PMC10456863_01
RFA at disease onset (A, C) and after 6 months from the start of induction therapy (B, D) in case 1 (A, B) and 2 (C, D). BRAOs are highlighted by a blue ovoid and AWH by dot arrows. In case 1, despite a complete disappearance of AWH in right eye (RE) after 6 months of cyclophosphamide therapy (B), two new AWH were observed in left eye (LE) compared to the examination performed at disease onset (A). Similarly, in case 2, new AWH in peripherals inferior temporal branches in RE were observed 6 months after rituximab commencement (D); no AWH were observed in LE.
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F1
AWH in both eyes (Figure 1C). The control RFA showed a few new AWH in the absence of recent BRAO (Figure 1D).
Comparison of axial CE-T1 (A, D), CE-FLAIR (B, E), and CE-VWI (C, F) sensibility in detecting LME in posterior fossa. (A-C) Show LME evaluation at onset in Case 1: LME is more evident in CE-VWI compared to CE-T1 and also to CE-FLAIR, located along the folia of the cerebellar hemispheres and vermis. (D-F) Show evaluation of LME regression in Case 2, 3 months after rituximab: despite CE-T1 and CE-FLAIR did not detect any residual LME, CE-VWI revealed a residual patch enhancement of the folia in left cerebellar hemisphere [blue ovoid, (F)].
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CE-FLAIR, and CE-VWI but it was more evident in the two latter, being the LME the posterior fossa more evident in the CE-VWI compared to the CE-FLAIR (Figures 2A-C). Cerebrospinal fluid (CSF) analysis revealed increased protein levels (1.6 g/L), absence of leucocytes, a normal glucose ratio, absence of oligoclonal bands and normal IgG index (<0.7). CSF PCR for neurotrophic viruses (HSV, CMV, VZV, EBV,
Brain MRI scan performed after 6 months of cyclophosphamide therapy in case 1. Axial VWI sequences without (A) and with (B) Gadolinium. A linear enhancement, compatible with a persistent inflamed vessel, is evident in CE-VWI [blue ovoid (B)]. In coronal scans without (C) and with (D) Gadolinium, an abnormal enhancement of semicircular canals is detected in CE-VWI [blue ovoid (D)].
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LME was no more detectable on CE-FLAIR, whereas CE-VWI revealed some thin linear enhancement attributable to inflamed vessel, as well as an abnormal enhancement of the right inner ear structures (Figure 3).
PMC10456863_02
RFA at disease onset (A, C) and after 6 months from the start of induction therapy (B, D) in case 1 (A, B) and 2 (C, D). BRAOs are highlighted by a blue ovoid and AWH by dot arrows. In case 1, despite a complete disappearance of AWH in right eye (RE) after 6 months of cyclophosphamide therapy (B), two new AWH were observed in left eye (LE) compared to the examination performed at disease onset (A). Similarly, in case 2, new AWH in peripherals inferior temporal branches in RE were observed 6 months after rituximab commencement (D); no AWH were observed in LE.
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F1
AWH in both eyes (Figure 1C). The control RFA showed a few new AWH in the absence of recent BRAO (Figure 1D).
Brain MRI scan after 6 months from rituximab start in Case 2: comparison of CE-T1, CE-FLAIR, and CE-VWI sensibility in detecting residual disease activity. Axial VWI without (A) and with Gadolinium (B). (C) Axial CE-T1. (D) CE-FLAIR. A punctiform persistent enhancement is observable on CE-VWI [blue ovoid, (B)] but not in CE-T1 (C) or CE-FLAIR (D). Note also that LME is completely absent on CE-FLAIR (D).
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The brain MRI control performed at month 6 after rituximab start showed no recent lesions and a complete disappearance of LME in CE-FLAIR, whereas CE-VWI demonstrated some residual punctiform enhancement (Figure 4).