The Cyprus Institute of Neurology and Genetics, Neurology, P.O.Box 23462, 1683, Nicosia, Cyprus.
The Cyprus Institute of Neurology and Genetics, Neurology, P.O.Box 23462, 1683, Nicosia, Cyprus.
Neuromuscul Disord. 2019 Oct;29(10):771-775. doi: 10.1016/j.nmd.2019.09.004. Epub 2019 Sep 6.
The co-existence of myasthenia gravis and other inflammatory myopathies has been reported in the literature before, but no clinical cases involving inclusion body myositis have been reported. We report a case of a 67-year-old patient who presented with dysphagia, exhibiting the typical electrophysiological features of postsynaptic neuromuscular junction defect with positive muscle acetylcholine receptor antibodies, consistent with the diagnosis of myasthenia gravis. Nevertheless, response to acetylcholinesterase inhibitors and immunomodulatory treatment was unexpectedly poor. As the disease progressed, the patient developed asymmetric muscle weakness, initially affecting mainly the quadriceps and the finger flexors. Muscle MRI imaging supported the presence of an inflammatory myopathy and muscle biopsy confirmed the diagnosis of inclusion body myositis. Thus, our patient represents the first reported overlap case of myasthenia gravis and inclusion body myositis.
重症肌无力和其他炎症性肌病共存以前在文献中有报道，但尚未有包涵体肌炎的临床病例报告。我们报告了一例 67 岁患者，其表现为吞咽困难，具有突触后神经肌肉接头缺陷的典型电生理特征，且肌肉乙酰胆碱受体抗体阳性，符合重症肌无力的诊断。然而，对乙酰胆碱酯酶抑制剂和免疫调节治疗的反应出乎意料地不佳。随着疾病的进展，患者出现非对称性肌无力，最初主要影响股四头肌和手指屈肌。肌肉 MRI 成像支持炎症性肌病的存在，肌肉活检证实了包涵体肌炎的诊断。因此，我们的患者代表了首例报告的重症肌无力和包涵体肌炎重叠病例。
