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Final ID: Poster #: EDU-087

MR Features of Pediatric Neurodegeneration with Brain Iron Accumulation

Purpose or Case Report: Neurodegeneration with Brain Iron Accumulation comprises a clinically and genetically heterogeneous group of disorders that share the feature of high levels of iron in the basal ganglia. Although very rare, these disorders may be suspected commonly by radiologists when iron deposits in the basal ganglia are encountered. Generally, radiologists are not familiar with the MR phenotypes of these disorders beyond the typical ‘eye of the tiger’, which may lead to overcalling since nonspecific iron deposits in the basal ganglia are way more common than NBIA. The aim of this educational poster is to discuss the MR phenotypes of the four most common NBIA disorders to include BPAN, PKAN, PLAN and MPAN, comprising about 95% of the cases. We will not only review the typical auntminnie presentation but also, we will describe the earliest signs, such as the T2 hyperintense streak along the medial border of the pallidi of PKAN, interestingly before any iron deposition is evident. When available, we will discuss the follow-up cases to see how the patterns evolve over time. Finally, we will briefly review the anatomy of the basal ganglia, subthalamic nuclei, substantia nigra and their connections since it is of the utmost importance for the pattern recognition. We hope that this review helps radiologists recognize the NBIA phenotypes to be more confident in their differentials.
Methods & Materials: NA
Results: NA
Conclusions: NA
  • Molto Garcia, Jose Francisco  ( Children's National Hospital , Washington , District of Columbia , United States )
  • Tochen, Laura  ( Children's National Hospital , Washington , District of Columbia , United States )
  • Rhee, Jullie  ( Children's National Hospital , Washington , District of Columbia , United States )
Meeting Info:
Session Info:

Posters - Educational

Neuroradiology

IPR Posters - Educational

More abstracts from these authors:
Subpial Hemorrhage of the Neonate: Focus on Initial Sonographic Findings

Molto Garcia Jose Francisco, Cielma Tara, Rao Jhanavi, Czarniecki Marcin B., Sanchez Ramon

MR Features of Pediatric MOGAD

Molto Garcia Jose Francisco, Nair Lakshmi, Kahn Ilana

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Poster____EDU-087.pdf
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