Contributions
Abstract: EP1346
Type: ePoster
Abstract Category: Clinical aspects of MS - MS Variants
Balo´s concentric sclerosis (BCS) is a rare demyelinating disease usually affecting young adults. The disease generally has a progressive course and is characterized pathologically and radiologically by the appearance of concentric rings of demyelinated and relatively myelin-preserved white matter, which look like "onion bulbs". Some authors consider this disease to be the type of multiple sclerosis (MS), but an individual subheading in the International Disease Classification determines individual treatment strategies. There is still no univocal opinion about administration of the disease-modifying therapies (DMTs) to the patients with BCS.
Since 2010 until present we have examined 10 patients with MRI findings typical for BCS. Nearly all of them were directed to us being suspected of brain tumor - the fact that reveals difficulties in BCS diagnosing.
Nine of ten patients had an acute manifestation. Their neurological symptoms were stopped by pulse therapy with methylprednisolone. In one case an additional course of plasmapheresis was given and a single mitoxantrone infusion was made. CSF and serum samples of 5 patients were submitted to isoelectric focusing with immunodetection of Ig G and all of them showed the second type of CSF and serum staining: oligoclonal IgG bands only in CSF, not in serum.
Currently 7 patients are under follow-up observation. White matter plaques typical for MS as well as concentric lesions typical for Balo disease were found on the MRI follow-up images. Two patients with the longest observation period had recurrent clinical attacks and new demyelinating lesions were found during further MRI examination. One woman who had suffered a clinical attack got pregnant in 2014 and had a trouble-free pregnancy and normal delivery. Afterwards her well-being, neurological status and MRI didn"t show negative dynamics.
Conclusion: Today BCS is a demyelinating disease which remains complicated in the diagnostic aspects. Legible diagnostic criteria and therapeutic standards haven´t been worked out until present. Emergence of new MS-type lesions together with the presence of intrathecal IgG production gives evidence that BCS is a specific form of MS.
In cases of remitting disease course characterized by new episodes of development or worsening of the clinical symptoms and/or emergence of “MRI activity” (appearance of new and/or cumulating contrast lesions on the MRI follow-up images), treatment by DMTs seems appropriate.
Disclosure: The authors declare no conflicts of interest in preparing this article.
Abstract: EP1346
Type: ePoster
Abstract Category: Clinical aspects of MS - MS Variants
Balo´s concentric sclerosis (BCS) is a rare demyelinating disease usually affecting young adults. The disease generally has a progressive course and is characterized pathologically and radiologically by the appearance of concentric rings of demyelinated and relatively myelin-preserved white matter, which look like "onion bulbs". Some authors consider this disease to be the type of multiple sclerosis (MS), but an individual subheading in the International Disease Classification determines individual treatment strategies. There is still no univocal opinion about administration of the disease-modifying therapies (DMTs) to the patients with BCS.
Since 2010 until present we have examined 10 patients with MRI findings typical for BCS. Nearly all of them were directed to us being suspected of brain tumor - the fact that reveals difficulties in BCS diagnosing.
Nine of ten patients had an acute manifestation. Their neurological symptoms were stopped by pulse therapy with methylprednisolone. In one case an additional course of plasmapheresis was given and a single mitoxantrone infusion was made. CSF and serum samples of 5 patients were submitted to isoelectric focusing with immunodetection of Ig G and all of them showed the second type of CSF and serum staining: oligoclonal IgG bands only in CSF, not in serum.
Currently 7 patients are under follow-up observation. White matter plaques typical for MS as well as concentric lesions typical for Balo disease were found on the MRI follow-up images. Two patients with the longest observation period had recurrent clinical attacks and new demyelinating lesions were found during further MRI examination. One woman who had suffered a clinical attack got pregnant in 2014 and had a trouble-free pregnancy and normal delivery. Afterwards her well-being, neurological status and MRI didn"t show negative dynamics.
Conclusion: Today BCS is a demyelinating disease which remains complicated in the diagnostic aspects. Legible diagnostic criteria and therapeutic standards haven´t been worked out until present. Emergence of new MS-type lesions together with the presence of intrathecal IgG production gives evidence that BCS is a specific form of MS.
In cases of remitting disease course characterized by new episodes of development or worsening of the clinical symptoms and/or emergence of “MRI activity” (appearance of new and/or cumulating contrast lesions on the MRI follow-up images), treatment by DMTs seems appropriate.
Disclosure: The authors declare no conflicts of interest in preparing this article.