Bhaskar Shalini, Abdul Rani Mohammed Fauzi Bin
Department of Medicine, Universiti Teknologi MARA, Thomson Hospital Kota Damansara, Shah Alam, Malaysia.
Case Rep Med. 2022 Jul 15;2022:4802538. doi: 10.1155/2022/4802538. eCollection 2022.
Muscle-specific tyrosine kinase (MuSK) antibody positive myasthenia gravis (MuSK-MG) is a rare clinical disorder, and diagnosing it can be challenging. Most of the patients present with predominant facial, oculo-bulbar, and neck muscle weakness along with respiratory muscle involvement. Such a presentation can be mistaken for bulbar onset motor neuron disease or as one of the rare oculopharyngeal myopathies. . We present a young female patient, who reported to us with neck muscle weakness, ocular and bulbar muscle paralysis, and breathing difficulty. She had been healthy till she was prescribed hydroxychloroquine (HCQ) tablets (400 mg per day) for a malar rash. By the end of the second week after commencing the HCQ therapy, she developed the muscle weakness. Her symptoms began to regress after stopping HCQ and starting steroids, pyridostigmine, and, subsequently, azathioprine. She was negative for anticholinesterase receptor antibodies (AChR-Ab) but was positive for MuSK antibodies (MuSK-Ab).
This report proves that MuSK-MG can also be unmasked by HCQ administration. Awareness of drug-induced/-unmasked MG is important, as failure to do so may result in a severe morbidity and a fatal outcome. The offending drug has to be promptly discontinued, and appropriate treatment should be instituted.
肌肉特异性酪氨酸激酶(MuSK)抗体阳性重症肌无力(MuSK-MG)是一种罕见的临床疾病,其诊断可能具有挑战性。大多数患者主要表现为面部、眼咽和颈部肌肉无力,并伴有呼吸肌受累。这种表现可能被误诊为球部起病的运动神经元病或罕见的眼咽肌病之一。我们报告一名年轻女性患者,她因颈部肌肉无力、眼肌和球部肌肉麻痹以及呼吸困难前来就诊。在因颧部皮疹被处方服用羟氯喹(HCQ)片(每日400毫克)之前,她一直健康。在开始HCQ治疗后的第二周结束时,她出现了肌肉无力。在停用HCQ并开始使用类固醇、吡啶斯的明,随后使用硫唑嘌呤后,她的症状开始缓解。她的抗胆碱酯酶受体抗体(AChR-Ab)呈阴性,但MuSK抗体(MuSK-Ab)呈阳性。
本报告证明HCQ给药也可引发MuSK-MG。认识到药物诱导/引发的重症肌无力很重要,因为未能认识到这一点可能导致严重的发病率和致命后果。必须立即停用致病药物,并应采取适当的治疗措施。