Wilson disease and other neurodegenerations with metal accumulations
Language English Country United States Media print
Document type Journal Article, Review, Video-Audio Media
PubMed
25432729
DOI
10.1016/j.ncl.2014.09.006
PII: S0733-8619(14)00074-7
Knihovny.cz E-resources
- Keywords
- Chelating therapy, Manganism, NBIA, Neurodegeneration with brain iron accumulation, Primary familial brain calcification, Wilson disease,
- MeSH
- Chelation Therapy MeSH
- Mental Disorders etiology MeSH
- Hepatolenticular Degeneration epidemiology etiology physiopathology therapy MeSH
- Metals metabolism toxicity MeSH
- Humans MeSH
- Brain metabolism MeSH
- Check Tag
- Humans MeSH
- Publication type
- Video-Audio Media MeSH
- Journal Article MeSH
- Review MeSH
- Names of Substances
- Metals MeSH
Trace elements, such as iron, copper, manganese, and calcium, which are essential constituents necessary for cellular homeostasis, become toxic when present in excess quantities. In this article, we describe disorders arising from endogenous dysregulation of metal homeostasis leading to their tissue accumulation. Although subgroups of these diseases lead to regional brain metal accumulation, mostly in globus pallidus, which is susceptible to accumulate divalent metal ions, other subgroups cause systemic metal accumulation affecting the whole brain, liver, and other parenchymal organs. The latter group comprises Wilson disease, manganese transporter deficiency, and aceruloplasminemia and responds favorably to chelation treatment.
References provided by Crossref.org
Psychiatric manifestations in Wilson's disease: possibilities and difficulties for treatment
