ECTRIMS eLearning

Baló's concentric sclerosis: study of clinico-radiological characteristics and therapeutic outcomes
Author(s): ,
D. Tzanetakos
Affiliations:
1st Department of Neurology, National and Kapodistrian University of Athens
,
I. Tzartos
Affiliations:
1st Department of Neurology, National and Kapodistrian University of Athens
,
M.E. Evangelopoulos
Affiliations:
1st Department of Neurology, National and Kapodistrian University of Athens
,
E. Andeadou
Affiliations:
1st Department of Neurology, National and Kapodistrian University of Athens
,
M. Anagnostouli
Affiliations:
1st Department of Neurology, National and Kapodistrian University of Athens
,
G. Koutsis
Affiliations:
1st Department of Neurology, National and Kapodistrian University of Athens
,
G. Velonakis
Affiliations:
Research Unit of Radiology - 2nd Department of Radiology, National and Kapodistrian University of Athens, Athens, Greece
,
P. Toulas
Affiliations:
Research Unit of Radiology - 2nd Department of Radiology, National and Kapodistrian University of Athens, Athens, Greece
C. Kilidireas
Affiliations:
1st Department of Neurology, National and Kapodistrian University of Athens
ECTRIMS Learn. Tzartos J. 10/10/18; 229155; EP1316
John Tzartos
John Tzartos
Contributions
Abstract

Abstract: EP1316

Type: Poster Sessions

Abstract Category: Clinical aspects of MS - MS Variants

Background and goals: Baló's Concentric Sclerosis(BCS) is a multiple sclerosis (MS) subtype characterized by concentric alternated lamenae of normal and destroyed myelin, histopathologically more consistent with type III pattern of MS. Our goal was to study MRI characteristics and therapeutic outcomes in BCS.
Methods: Our study included three patients with acute symptom onset due to a BCS lesion on brain MRI.
Results: Patient-A a 30-year-old female, experienced dysarthria, left facial droop and left hand dexterity problems for four days, due to a mass lesion (3.10cm x2.85cm x3.05cm)involving the right centrum semiovale with contrast enhancement (Gd+). Patient-B a 24-year-old man presented with right-sided hemiparesis and hemianaesthesia; a large Gd+ lesion (2.8cm x2.47cm x2.45cm) of the left corona radiata was revealed. Patient-C developed Wernicke´s aphasia and right-sided hemianaesthesia as a result of a Gd+ concentric lesion(2.6cm x1.8cm x1.2cm) involving the left corona radiata and the adjacent parietal subcortical white matter. These lesions were solitary, tumefactive, with hyperintense-isointense-hypointense concentric rings on T2-weighted images. On DWI images concentric layers of high and low intensity were noticed without low signal on ADC map(T2 shine-through)with the exception of a part of the outer layer which showed minimal restriction. All the patients initially received intravenous methylprednisolone course with poor response, while one underwent seven plasmapheresis sessions with subsequent MRI activity deterioration. However, after immunosuppresive treatment they all had a remarkable clinical response, no Gd+ and significant reduction of lesion size : patient-A 1.88cm x1.35cm x1.75cm, patient-B 1.74cm x1.37 x1cm, patient-C 1.93cm x1.61cm x1cm. Two patients after six monthly IV pulses of cyclophosphamide(800mg/m2/pulse), while the third after four doses of IV mitoxantrone(10mg/m2 every 3months).
Conclusions: Immunosuppresive treatment with cyclophoshamide or mitoxantrone seems to be the basis of therapeutics in BCS as it can affect both activated microglia and macrophages that highly express nitric oxide synthetase associated with apoptosis.
Disclosure: C. Kilidireas has received grants and honoraria from: Bayer, Biogen, Genesis Pharma, Merck-Serono, Novartis, Sanofi - Genzyme, Teva.
G. Koutsis has received research grants from Genesis Pharma and Teva, consultation fees, advisory board renumeration and honoraria from Genzyme, Genesis Pharma, Teva and Novartis.
M.E. Evangelopoulos has provided consultation services for and received honoraria from Novartis, Biogen and Teva.
M. Anagnostouli has received research grants from Biogen, Merck-Serono, Novartis, Teva, Bayer and Genzyme, as well as lecture-fees from Novartis, Teva, Biogen and Genzyme.
E. Andreadou has received research grants from Biogen, Merck-Serono, Novartis, and Sanofi Aventis, as well as lecture-fees from Teva.
D. Tzanetakos : nothing to disclose.
G. Velonakis : nothing to disclose.
P. Toulas : nothing to disclose.
J. Tzartos : nothing to disclose.

