title: Neurodegeneration with brain iron accumulation: an overview.
abstract: 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.
authors: Pediatric Neurology Research Center, Shahid Beheshti University of Medical Sciences (SBMU), Tehran, Iran ; Pediatric Neurology Center of Excellence, Department of Pediatric Neurology, Mofid Children Hospital, Faculty of Medicine, Shahid Beheshti University of Medical Sciences (SBMU), Tehran, Iran.
authors: Seyed Hassan
authors: SH
authors: Tonekaboni
authors: Pediatric Neurology Department, Hazrat Fatemeh Masoumeh Hospital, Qom University of Medical Sciences, Qom, Iran.
authors: Mohsen
authors: M
authors: Mollamohammadi
keywords: Hallervorden- Spatz syndrome; Neuroaxonal Dystrophy; Neurodegeneration With Brain Iron Accumulation; Pantothenate Kinase Associated Neurodegeneration
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