Qi Xiao-kun, Liu Jian-guo, Qian Hai-rong, Qiu Feng, Yao Sheng, Li Chang-qing, Wang Ya-ming
Department of Neurology, Navy General Hospital of PLA, Beijing 100048, China.
Zhonghua Nei Ke Za Zhi. 2010 Sep;49(9):750-3.
To summarize the clinical features, neuroimaging findings and pathological characteristics of 26 patients with tumor-like inflammatory demyelinating diseases (TIDD) confirmed by histopathology for better diagnosis and differential diagnosis.
The clinical features, neuroimaging findings and pathological characteristics of 26 patients (14 male, 12 female) with pathologically proven TIDD (24 brain-type and 2 spinal cord-type) were retrospectively analysed.
The mean onset age was 6 - 69 (36.7 ± 13.8) years. Twenty-one patients had good prognosis with a median followed-up duration of 51.0 months. Two patients were died of post-operative complication and pulmonary infection respectively and the remaining 3 patients were lost to followed up. The TIDD patients almost showed monophasic clinical setting. Headache, indifference accompanied with hypothesis were the commonest initial symptoms. The positive or abnormal rates of cerebrospinal fluid oligoclonal bands (OCB) and myelin basic protein (MBP) in TIDD patients were high. The involvements of bilateral and multi-lesions were commonest in TIDD (61.5%, 65.4% respectively). Twenty-two patients with CT unenhanced scanning showed hypodense lesions. Long T(1) and long T(2) signal intensity was showed on MRI and most cases appeared round-like lesion in shape. According to the shape of enhancement of the 23 patients performed with contrast agents, 11 were shown with open-ring enhancement, 4 cases (including 2 accompanied with open-ring enhancement) with complete ring enhancement, 3 with asymmetrical dotted enhancement, 2 with diffused even enhancement, and no enhancement was seen in the other 6. Furthermore, 14 cases with DWI and 12 with FLAIR all appeared hyperdensity. The typical pathological changes were demyelinating, perivascular inflammatory cells infiltration and reactive gliosis. Occasionally, the Creutzfeldt cells were also found in brain tissue of some patients.
TIDD is a distinct demyelinating disease entity. In spite of being apt to be confused with the neoplasm in brain and spinal cord. TIDD has its own-features, for example, OCB is frequently positive in patients with TIDD and the level of MBP may be significantly increased. Furthermore, the involvements of bilateral and multi-lesions are the common in TIDD, and most cases showed open-ring enhancement or complete rim enhancement on MRI. In addition, all cases present hypodense lesions on unenhanced CT and patients with hyperdense seemed not to be considered as TIDD.
总结26例经组织病理学确诊的肿瘤样炎性脱髓鞘疾病（TIDD）患者的临床特征、神经影像学表现及病理特点，以提高诊断及鉴别诊断水平。
回顾性分析26例经病理证实的TIDD患者（男14例，女12例）的临床特征、神经影像学表现及病理特点，其中脑型24例，脊髓型2例。
平均发病年龄6～69岁（36.7±13.8岁）。21例预后良好，中位随访时间51.0个月。2例分别死于术后并发症及肺部感染，其余3例失访。TIDD患者临床多表现为单相病程。头痛、淡漠伴假象为最常见的首发症状。TIDD患者脑脊液寡克隆带（OCB）及髓鞘碱性蛋白（MBP）阳性或异常率较高。双侧及多发病灶受累在TIDD中最为常见（分别为61.5%、65.4%）。22例行CT平扫的患者表现为低密度病灶。MRI表现为长T1、长T2信号，多数病灶呈类圆形。23例行增强扫描的患者，根据强化形态，11例呈开环强化，4例（包括2例合并开环强化）呈完整环形强化，3例呈不对称点状强化，2例呈弥漫均匀强化，其余6例无强化。此外，14例行DWI检查及12例行FLAIR检查均呈高信号。典型病理改变为脱髓鞘、血管周围炎性细胞浸润及反应性胶质增生。部分患者脑组织中偶尔可见克-雅氏细胞。
TIDD是一种独特的脱髓鞘疾病实体。尽管易与脑和脊髓肿瘤混淆，但TIDD有其自身特点，如TIDD患者OCB常为阳性，MBP水平可能显著升高。此外，双侧及多发病灶受累在TIDD中常见，多数病例MRI表现为开环强化或完整边缘强化。另外，所有病例CT平扫均表现为低密度病灶，呈高信号的患者似乎不应考虑为TIDD。
