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Abstract:
We thank Dr. Parnes for his highly valuable comment in his letter to the editor in reply to our recent publication,1 and we agree that duplicate terms for phenotypic or imaging characteristics of different diseases may be misleading in general.
However, we believe that the descriptive term “double cortex sign” referring to the pattern of iron deposition subcortical to the entire cortical ribbon on susceptibility-weighted imaging in our patient with PLA2G6-associated neurodegeneration (PLAN)1 is clearly distinguishable from the band heterotopia seen on standard T1- and T2-weighted sequences underlying the imaging pattern termed “double cortex syndrome” caused by pathogenic variants in the DCX gene.2-6
Therefore, in our view, given the similar but distinguishable imaging pattern in PLAN and DCX-associated disease, the descriptive term “double cortex” resulting from the visual appearances on different magnetic resonance imaging (MRI) sequences could be considered adequate in both PLAN and DCX-associated disease. This might even stimulate and facilitate thorough differential diagnostic considerations in disorders with imaging patterns reminiscent of a “double cortex,” eventually leading to appropriate selection of suitable MRI sequences in the diagnostic workup of such patients.
