Evan A Jolliffe1, Yong Guo1, Todd A Hardy1, P Pearse Morris1, Eoin P Flanagan1, Claudia F Lucchinetti1, W Oliver Tobin2. 1. From the Departments of Neurology (E.A.J., Y.G., E.P.F., C.F.L., W.O.T.) and Radiology (P.P.M.), Mayo Clinic, Rochester, MN; Department of Neurology (E.A.J.), Capital and Coast District Health Board, Wellington, New Zealand; Brain & Mind Centre (T.A.H.), University of Sydney; and Department of Neurology (T.A.H.), Concord Repatriation General Hospital, Sydney, NSW, Australia. 2. From the Departments of Neurology (E.A.J., Y.G., E.P.F., C.F.L., W.O.T.) and Radiology (P.P.M.), Mayo Clinic, Rochester, MN; Department of Neurology (E.A.J.), Capital and Coast District Health Board, Wellington, New Zealand; Brain & Mind Centre (T.A.H.), University of Sydney; and Department of Neurology (T.A.H.), Concord Repatriation General Hospital, Sydney, NSW, Australia. tobin.oliver@mayo.edu.
Abstract
OBJECTIVE: To describe clinical, radiologic, and pathologic features of Baló concentric sclerosis (BCS) and assess overlap between BCS and other CNS inflammatory demyelinating diseases. METHODS: Retrospective review of BCS cases from US and Australian tertiary care centers. RESULTS: We identified 40 BCS cases with 38 available MRIs. Solitary MRI lesions were present in 26% (10/38). We saw >1 active concurrent BCS lesion in 45% (17/38). A third (13/38) had multiple sclerosis-suggestive lesions on the index MRI, of which 10 fulfilled Barkhof criteria. In patients with serial MRI performed within 1 month of the index MRI, lesions expanded radially with sequentially increased numbers of T2 hyperintense rings 52% (14/27). Initially nonenhancing or centrally enhancing lesions subsequently developed single or multiple enhancing rings (41%; 9/22) and incomplete enhancing rings (14%; 3/22). Discordance between rings as they appear on apparent diffusion coefficient, diffusion-weighted imaging, and gadolinium-enhanced imaging was observed in 67% (22/33). Aquaporin-4 immunoglobulin G (n = 26) and myelin oligodendrocyte glycoprotein immunoglobulin G (n = 21) were negative in all patients with serum available. Clinical response to steroid treatment was seen in 46% (13/28). A monophasic clinical course was present in 56% (18/32) at last follow-up (median 27.5 months; range 3-100 months). The initial attack was fatal in 10% (4/40). Median time from symptom onset to death was 23 days (range 19-49 days). All 17 patients with pathology available demonstrated typical findings of multiple sclerosis. Patients with active demyelinating lesions all demonstrated oligodendrocytopathy (pattern III). CONCLUSIONS: BCS may be a distinct subtype of multiple sclerosis characterized by pattern III immunopathology.
OBJECTIVE: To describe clinical, radiologic, and pathologic features of Baló concentric sclerosis (BCS) and assess overlap between BCS and other CNS inflammatory demyelinating diseases. METHODS: Retrospective review of BCS cases from US and Australian tertiary care centers. RESULTS: We identified 40 BCS cases with 38 available MRIs. Solitary MRI lesions were present in 26% (10/38). We saw >1 active concurrent BCS lesion in 45% (17/38). A third (13/38) had multiple sclerosis-suggestive lesions on the index MRI, of which 10 fulfilled Barkhof criteria. In patients with serial MRI performed within 1 month of the index MRI, lesions expanded radially with sequentially increased numbers of T2 hyperintense rings 52% (14/27). Initially nonenhancing or centrally enhancing lesions subsequently developed single or multiple enhancing rings (41%; 9/22) and incomplete enhancing rings (14%; 3/22). Discordance between rings as they appear on apparent diffusion coefficient, diffusion-weighted imaging, and gadolinium-enhanced imaging was observed in 67% (22/33). Aquaporin-4 immunoglobulin G (n = 26) and myelin oligodendrocyte glycoprotein immunoglobulin G (n = 21) were negative in all patients with serum available. Clinical response to steroid treatment was seen in 46% (13/28). A monophasic clinical course was present in 56% (18/32) at last follow-up (median 27.5 months; range 3-100 months). The initial attack was fatal in 10% (4/40). Median time from symptom onset to death was 23 days (range 19-49 days). All 17 patients with pathology available demonstrated typical findings of multiple sclerosis. Patients with active demyelinating lesions all demonstrated oligodendrocytopathy (pattern III). CONCLUSIONS: BCS may be a distinct subtype of multiple sclerosis characterized by pattern III immunopathology.
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