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一名四肢瘫痪患者的眼肌型重症肌无力在诊断和管理方面存在挑战。

Ocular myasthenia gravis in a person with tetraplegia presenting challenges in diagnosis and management.

作者信息

Gounden Sumitha, Lee Bonne, Mellick Ross, Rutkowski Susan B, Middleton James W

机构信息

Rehabilitation Unit, Orange Health Service , Orange, NSW, Australia.

Spinal Cord Injuries Unit, Prince of Wales Hospital , Sydney, NSW, Australia.

出版信息

Spinal Cord Ser Cases. 2016 Apr 7;2:15037. doi: 10.1038/scsandc.2015.37. eCollection 2016.

Abstract

We report the first case of ocular myasthenia gravis (OMG) in a patient with complete tetraplegia, highlighting diagnostic and management challenges. Spinal multidisciplinary rural clinic and specialised inpatient Spinal Cord Injury Unit, NSW, Australia. A 61-year-old man with established C5 AIS A tetraplegia, presented with sudden onset of diplopia and bilateral ptosis, later diagnosed as OMG, in context of other complex co-morbidities, including a cervical cord syrinx, obstructive sleep apnoea and labile blood pressure. Clinical findings were consistent with fluctuating bilateral partial third and sixth nerve palsies. Acetylcholine receptor antibodies were negative, but electromyography demonstrated muscle fatigue. The ocular signs responded well to pyridostigmine. Medications taken before diagnosis, including solifenacin for neurogenic bladder overactivity, were ceased to avoid attenuating the anti-cholinesterase effect. However, the unopposed anti-cholinesterase activity led to frequent and painful abdominal spasms, associated with uncontrolled detrusor hyperreflexia and worsening autonomic dysreflexia (AD). A trans-vesical phenol block to treat this provided only short-lasting benefit. Pyridostigmine was ceased to avoid provoking his abdominal spasms and his regular medications were recommenced. It was decided that the most appropriate treatment for his distressing diplopia was an eye patch. After discharge home, he continued to experience problems with recurrent urinary tract infections, abdominal spasms, episodic postural hypotension and AD. After 5 months, the patient died from an acute myocardial infarction. This case report contributes new knowledge about the rare presentation of OMG in a person with chronic tetraplegia.

摘要

我们报告了首例患有完全性四肢瘫痪患者的眼肌型重症肌无力(OMG)病例,突出了诊断和管理方面的挑战。澳大利亚新南威尔士州的脊髓多学科农村诊所和专门的脊髓损伤住院病房。一名61岁已确诊为C5 AIS A级四肢瘫痪的男性,出现突发复视和双侧上睑下垂,后来在存在其他复杂合并症的情况下被诊断为OMG,这些合并症包括颈髓空洞症、阻塞性睡眠呼吸暂停和血压不稳定。临床检查结果与双侧波动性部分动眼神经和展神经麻痹一致。乙酰胆碱受体抗体为阴性,但肌电图显示肌肉疲劳。眼部症状对吡啶斯的明反应良好。诊断前服用的药物,包括用于神经源性膀胱过度活动症的索利那新,均已停用,以避免减弱抗胆碱酯酶的作用。然而,无对抗的抗胆碱酯酶活性导致频繁且疼痛的腹部痉挛,伴有无法控制的逼尿肌反射亢进和自主神经反射亢进(AD)加重。经膀胱酚阻滞治疗仅提供了短期益处。停用吡啶斯的明以避免引发他的腹部痉挛,并重新开始使用他的常规药物。决定对其令人痛苦的复视最合适的治疗方法是使用眼罩。出院回家后,他继续经历反复尿路感染、腹部痉挛、发作性体位性低血压和AD等问题。5个月后,患者死于急性心肌梗死。本病例报告为慢性四肢瘫痪患者中OMG的罕见表现提供了新知识。

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