Article Figures & Data
Figures
- FIG 1.
Imaging appearance and follow-up in the early infantile age group. Axial (A and B) and coronal (C) T2-weighted images in patient 4 at 11 weeks of age at the acute stage show diffuse white matter T2 hyperintensity. D–F, The findings fully resolved on treatment with biotin.
- FIG 2.
Other imaging patterns in the early infantile age group. Coronal T2-weighted images in patient 10 at 8 weeks of age show predominant superior and inferior semilunar lobule involvement in the acute stage (A, arrow), with atrophy on follow-up MR imaging (B, arrow). C and D, Axial DWI and ADC image in patient 4 at 11 weeks of age show perirolandic restricted diffusion (arrows). Axial T2-weighted image of patient 3 in the acute stage (10 weeks of age) (E) shows predominant involvement of the frontal lobes (arrow). Axial T2-weighted image of patient 10 (F) on the follow-up image at 7 months of age shows frontal-predominant atrophy (arrow).
- FIG 3.
Imaging appearance in the early childhood age group (patient 8, 18 months of age). Axial T2-weighted images show hyperintense lesions involving the genu and splenium of corpus callosum (A, arrows), forniceal columns (A, arrowhead); dorsal midbrain including the periaqueductal gray matter (B, arrow); dorsal pons (C, arrow), medullary pyramids (D, black arrowhead), and dorsal medulla (D, white arrowhead). Axial DWI (E) and ADC (F) images show restricted diffusion involving the corpus callosum (arrows), fornices (arrowheads), and tapetum (dashed arrow). Coronal T2-weighted image (G) shows involvement of the optic chiasm (black arrowhead). Sagittal T2-weighted image of the cervical spine (H) shows faint hyperintensity involving the dorsal cervical cord (white arrowheads).
- FIG 4.
Imaging appearance and follow-up in the late childhood age group (patient 14, six years of age). A, Axial DWI (b-value = 1000) shows hyperintense signal involving the forniceal columns (arrow, A2) and the left forniceal crus (arrowhead, A1). Axial FLAIR (B1) and axial DWI (b-value = 1000) (B2) show hyperintense signal involving the right optic nerve (arrow, B1), with persistent signal abnormality in the fornices (arrow, B2). Axial T2-weighted image of the cervical spine (B3) shows hyperintense lesions involving the dorsal (black arrowhead) and lateral columns (white arrowheads). Coronal T2-weighted images (C1 and C2) show extension of signal abnormality to involve the optic chiasm (arrow, C1) and mamillary bodies (arrow, C2). Axial T2-weighted image of the cervical spine (C3) shows increased signal abnormality in the lateral columns (arrowhead). Axial DWI (b-value = 1000) (D1), coronal T2 (D2), and axial T2 (D3) weighted images show resolution of the forniceal and mamillary body hyperintensity, with faint residual signal change in the optic chiasm on the right (arrow, D2). Mths indicates months.
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