Witt N J, Bolton C F
Department of Clinical Neurological Sciences, Victoria Hospital, University of Western Ontario, London, Canada.
Muscle Nerve. 1988 Apr;11(4):398-405. doi: 10.1002/mus.880110418.
We describe a patient who had asymmetrical atrophy of limb muscles and myasthenic weakness of neck, facial, and bulbar muscles. Electrophysiological tests indicated myasthenia gravis of facial muscles and changes consistent with an asymmetrical motor neuropathy as a cause of the muscle atrophy. Both conditions occurred as complications of a locally invasive thymoma, and both failed to improve after surgery and radiation but substantially improved with subsequent treatment by corticosteroids, azathioprine, and plasmapheresis. Review of the literature disclosed that several neuromuscular conditions may be associated with thymoma, the commonest being myasthenia gravis. Muscle atrophy may occur in 10% of patients who have myasthenia gravis, whether associated with or without thymoma. Its mechanism is debated, and further studies are needed, but observations in our patient suggest the atrophy is due to a motor neuropathy. Primary treatment of the thymoma by surgery, irradiation if the tumor is invasive, and immunosuppressive therapy for neuromuscular complications offers a relatively good prognosis for this group of patients.
我们描述了一名患者,其肢体肌肉出现不对称萎缩,颈部、面部和延髓肌肉存在肌无力。电生理测试表明面部肌肉患有重症肌无力,且存在与不对称性运动神经病相符的变化,后者是肌肉萎缩的病因。这两种情况均为局部侵袭性胸腺瘤的并发症,术后和放疗后均未见改善,但随后使用皮质类固醇、硫唑嘌呤和血浆置换治疗后有显著改善。文献回顾显示,几种神经肌肉疾病可能与胸腺瘤有关,最常见的是重症肌无力。重症肌无力患者中有10%可能会出现肌肉萎缩,无论是否伴有胸腺瘤。其机制存在争议,仍需进一步研究,但我们患者的观察结果表明,萎缩是由运动神经病所致。胸腺瘤的主要治疗方法为手术,如果肿瘤具有侵袭性则进行放疗,针对神经肌肉并发症进行免疫抑制治疗,这为这类患者提供了相对较好的预后。