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Vol. 2, No. 2, 2010
Issue release date: May – August
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Case Rep Neurol 2010;2:46–51
(DOI:10.1159/000313920)

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
email Corresponding Author

Abstract

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.


 Outline


 goto top of outline Key Words

  • Fahr’s disease
  • Calcification
  • Copper
  • Zinc
  • Dementia
  • Parkinsonism

 goto top of outline Abstract

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.

Copyright © 2010 S. Karger AG, Basel


 goto top of outline 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 ihozumi@gifu-u.ac.jp


 goto top of outline Article Information

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


 goto top of outline 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)

For additional information: http://www.karger.com/CRN


Open Access License / Drug Dosage / Disclaimer

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) (www.karger.com/OA-license), applicable to the online version of the article only. Distribution permitted for non-commercial purposes only.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

Abstract

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.



 goto top of outline 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 ihozumi@gifu-u.ac.jp


 goto top of outline Article Information

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


 goto top of outline 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)

For additional information: http://www.karger.com/CRN


Open Access License / Drug Dosage

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) (www.karger.com/OA-license), applicable to the online version of the article only. Distribution permitted for non-commercial purposes only.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.