Abstract: EP1316

Type: Poster Sessions

Abstract Category: Clinical aspects of MS - MS Variants

Background and goals: Baló's Concentric Sclerosis(BCS) is a multiple sclerosis (MS) subtype characterized by concentric alternated lamenae of normal and destroyed myelin, histopathologically more consistent with type III pattern of MS. Our goal was to study MRI characteristics and therapeutic outcomes in BCS.
Methods: Our study included three patients with acute symptom onset due to a BCS lesion on brain MRI.
Results: Patient-A a 30-year-old female, experienced dysarthria, left facial droop and left hand dexterity problems for four days, due to a mass lesion (3.10cm x2.85cm x3.05cm)involving the right centrum semiovale with contrast enhancement (Gd+). Patient-B a 24-year-old man presented with right-sided hemiparesis and hemianaesthesia; a large Gd+ lesion (2.8cm x2.47cm x2.45cm) of the left corona radiata was revealed. Patient-C developed Wernicke´s aphasia and right-sided hemianaesthesia as a result of a Gd+ concentric lesion(2.6cm x1.8cm x1.2cm) involving the left corona radiata and the adjacent parietal subcortical white matter. These lesions were solitary, tumefactive, with hyperintense-isointense-hypointense concentric rings on T2-weighted images. On DWI images concentric layers of high and low intensity were noticed without low signal on ADC map(T2 shine-through)with the exception of a part of the outer layer which showed minimal restriction. All the patients initially received intravenous methylprednisolone course with poor response, while one underwent seven plasmapheresis sessions with subsequent MRI activity deterioration. However, after immunosuppresive treatment they all had a remarkable clinical response, no Gd+ and significant reduction of lesion size : patient-A 1.88cm x1.35cm x1.75cm, patient-B 1.74cm x1.37 x1cm, patient-C 1.93cm x1.61cm x1cm. Two patients after six monthly IV pulses of cyclophosphamide(800mg/m2/pulse), while the third after four doses of IV mitoxantrone(10mg/m2 every 3months).
Conclusions: Immunosuppresive treatment with cyclophoshamide or mitoxantrone seems to be the basis of therapeutics in BCS as it can affect both activated microglia and macrophages that highly express nitric oxide synthetase associated with apoptosis.
Disclosure: C. Kilidireas has received grants and honoraria from: Bayer, Biogen, Genesis Pharma, Merck-Serono, Novartis, Sanofi - Genzyme, Teva.
G. Koutsis has received research grants from Genesis Pharma and Teva, consultation fees, advisory board renumeration and honoraria from Genzyme, Genesis Pharma, Teva and Novartis.
M.E. Evangelopoulos has provided consultation services for and received honoraria from Novartis, Biogen and Teva.
M. Anagnostouli has received research grants from Biogen, Merck-Serono, Novartis, Teva, Bayer and Genzyme, as well as lecture-fees from Novartis, Teva, Biogen and Genzyme.
E. Andreadou has received research grants from Biogen, Merck-Serono, Novartis, and Sanofi Aventis, as well as lecture-fees from Teva.
D. Tzanetakos : nothing to disclose.
G. Velonakis : nothing to disclose.
P. Toulas : nothing to disclose.
J. Tzartos : nothing to disclose.

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