Hair trace elements in Friedreich's disease

Barbeau, A.; Roy, Madeleine; Paris, Suzanne

doi:10.1017/s0317167100035162

Cited by 2 publications

(1 citation statement)

References 13 publications

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“…Friedreich's ataxia (FRDA) [1][2][3], ataxia with isolated vitamin E deficiency (AVED) [4], X-linked sideroblastic anemia with ataxia (XLSA/A) [5,6], ataxiatelangiectasia (A-T) [7,8], and infantile-onset spinocerebellar ataxia [9,10] are examples of SCA which are caused by or lead to disturbances in iron homeostasis with oxidative damage as a common biochemical feature. Moreover, even though early work suggested a possible dysregulation of copper and zinc metabolism in some SCA, especially FRDA [11][12][13], the involvement of other metals in SCA neurodegeneration has not yet been investigated.…”

Section: Introductionmentioning

confidence: 99%

Synchrotron X-ray Fluorescence Reveals Abnormal Metal Distributions in Brain and Spinal Cord in Spinocerebellar Ataxia: A Case Report

et al. 2009

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For the first time, synchrotron rapid-scanning X-ray fluorescence (RS-XRF) was used to simultaneously localize and quantify iron, copper, and zinc in spinal cord and brain in a case of spinocerebellar ataxia (SCA). In the normal medulla, a previously undescribed copper enrichment was seen associated with spinocerebellar fibers and amiculum olivae. This region was virtually devoid of all metals in the SCA case. Regions with neuronal loss and gliosis in the cerebellar cortex, inferior olivary, and dentate nuclei and areas showing loss of myelinated fibers were also low in all metals in SCA compared to control. In contrast, the ventral columns of the spinal cord that exhibited only moderate myelin pallor had increased metal levels. Iron and zinc were also elevated in the globus pallidus pars externa in SCA relative to control. We hypothesize that metals increase as part of the initial neurodegenerative process, but once degeneration is advanced, the metal levels drop. This implies a role for multiple metals in SCA neurodegeneration, but further study is required to establish a causative role. We suggest that if these findings are generally true of at least some cases of SCA, not only iron but also copper and zinc should be considered as possible therapeutic targets.

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Section: Introductionmentioning

confidence: 99%

Synchrotron X-ray Fluorescence Reveals Abnormal Metal Distributions in Brain and Spinal Cord in Spinocerebellar Ataxia: A Case Report

et al. 2009

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Hair trace elements in cerebellar degeneration: low copper levels in late cortical cerebellar atrophy

et al. 1990

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The levels of ten elements in the hair of 7 patients with olivopontocerebellar atrophy (OPCA), 10 patients with late cortical cerebellar atrophy (LCCA) and 1489 healthy persons were determined by inductively coupled plasma emission spectrometry. The phosphorus, zinc, selenium, calcium, magnesium, iron, manganese, aluminium and mercury concentrations did not show any statistically significant difference among the three groups. The copper concentration was significantly lower in the patients with LCCA than in the patients with OPCA, and in the healthy controls. The possibility that LCCA is associated with a mild degree of copper deficiency is considered.

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Hair trace elements in Friedreich's disease

Cited by 2 publications

References 13 publications

Synchrotron X-ray Fluorescence Reveals Abnormal Metal Distributions in Brain and Spinal Cord in Spinocerebellar Ataxia: A Case Report

Synchrotron X-ray Fluorescence Reveals Abnormal Metal Distributions in Brain and Spinal Cord in Spinocerebellar Ataxia: A Case Report

Hair trace elements in cerebellar degeneration: low copper levels in late cortical cerebellar atrophy

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