title: High Levels of Copper, Zinc, Iron and Magnesium, but not Calcium, in the Cerebrospinal Fluid of Patients with Fahr's Disease.
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.
authors.affiliation: Department of Neurology and Geriatrics, Gifu University, Graduate School of Medicine, Gifu, Japan.
authors.forename: Isao
authors.initials: I
authors.lastname: Hozumi
authors.forename: Akihiro
authors.initials: A
authors.lastname: Kohmura
authors.forename: Akio
authors.initials: A
authors.lastname: Kimura
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Hasegawa
authors.forename: Akiko
authors.initials: A
authors.lastname: Honda
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Hayashi
authors.forename: Kazunori
authors.initials: K
authors.lastname: Hashimoto
authors.forename: Megumi
authors.initials: M
authors.lastname: Yamada
authors.forename: Takeo
authors.initials: T
authors.lastname: Sakurai
authors.forename: Yuji
authors.initials: Y
authors.lastname: Tanaka
authors.forename: Masahiko
authors.initials: M
authors.lastname: Satoh
authors.forename: Takashi
authors.initials: T
authors.lastname: Inuzuka