Maarouf M, Kuchta J, Miletic H, Ebel H, Hesselmann V, Hilker R, Sturm V
Department of Stereotactic and Functional Neurosurgery, Cologne University, Cologne, Germany.
Acta Neurochir (Wien). 2003 Nov;145(11):961-9; discussion 969. doi: 10.1007/s00701-003-0113-3.
Despite the rapid development in neuro-imaging over the past two decades, ring like contrast-enhancing lesions on CCT or MRI still may pose a diagnostic challenge. The main differential diagnoses of these lesions include metastatic carcinoma, high-grade glioma and brain abscess. Acute demyelination seldom turns out to be the underlying pathology.
Retrospective analysis was done on six patients with acute demyelination treated at our neurosurgical department between 1990 and 2001. Clinical, radiological, PET, intra-operative and histological findings were evaluated.
In five patients, the diagnosis of acute demyelination was established by histopathological evaluation of stereotactic biopsy specimen, in the sixth patient following microsurgical extirpation of the lesion. Neuropathology revealed demyelination with the presence of myelin-phagocytosing macrophages. In addition, lymphocytic infiltrates were present. Symptoms and signs improved significantly after high-dose steroid therapy.
Despite CNS tissue destruction, necrosis and cyst formation are not usually found in demyelinating disease, being rather more common in young patients with ring-like contrast-enhancing lesions on CCT and MRI. Though an incorrect diagnosis can lead to a potentially fatal therapeutic intervention, histological diagnosis should be made in all cases. Due to minimum morbidity, stereotactic biopsy is the method of choice to obtain representative specimens for histological diagnosis. Open microsurgery of these lesions is not indicated since conservative medical treatment with steroids results in a favourable outcome in most cases.
尽管在过去二十年神经影像学发展迅速，但计算机断层扫描（CCT）或磁共振成像（MRI）上出现的环形强化病变仍可能带来诊断挑战。这些病变的主要鉴别诊断包括转移性癌、高级别胶质瘤和脑脓肿。急性脱髓鞘很少是潜在的病理情况。
对1990年至2001年间在我们神经外科接受治疗的6例急性脱髓鞘患者进行回顾性分析。评估临床、放射学、正电子发射断层扫描（PET）、术中及组织学检查结果。
5例患者通过立体定向活检标本的组织病理学评估确诊为急性脱髓鞘，第6例患者在病变显微手术切除后确诊。神经病理学显示存在脱髓鞘，伴有吞噬髓磷脂的巨噬细胞。此外，还有淋巴细胞浸润。大剂量类固醇治疗后症状和体征明显改善。
尽管存在中枢神经系统组织破坏，但脱髓鞘疾病通常不会出现坏死和囊肿形成，这种情况在CCT和MRI上有环形强化病变的年轻患者中更为常见。尽管错误诊断可能导致潜在的致命治疗干预，但所有病例均应进行组织学诊断。由于发病率最低，立体定向活检是获取组织学诊断代表性标本的首选方法。对于这些病变，不建议进行开放性显微手术，因为在大多数情况下，类固醇保守治疗会取得良好效果。
