Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
Muscle Nerve. 2014 Feb;49(2):287-9. doi: 10.1002/mus.24060. Epub 2013 Dec 18.
The hallmark clinical presentation of inclusion-body myositis (IBM) is slowly progressive weakness that characteristically affects the quadriceps and finger and wrist finger flexor muscles. Facial weakness can also occur, but it is typically mild and not a prominent finding.
We describe the clinical features, laboratory investigations, and muscle biopsy findings in a 58-year old man who presented with a 6-year history of marked progressive symmetrical facial weakness. Examination also showed shoulder abduction and hip extensor weakness.
The patient's serum creatine kinase level was 655 U/L, and electromyography showed fibrillation potentials and myopathic motor unit potentials. A biopsy specimen of the left biceps muscle was pathognomonic for IBM.
This patient did not have a typical presentation for IBM but rather fulfilled the pathological criteria for IBM. To our knowledge, facial diplegia has not been reported previously as a presenting manifestation of IBM.
包涵体肌炎（IBM）的显著临床特征是进行性缓慢无力，主要影响股四头肌和手指及腕部手指屈肌。也可能出现面部无力，但通常较为轻微，不是显著发现。
我们描述了一位 58 岁男性的临床特征、实验室检查和肌肉活检结果，该患者表现为进行性对称性面部无力 6 年。检查还显示肩外展和髋外展无力。
患者的血清肌酸激酶水平为 655 U/L，肌电图显示纤颤电位和肌病性运动单位电位。左肱二头肌的活检标本具有 IBM 的特征性表现。
该患者的表现不典型，但符合 IBM 的病理标准。据我们所知，面部弛缓性瘫痪以前没有报道过是 IBM 的首发表现。
