title: Iron chelation in the treatment of neurodegenerative diseases.
abstract: Disturbance of cerebral iron regulation is almost universal in neurodegenerative disorders. There is a growing body of evidence that increased iron deposits may contribute to degenerative changes. Thus, the effect of iron chelation therapy has been investigated in many neurological disorders including rare genetic syndromes with neurodegeneration with brain iron accumulation as well as common sporadic disorders such as Parkinson's disease, Alzheimer's disease, and multiple sclerosis. This review summarizes recent advances in understanding the role of iron in the etiology of neurodegeneration. Outcomes of studies investigating the effect of iron chelation therapy in neurodegenerative disorders are systematically presented in tables. Iron chelators, particularly the blood brain barrier-crossing compound deferiprone, are capable of decreasing cerebral iron in areas with abnormally high concentrations as documented by MRI. Yet, currently, there is no compelling evidence of the clinical effect of iron removal therapy on any neurological disorder. However, several studies indicate that it may prevent or slow down disease progression of several disorders such as aceruloplasminemia, pantothenate kinase-associated neurodegeneration or Parkinson's disease.
authors.affiliation: Department of Neurology and Center of Clinical Neuroscience, Charles University in Prague, 1st Faculty of Medicine and General University Hospital in Prague, Czech Republic; Institute of Neuroradiology, University Göttingen, Göttingen, Germany. Electronic address: petr.dusek@vfn.cz.
authors.forename: Petr
authors.initials: P
authors.lastname: Dusek
authors.affiliation: Department of Neurology, Ludwig-Maximilians-University, Munich, Germany.
authors.forename: Susanne A
authors.initials: SA
authors.lastname: Schneider
authors.affiliation: Innlandet Hospital Trust, Kongsvinger, Norway; Hedmark University College, Elverum, Norway.
authors.forename: Jan
authors.initials: J
authors.lastname: Aaseth
mesh_terms: D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use*; D019636:Neurodegenerative Diseases / Q000188:drug therapy*
chemical_list: D007502:Iron Chelating Agents
keywords: Friedreich’s ataxia; Iron chelation; Multiple sclerosis; NBIA; Parkinson’s disease; Superficial siderosis