Table 1.
Case | Age at onset (years) |
Sex | Symptoms at BCS onset | BCS-type lesion size (cm) at onset |
Pattern of BCS-type lesion Gd+ | Coexistence of brain MS-like plaques |
Barkof criteria fulfillment at BCS onset |
Spinal cord lesions at BCS onset | CSF OCBs |
Time to follow-up from BCS onset (months) |
Response to IVMP | Long-term Treatment |
---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | 24 | M | right-sided hemiparesis and hemianaesthesia | 2.8 | Peripheral Gd + at outer layer | (+) | No | (−) | (+) | 85 | poor | CYC |
2 | 30 | F | dysarthria,left facial palsy, left hand dexterity problems | 3.6 | Peripheral ring-like Gd + at outer layer | (−) | No | (−) | (+) | 34 | poor | CYC |
3 | 41 | M | Wernicke’s aphasia and right-sided hemianaesthesia | 2.6 | Peripheral ring-like Gd + at outer two layers | (+) | No | (−) | Not tested | 132 | poor |
1st MTX 2nd GA |
4 | 18 | F | blurred vision | 3.1 | Peripheral incomplete ring-like Gd + at outer two layers | (+) | Yes | (−) | (+) | 16 | excellent |
1st GA, 2nd NTZ |
5 | 18 | F | right-sided hemiparesis | 2 | Peripheral ringlike Gd + at outer layer | (+) | Yes | (+) | Not tested | 127 | good |
1st IFN-beta-1b, 2nd NTZ |
6 | 26 | M | dysarthria | 3.6 | Peripheral incomplete ringlike Gd + at outer two layers | (+) | Yes | (−) | (−) | 23 | poor | RTX |
7 | 26 | F | gait instability, mild left hemiparesis | 1.7 | peripheral Gd + at outer layer | (+) | No | (+) | (+) | 29 | poor | RTX |
8 | 27 | M | left facial nerve palsy | 1.8 | partial ringlike Gd+ | (+) | No | (−) | (−) | 9 | poor | CYC |
Abbreviations: BCS Baló’s Concentric Sclerosis, F Female, M: Male, Gd+ Gadolinium Enhancement, CSF OCBs Cerebrospinal Fluid Oligoclonal Bands, (+) presence, (−) absence, MS Multiple Sclerosis, IVMP intravenous methylprednisolone, CYC Cyclophosphamide, MTX Mitoxantrone, RTX Rituximab, GA Glatiramer acetate