Dancing muscles: the value of real-time ultrasound evaluation of muscle in myositis and mimics.
Dancing muscles: the value of real-time ultrasound evaluation of muscle in myositis and mimics.
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跳舞的肌肉：实时超声评估肌肉在肌炎和模仿中的价值。
DOI：
10.1093/rheumatology/keab088
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发表时间：
2021
期刊：
影响因子：
--
通讯作者：
Christopher-Stine,Lisa
中科院分区：
文献类型：
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作者：
Albayda,Jemima;Hayes,LindseyR;Christopher-Stine,Lisa
尊敬的编辑，包涵体肌炎（IBM）是50岁以上成人中最常见的肌病，表现为缓慢进行性无力，可导致严重残疾。尽管越来越多的人认识到IBM是一个临床实体，但鉴别诊断是广泛的，特别是当手指屈肌和膝伸肌无力的主要特征不完全明显时[1]。在非典型或早期表现中，通常需要进行辅助检查。作为一种非侵入性的工具，肌肉超声成像可能有助于确认或建议替代诊断。一名67岁的妇女到流变学评估手无力，推测诊断为IBM。她的症状从掉落物品发展到因虚弱而无法闭合双手超过10个月。脑部MRI正常，脊髓MRI显示多节段椎间盘疾病。包括肌肉酶在内的实验室检查正常。体格检查时，她的下肢肌肉力量保留，手动肌肉测试为5/5，但臂外展肌2/5无力，手指屈肌4/5无力。双臂肌肉张力降低，肩带周围、前臂和手部固有肌肉消瘦。手臂的反应迟钝，双膝的反应活跃。鉴于手无力，进行了前臂床旁超声检查，显示远端手指屈肌（指深屈肌）的肌肉回声正常，与IBM不兼容。然而，超声检测到多个肌群的异常肌束震颤，这在体格检查中是不可见的（图1和补充视频S1，可在Rheologyonline获得）。然后完成EMG，并显示颈部和胸部肌节不对称的慢性急性去神经支配的证据，涉及运动神经元疾病。这导致随后转诊到神经科，诊断患者患有肌萎缩侧索硬化症（ALS）的连枷臂变体。患者已开始接受利鲁唑治疗。
Dear Editor, Inclusion body myositis (IBM) is the most common myopathy in adults> 50, presenting with slowly progressive weakness that can lead to significant disability. Despite increasing recognition of IBM as a clinical entity, the differential diagnosis is broad especially when cardinal features of finger flexor and knee extensor weakness are not fully apparent [1]. In atypical or early presentations, ancillary testing is often needed. As a non-invasive tool, imaging with muscle ultrasound may help to confirm or suggest alternative diagnoses.A 67-year-old woman presented to Rheumatology for evaluation of hand weakness with a presumed diagnosis of IBM. Her symptoms had progressed over 10 months from dropping items to inability to close her hands secondary to weakness. An MRI of the brain was normal and an MRI of the spinal cord showed multilevel disc disease. Laboratory investigations including muscle enzymes were normal. On physical examination, she had preserved muscle strength in the lower extremities with 5/5 strength by manual muscle testing, but 2/5 weakness at the arm abductors and 4/5 at the finger flexors. There was reduced muscle tone in both arms with wasting around the shoulder girdle, forearms and intrinsic hand muscles. Reflexes were hypoactive in the arms and brisk at both knees. Given the hand weakness, a bedside ultrasound of the forearm was done that demonstrated normal muscle echogenicity of the distal finger flexors (flexor digitorum profundus), not compatible with IBM. However, abnormal fasciculations of multiple muscle groups were detected by ultrasound, which were not visible on physical exam (Fig. 1 and Supplementary Video S1, available at Rheumatology online). An EMG was then completed and showed evidence of acute-on-chronic denervation in cervical and thoracic myotomes asymmetrically, concerning for motor neuron disease. This led to a subsequent referral to Neurology who diagnosed the patient with flail arm variant of amyotrophic lateral sclerosis (ALS). The patient has been started on riluzole.