Tumefactive Multiple Sclerosis Variants: Report of Two Cases of Schilder and Balo Diseases

Abstract

A tumefactive lesion of central nervous system (CNS) is defined as a mass-like lesion with a size greater than 2 cm in brain detected by magnetic resonance imaging (MRI). Neuroimaging may help to distinguish the nature of a tumefactive lesion and therefore, can prevent an unnecessary brain biopsy. Here we emphasized on determining the nature of a CNS tumefactive lesions with the help of MRI and more explanations about demyelinating lesions with focus on Schilder and Balo diseases as two multiple sclerosis variants. We have reported here two boys of 10 and 8 years of age respectively of multiple sclerosis (MS) variants who presented with acute neurologic complications to our hospital as one of the two referral children hospital in Tehran, Iran. Tumefactive demyelinating lesions can be considered a separate entity that itself can contain Schilder disease, Balo disease, some cases of acute disseminated encephalomyelitis (ADEM) or classic MS. MRI can help to establish a diagnosis of a tumefactive lesion and to differentiate among different underlying etiologies.

Keywords: Balo Disease; Central Nervous System; Demyelination; Multiple Sclerosis Variants; Schilder Disease; Tumefactive Lesion.

Fig 1.

Initial brain MRI without contrast shows bilateral abnormal high signal lesions in the subcortical and deep posterior white matter in T2-WI sequence, almost isointense with CSF in the center, surrounded by a peripheral halo (a): After contrast injection In T1-WI images, low signal center with peripheral complete and incomplete ring enhancement is seen, suggesting necrotic or tumefactive center (b, c, d). Some cortical extension of the lesion in the right posterior parietal lobe is seen (Black arrow in d

Fig 2

Brain MRI with contrast showed remaining hyper signal in T2- FLARE from sequel of treated demyelinating lesions (a,b) and just disappearance of tumefactive lesions in T1-WI (c,d)

Fig 3

Brain CT scan with contrast demonstrated low attenuated subcortical areas in the right frontal and right lentiform nucleus without significant mass effect or post contrast enhancement (a-White arrow). The first brain MRI of the patient showed a couple of hypersignal lesions in T2-WI. The larger one is located in the right lentiform nucleus and insular subcortical white matter with surrounding halo (Large white arrow-b). The smaller one was located in the posterior limb of right internal capsule (Small white arrow-c

Fig 4

The second brain MRI revealed very large mass like concentric or layered high signal lesion in FLARE sequence in the right frontal lobe white matter and adjacent basal ganglia

Fig 5

The third brain MRI has done 6 months after biopsy, demonstrated volume loss in the right hemisphere with secondary ventricular prominence. Mass like lesion in the second brain MRI in the right frontal lobe could not be seen any longer (a,b) and abnormal periventricular white matter signal would suggest diffuse gliotic changes or less likely sequel of previous demyelinating lesion (c,d