neurodegeneration with brain iron accumulation


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neurodegeneration with brain iron accumulation

A group of genetically heterogeneous disorders—the prototypic form of which is Hallervorden-Spatz disease—characterised by progressive iron accumulation in the basal ganglia and other regions of the brain, resulting in extrapyramidal movements—e.g., parkinsonism and dystonia.
References in periodicals archive ?
Absence of an orphan mitochondrial protein, c19orf12, causes a distinct clinical subtype of neurodegeneration with brain iron accumulation.
Syndromes of neurodegeneration with brain iron accumulation (NBIA): an update on clinical presentations, histological and genetic underpinnings, and treatment considerations.
Childhood disorders of neurodegeneration with brain iron accumulation (NBIA).
The sign is considered the distinctive radiologic feature of Neurodegeneration with Brain Iron Accumulation (NBIA) type 1, also known as pantothenate kinase associated neurodegeneration (PKAN).
Neuroimaging features of neurodegeneration with brain iron accumulation.