Xavier Ayrignac1, Laurent Letourneau-Guillon2, Clarisse Carra-Dallière3, Pierre Duquette4, Marc Girard4, José Poirier4, Boaz Lahav4, Catherine Larochelle4, Alexandre Prat4. 1. Clinique de Sclérose en plaques du Centre de Recherche du Centre Hospitalier de l'Université de Montréal (CRCHUM), and department of Neurosciences, Faculty of Medicine, Université de Montréal, Montreal, Quebec, Canada; Département de Neurologie, CRC sclérose en plaques, CHU Montpellier, INSERM, Univ Montpellier, Montpellier, France. Electronic address: xavier.ayrignac@yahoo.fr. 2. Département de Radiologie et Centre de Recherche du Centre Hospitalier de l'Université de Montréal (CRCHUM), Centre Hospitalier de l'Université de Montréal (CHUM), département de radiologie, radio-oncologie et médecine nucléaire, faculté de médecine, Université de Montréal, Montréal, Québec, Canada. 3. Département de Neurologie, CRC sclérose en plaques, CHU Montpellier, INSERM, Univ Montpellier, Montpellier, France. 4. Clinique de Sclérose en plaques du Centre de Recherche du Centre Hospitalier de l'Université de Montréal (CRCHUM), and department of Neurosciences, Faculty of Medicine, Université de Montréal, Montreal, Quebec, Canada.
Abstract
INTRODUCTION: Baló's concentric sclerosis (BCS) is a rare CNS disorder characterized by alternating bands of demyelination on MRI. One of the main issues is its relationship with multiple sclerosis (MS). OBJECTIVES: To describe 6 BCS patients. To review the risk of developing MS in BCS patients. METHODS: We retrospectively recorded clinical and radiological findings of 6 BCS patients and performed a review of the literature. RESULTS: Six patients (5 women) with a mean age of 25 years old were included. Main symptoms were hemiparesis/hemihypoesthesia. On MRI, two patients had a single BCS lesion and four had additional MS-like lesions. Alternating bands were usually more visible on DWI. A patient had reduced central perfusion and SWI hypointensity suggestive of a central vein. Oligoclonal bands were identified in 5/6 patients. After 7 years of follow-up, all patients achieved MS criteria with mild disability (mean EDSS 1.75; 0-4). Our literature review included 65 BCS patients from 30 studies: although CSF oligoclonal bands and the presence of additional MS lesions were associated with subsequent relapses, this was not significant. DISCUSSION/ CONCLUSION: Our series allows a detailed MRI description in BCS and gives a new insight into BCS evolution and its strong relationship with MS.
INTRODUCTION: Baló's concentric sclerosis (BCS) is a rare CNS disorder characterized by alternating bands of demyelination on MRI. One of the main issues is its relationship with multiple sclerosis (MS). OBJECTIVES: To describe 6 BCS patients. To review the risk of developing MS in BCS patients. METHODS: We retrospectively recorded clinical and radiological findings of 6 BCS patients and performed a review of the literature. RESULTS: Six patients (5 women) with a mean age of 25 years old were included. Main symptoms were hemiparesis/hemihypoesthesia. On MRI, two patients had a single BCS lesion and four had additional MS-like lesions. Alternating bands were usually more visible on DWI. A patient had reduced central perfusion and SWI hypointensity suggestive of a central vein. Oligoclonal bands were identified in 5/6 patients. After 7 years of follow-up, all patients achieved MS criteria with mild disability (mean EDSS 1.75; 0-4). Our literature review included 65 BCS patients from 30 studies: although CSF oligoclonal bands and the presence of additional MS lesions were associated with subsequent relapses, this was not significant. DISCUSSION/ CONCLUSION: Our series allows a detailed MRI description in BCS and gives a new insight into BCS evolution and its strong relationship with MS.
Authors: D Tzanetakos; A G Vakrakou; J S Tzartos; G Velonakis; M E Evangelopoulos; M Anagnostouli; G Koutsis; E Dardiotis; E Karavasilis; P Toulas; L Stefanis; C Kilidireas Journal: BMC Neurol Date: 2020-11-02 Impact factor: 2.474