PDF(Pubmed)
Abstract：
BACKGROUND: Myasthenia gravis is an autoimmune condition affecting the neuromuscular junction and causing muscle weakness along with fatigue (myasthenia). When the clinical manifestations of myasthenia gravis are isolated to the eye muscles, only causing weak eye movements, it is referred to as ocular myasthenia gravis, which can mimic a 1 and ½ syndrome.
METHODS: An African-American female in her fifties with past medical history of hypertension presented to our outpatient clinic with complaints of blurred vision for two weeks. Her symptoms were associated with facial discomfort and a generalized headache. On physical examination upon her initial presentation, there was demonstratable swelling of the left upper eyelid with drooping. Her extraocular movements revealed defects with the abduction and adduction of the right eye, and the left eye would not adduct, although the outward movement was normal. The left eye failed to lift/elevate completely when looking upwards, a pseudo 1 and ½ syndrome. A positive Cogan lid twitch was also noticed. Imaging of the brain and orbit ruled out central causes. Diagnosis of ocular myasthenia gravis was made in accordance with positive anti-acetylcholine receptor antibodies. With 120 mg pyridostigmine oral dose, the patient experienced improvement subjectively and objectively, and the patient was discharged on oral pyridostigmine and prednisone. Six months later, with prednisone having been tapered off, the patient developed a myasthenic crisis and was treated with plasmapheresis and intravenous immunoglobulins. After recovering from the myasthenic crisis, efgartigimod infusions were instituted, which helped our patient restore normal life.
CONCLUSIONS: Our patient who presented with \"blurred vision\" was discovered to have binocular diplopia due to significant dysconjugate eye movements. After diligently ruling out central etiologies, we concluded that her presentation was due to a peripheral etiology. Her serologies and her presentation helped confirm a diagnosis of ocular myasthenia gravis. Also, as in most cases, our patient also progressed to develop generalized myasthenia gravis while on pyridostigmine. Efgartigimod infusions instituted after our patient recovered from a myasthenic crisis have helped her restore a normal life.
METHODS: An African-American female in her fifties with past medical history of hypertension presented to our outpatient clinic with complaints of blurred vision for two weeks. Her symptoms were associated with facial discomfort and a generalized headache. On physical examination upon her initial presentation, there was demonstratable swelling of the left upper eyelid with drooping. Her extraocular movements revealed defects with the abduction and adduction of the right eye, and the left eye would not adduct, although the outward movement was normal. The left eye failed to lift/elevate completely when looking upwards, a pseudo 1 and ½ syndrome. A positive Cogan lid twitch was also noticed. Imaging of the brain and orbit ruled out central causes. Diagnosis of ocular myasthenia gravis was made in accordance with positive anti-acetylcholine receptor antibodies. With 120 mg pyridostigmine oral dose, the patient experienced improvement subjectively and objectively, and the patient was discharged on oral pyridostigmine and prednisone. Six months later, with prednisone having been tapered off, the patient developed a myasthenic crisis and was treated with plasmapheresis and intravenous immunoglobulins. After recovering from the myasthenic crisis, efgartigimod infusions were instituted, which helped our patient restore normal life.
CONCLUSIONS: Our patient who presented with \"blurred vision\" was discovered to have binocular diplopia due to significant dysconjugate eye movements. After diligently ruling out central etiologies, we concluded that her presentation was due to a peripheral etiology. Her serologies and her presentation helped confirm a diagnosis of ocular myasthenia gravis. Also, as in most cases, our patient also progressed to develop generalized myasthenia gravis while on pyridostigmine. Efgartigimod infusions instituted after our patient recovered from a myasthenic crisis have helped her restore a normal life.
摘要：
背景：重症肌无力是一种自身免疫性疾病，影响神经肌肉接头并引起肌肉无力和疲劳（肌无力）。当重症肌无力的临床表现被隔离到眼部肌肉时，只会导致微弱的眼球运动，它被称为眼部重症肌无力，可以模拟1和1/2综合征。
方法：一名50多岁的非裔美国女性，既往有高血压病史，出现在我们的门诊，主诉视力模糊两周。她的症状与面部不适和全身头痛有关。在她初次陈述时的身体检查中，左上眼睑有明显肿胀伴下垂。她的眼外运动显示出右眼外展和内收的缺陷，左眼不会加成，虽然向外运动是正常的。向上看时，左眼未能完全抬起/抬起，伪1和½综合征。还注意到Cogan盖子抽搐。大脑和眼眶的成像排除了中央原因。根据抗乙酰胆碱受体抗体阳性对眼肌重症肌无力进行诊断。口服120毫克吡啶斯的明，患者主观和客观地经历了改善，患者口服吡啶斯的明和泼尼松出院。六个月后,泼尼松已经逐渐减少，患者出现肌无力危象，接受血浆置换和静脉注射免疫球蛋白治疗。从肌无力危机中恢复过来后，开始了efgartigimod输注，帮助我们的病人恢复正常生活.
结论：我们的患者出现“视力模糊”，被发现是由于明显的眼球运动异常而导致的双眼复视。在努力排除中心病因后，我们的结论是，她的表现是由于外周病因。她的血清学和表现有助于确认眼部重症肌无力的诊断。此外，在大多数情况下，我们的患者在服用吡啶斯的明时也发展为全身性重症肌无力。在我们的患者从肌无力危机中康复后开始的Efgartigimod输注帮助她恢复正常生活。
方法：一名50多岁的非裔美国女性，既往有高血压病史，出现在我们的门诊，主诉视力模糊两周。她的症状与面部不适和全身头痛有关。在她初次陈述时的身体检查中，左上眼睑有明显肿胀伴下垂。她的眼外运动显示出右眼外展和内收的缺陷，左眼不会加成，虽然向外运动是正常的。向上看时，左眼未能完全抬起/抬起，伪1和½综合征。还注意到Cogan盖子抽搐。大脑和眼眶的成像排除了中央原因。根据抗乙酰胆碱受体抗体阳性对眼肌重症肌无力进行诊断。口服120毫克吡啶斯的明，患者主观和客观地经历了改善，患者口服吡啶斯的明和泼尼松出院。六个月后,泼尼松已经逐渐减少，患者出现肌无力危象，接受血浆置换和静脉注射免疫球蛋白治疗。从肌无力危机中恢复过来后，开始了efgartigimod输注，帮助我们的病人恢复正常生活.
结论：我们的患者出现“视力模糊”，被发现是由于明显的眼球运动异常而导致的双眼复视。在努力排除中心病因后，我们的结论是，她的表现是由于外周病因。她的血清学和表现有助于确认眼部重症肌无力的诊断。此外，在大多数情况下，我们的患者在服用吡啶斯的明时也发展为全身性重症肌无力。在我们的患者从肌无力危机中康复后开始的Efgartigimod输注帮助她恢复正常生活。
