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 REVIEW ARTICLE
Year : 2003  |  Volume : 51  |  Issue : 2  |  Page : 176--182

Friedreich’s ataxia – yesterday, today and tomorrow


Department of Neurology, Vivekananda Institute of Medical Science, Calcutta, India

Correspondence Address:
A Chakravarty
59, Beadon Street, Calcutta-700006
India
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Source of Support: None, Conflict of Interest: None


PMID: 14570998

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The present review traces the origin of Friedreich’s Ataxia (FA) from the time of Nikolaus Friedreich in the mid-nineteenth century. The early hesitation on the part of the neurological community in accepting FA as a distinct entity, rather than a variant form of tabes dorsalis and multiple sclerosis, has been highlighted. Research within the last 6-7 years, has firmly established FA as a trinucleotide repeat disorder, the location of the offending gene, and the disease-related gene product, frataxin. Frataxin is now thought to interfere with the mitochondrial oxidative process and enhance iron accumulation. However, whether this iron accumulation is a primary causative event for symptom production is not clear and iron chelators are unlikely to be helpful in therapy. Of great promise is the use of free radical scavengers and antioxidants. One such agent idebenone, a short chain analogue of co-enzyme Q10, may have a future.






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Online since 20th March '04
Published by Wolters Kluwer - Medknow