Epub: Berghoff et al. 7 Tesla MRI demonstrates vascular pathology in Balo’s concentric sclerosis.Mult Scler. 2012 May 28.
Baló’s concentric sclerosis (BCS) is an inflammatory demyelinating disease related to MS; its underlying pathology remains unclear. At 7 T MRI in a 19-year-old female BCS patient, microhaemorrhages and ectatic veins were found in T2 hyperintense regions (white lesions), features which have not been previously reported in conjunction with BCS, and these findings may support the view that vascular pathology plays a role in BCS. MRS data suggest that neuron loss and lipid turnover still took place months after a remission. Plasma exchange was effective in treating a relapse with severe motor deficits, and the off-label use of natalizumab was successful in maintaining remission in this patient.
Balo disease, which is also known as concentric sclerosis, or encephalitis periaxialsis concentrica, or leukoencephalitis periaxalis concentric, is a rare progressive form or variant of MS. Although Balo disease usually develops in adults, primarily young adults, it can also occur as a variant of MS in children. Balo concentric sclerosis is a demyelinating disease similar to standard MS, but with the particularity that the demyelinated tissues form concentric layers. Balo’s concentric sclerosis, the rings may be caused by a physiological hypoxia (similar to that caused by some toxins or viruses) in the lesion, which is in turn countered by expression of stress proteins at the border. This expression and counter-expression forms rings of preserved tissue within the lesion and rings of demyelinated tissue just beyond where the previous attack had induced the protective stress proteins. Hence, subsequent attacks form concentric rings.
“The observation of vascular involvement in the lesion, which is common to most MS lesions, i.e. they are centred on small veins, is not support for CCSVI. The observation simply means that whatever triggers MS lesions is vascular centric; this is not surprising as the peripheral immune system communicates with the central nervous system via blood vessels in particular small venules. White blood cells cross over from the blood into the brain and spinal cord at specialised areas on veins. The observation that this patient responded to plasma exchange suggests an antibody mediated event.”
3 thoughts on “Research: Balos concentric sclerois”
The patient was originally diagnosed with RRMS and had been taking interferon B-1a 22mg (Rebif) for two years. She was then switched to natalizumab due to severe worsening of her condition, and after that her diagnosis was revised to BCS. She continued on natalizumab off-label.The writers of the study want to make a clear point on vascular pathology in BCS, beyond the simple vascular origin of the lesions. That's why they say:"The imaging findings of this case study support the view that vascular pathology contributes to the pathogenesis of BCS. BCS is classically considered to be a demyelinating disease; however, there is evidence for a vascular disease mechanism which may account for the peculiar ring structure."ps. ectatic vein is a swollen vein. By no means a healthy one, whatever the cause.
Is anyone doing current research. I may be able to help and provide info.
I am not aware of people working on this, ProfG may know