PDF(Pubmed)
Abstract：
OBJECTIVE: To report a case of sudden onset vertical diplopia, blurred vision, and muscle spasms.
METHODS: This is a case report of a 57-year-old female who presented to the accident and emergency department with a one day history of vertical diplopia and a two week history of lower limb spasticity secondary to muscle spasms.
RESULTS: The patient had no significant medical or ocular history. Orthoptic investigation initially revealed a left inferior rectus (IR) underaction. Possible diagnoses at this point were thought to be isolated IR weakness, myasthenia gravis or skew deviation. An urgent MRI scan was arranged and blood tests were taken. MRI showed no abnormalities. Blood tests were normal, however, the acetylcholine receptor antibody serum test (ACH-R) was 0.43 nmol/L, which is at the high end of normal. At the follow-up visit, the IR weakness had deteriorated and the patient had also developed gaze-evoked nystagmus. An appointment with the neurologist and neuro-ophthalmologist was expedited. When the patient returned, she reported that her neurologist had diagnosed her with stiff-person syndrome (SPS). The patient had also developed a laterally alternating skew deviation and reported that she had undergone a course of intravenous immunoglobulin (IVIG). The patient was prescribed diazepam and gabapentin. Due to the lack of recovery, persistent diplopia and oscillopsia, monthly IVIG have been prescribed.
CONCLUSIONS: There is currently a paucity of literature relating to ophthalmic problems with SPS and how they are best treated. Previous reports have established that there is a link with myasthenia gravis, with many patients going on to develop myasthenia. Treatment of SPS is lacking large evidence-based studies. However, treatment with muscle relaxants and anticonvulsants has provided a good outcome for some. Further research is required to develop an evidence-based approach to treating the ophthalmological problems patients with SPS experience. This case report highlights the importance and value of orthoptists in investigating and monitoring patients with stiff-person syndrome.
METHODS: This is a case report of a 57-year-old female who presented to the accident and emergency department with a one day history of vertical diplopia and a two week history of lower limb spasticity secondary to muscle spasms.
RESULTS: The patient had no significant medical or ocular history. Orthoptic investigation initially revealed a left inferior rectus (IR) underaction. Possible diagnoses at this point were thought to be isolated IR weakness, myasthenia gravis or skew deviation. An urgent MRI scan was arranged and blood tests were taken. MRI showed no abnormalities. Blood tests were normal, however, the acetylcholine receptor antibody serum test (ACH-R) was 0.43 nmol/L, which is at the high end of normal. At the follow-up visit, the IR weakness had deteriorated and the patient had also developed gaze-evoked nystagmus. An appointment with the neurologist and neuro-ophthalmologist was expedited. When the patient returned, she reported that her neurologist had diagnosed her with stiff-person syndrome (SPS). The patient had also developed a laterally alternating skew deviation and reported that she had undergone a course of intravenous immunoglobulin (IVIG). The patient was prescribed diazepam and gabapentin. Due to the lack of recovery, persistent diplopia and oscillopsia, monthly IVIG have been prescribed.
CONCLUSIONS: There is currently a paucity of literature relating to ophthalmic problems with SPS and how they are best treated. Previous reports have established that there is a link with myasthenia gravis, with many patients going on to develop myasthenia. Treatment of SPS is lacking large evidence-based studies. However, treatment with muscle relaxants and anticonvulsants has provided a good outcome for some. Further research is required to develop an evidence-based approach to treating the ophthalmological problems patients with SPS experience. This case report highlights the importance and value of orthoptists in investigating and monitoring patients with stiff-person syndrome.
摘要：
目的：报告一例突发性垂直复视，视力模糊,肌肉痉挛.
方法：这是一例57岁女性患者就诊于急症室的病例报告，患者有1天的垂直复视病史和2周的继发于肌肉痉挛的下肢痉挛病史。
结果：患者无明显病史或眼部病史。视神经检查最初显示左下直肌（IR）作用不足。在这一点上可能的诊断被认为是孤立的IR弱点，重症肌无力或偏斜。安排了紧急MRI扫描并进行了血液检查。MRI未见异常。验血正常,然而,乙酰胆碱受体抗体血清试验(ACH-R)为0.43nmol/L,这是正常的高端。在后续访问中，IR无力恶化,患者也出现了凝视诱发的眼球震颤.加快了与神经科医生和神经眼科医生的约会。当病人回来时，她报告说,她的神经科医生诊断她患有僵硬人综合征(SPS).该患者还出现了横向交替的偏斜，并报告说她接受了一个疗程的静脉注射免疫球蛋白（IVIG）。患者服用地西泮和加巴喷丁。由于缺乏恢复，持续性复视和示波，每月IVIG已开处方。
结论:目前缺乏关于SPS的眼科问题以及如何最好地治疗它们的文献。以前的报告已经确定与重症肌无力有联系，许多患者继续发展为肌无力。SPS的治疗缺乏大量的循证研究。然而,肌肉松弛剂和抗惊厥剂的治疗为一些人提供了良好的结果。需要进一步的研究来开发一种基于证据的方法来治疗具有SPS经验的眼科问题患者。此病例报告强调了骨科医师在调查和监测僵硬人综合征患者中的重要性和价值。
方法：这是一例57岁女性患者就诊于急症室的病例报告，患者有1天的垂直复视病史和2周的继发于肌肉痉挛的下肢痉挛病史。
结果：患者无明显病史或眼部病史。视神经检查最初显示左下直肌（IR）作用不足。在这一点上可能的诊断被认为是孤立的IR弱点，重症肌无力或偏斜。安排了紧急MRI扫描并进行了血液检查。MRI未见异常。验血正常,然而,乙酰胆碱受体抗体血清试验(ACH-R)为0.43nmol/L,这是正常的高端。在后续访问中，IR无力恶化,患者也出现了凝视诱发的眼球震颤.加快了与神经科医生和神经眼科医生的约会。当病人回来时，她报告说,她的神经科医生诊断她患有僵硬人综合征(SPS).该患者还出现了横向交替的偏斜，并报告说她接受了一个疗程的静脉注射免疫球蛋白（IVIG）。患者服用地西泮和加巴喷丁。由于缺乏恢复，持续性复视和示波，每月IVIG已开处方。
结论:目前缺乏关于SPS的眼科问题以及如何最好地治疗它们的文献。以前的报告已经确定与重症肌无力有联系，许多患者继续发展为肌无力。SPS的治疗缺乏大量的循证研究。然而,肌肉松弛剂和抗惊厥剂的治疗为一些人提供了良好的结果。需要进一步的研究来开发一种基于证据的方法来治疗具有SPS经验的眼科问题患者。此病例报告强调了骨科医师在调查和监测僵硬人综合征患者中的重要性和价值。
