Clinical and radiographic spectrum of pathologically confirmed tumefactive multiple sclerosis.
Clinical and radiographic spectrum of pathologically confirmed tumefactive multiple sclerosis.
复制标题
病理确认的曲霉多发性硬化症的临床和放射学光谱。
DOI：
10.1093/brain/awn098
复制
发表时间：
2008-07
期刊：
影响因子：
14.5
通讯作者：
Brueck, W.
中科院分区：
文献类型：
--
作者：
Lucchinetti, C. F.;Gavrilova, R. H.;Metz, I.;Parisi, J. E.;Scheithauer, B. W.;Weigand, S.;Thomsen, K.;Mandrekar, J.;Altintas, A.;Erickson, B. J.;Koenig, F.;Giannini, C.;Lassmann, H.;Linbo, L.;Pittock, S. J.;Brueck, W.
多发性硬化症病变的非典型影像学特征包括大小> 2 cm、占位效应、水肿和/或环状增强。这种现象通常被称为“肿胀性多发性硬化症”。之前的系列强调其单灶性和临床孤立性，然而，这些病变的演变尚不清楚。诊断可能需要活检。我们描述了 168 名活检确诊为 CNS 炎症性脱髓鞘疾病 (IDD) 的患者的临床和影像学特征。在活检前和活检后磁共振成像（MRI）上分析病变的位置、大小、占位效应/水肿、增强、多灶性和巴克霍夫标准的满足情况。临床数据与 MRI 相关。女性与男性的比例为 1.2:1，中位发病年龄为 37 岁，症状出现和活检之间的持续时间为 7.1 周，总病程为 3.9 年。活检前的临床病程为首次神经系统事件（61%），复发缓解型（29%），进展型（4%）。表现通常是多症状的，以运动、认知和感觉症状为主。观察到失语、失认、癫痫发作和视野缺损。随访时，70% 的人患有明确的多发性硬化症，14% 的人患有孤立性脱髓鞘综合征。至第二次发作的中位时间为 4.8 年，随访时的中位 EDSS 为 3.0。活检前 MRI 中 70% 存在多个病变，最后一次 MRI 中 83% 存在多个病变，活检前 46% 满足 Barkhof 标准，随访时 55% 满足 Barkhof 标准。只有 17% 的病例保持单灶性。 T2 加权图像上的中位最大病变尺寸为 4 厘米（范围 0.5-12），可辨别尺寸为 2.1 厘米（范围 0.5-7.5）。活检病变显示 45% 存在占位效应，77% 存在水肿。发现病变大小与占位效应和/或水肿之间存在很强的相关性（P < 0.001）。环增强频繁。大多数肿胀特征与性别、病程或诊断无关。尽管病变大小 >5 cm 与末次随访时 EDSS 稍高相关，但与基于人群的与病程匹配的多发性硬化症队列 (EDSS 3.5；P < 0.001) 相比，病程 > 10 年患者的长期预后 (EDSS 1.5) 更好。鉴于该研究的回顾性，并不总是能够确定活检的确切原因。这项研究强调了具有非典型临床或放射学特征的 CNS IDD 的诊断挑战性。大多数患者在发病时患有多灶性疾病，并通过随访发展为 RRMS。尽管提高对这种广谱的认识可能会在许多情况下消除对活检的需要，但在某些情况下可能需要诊断性脑活检发挥重要作用。
Atypical imaging features of multiple sclerosis lesions include size >2 cm, mass effect, oedema and/or ring enhancement. This constellation is often referred to as ‘tumefactive multiple sclerosis’. Previous series emphasize their unifocal and clinically isolated nature, however, evolution of these lesions is not well defined. Biopsy may be required for diagnosis. We describe clinical and radiographic features in 168 patients with biopsy confirmed CNS inflammatory demyelinating disease (IDD). Lesions were analysed on pre- and post-biopsy magnetic resonance imaging (MRI) for location, size, mass effect/oedema, enhancement, multifocality and fulfilment of Barkhof criteria. Clinical data were correlated to MRI. Female to male ratio was 1.2 : 1, median age at onset, 37 years, duration between symptom onset and biopsy, 7.1 weeks and total disease duration, 3.9 years. Clinical course prior to biopsy was a first neurological event in 61%, relapsing–remitting in 29% and progressive in 4%. Presentations were typically polysymptomatic, with motor, cognitive and sensory symptoms predominating. Aphasia, agnosia, seizures and visual field defects were observed. At follow-up, 70% developed definite multiple sclerosis, and 14% had an isolated demyelinating syndrome. Median time to second attack was 4.8 years, and median EDSS at follow-up was 3.0. Multiple lesions were present in 70% on pre-biopsy MRI, and in 83% by last MRI, with Barkhof criteria fulfilled in 46% prior to biopsy and 55% by follow-up. Only 17% of cases remained unifocal. Median largest lesion size on T2-weighted images was 4 cm (range 0.5–12), with a discernible size of 2.1 cm (range 0.5–7.5). Biopsied lesions demonstrated mass effect in 45% and oedema in 77%. A strong association was found between lesion size, and presence of mass effect and/or oedema (P < 0.001). Ring enhancement was frequent. Most tumefactive features did not correlate with gender, course or diagnosis. Although lesion size >5 cm was associated with a slightly higher EDSS at last follow-up, long-term prognosis in patients with disease duration >10 years was better (EDSS 1.5) compared with a population-based multiple sclerosis cohort matched for disease duration (EDSS 3.5; P < 0.001). Given the retrospective nature of the study, the precise reason for biopsy could not always be determined. This study underscores the diagnostically challenging nature of CNS IDDs that present with atypical clinical or radiographic features. Most have multifocal disease at onset, and develop RRMS by follow-up. Although increased awareness of this broad spectrum may obviate need for biopsy in many circumstances, an important role for diagnostic brain biopsy may be required in some cases.
登录
查看更多内容
影响因子：
4.4
作者：
Annunziata, Pasquale;Giorgio, Antonio;Maimone, Davide
通讯作者：
Maimone, Davide
影响因子：
2.8
作者：
GIANG, DW;PODURI, KR;HERNDON, RM
通讯作者：
HERNDON, RM
影响因子：
14.5
作者：
Barkhof, F;Filippi, M;Valk, J
通讯作者：
Valk, J
影响因子：
11.2
作者：
Barkhof, F;Rocca, M;Filippi, M
通讯作者：
Filippi, M
影响因子：
1.9
作者：
Brinar, VV
通讯作者：
Brinar, VV