Romano Alissa E, Al-Qudah Zaid, Kaminski Henry J, Katirji Bashar, Salame Karim
*Department of Neurology, Lehigh Valley Health Network, Allentown, PA; †Department of Neurology, University Hospitals Case Medical Center, Case Western Reserve University Cleveland, OH; and ‡Department of Neurology, The George Washington University Washington DC.
J Clin Neuromuscul Dis. 2017 Jun;18(4):218-222. doi: 10.1097/CND.0000000000000165.
Paraspinous myopathy is a rare neuromuscular disorder characterized by selective involvement of the cervical, thoracic, or lumbar muscles. Leading clinical features include a bent spine or dropped head (antecollis). In myasthenia gravis (MG), patients may have camptocormia secondary to neuromuscular junction dysfunction of the paraspinal muscles, and this condition usually responds to acetylcholinesterase inhibitors or immunosuppressive treatments. However, concomitant MG and paraspinous myopathy with histologic and electrophysiologic evidence of myopathic changes of the paraspinal muscles has only been reported twice in the literature. In this case series, 5 patients with MG with paraspinous myopathy are reported. While neck and extension truncal weakness is not uncommon in MG, most often the weakness is due to neuromuscular junction dysfunction. Coexisting paraspinous myopathy and MG is under-recognized, is often resistant to mainstay treatments, and should be considered in patients presenting with neck or trunk extensor weakness.
椎旁肌病是一种罕见的神经肌肉疾病,其特征是颈椎、胸椎或腰椎肌肉选择性受累。主要临床特征包括脊柱弯曲或头部下垂(颈前屈)。在重症肌无力(MG)中,患者可能因椎旁肌神经肌肉接头功能障碍而出现脊柱前凸,这种情况通常对乙酰胆碱酯酶抑制剂或免疫抑制治疗有反应。然而,伴有组织学和电生理学证据显示椎旁肌存在肌病性改变的MG和椎旁肌病,在文献中仅报道过两例。在本病例系列中,报告了5例患有MG合并椎旁肌病的患者。虽然颈部和躯干伸展无力在MG中并不罕见,但大多数情况下,这种无力是由于神经肌肉接头功能障碍所致。并存的椎旁肌病和MG未得到充分认识,通常对主要治疗方法耐药,对于出现颈部或躯干伸肌无力的患者应考虑到这种情况。