A 38-year-old man presented to our centre with subacute cognitive decline and right limb weakness. His memory had been normal until 5 months before, initially having difficulty recalling friends’ names. Over the next 3 months, he became sleepy and lacked interest in social activities. Gradually his right limbs became inflexible, interfering with tooth brushing and walking. An MR scan of brain at another hospital showed asymmetric confluent white matter hyperintensities, mild temporal lobe atrophy and normal intracranial arteries (figure 1). He had been treated elsewhere with intravenous immunoglobulins and corticosteroids for presumed antibody-negative autoimmune encephalitis, but his symptoms had continued to worsen despite regular immunotherapy, such that he could no longer talk or walk. Figure 1 MR scan of brain series (A) FLAIR and (B) enhanced MR scan of brain 3 months after onset showed confluent white matter hyperintensities involving left fronto-temporo-insular lobe, basal ganglia and thalamus, with mild temporal lobe atrophy and faint patchy enhancement. Repeat (C) FLAIR and (D) enhanced MR scan of brain 5 months after onset showed prominent left temporal lobe atrophy. FLAIR, fluid-attenuated inversion recovery. He had been previously well and was immunocompetent. There was no history of substance abuse or toxin exposure and no relevant family history. On examination, he was alert with mixed aphasia. Cognitive assessment identified impaired temporal and spatial orientation and problems with long-term and short-term memory. He could not complete calculation or the Mini-Mental State Examination. There was no meningism. He had a right facial palsy. The strength in his right arm and leg was rated as 3/5 proximally and 0/5 distally; left-sided limb strength was normal. Deep tendon reflexes were 4+ on the right, with inducible ankle clonus, and 3+ on the left. The right plantar response was extensor. Sensation testing was limited by cognitive deficits. His sensation to painful stimuli …

中文翻译：

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伴有进行性脑萎缩的白质脑病

一名 38 岁的男性因亚急性认知能力下降和右肢无力而到我们中心就诊。直到 5 个月前他的记忆力一直正常，最初难以回忆朋友的名字。在接下来的 3 个月里，他变得困倦，对社交活动缺乏兴趣。渐渐地，他的右肢变得僵硬，妨碍了刷牙和行走。另一家医院的脑部 MR 扫描显示不对称的融合白质高信号，颞叶轻度萎缩和颅内动脉正常（图 1）。他曾在其他地方接受过静脉注射免疫球蛋白和皮质类固醇治疗推测的抗体阴性自身免疫性脑炎，但尽管进行了常规免疫治疗，但他的症状继续恶化，以至于他不再能说话或走路。图 1 脑部 MR 扫描系列 (A) FLAIR 和 (B) 脑部增强 MR 扫描 发病 3 个月后显示融合的白质高信号，累及左侧额颞岛叶、基底节和丘脑，伴有轻度颞叶萎缩和微弱斑驳的增强。发病 5 个月后重复 (C) FLAIR 和 (D) 增强的脑部 MR 扫描显示明显的左颞叶萎缩。FLAIR，流体衰减反转恢复。他以前身体很好，免疫能力强。没有药物滥用或毒素暴露史，也没有相关家族史。检查时，他有混合性失语症的警觉。认知评估确定了受损的时间和空间方向以及长期和短期记忆的问题。他无法完成计算或简易精神状态检查。没有脑膜炎。他患有右侧面瘫。他的右臂和右腿的力量被评为近端 3/5 和远端 0/5；左侧肢体力量正常。右侧深腱反射为 4+，可诱发踝阵挛，左侧为 3+。正确的足底反应是伸肌。感觉测试受到认知缺陷的限制。他对疼痛刺激的感觉……