PMC10033939_01
Pre-surgical evaluation. (A, B) Facial dysmorphic features of Sotos syndrome showing prominent forehead, pointed chin and macrodolicocephaly. (C, D) Plain spine radiograph shows thoraco-lumbar scoliosis. (E) T2-weighted coronal brain MRI showing global atrophy with marked sulcal and ventricular volume increase. Note left hippocampal malrotation/atrophy (black triangle) compared to right hippocampus (white triangle). (F) FLAIR-weighted axial brain MRI. Left hippocampal malrotation/atrophy with slight hyperintense signal (black triangle). (G) Interictal EEG monitoring showing bilateral epileptic discharges with left temporal lobe lateralization.
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F1
Patient's family reported learning disabilities and phenotypic features of Sotos syndrome (Figure 1) in clinical and imaging exams were present. Figure 1G) and six electroclinical seizures. Five focal onset impaired awareness motor seizures were during awake period and only one during sleep. All seizures recorded had the ictal onset and sytomatogenic area in the left temporal lobe, and brain MRI showed left hippocampus atrophy and right hippocampus malrotation (Figure 1).
Late post-surgical brain MRI. (A) T2-weighted axial brain MRI shows left hippocampus resection (black triangle). (B, C) Coronal brain images showing complete resection of left amygdala and uncus on image (B) and hippocampal resection through transcortical approach (T2, white triangle).
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F2
During 3-years follow-up, the patient is under previous antiseizure medication dosage (carbamazepine 400 mg q8h and clobazam 20 mg q12h), with post-operative brain MRI showing complete hippocampal and temporal resection (Figure 2).