PMC9935935_01
PMC9935935_02
Magnetic resonance imaging of each patient at presentation and various treatment time points. (A) Case 1: MRI following presentation demonstrates a giant hyperintense confluent right sided periventricular lesion on axial T2 FLAIR (a/b), with coronal oblique T2 FLAIR reconstruction (d), and sagittal oblique (e) reconstruction. Maximum dimension of the dominant lesion measuring up to 72 mm on sagittal imaging. Evidence of internal diffusion hyperintensity (green circle) on axial DWI (c) and internal punctate foci of enhancement (blue arrow) on sagittal T1 post contrast (f). There was evidence of further periventricular T2 FLAIR hyperintense lesion adjacent to the contralateral left occipital horn (red arrows). (B) Case 2: MRI at presentation (a-d) and 4 months follow up (e-h). Scan at diagnosis demonstrates a large solitary T2 FLAIR (a) hyperintense lesion centered on the right frontal white matter with no significant mass effect. This lesion measured up to 66 mm with peripheral enhancement (red arrows) on post contrast T1 imaging (b) with patchy internal diffusion restriction (blue circle) on DWI (c), and subtle peripheral restricting peripheral margin (white arrow) on ADC (d). Near complete resolution on follow up imaging with minimal residual T2 FLAIR hyperintensity (e). (C) Case 3: MRI at presentation (a-e), multiple follow up times points post contrast T1 imaging (f), and 4 month follow up DWI (g) and ADC (h). On multiplanar T2 FLAIR (a-c) there is a large periventricular lesion (yellow circle) measuring up to 47 mm with marked associated diffusion restriction (blue arrows) on DWI (d) and ADC (e). No enhancement was demonstrated on scan at diagnosis (c) however the patient progressively developed classic interrupted peripheral enhancement (red arrows). On delayed 4 month follow up scan enhancement and diffusion restriction had resolved (g/h). (D) Case 4: MRI at presentation with sagittal T2 FLAIR (a), coronal T2 (b), coronal T1 post contrast (c), and DWI (d) demonstrates a large T2/FLAIR hyperintensity extending from the periventricular margin to the cortex of the right posterior frontal lobe. There was wispy associated linear enhancement (blue arrow) at diagnosis on post contrast T1 imaging (c) and regions of internal and peripheral diffusion restriction (red circle) on DWI (d). Six year follow up scan (e-h) shows marked reduction in FLAIR signal, some associated cortical atrophy and volume loss (yellow circle) and resolution of previous enhancement and diffusion restriction.
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F1
MRI brain demonstrated TDLs in all cases (Figures 1A-D), and the mean size of the lesions at maximum was 59.75 mm (Table 2). All lesions demonstrated T2/FLAIR hyperintensity with T1 hypointensity and variable patterns of diffusion restriction and gadolinium enhancement (Figure 1). One patient exhibited a Balo-like lesion with a subsequent second much smaller lesion in the contralateral hemisphere (Figure 1A). There was substantial radiological improvement in all cases, with case 1 and 2 showing near-complete resolution of the GDLs (Figures 1A, B).
PMC9935935_03
PMC9935935_04
Graphs illustrating the clinical response of each patient according to timing and nature of treatment, as represented by the Extended Disability Status Scale (EDSS).
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F2
One patient had cyclophosphamide as an adjunctive treatment (outlined in Table 1; Figure 2). The clinical response to treatment, as measured by the Extended Disability Status Scale (EDSS) is shown in Figure 2.