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. 2022 Feb 15;12(2):502. doi: 10.3390/diagnostics12020502

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© 2022 by the authors.

Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).

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Patient 2, progression of the disease in 21 years. Axial (a,b,d,e) T2-weighted images, coronal T1-inversion recovery (c) and T1-weighted image (f) at (a–c) 5 years (first MRI scan) and (d–f) 21 years from clinical onset (last MRI scan). The disease showed mild progression over time, involving left perisylvian region (including frontal, insular and temporal structures, arrowhead). Clinical symptoms progressed from seizures at onset to seizures, focal deficits, EPC, cognitive and behavioral disorders.