Neurodegenerations with Brain Iron Accumulation

Parkinsonism & Related Disorders
Susanne A Schneider

Abstract

Syndromes with Neurodegeneration with Brain Iron Accumulation (NBIA) are a group of neurodegenerative disorders characterized by excess iron mainly in the globus pallidus and sometimes adjacent areas. They clinically present as hypo- and/or hyperkinetic movement disorders and a variable degree of pyramidal, cerebellar, peripheral nerve, autonomic, cognitive and psychiatric involvement and visual dysfunction. Several causative genes underlying NBIA have been identified which explain about 65% of cases. Pathophysiologically, many of the NBIA syndromes map into related biochemical pathways and gene networks including lipid metabolism. Treatment for NBIA disorders remains symptomatic.

References

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Citations

Feb 2, 2016·Journal of Movement Disorders·Jae-Hyeok LeeMyung Sik Lee
Oct 11, 2017·The Journal of Veterinary Medical Science·Miyuu TanakaMitsuru Kuwamura
Dec 18, 2018·Movement Disorders : Official Journal of the Movement Disorder Society·Darius Ebrahimi-FakhariAlexander Münchau
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Jul 20, 2020·La Revue de médecine interne·H LobbesS Durupt
Jun 5, 2021·The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques·Reza HajatiAfagh Alavi

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