PMC5988887_01
H&E staining of skeletal muscle transverse section performed at the age of 14. Rare atrophic fibers and few centralized nuclei were present.
fneur-09-00385-g0001.jpg
PMC5988887_01_fneur-09-00385-g0001.jpg
F1
Skeletal muscle biopsy, performed at the age of 14, revealed only moderate fiber size variability, with mild atrophy of type 1 fibers and rare centralized nuclei (Figure 1).
(A) Baseline 12 lead ECG; (B) ECG while starting ajmaline infusion; (C) ECG 5 min after ajmaline infusion starting.
fneur-09-00385-g0002.jpg
PMC5988887_01_fneur-09-00385-g0002.jpg
F2
After an appropriate pharmacological wash-out, ajmaline challenge was performed and resulted positive for the appearance of Brugada pattern type I (Figure 2).