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.
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.
Mult Scler Relat Disord. 2020 Jul;42:102078. doi: 10.1016/j.msard.2020.102078. Epub 2020 May 4.
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).
To describe 6 BCS patients. To review the risk of developing MS in BCS patients.
We retrospectively recorded clinical and radiological findings of 6 BCS patients and performed a review of the literature.
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.
巴洛氏同心性硬化症(BCS)是一种罕见的中枢神经系统疾病,其特征是 MRI 上出现脱髓鞘的交替带。其中一个主要问题是它与多发性硬化症(MS)的关系。
描述 6 例 BCS 患者。回顾 BCS 患者发展为 MS 的风险。
我们回顾性地记录了 6 例 BCS 患者的临床和影像学发现,并对文献进行了回顾。
纳入 6 名(5 名女性)平均年龄 25 岁的患者。主要症状为偏瘫/偏身感觉减退。在 MRI 上,2 例患者有单个 BCS 病变,4 例患者有类似 MS 的病变。交替带通常在 DWI 上更明显。1 例患者存在中央灌注减少和 SWI 低信号,提示中央静脉。5/6 例患者检测到寡克隆带。经过 7 年的随访,所有患者均符合 MS 标准,且残疾程度较轻(平均 EDSS 1.75;0-4)。我们的文献复习包括来自 30 项研究的 65 例 BCS 患者:尽管 CSF 寡克隆带和存在额外的 MS 病变与随后的复发相关,但这并不显著。
讨论/结论:我们的系列研究允许对 BCS 进行详细的 MRI 描述,并对 BCS 的演变及其与 MS 的密切关系提供新的见解。