Abstract Details

Title The Brain Metals Accumulation in Wilson's Disease

Topic Movement Disorders

Presentation(s) P03 - (-)

Poster/Presentation
Number 061

Objective

Background BACKGROUND: Wilson's Disease (WD) is an inherited copper metabolism disorder with pathological copper tissue accumulation, mainly in liver and brain. Previous studies have shown that accumulation of other metals in the brain tissue occurs in numerous neurodegenerative disorders. However, until now this issue have not been quantitatively examined in WD.

Design/Methods DESIGN/METHODS: The study material consisted of 17 brains (12 WD patients, 5 controls) obtained upon autopsy. Samples were taken from 4 different regions of each brain: frontal cortex, putamen, nucleus dentatus and pons. The copper, manganese and zinc levels were assessed using plasma mass spectrometry. Iron concentration was measured with atomic absorption spectroscopy. Clinical presentation of WD was considered hepatic or neuropsychiatric.

Results RESULTS: Copper content was homogenously increased in all investigated structures of WD brains compared to controls (41.0±18.6 vs. 5.4±1.8 mg/g, p<0.01). Concentrations of manganese and zinc in WD brains were similar to the controls, and did not differ between analyzed brains parts. However, the concentration of iron in nucleus dentatus was significantly higher in WD brains (56.8±14.1 vs. 32.6±6.0 ug/g, p<0.05). There were no associations between the concentration of any metal in any part of brain and the clinical presentation of WD.

Conclusions CONCLUSIONS: According to our findings, in the course of WD copper accumulate in different parts of the brain irrespectively of predominant phenotypic presentation. Zinc and manganese does not seem to be involved in the pathology. However, increased amounts of iron appear to accumulate in nucleus dentatus. We speculate that this effect may be due to diminished level of ceruloplasmin, but further studies are needed.

Authors/Disclosures
Anna Czlonkowska, MD (Institute of Psychiatry and Neurology) PRESENTER	Dr. Czlonkowska has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Alexion.
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file

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