PT  - JOURNAL ARTICLE
AU  - Kruer, M.C.
AU  - Boddaert, N.
AU  - Schneider, S.A.
AU  - Houlden, H.
AU  - Bhatia, K.P.
AU  - Gregory, A.
AU  - Anderson, J.C.
AU  - Rooney, W.D.
AU  - Hogarth, P.
AU  - Hayflick, S.J.
TI  - Neuroimaging Features of Neurodegeneration with Brain Iron Accumulation
AID  - 10.3174/ajnr.A2677
DP  - 2012 Mar 01
TA  - American Journal of Neuroradiology
PG  - 407--414
VI  - 33
IP  - 3
4099  - http://www.ajnr.org/content/33/3/407.short
4100  - http://www.ajnr.org/content/33/3/407.full
SO  - Am. J. Neuroradiol.2012 Mar 01; 33
AB  - NBIA characterizes a class of neurodegenerative diseases that feature a prominent extrapyramidal movement disorder, intellectual deterioration, and a characteristic deposition of iron in the basal ganglia. The diagnosis of NBIA is made on the basis of the combination of representative clinical features along with MR imaging evidence of iron accumulation. In many cases, confirmatory molecular genetic testing is now available as well. A number of new subtypes of NBIA have recently been described, with distinct neuroradiologic and clinical features. This article outlines the known subtypes of NBIA, delineates their clinical and radiographic features, and suggests an algorithm for evaluation. ACPaceruloplasminemiaCNScentral nervous systemFAHNfatty acid hydroxylase-associated neurodegenerationINADinfantile neuroaxonal dystrophyKRSKufor-Rakeb syndromeNADneuroaxonal dystrophyNBIAneurodegeneration with brain iron accumulationNFTneuroferritinopathyPKANpantothenate kinase-associated neurodegenerationPLANphospholipase-associated neurodegenerationSENDAstatic encephalopathy of childhood with neurodegeneration in adulthoodWSSWoodhouse-Sakati syndrome