Clinical and Radiologic Features, Pathology, and Treatment of Baló Concentric Sclerosis

Abstract

Objective:To describe clinical, radiological and pathological features of patients with Baló’s concentric sclerosis (BCS) and assess overlap between BCS and other central nervous system 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 one month of the index MRI, lesions expanded radially with sequentially increased numbers of T2 hyperintense rings 52% (14/27). Initially non-enhancing 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 ADC, DWI, and gadolinium-enhanced imaging was observed in 67% (22/33). AQP4-IgG (n=26) and MOG-IgG (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.

Population‐based incidence and clinico‐radiological characteristics of tumefactive demyelination in Olmsted County, Minnesota, United States

“…Tumefactive demyelination (TD) is an atypical form of demyelination, encompassing various entities such as Marburg disease and Balo's concentric sclerosis (BCS) [1,2]. Clinically, there is evidence that BCS and TD commonly occur as tumefactive variants of multiple sclerosis (MS) [3]. Tumefactive MS is characterized by large inflammatory demyelinating lesions with an atypical enhancement pattern, edema, or mass effect [4].…”

Increasing role of imaging in differentiating MS from non-MS and defining indeterminate borderline cases

“…Recognition of AQP4-Ab NMOSD and MOGAD as new disease entities having a separate non-MS-like radiological appearances has sparked interest in other types of atypical demyelination with an unclear link to MS, in particular tumefactive demyelinating lesions (TDLs) and Balo's concentric sclerosis [49,50].…”

“…Alternating bands are characterised by at least two T2 hyperintense bands separated by one hypointense band. The number of T2 hyperintense bands can increase on subsequent scans performed within one month [50]. Evolution of signal abnormalities at the lesion edge is particularly interesting with diffusion restriction seen before contrast enhancement, which in turn is followed by the rim of T2 hyperintensity [58].…”

Case Report: Baló's Concentric Sclerosis-Like Lesion in a Patient With Relapsing-Remitting Multiple Sclerosis Treated With Dimethyl Fumarate

“…This typical pathology is reflected by magnetic resonance (MR) images of concentric lamellar-like zones of hyper- and hypointensities on T2-weighted sequences, corresponding to myelinated and demyelinated layers. The onion bulb (or wood grain) appearance is distinctive on gadolinium-enhanced T1-weighted sequences ( 1 , 2 ).…”

“…A fulminant disease course, resulting in death, is not uncommon and has recently been reported as 10%. However, complete recovery has also been described ( 2 ).…”