Siffrin Volker, Müller-Forell Wibke, von Pein Harald, Zipp Frauke
Department of Neurology, Johannes Gutenberg University Mainz, Germany.
Mult Scler. 2014 Apr;20(5):631-3. doi: 10.1177/1352458513516891. Epub 2013 Dec 17.
Glioma-like inflammatory demyelinating lesions can be found in patients with pre-diagnosed multiple sclerosis, but they have also been described as an isolated disease entity. The initial diagnostic work-up usually includes a biopsy for histopathological analysis. However, even after unambiguous histopathologic classification, tumefactive lesions pose a therapeutic challenge. Until now, there have been no guidelines on how to treat patients with these rare and extreme lesion phenotypes. Here we report a patient with a relapsing unifocal tumefactive demyelinating lesion. The patient initially showed a good response to steroid treatment, with full clinical recovery. However, after relapse of the same lesion, recovery was incomplete. Although immunosuppression was initiated, the patient presented with subsequent further deterioration. Only maximal escalation of immunosuppression was able to stop the inflammatory activity. Due to the length of time of the step-wise escalation treatment however, the lengthy lesion activity led to irreversible tissue destruction and residual non-remitting disability. Early aggressive treatment with an induction therapy regimen might be more appropriate for these rare and often strongly disabling lesion subtypes.
类胶质瘤样炎性脱髓鞘病变可见于预先诊断为多发性硬化症的患者，但也被描述为一种独立的疾病实体。初始诊断检查通常包括活检以进行组织病理学分析。然而，即使在明确的组织病理学分类之后，瘤样病变仍构成治疗挑战。到目前为止，对于如何治疗这些罕见且极端病变表型的患者尚无指南。在此，我们报告一例复发性单灶性瘤样脱髓鞘病变患者。该患者最初对类固醇治疗反应良好，临床完全恢复。然而，同一病变复发后，恢复不完全。尽管开始了免疫抑制治疗，但患者随后病情进一步恶化。只有最大程度地加强免疫抑制才能阻止炎症活动。然而，由于逐步强化治疗的时间长度，长时间的病变活动导致了不可逆的组织破坏和残留的持续性残疾。对于这些罕见且通常严重致残的病变亚型，早期采用诱导治疗方案进行积极治疗可能更为合适。
