PDF(Pubmed)
Abstract：
BACKGROUND: Uraemia causes a generalised encephalopathy as its most common neurological complication. Isolated brainstem uraemic encephalopathy is rare. We report a case of fatigable ptosis and complex ophthalmoplegia in brainstem uraemic encephalopathy.
METHODS: A 22-year-old Sri Lankan man with end stage renal failure presented with acute onset diplopia and drooping of eyelids progressively worsening over one week. The patient had not complied with the prescribed renal replacement therapy which was planned to be initiated 5 months previously. On examination, his Glasgow coma scale score was 15/15, He had a fatigable asymmetrical bilateral ptosis. The ice-pack test was negative. There was a complex ophthalmoplegia with bilateral abduction failure and elevation failure of the right eye. The diplopia did not worsen with prolonged stare. The rest of the neurological examination was normal. Serum creatinine on admission was 21.81 mg/dl. The repetitive nerve stimulation did not show a decremental pattern. Magnetic resonance imaging (MRI) of the brain demonstrated diffuse midbrain and pontine oedema with T2 weighted/FLAIR hyperintensities. The patient was haemodialyzed on alternate days and his neurological deficits completely resolved by the end of the second week of dialysis. The follow up brain MRI done two weeks later demonstrated marked improvement of the brainstem oedema with residual T2 weighted/FLAIR hyperintensities in the midbrain.
CONCLUSIONS: Uraemia may rarely cause an isolated brainstem encephalopathy mimicking ocular myasthenia, which resolves with correction of the uraemia.
METHODS: A 22-year-old Sri Lankan man with end stage renal failure presented with acute onset diplopia and drooping of eyelids progressively worsening over one week. The patient had not complied with the prescribed renal replacement therapy which was planned to be initiated 5 months previously. On examination, his Glasgow coma scale score was 15/15, He had a fatigable asymmetrical bilateral ptosis. The ice-pack test was negative. There was a complex ophthalmoplegia with bilateral abduction failure and elevation failure of the right eye. The diplopia did not worsen with prolonged stare. The rest of the neurological examination was normal. Serum creatinine on admission was 21.81 mg/dl. The repetitive nerve stimulation did not show a decremental pattern. Magnetic resonance imaging (MRI) of the brain demonstrated diffuse midbrain and pontine oedema with T2 weighted/FLAIR hyperintensities. The patient was haemodialyzed on alternate days and his neurological deficits completely resolved by the end of the second week of dialysis. The follow up brain MRI done two weeks later demonstrated marked improvement of the brainstem oedema with residual T2 weighted/FLAIR hyperintensities in the midbrain.
CONCLUSIONS: Uraemia may rarely cause an isolated brainstem encephalopathy mimicking ocular myasthenia, which resolves with correction of the uraemia.
摘要：
背景：尿毒症会导致广泛性脑病，是其最常见的神经系统并发症。孤立的脑干尿毒症脑病是罕见的。我们报告了一例脑干尿毒症脑病的疲劳下垂和复杂的眼肌麻痹。
方法：一名22岁的斯里兰卡晚期肾衰竭男子表现为急性复视，眼睑下垂在一周内逐渐恶化。患者未遵守计划于5个月前开始的规定的肾脏替代疗法。在检查中，他的格拉斯哥昏迷评分为15/15，他有一个疲劳不对称的双侧下垂。冰袋测试是阴性的。有一个复杂的眼肌麻痹，双侧外展失败和右眼抬高失败。长时间凝视并没有使复视恶化。其余神经系统检查正常。入院时血清肌酐为21.81mg/dl。重复的神经刺激没有显示出衰减模式。大脑的磁共振成像（MRI）显示弥漫性中脑和脑桥水肿，T2加权/FLAIR高强度。患者隔天进行血液透析，在透析的第二周结束时，他的神经功能缺损完全解决。两周后进行的随访脑MRI显示，脑干水肿的明显改善，中脑中残留的T2加权/FLAIR高信号。
结论：尿毒症很少会引起孤立的脑干脑病，模仿眼部肌无力，通过纠正尿毒症来解决。
方法：一名22岁的斯里兰卡晚期肾衰竭男子表现为急性复视，眼睑下垂在一周内逐渐恶化。患者未遵守计划于5个月前开始的规定的肾脏替代疗法。在检查中，他的格拉斯哥昏迷评分为15/15，他有一个疲劳不对称的双侧下垂。冰袋测试是阴性的。有一个复杂的眼肌麻痹，双侧外展失败和右眼抬高失败。长时间凝视并没有使复视恶化。其余神经系统检查正常。入院时血清肌酐为21.81mg/dl。重复的神经刺激没有显示出衰减模式。大脑的磁共振成像（MRI）显示弥漫性中脑和脑桥水肿，T2加权/FLAIR高强度。患者隔天进行血液透析，在透析的第二周结束时，他的神经功能缺损完全解决。两周后进行的随访脑MRI显示，脑干水肿的明显改善，中脑中残留的T2加权/FLAIR高信号。
结论：尿毒症很少会引起孤立的脑干脑病，模仿眼部肌无力，通过纠正尿毒症来解决。
