Zhang Yin-Xi, Fang Gao-Li, Tang Jin-Long, Lai Qi-Lun
Department of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, 310009, China.
Department of Neurology, Zhejiang Chinese Medicine and Western Medicine Integrated Hospital, Hangzhou, 310003, China.
Heliyon. 2024 Jun 21;10(12):e33386. doi: 10.1016/j.heliyon.2024.e33386. eCollection 2024 Jun 30.
Baló's concentric sclerosis (BCS) is a rare type of central nervous system demyelinating disorder. Most patients with BCS are treated with corticosteroids, and spontaneous remission has seldom been described.
A 46-year-old man presented with a subacute-onset headache and memory loss. Brain magnetic resonance imaging (MRI) revealed multiple onion-shaped ring lesions with mild enhancement in the outermost ring. A brain biopsy revealed significant myelin loss. The diagnosis of BCS was established based on the MRI results and pathological findings. Interestingly, the patient recovered almost completely without immunotherapy, with repeated brain MRI at the 1-year follow-up showing an obvious reduction in the extent of the lesions.
Neurologists should improve the recognition of the typical MRI features of BCS to avoid unnecessary biopsies. Although rare, spontaneous remission can be observed in clinical practice.
巴洛同心圆性硬化(BCS)是一种罕见的中枢神经系统脱髓鞘疾病。大多数BCS患者接受皮质类固醇治疗,很少有自发缓解的报道。
一名46岁男性出现亚急性起病的头痛和记忆力减退。脑部磁共振成像(MRI)显示多个洋葱状环形病变,最外层有轻度强化。脑活检显示明显的髓鞘丢失。根据MRI结果和病理发现确诊为BCS。有趣的是,该患者未经免疫治疗几乎完全康复,1年随访时重复脑部MRI显示病变范围明显缩小。
神经科医生应提高对BCS典型MRI特征的认识,以避免不必要的活检。虽然罕见,但在临床实践中可观察到自发缓解。