Neurodegeneration with Brain Iron Accumulation: An Overview




How to Cite This Article: Tonekaboni SH, Mollamohammadi M. Neurodegeneration with Brain Iron Accumulation: An Overview. Iran J Child Neurol. 2014 Autumn;8(4): 1-8.

Neurodegeneration with brain iron accumulation (NBIA) is a group of neurodegenerative disorder with deposition of iron in the brain (mainly Basal Ganglia) leading to a progressive Parkinsonism, spasticity, dystonia, retinal degeneration, optic atrophy often accompanied by psychiatric manifestations and cognitive decline. 8 of the 10 genetically defined NBIA types are inherited as autosomal recessive and the remaining two by autosomal dominant and X-linked dominant manner. Brain MRI findings are almost specific and show abnormal brain iron deposition in basal ganglia some other related anatomical
locations. In some types of NBIA cerebellar atrophy is the major finding in MRI.


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Pantothenate Kinase Associated Neurodegeneration; Neurodegeneration With Brain Iron Accumulation; Hallervorden- Spatz syndrome; Neuroaxonal Dystrophy

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