Kutsuna Fumiya, Ueno Miki, Tokuda Masahiro, Iwanaga Hiroshi, Tsutsumi Keisuke
Department of Neurology, National Hospital Organization Nagasaki Medical Center.
Present address: Department of Internal Medicine, Terasawa Hospital.
Rinsho Shinkeigaku. 2022 Mar 29;62(3):224-230. doi: 10.5692/clinicalneurol.cn-001697. Epub 2022 Mar 25.
We present an adult case of mild encephalitis/encephalopathy with a reversible splenial lesion (MERS). A 26-year-old man with a family history of MERS presented to our hospital owing to dysarthria and dysesthesia of the right side of his body. The duration of these symptoms was approximately 4 hours. T and diffusion weighted MRI showed high signal intensity lesions in the bilateral deep white matter. On a 3-week follow-up MRI, the lesions had completely disappeared. We attributed this clinical course and image findings to MERS. The patient had experienced similar symptoms at the age of 8 years old. Furthermore, his younger brother showed a similar clinical history and experienced a few recurrence events during the age of 9-16 years old. The mechanism of MERS remains controversial. However, similar to our case, there are some case reports with a family history. In addition, a previous report has confirmed the existence of a heterozygous variant in the myelin regulatory factor gene in patients with MERS. Some genetic factors may induce MERS, especially with extensive white matter lesions.
我们报告一例成人轻度脑炎/脑病伴可逆性胼胝体压部病变（MERS）。一名有MERS家族史的26岁男性因构音障碍和身体右侧感觉异常前来我院就诊。这些症状持续时间约4小时。T加权和弥散加权磁共振成像（MRI）显示双侧深部白质有高信号强度病变。在3周后的随访MRI检查中，病变已完全消失。我们将此临床过程和影像表现归因于MERS。该患者8岁时曾出现过类似症状。此外，他的弟弟有相似的临床病史，在9至16岁期间经历过几次复发事件。MERS的发病机制仍存在争议。然而，与我们的病例相似，有一些病例报告也有家族史。此外，先前的一份报告已证实MERS患者的髓鞘调节因子基因存在杂合变异。一些遗传因素可能诱发MERS，尤其是伴有广泛白质病变时。
