Imaging of Fulminant Demyelinating Disorders of the Central Nervous System

Abstract

We aim to review the imaging appearance of fulminant demyelinating disorders of central nervous system that have different pathological features, clinical course, clinical features, and imaging findings different from classic multiple sclerosis. Routine magnetic resonance imaging (MRI) can help in accurate localization of the lesions, detection of associated lesions, and monitoring of these patients. Advanced MRI combined with routine MRI can aid in differentiation fulminant demyelinating lesions from simulating malignancy. Tumefactive demyelination lesions are located in supratentorial white matter mainly frontal and parietal regions with incomplete rim enhancement. Baló concentric sclerosis shows characteristic concentric onion skin appearance. Schilder disease is subacute or acute demyelinating disorders with one or more lesions commonly involving the centrum semiovale. Marburg disease is the most severe demyelinating disorder with diffuse infiltrative lesions and massive edema involving both the cerebral hemisphere and brain stem.

A rare case of Balo's disease in a young adult: A clinical presentation and management

“…16 In our case, the well-defined lesions with concentric rings observed in the bilateral cerebral hemispheres prompted consideration of both BCS and ADEM. 17 In our case, treatment with intravenous methylprednisolone, which is a common method for immune-mediated demyelinating disorders, 19 did not lead to the expected improvements in motor function and speech. This lack of response underscored the necessity of accurate diagnosis, as different demyelinating disorders may exhibit varied responses to similar therapeutic approaches.…”

“…In contrast, BCS typically presents with the characteristic concentric ring pattern on MRI, while ADEM tends to exhibit more diffuse and asymmetrical lesions 16 . In our case, the well‐defined lesions with concentric rings observed in the bilateral cerebral hemispheres prompted consideration of both BCS and ADEM 17 …”

“…One of the distinguishing features of BCS is its unique concentric ring pattern on neuroimaging, which sets it apart from more common forms of MS and other demyelinating disorders. 17 The hallmark radiological feature of BCS is the presence of alternating layers of demyelinated and preserved myelinated tissue, creating a target-like appearance on magnetic resonance imaging (MRI) scans. 18 This pattern of concentric rings makes me think it might be BCS, and I need more tests to tell it apart from other demyelinating diseases like ADEM.…”

Clinical applications of artificial intelligence and radiomics in neuro-oncology imaging

“…Tumefactive multiple sclerosis lesions (MS) with atypical features can mimic high-grade gliomas on conventional MR imaging [ 35 ]. A study used dynamic texture parameter analysis (DTPA) and extracted features from the first pass of contrast phase of DSC-enhanced perfusion maps and differentiated glioblastomas from tumefactive MS [ 36 ].…”

Heterogeneity of Baló’s concentric sclerosis: a study of eight cases with different therapeutic concepts

“…Baló’s concentric sclerosis (BCS) is a rare demyelinating disease, histopathologically characterized by large concentric lesions with circumferential rings of myelin loss alternating with rings of myelin preservation. BCS along with tumefactive Multiple Sclerosis (MS), Marburg and Schilder disease are considered types of fulminant demyelinating disorders of the central nervous system (CNS) [ 1 ]. BCS lesions have been considered to be a variant of pattern III of MS [ 2 , 3 ].…”