Nilsson P, Larsson E-M, Kahlon B, Nordström C-H, Norrving B
Department of Neurology, Clinical Sciences Lund, Lund University, Sweden.
Eur J Neurol. 2009 May;16(5):639-42. doi: 10.1111/j.1468-1331.2009.02547.x. Epub 2009 Mar 20.
Tumefactive demyelinating disease (TDD) is a rare primary demyelinating disease with diagnostic and therapeutic challenges.
We report a 50-year old woman with TDD successfully treated with decompressive craniectomy and corticosteroids. The patient presented with seizures, subacute progressive hemispheric syndrome, and a tumourlike abnormality on MRI. Demyelinating disease was initially considered unlikely. Due to a rapidly evolving herniation syndrome hemicraniectomy was performed. Outcome was favourable with only very mild neurological deficits 6 weeks later.
TDD should be considered as a differential diagnosis in tumour-like presentations, and appears to have distinctive neuroimaging features. In the advent of treatement failure from high dose corticosteroids and plasmapheresis and development of severe mass effect, decompressive hemicraniectomy is an important treatment option.
瘤样脱髓鞘疾病（TDD）是一种罕见的原发性脱髓鞘疾病，在诊断和治疗方面存在挑战。
我们报告了一名50岁患有TDD的女性患者，通过减压颅骨切除术和皮质类固醇成功治疗。该患者出现癫痫发作、亚急性进行性半球综合征，且MRI显示有肿瘤样异常。最初认为脱髓鞘疾病不太可能。由于疝综合征迅速发展，进行了颅骨切除术。6周后结果良好，仅遗留非常轻微的神经功能缺损。
在出现肿瘤样表现时应将TDD视为鉴别诊断，且其似乎具有独特的神经影像学特征。在大剂量皮质类固醇和血浆置换治疗失败且出现严重占位效应时，减压性颅骨切除术是一种重要的治疗选择。
