Bioavailable Trace Metals in Neurological Diseases.
Medical treatment in Wilson's disease includes chelators (D-penicillamine and trientine) or zinc salts that have to be maintain all the lifelong. This pharmacological treatment is categorised into two phases; the first being a de-coppering phase and the second a maintenance one. The best therapeutic approach remains controversial, as only a few non-controlled trials have compared these treatments. During the initial phase, progressive increase of chelators' doses adjusted to exchangeable copper and urinary copper might help to avoid neurological deterioration. Liver transplantation is indicated in acute fulminant liver failure and decompensated cirrhosis; in cases of neurologic deterioration, it must be individually discussed. During the maintenance phase, the most important challenge is to obtain a good adherence to lifelong medical therapy. Neurodegenerative diseases that lead to a mislocalisation of iron can be caused by a culmination of localised overload (pro-oxidant siderosis) and localised deficiency (metabolic distress). A new therapeutic concept with conservative iron chelation rescues iron-overloaded neurons by scavenging labile iron and, by delivering this chelated metal to endogenous apo-transferrin, allows iron redistribution to avoid systemic loss of iron.
French National Reference Centre for Wilson's Disease, University Hospital Lariboisière, Assistance Publique-Hôpitaux de Paris, Paris, France. aurelia.poujois@aphp.fr.|Department of Neurology, University Hospital Lariboisière, Assistance Publique-Hôpitaux de Paris, Paris, France. aurelia.poujois@aphp.fr.
Aurélia
A
Poujois
INSERM U1171, CHU Lille, Lille University, Lille, France.|Department of Medical Pharmacology, CHU Lille, Lille University, Lille, France.
Jean-Christophe
JC
Devedjian
INSERM U1171, CHU Lille, Lille University, Lille, France.|Department of Medical Pharmacology, CHU Lille, Lille University, Lille, France.
Caroline
C
Moreau
INSERM U1171, CHU Lille, Lille University, Lille, France.|Department of Medical Pharmacology, CHU Lille, Lille University, Lille, France.|Department of Movement Disorders and Neurology, CHU Lille, Lille University, Lille, France.
David
D
Devos
French National Reference Centre for Wilson's Disease, University Hospital Lariboisière, Assistance Publique-Hôpitaux de Paris, Paris, France.|Department of Neurology, University Hospital Lariboisière, Assistance Publique-Hôpitaux de Paris, Paris, France.
Pascal
P
Chaine
French National Reference Centre for Wilson's Disease, University Hospital Lariboisière, Assistance Publique-Hôpitaux de Paris, Paris, France.|Department of Neurology, University Hospital Lariboisière, Assistance Publique-Hôpitaux de Paris, Paris, France.
France
F
Woimant
School of Biomedical Sciences, Faculty of Biological Sciences, University of Leeds, Leeds, West Yorkshire, UK.|Oxidation Biology Unit, The Florey Institute of Neuroscience and Mental Health, The University of Melbourne, Parkville, Victoria, Australia.
James A
JA
Duce
Chelation therapy; Copper; Iron; Manganese; Neurodegeneration; Reactive species
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