Autor*in
Markowitsch, Hans J.UniBi;
Staniloiu, AngelicaUniBi;
Wahl-Kordon, Andreas
Abstract / Bemerkung
Urbach-Wiethe disease is an extremely rare genetically-based syndrome which usually leads to dermatological and neurological changes. Neurologically, the amygdaloid region is primarily bilaterally affected. Therefore, several functions modulated by the amygdala are changed in patients with Urbach-Wiethe disease. As the neurological alterations evolve only gradually, it is particularly important to determine the cognitive and brain status of a juvenile. The patient described here was seen briefly at age 9 and tested neuropsychologically at age 19; furthermore, computer tomography and magnetic resonance imaging of his head was done. There were no important abnormalities in the brain, which is unusual in the light of previous data from other patients. On the cognitive level, the patient was generally within normal limits. However, he had mild problems in attention and concentration, and more prominent problems in emotional processing domain, and in personality dimensions. It is concluded that amygdala calcifications in Urbach-Wiethe disease take place progressively-possibly under-pinned by genetic and gender variables; this can subsequently allow psychosocial-social factors (such a proper education and socialization) and biological factors (compensatory neuroplasticity) to retard and diminish the development of socio-emotional and cognitive deteriorations, though the outcome of questionnaires indicates that such patients may develop substantial concerns as to their future life and well-being.
Stichworte
Lipoid proteinosis;
Hyalinosis cutis et mucosae;
Autonoetic;
consciousness;
Effort;
Emotion
Erscheinungsjahr
2023
Zeitschriftentitel
Neuropsychologia
Band
183
Art.-Nr.
108505
ISSN
0028-3932
eISSN
1873-3514
Page URI
https://pub.uni-bielefeld.de/record/2978298
Zitieren
Markowitsch HJ, Staniloiu A, Wahl-Kordon A. Urbach-Wiethe disease in a young patient without apparent amygdala calcification. Neuropsychologia. 2023;183: 108505.
Markowitsch, H. J., Staniloiu, A., & Wahl-Kordon, A. (2023). Urbach-Wiethe disease in a young patient without apparent amygdala calcification. Neuropsychologia, 183, 108505. https://doi.org/10.1016/j.neuropsychologia.2023.108505
Markowitsch, Hans J., Staniloiu, Angelica, and Wahl-Kordon, Andreas. 2023. “Urbach-Wiethe disease in a young patient without apparent amygdala calcification”. Neuropsychologia 183: 108505.
Markowitsch, H. J., Staniloiu, A., and Wahl-Kordon, A. (2023). Urbach-Wiethe disease in a young patient without apparent amygdala calcification. Neuropsychologia 183:108505.
Markowitsch, H.J., Staniloiu, A., & Wahl-Kordon, A., 2023. Urbach-Wiethe disease in a young patient without apparent amygdala calcification. Neuropsychologia, 183: 108505.
H.J. Markowitsch, A. Staniloiu, and A. Wahl-Kordon, “Urbach-Wiethe disease in a young patient without apparent amygdala calcification”, Neuropsychologia, vol. 183, 2023, : 108505.
Markowitsch, H.J., Staniloiu, A., Wahl-Kordon, A.: Urbach-Wiethe disease in a young patient without apparent amygdala calcification. Neuropsychologia. 183, : 108505 (2023).
Markowitsch, Hans J., Staniloiu, Angelica, and Wahl-Kordon, Andreas. “Urbach-Wiethe disease in a young patient without apparent amygdala calcification”. Neuropsychologia 183 (2023): 108505.
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Daten bereitgestellt von Europe PubMed Central.
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