A previously independent 64-year-old man presented to his local hospital with new-onset diplopia and dysarthria while watching television. Over the following days, he developed painless perioral and upper limb sensory disturbance, gait unsteadiness and limb ataxia, most pronounced in the upper limbs. He was initially admitted and treated for a cerebrovascular event, followed by a period of failed rehabilitation. At 6-week review, his symptoms had worsened, and he required the assistance of two people to walk short distances. He was then transferred to a regional neurology unit. It was uncertain whether his symptoms had stabilised or progressed over the preceding fortnight. He had no other neurological symptoms, normal bladder and bowel function, and no history of preceding illness. Table 1 provides the background history. View this table: Table 1 Patient’s clinical and social background Assessment of eye movements identified a complex ophthalmoplegia, with complete failure of right eye abduction. Upgaze was bilaterally limited to 45° and adduction was partially restricted. There was no nystagmus. He described perioral numbness and was unaware of food boluses within his mouth, though objective testing was normal. Tongue movements were slowed but symmetrical. There was no fasciculation and his swallow appeared normal. His speech was dysarthric but intelligible and had a subtle nasal character. There were no other cranial nerve signs. Neck flexion and extension were normal. Tone was normal in all four limbs. His strength was symmetrically reduced in the upper limbs with the Medical Research Council (MRC) grade 4 proximally and 4− in the small finger muscles. Lower limb strength was MRC grade 4+ at hip flexion and 5 in all other muscle groups. He could not stand due to unsteadiness and had pronounced ataxia in all limbs. There was profound loss of proprioception to the shoulders in his arms with pseudoathetosis, with no corresponding lower limb involvement. Vibration …

中文翻译：

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伴有颅神经麻痹的感觉性共济失调

一名以前独立的 64 岁男子在看电视时因新发复视和构音障碍到当地医院就诊。在接下来的几天里，他出现了无痛的口周和上肢感觉障碍、步态不稳和肢体共济失调，最明显的是上肢。他最初因脑血管事件入院并接受治疗，随后一段时间的康复失败。在 6 周复查时，他的症状恶化了，他需要两个人的帮助才能步行短距离。然后他被转移到一个区域神经病学单位。不确定他的症状在前两周是否稳定或进展。他没有其他神经系统症状，膀胱和肠道功能正常，也没有既往病史。表 1 提供了背景历史。查看此表：表 1 患者的临床和社会背景 眼球运动评估确定了复杂的眼肌麻痹，右眼外展完全失败。双侧向上凝视限制在 45°，内收部分受限。没有眼球震颤。他描述了口周麻木并且不知道他嘴里有食物团块，尽管客观测试是正常的。舌头运动缓慢但对称。没有震颤，他的吞咽看起来很正常。他的讲话是构音障碍，但可以理解，并带有微妙的鼻音特征。没有其他颅神经体征。颈部屈伸正常。四肢的音调正常。他的上肢力量对称性降低，医学研究委员会 (MRC) 近端 4 级和小指肌肉 4- 级。下肢力量在髋关节屈曲时为 MRC 4+ 级，在所有其他肌肉群中为 5 级。他因不稳而无法站立，四肢明显共济失调。假性手足徐动症患者双臂肩部本体感觉严重丧失，下肢无相应受累。振动 …