PMC10327559_01
(A,B) First MRI study: FLAIR (A) and ADC (B) images show diffuse thalamic and internal capsule bilateral involvement with increased diffusion as for vasogenic edema.
fped-11-1175584-g001.jpg
PMC10327559_01_fped-11-1175584-g001.jpg
F1
Brain magnetic resonance imaging (MRI) showed symmetrical thalamic lesions, with extension to the hemispheric white matter and to the right optic tract, without contrast enhancement (Figure 1A). A diagnosis of acute disseminated encephalomyelitis (ADEM) was made, and endovenous high-dose methylprednisolone therapy was started for 7 days, which involved a cycle of high-dose intravenous immunoglobulins (IVIg), leading to rapid clinical recovery and radiological response at the 3-month control brain MRI, without any radiological progression (Figure 1B). An MRI performed at 29 months of age did not show any new brain or spinal cord lesion but highlighted the presence of active arthritis of the right temporomandibular joint (Supplementary Figure S1).
(A-C) Third MRI study: FLAIR (A) and postcontrast T1WI (B) of the cerebellum and a STIR midsagittal image of the cervical spine (C). New lesions on deep cerebellar white matter and V cranial nerves origin are visible, with a postcontrast enhancement of the right lesion. A new lesion in the cervical spine is also visible at the C3-C4 level.
fped-11-1175584-g002.jpg
PMC10327559_01_fped-11-1175584-g002.jpg
F2
The patient again underwent a brain and spinal cord MRI, which showed new lesions in the supra- and infratentorial white matter, optic nerves, some of which with gadolinium enhancement, and longitudinally extensive transverse myelitis (LTEM) lesion at the C2-C4 level, suggestive of neuromyelitis optica spectrum disorder (NMOSD) (Figure 2).