PROJECT: Wilson's disease (WD) is an inherited disorder of copper metabolism characterised by juvenile liver cirrhosis and by neurological symptoms. Copper levels in brain in WD have been reported to be 10 to 15 fold normal values, depending on the different brain regions. Being very few data on copper distribution in central nervous system in WD available, it seemed of interest to study the concentration of copper and of other trace elements (Zn, P, Mg, Ca, Fe and S) in the brain of a patient died for WD. PROCEDURE: a 56 year old woman affected by WD was admitted to our hospital with signs of hepatic failure and died few days later. At autopsy, a brain slice extending from the left to the right hemisphere was divided in 28 samples. On each sample Copper, Iron, Magnesium, Phosphorus, Sulphur, Zinc and Calcium were determined by Induced Coupled Plasma Atomic Emission Spectroscopy. RESULTS: the mean concentration of copper, ranging from 88 to 158 microg/g of dry tissue in all the brain specimens was higher than literature reference values, while that of the other tested elements was considerably lower. CONCLUSIONS: 1) In the brain of WD patient examined the status of trace elements was extensively altered. Further studies are necessary to correlate the concentration of trace elements with pathological lesions and with clinical pictures. 2) The elements considered in our study showed an uneven distribution in different brain areas.
PROJECT: Wilson's disease (WD) is an inherited disorder of copper metabolism characterised by juvenile liver cirrhosis and by neurological symptoms. Copper levels in brain in WD have been reported to be 10 to 15 fold normal values, depending on the different brain regions. Being very few data on copper distribution in central nervous system in WD available, it seemed of interest to study the concentration of copper and of other trace elements (Zn, P, Mg, Ca, Fe and S) in the brain of a patientdied for WD. PROCEDURE: a 56 year old woman affected by WD was admitted to our hospital with signs of hepatic failure and diedfew days later. At autopsy, a brain slice extending from the left to the right hemisphere was divided in 28 samples. On each sample Copper, Iron, Magnesium, Phosphorus, Sulphur, Zinc and Calcium were determined by Induced Coupled Plasma Atomic Emission Spectroscopy. RESULTS: the mean concentration of copper, ranging from 88 to 158 microg/g of dry tissue in all the brain specimens was higher than literature reference values, while that of the other tested elements was considerably lower. CONCLUSIONS: 1) In the brain of WDpatient examined the status of trace elements was extensively altered. Further studies are necessary to correlate the concentration of trace elements with pathological lesions and with clinical pictures. 2) The elements considered in our study showed an uneven distribution in different brain areas.
Authors: Hsien W Chan; Tianbing Liu; Giuseppe Verdile; Glenda Bishop; Ryan J Haasl; Mark A Smith; George Perry; Ralph N Martins; Craig S Atwood Journal: Int J Clin Exp Med Date: 2008-01-20
Authors: Sabine Borchard; Stefanie Raschke; Krzysztof M Zak; Carola Eberhagen; Claudia Einer; Elisabeth Weber; Sandra M Müller; Bernhard Michalke; Josef Lichtmannegger; Albrecht Wieser; Tamara Rieder; Grzegorz M Popowicz; Jerzy Adamski; Martin Klingenspor; Andrew H Coles; Ruth Viana; Mikkel H Vendelbo; Thomas D Sandahl; Tanja Schwerdtle; Thomas Plitz; Hans Zischka Journal: Life Sci Alliance Date: 2021-12-02
Authors: Anh Le; Noreene M Shibata; Samuel W French; Kyoungmi Kim; Kusum K Kharbanda; Mohammad S Islam; Janine M LaSalle; Charles H Halsted; Carl L Keen; Valentina Medici Journal: Int J Mol Sci Date: 2014-05-07 Impact factor: 5.923