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Susceptibility-Weighted Imaging Reveals Subcortical Iron Deposition in PLA2G6-associated Neurodegeneration: The "Double Cortex Sign"

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Hagberg,  GE       
Department High-Field Magnetic Resonance, Max Planck Institute for Biological Cybernetics, Max Planck Society;

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Scheffler,  K       
Department High-Field Magnetic Resonance, Max Planck Institute for Biological Cybernetics, Max Planck Society;

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Citation

Roeben, B., Zeltner, L., Hagberg, G., Scheffler, K., Schöls, L., & Bender, B. (2023). Susceptibility-Weighted Imaging Reveals Subcortical Iron Deposition in PLA2G6-associated Neurodegeneration: The "Double Cortex Sign". Movement Disorders, 38(5), 904-906. doi:10.1002/mds.29364.


Cite as: https://hdl.handle.net/21.11116/0000-000C-B1CF-3
Abstract
Magnetic resonance imaging (MRI) findings in PLA2G6-associated Neurodegeneration (PLAN), a “Neurodegeneration with Brain Iron Accumulation” (NBIA) disorder caused by autosomal-recessive mutations in the PLA2G6 gene,1 have been reported to show varying degrees of cerebral and cerebellar atrophy as well as basal ganglia iron deposition on susceptibility-weighted imaging (SWI), although some patients do not show any of these imaging features despite genetically proven PLAN.