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 ?
Childhood disorders of neurodegeneration with brain iron accumulation (NBIA).
T2* and FSE MRI distinguishes four subtypes of neurodegeneration with brain iron accumulation. Neurology 2008 Apr; 70(18):1614-1619.
Bhatia, "Genetics and pathophysiology of neurodegeneration with brain iron accumulation (NBIA)," Current Neuropharmacology, vol.
Bhatia, "Syndromes of neurodegeneration with brain iron accumulation (NBIA): an update on clinical presentations, histological and genetic underpinnings, and treatment considerations," Movement Disorders, vol.
Garavaglia et al., "Genotypic and phenotypic spectrum of PANK2 mutations in patients with neurodegeneration with brain iron accumulation, " Annals of Neurology, vol.
Jankovic, "Clinical heterogeneity of neurodegeneration with brain iron accumulation (Hallervorden-Spatz syndrome) and pantothenate kinase-associated neurodegeneration, " Movement Disorders, vol.
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. AJNR Am J Neuroradiol 2012;33(3):407-14.
Other case shows eye of the tiger sign.(10) consistent with that of Hallervorden Spatz disease (NBIA- Neurodegeneration with Brain Iron Accumulation).