MRI study of Bal�'s concentric sclerosis before and after immunosuppressive therapy

Abstract

An intra vitam diagnosis of Baló's concentric sclerosis in a 39-year-old male is described. To our knowledge this is the first reported case of ante-mortem diagnosis based solely on magnetic resonance imaging (MRI) findings. MRI showed features unique to this process. After high-dose intravenous therapy with prednisone, the clinical symptoms improved, and MRI showed marked changes of the concentric lesions.

MRI features of Balo's concentric sclerosis.

“…Over time, the MR abnormalities of BCS decrease in size and do not enhance with contrast medium. The duration of the characteristic concentric pattern is variable, ranging from 6.5 weeks to 1 year [3,6,8,9]. Bolay et al [9] suggested that the concentric pattern might be a transient feature in the development of BCS.…”

Balo‐s Concentric Sclerosis Report of Two Patients with Magnetic Resonance Imaging Follow‐up

“…The diagnosis has long been based on characteristic histopathological findings. However, Spiegel and coauthors [3] reported the first diagnosis of Balo's concentric sclerosis made solely by MR imaging. Nagara and colleagues [12] stated that MR study of formalin-fixed brain of a patient with Balo's disease was useful for correlating MR findings and the corresponding neuropathology.…”

“…Balo's concentric sclerosis is a rare demyelinating disease characterized by a concentric pattern of alternating bands containing demyelinated and partially myelinated white matter. It is known to affect young adults, have a rapid onset, and run a rather fulminant course [1][2][3]. Since the first description in 1928 [1], more than 50 cases have been reported.…”

“…In most cases, the diagnoses were made by histopathological examination of autopsy material [2]. The first antemortem diagnosis was made by needle biopsy in 1986 [4] and 5 more premortem diagnoses [3][4][5][6][7][8] have been reported so far. The diagnosis was made only by findings of the characteristic lamellar structure on magnetic resonance (MR) scans in 4 patients [3,[6][7][8].…”

Balo's concentric sclerosis associated with primary human herpesvirus 6 infection

“…[1][2][3] Although the conclusive diagnosis of BCS is confirmed via histopathology, MRI has made it possible to diagnose BCS intra vitam, demonstrating the characteristic onion bulb-like structure of the lesions. [4][5][6][7][8][9][10][11][12][13][14][15] Treatment of BCS with corticosteroids, [3][4][5][6][7][8][9][10][11][12][13] other immunosuppressants, [10][11][12] and plasmapheresis 13 is of varying efficacy.…”