Blood. 2007 Mar 22; [Epub ahead of print]Selective iron chelation in
Friedreich ataxia. Biological and clinical implications.Boddaert N, Le
Quan Sang KH, Rotig A, Leroy-Willig A, Gallet S, Brunelle F, Sidi D,
Thalabard JC, Munnich A, Cabantchik ZI.
Pediatric Radiology Unit, ERM0205, Hopital Necker-Enfants Malades &
Universite Paris V Rene Descartes, Paris, France.

Genetic disorders of iron metabolism and chronic inflammation often
evoke local iron accumulation. In Friedreich-ataxia, decreased iron-
sulphur-cluster and haem formation leads to mitochondrial iron
accumulation and ensuing oxidative damage that affect primarily
sensory neurons, myocardium and endocrine glands. We assessed the
possibility of reducing brain iron accumulation in Friedreich-ataxia
patients with a membrane-permeant chelator capable of shuttling
chelated-iron from cells to transferrin, using regimens suitable for
patients with no systemic iron overload. Brain MRI of Friedreich-
ataxia patients compared to age-matched controls revealed smaller and
irregularly shaped dentate-nuclei with significantly (p<0.027) higher
H-relaxation rates R2*, indicating regional iron accumulation. A six-
month treatment with 20-30mg/kg/d deferiprone applied on 11/20
adolescent patients with no overt cardiomyopathy reduced R2* from
18.3+/-1.6 to 15.7+/-0.7msec(-1) (p<0.002) specifically in dentate
nuclei and proportionally to the initial R2* (r=0.90). Chelator-
treatment caused no apparent haematological or neurological side-
effects, while reducing neuropathy and ataxic-gait in the youngest
patients. To our knowledge, this is the first clinical demonstration
of chelation removing labile iron accumulated in a specific brain area
implicated in a neurodegenerative disease. The use of moderate
chelation for relocating iron from areas of deposition to areas of
deprivation has clinical implications for various neurodegenerative
and haematological disorders.

PMID: 17379741 [PubMed - as supplied by publisher]

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