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Vol. 2, No. 2, 2010
Issue release date: May – August
Section title: Published: May 2010

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Case Rep Neurol 2010;2:46–51

High Levels of Copper, Zinc, Iron and Magnesium, but not Calcium, in the Cerebrospinal Fluid of Patients with Fahr’s Disease

Hozumi I.a · Kohmura A.a · Kimura A.a · Hasegawa T.b · Honda A.c · Hayashi Y.a · Hashimoto K.a · Yamada M.a · Sakurai T.a · Tanaka Y.a · Satoh M.c · Inuzuka T.a
aDepartment of Neurology and Geriatrics, Gifu University, Graduate School of Medicine, Gifu, bLaboratory of Environmental Biochemistry, Yamanashi Prefectural Environmental Science Institute, Fuji-Yoshida, and cLaboratory of Pharmaceutical Health Sciences, School of Pharmacy, Aichi Gakuin University, Nagoya, Japan

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Patients with marked calcification of the basal ganglia and cerebellum have traditionally been referred to as having Fahr’s disease, but the nomenclature has been criticized for including heterogeneous etiology. We describe 3 patients with idiopathic bilateral striatopallidodentate calcinosis (IBSPDC). The patients were a 24-year-old man with mental deterioration, a 57-year-old man with parkinsonism and dementia, and a 76-year-old woman with dementia and mild parkinsonism. The former 2 patients showed severe calcification of the basal ganglia and cerebellum, and the latter patient showed severe calcification of the cerebellum. We found significantly increased levels of copper (Cu), zinc (Zn), iron (Fe) and magnesium (Mg), using inductively coupled plasma mass spectrometry in the CSF of all these 3 patients. The increased levels of Cu, Zn, Fe and Mg reflect the involvement of metabolism of several metals and/or metal-binding proteins during the progression of IBSPDC. More numerous patients with IBSPDC should be examined in other races to clarify the common mechanism of the disease and to investigate the specific treatment.

© 2010 S. Karger AG, Basel


Author Contacts

Isao Hozumi
Department of Neurology and Geriatrics, Gifu University
Graduate School of Medicine, 1-1 Yanagido
Gifu 501-1194 (Japan)
Tel. +81 58 230 6253, Fax +81 58 230 6256, E-Mail


Article Information

Published online: May 7, 2010
Number of Print Pages : 6
Number of Figures : 2, Number of Tables : 1,


Publication Details

Case Reports in Neurology

Vol. 2, No. 2, Year 2010 (Cover Date: May - August)

Journal Editor: Tatlisumak T. (Helsinki)
ISSN: NIL (Print), eISSN: 1662-680X (Online)

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Article / Publication Details

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Abstract of Published: May 2010

Published online: 5/7/2010
Issue release date: May – August

Number of Print Pages: 6
Number of Figures: 2
Number of Tables: 1

ISSN: (Print)
eISSN: 1662-680X (Online)

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Open Access License: This is an Open Access article licensed under the terms of the Creative Commons Attribution-NonCommercial 3.0 Unported license (CC BY-NC) (, applicable to the online version of the article only. Distribution permitted for non-commercial purposes only.
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