Neuroimaging features of neurodegeneration with brain iron accumulation

Michael C. Kruer, N. Boddaert, A. Schneider, H. Houlden, K. P. Bhatia, A. Gregory, J. C. Anderson, W. D. Rooney, P. Hogarth, S. J. Hayflick

Research output: Contribution to journalReview article

133 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)407-414
Number of pages8
JournalAmerican Journal of Neuroradiology
Volume33
Issue number3
DOIs
StatePublished - Mar 2012

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Clinical Neurology

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