Author Of 2 Presentations
P0704 - Cortical involvement in MOG IgG–positive patients : a multicenter MRI study (ID 1853)
Abstract
Background
Cortical involvement in neuropathological studies has been identified in Multiple Sclerosis (MS) and recently in myelin oligodendrocyte glycoprotein antibody disease (MOGAD) patients. Neuroimaging findings also seem to confirm cortical involvement as cerebral cortical lesions (CCLs) and leptomeningeal contrast enhancement (LMCE) have been shown in 3D-FLAIR sequences in MS patients.We described recently LMCE in MOGAD-patients with 3D-FLAIR post-gadolinium (3D-FLAIRED) sequences.
Objectives
To assess the presence and type of CCLs and the possible relation between CCLs and LMCE in MOGAD-patients using a 3 Tesla-MRI-scanner.
Methods
We conducted a brain MRI study including 11 MOGAD patients (MOG-IgG1 serum detection with cell-based-assay) with CNS demyelination and 12 Relapsing-Remitting MS (RRMS) patients as a control-group. In these groups, 8/11 and 6/12 were females, with a mean age at MRI acquisition 45.2 years and 38.25years in MOGAD and RRMS groups respectively. Exclusion criteria of this study were a clinical relapse or administration of intravenous corticosteroids within one month preceding MRI acquisition. LMCE foci were identified as hyperintensities on 3D-FLAIRED and not on 3D-T1-weighted-post-gadolinium sequences. For the detection of CCLs both high signal on 3D-FLAIR and low in 3D-T1-weighted sequences were required. Due to limitations of 3Tesla, CCLs were classified in two subgroups: a) intracortical/subpial or b) leukocortical lesions.
Results
CCLs were detected in 8 out of 11 patients in the MOGAD-group with mean-number-of-lesions (MNLs) 4.375 and in all of the RRMS-group with MNLs 12.25. In MOGAD, leukocortical-MNLs (2.143) was similar to intracortical/subpial-MNLs (2.714), whereas a predomidance of leukocortical (MNLs 9.82) versus intracortical/subpial (MNLs 4.82) lesions was observed in RRMS. LMCE was observed in 3 out of 11 in the MOGAD-group and in 1 out of 12 in the RRMS–group. In the MOGAD-group, LMCE presence was related to a higher lesion number in both lesion subtypes when compared to the MOGAD patients without LMCE.
Conclusions
Our study showed the ability of 3D-FLAIR joint with 3D-T1-weighted sequences in disclosing and classifying CCLs in MOGAD. An association between LMCE and the extent of cortical demyelination in MOGAD was evident, suggesting that meningeal inflammation may contribute in cortical lesion development. Moreover, cerebral cortical lesion number was higher in RRMS compared to MOGAD.
P0983 - Multiple therapeutic approaches for Baló’s Concentric Sclerosis : study of 10 cases (ID 1877)
Abstract
Background
Baló’s concentric sclerosis (BCS) is a rare demyelinating disease, histopathologically characterized by large concentric lesions with rings of myelin loss alternating with rings of myelin preservation; BCS is considered to be more consistent with pattern III Multiple Sclerosis (MS) lesions in neuropathological studies. The typical MRI finding of BCS is a tumefactive brain lesion with hyperintense-isointense-hypointense concentric rings on T2-weighted images; however other typical lesions for MS can also be observed. Regarding treatments there are no established algorithms yet.
Objectives
To present a group of patients with BCS and introduce the concept of distinct BCS subtypes with different response to therapies.
Methods
Retrospective clinico-radiologic analysis of 10 treated patients with a tumefactive (size ≥ 2cm) BCS lesion at first clinical attack, diagnosed from 2009 to present. Mean age at onset was 25.6 years (range 18-41) and mean follow-up was 50 months (range 2-136).
Results
In our cohort the mean BCS lesion size was 2.763cm. All patients received induction therapy with high doses of intravenous methylprednisolone (IVMP) at symptom onset and 9 out of 10 continued with maintenance treatment. According to radiological characteristics and response to therapies we categorized them into 4 subtypes : i) solitary BCS tumefactive lesion ; 2 patients non-responders to IVMP, one of the two with significant reponse following i.v. cyclophosphamide, ii) BCS lesion with coexisting non-specific brain lesions; 3 patients with poor response to IVMP but with remarkable response to immunosupression (cyclophosphamide n=2, mitoxantrone n=1), iii) BCS lesion with typical MS lesions; 2 patients that responded initially to IVMP and followingly to natalizumab, iv) BCS lesion with coexistence of another demyelinating tumefactive lesion; 3 patients moderate responders to IVMP but with excellent clinicoradiologic outcomes under rituximab.
Conclusions
We described 4 distinct subtypes of Balo successfully treated with different immunotherapies; after high doses of i.v. corticosteroids, maintenance therapy should be decided according to the specific subtype. Immunosupression with cyclophosphamide or mitoxantrone seems to be an effective choice, however the use of immunomodulatory MS drugs or cell depletion therapies should also be considered in BCS cases where MS characteristics are also present.