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重症肌无力合并低钾性周期性麻痹:一种罕见的表现。

Coexistence of myasthenia gravis with hypokalemic periodic paralysis: a rare presentation.

作者信息

Khan Farah Gul, Namran Sidra

机构信息

Medicine, Aga Khan University Hospital, Karachi, Pakistan

Medicine, Aga Khan University Hospital, Karachi, Pakistan.

出版信息

BMJ Case Rep. 2019 Oct 5;12(10):e231241. doi: 10.1136/bcr-2019-231241.

Abstract

Bilateral symmetrical weakness of acute onset is not very uncommon and the differential varies widely from life-threatening neurological illnesses to metabolic and electrolyte derangements. We report the case of a young female with severe muscle weakness, respiratory distress and hypokalemia who required immediate intubation on arrival to emergency department. During hospital course, even after normalisation of serum potassium and some improvement in limb weakness, patient failed multiple attempts of extubation because of type II respiratory failure. Subsequently, acetyl cholinesterase antibodies were checked which came out positive, and diagnosis of myasthenia gravis and hypokalemic periodic paralysis was made. She was successfully extubated after intravenous pulse steroids, pyridostigmine and plasmapheresis. Patient was finally discharged home on oral steroids, pyridostigmine and azathioprine. In a patient presenting with hypokalemic weakness, the suspicion of a second disorder should be very high if weakness fails to resolve following correction of hypokalemia.

摘要

急性起病的双侧对称性肌无力并非十分罕见,其鉴别诊断范围广泛,涵盖从危及生命的神经系统疾病到代谢及电解质紊乱等多种情况。我们报告一例年轻女性病例,该患者有严重肌无力、呼吸窘迫及低钾血症,抵达急诊科时即需立即插管。在住院期间,即便血清钾恢复正常且肢体无力有所改善,但患者因Ⅱ型呼吸衰竭多次拔管尝试均失败。随后检查乙酰胆碱酯酶抗体呈阳性,从而诊断为重症肌无力合并低钾性周期性麻痹。经静脉注射冲击剂量类固醇、吡啶斯的明及血浆置换后,她成功拔管。患者最终口服类固醇、吡啶斯的明及硫唑嘌呤出院回家。对于出现低钾性肌无力的患者,如果低钾血症纠正后肌无力仍未缓解,应高度怀疑存在第二种疾病。

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本文引用的文献

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Repetitive nerve stimulation testing in myasthenic crisis.重症肌无力危象中的重复神经刺激试验
Muscle Nerve. 2019 May;59(5):528-530. doi: 10.1002/mus.26472. Epub 2019 Mar 25.
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N Engl J Med. 2016 Dec 29;375(26):2570-2581. doi: 10.1056/NEJMra1602678.
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Thyrotoxic periodic paralysis: clinical challenges.甲状腺毒症性周期性瘫痪:临床挑战
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