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Publication Detail
Neuroimaging features of neurodegeneration with brain iron accumulation.
  • Publication Type:
    Journal article
  • Publication Sub Type:
    Journal Article
  • Authors:
    Kruer MC, Boddaert N, Schneider SA, Houlden H, Bhatia KP, Gregory A, Anderson JC, Rooney WD, Hogarth P, Hayflick SJ
  • Publication date:
    03/2012
  • Pagination:
    407, 414
  • Journal:
    AJNR Am J Neuroradiol
  • Volume:
    33
  • Issue:
    3
  • Status:
    Published
  • Country:
    United States
  • PII:
    ajnr.A2677
  • Language:
    eng
  • Keywords:
    Algorithms, Brain, Brain Diseases, Metabolic, Humans, Image Enhancement, Iron Overload, Neurodegenerative Diseases, Neuroimaging, Radiography
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.
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Clinical and Movement Neurosciences
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Department of Neuromuscular Diseases
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