Leupold Daniela, Felbecker Ansgar, Tettenborn Barbara, Hundsberger Thomas
Departments of Neurology, Cantonal Hospital, St. Gallen, Switzerland.
Departments of Neurology, Cantonal Hospital, St. Gallen, Switzerland; Departments of Haematology and Oncology, Cantonal Hospital, St. Gallen, Switzerland.
Case Rep Neurol. 2016 May 25;8(2):108-14. doi: 10.1159/000446314. eCollection 2016 May-Aug.
The additional value of peripheral nerve ultrasound in acquired immune-mediated neuropathies has recently been reported. Its impact in vasculitic neuropathy is yet to be defined. We report electrophysiological and nerve ultrasound studies in a patient with nonsystemic vasculitic neuropathy at first diagnosis and in response to immunosuppression.
A 44-year-old female presented with painful neuropathy and weakness of the intrinsic hand muscles. Electrodiagnostic studies revealed severe axonal neuropathy of the nerves of the left arm. On nerve ultrasound, massive and patchy swelling of these nerves was detected. Clinical, laboratory, and radiological evidence of nonneuromuscular involvement and systemic vasculitic diseases was absent. Hence, nonsystemic vasculitic neuropathy was diagnosed without the possibility of histological verification. After 6 months of systemic immunosuppression with steroids and cyclophosphamide, clinical symptoms improved in parallel with neurosonography. In contrast, electrophysiological studies remained pathological despite clinical improvement.
Neurosonography studies in nonsystemic vasculitic neuropathy are rare but might be an ancillary technique to guide noninvasive diagnosis and therapeutic monitoring. Morphological analysis of nerves and changes in response to treatment could be well visualized. Additionally, neurosonography might be useful to target nerve biopsy.
外周神经超声在获得性免疫介导性神经病中的附加价值最近已有报道。其在血管炎性神经病中的作用尚待明确。我们报告了一名初诊为非系统性血管炎性神经病且接受免疫抑制治疗患者的电生理和神经超声研究结果。
一名44岁女性，出现手部固有肌疼痛性神经病及肌无力。电诊断研究显示左臂神经存在严重轴索性神经病。神经超声检查发现这些神经有大量片状肿胀。未发现非神经肌肉受累及系统性血管炎性疾病的临床、实验室及影像学证据。因此，在无法进行组织学验证的情况下诊断为非系统性血管炎性神经病。经类固醇和环磷酰胺进行6个月的全身免疫抑制治疗后，临床症状改善，同时神经超声检查结果也有所改善。相比之下，尽管临床症状改善，但电生理研究仍显示异常。
非系统性血管炎性神经病的神经超声研究较少，但可能是指导无创诊断和治疗监测的辅助技术。神经的形态学分析及治疗反应变化可得到很好的显示。此外，神经超声可能有助于确定神经活检部位。
