Population-based incidence and clinico-radiological characteristics of tumefactive demyelination in Olmsted County, Minnesota, United States.
Population-based incidence and clinico-radiological characteristics of tumefactive demyelination in Olmsted County, Minnesota, United States.
复制标题
美国明尼苏达州奥尔姆斯特德县Tumefactive脱髓鞘的基于人群的发病率和临床 - 辐射学特征。
DOI：
10.1111/ene.15182
复制
发表时间：
2022-03
影响因子：
5.1
通讯作者：
Tobin WO
中科院分区：
文献类型：
--
作者：
Fereidan-Esfahani M;Decker PA;Eckel Passow JE;Lucchinetti CF;Flanagan EP;Tobin WO
肿胀性脱髓鞘（TD）表现为类似肿瘤或其他占位性病变的大型炎性病变。流行病学、临床和放射学数据有限。我们报告了明尼苏达州奥姆斯特德县的发病率、临床和放射学特征。我们通过罗切斯特流行病学项目数据库回顾性分析了CNS炎性脱髓鞘相关诊断代码（1998年1月1日至1998年12月31日）的患者，根据2010年美国总人群调整了年龄和性别的发病率。我们使用扩展残疾状态量表评分（EDSS）评估结局（首次发作和末次随访）。15/792例多发性硬化（MS）患者（8例男性，7例女性）患有肿瘤性MS，占MS人群的1.9%。中位发作年龄为34.2岁（范围2-61岁）。8/16例患者的MS首次临床发作为肿瘤样病变。8/12例患者存在CSF寡克隆带（OCB）。11/16例患者符合Barkhof空间传播标准。中位随访时间为10.5年（范围1-20.5年）后，大多数患者仍能完全行走（13/16例EDSS ≤4 [81%]）。女性和男性经统计学校正的年发病率分别为0.46/100，000 [95%CI：0.12-0.81]和0.66/100，000 [95%CI：0.23-1.02]，总体为0.56/100，000 [95%CI：0.28-0.83]。根据2010年美国总人群调整年龄和性别后，总体年发病率为0.57 [95% CI：0.28-0.84]。尽管在疾病发作时临床表现为侵袭性，但大多数患者仍然完全行走（13/16例EDSS≤4），具有复发-缓解病程。虽然发病率很低，但在亚急性进行性神经功能缺损伴MRI环增强、ADC限制和脊髓液分析OCB的患者中，应怀疑TD。
Tumefactive demyelination (TD) presents with large inflammatory lesions mimicking tumors or other space-occupying lesions. Limited epidemiology, clinical and radiologic data exist. We report incidence rate, clinical and radiological features in Olmsted County, Minnesota. We retrospectively reviewed patients with CNS inflammatory demyelination-related diagnostic codes (1/1/98–12/31/18) via the Rochester Epidemiology Project database with incidence rates by age and sex adjusted to the 2010 US total population. We used the Expanded Disability Status Scale score (EDSS) to assess outcomes (index attack and last follow-up). 15/792 multiple sclerosis (MS) patients (8 males,7 females) had Tumefactive MS, representing 1.9% of the MS population. Median attack-onset age was 34.2 years (range 2–61). Tumefactive lesion was the first clinical MS attack in 8/16 patients. CSF oligoclonal bands (OCBs) were present in 8/12 patients. 11/16 patients met Barkhof criteria for dissemination in space. Most remained fully ambulatory (EDSS ≤4 in 13/16 [81%]) after median follow-up duration of 10.5 years (range 1–20.5). Age-adjusted annual incidence rates were 0.46/100,000 [95% CI: 0.12–0.81] for females, 0.66/100,000 [95% CI: 0.23–1.02] for males, and overall, 0.56/100,000 [95% CI: 0.28–0.83]. When age- and sex-adjusted to the 2010 US total population, overall annual incidence rate was 0.57 [95% CI: 0.28–0.84]. Despite aggressive clinical presentation at disease onset, most patients remained fully ambulatory (EDSS≤4 in 13/16) with a relapsing-remitting course. Although incidence is rare, TD should be suspected in patients presenting with subacutely progressive neurological deficits associated with MRI findings of ring enhancement, ADC restriction, and OCB on spinal fluid analysis.
登录
查看更多内容
影响因子：
--
作者：
Sastre-Garriga, J;Tintoré, M;Montalban, X
通讯作者：
Montalban, X
影响因子：
48
作者：
Thompson, Alan J.;Banwell, Brenda L.;Cohen, Jeffrey A.
通讯作者：
Cohen, Jeffrey A.
影响因子：
11
作者：
Pittock, SJ;McClelland, RL;Lucchinetti, CF
通讯作者：
Lucchinetti, CF
DOI：
10.1212/nxi.0000000000000231
发表时间：
2016-06
期刊：
Neurology(R) neuroimmunology & neuroinflammation
影响因子：
--
作者：
Majed M;Fryer JP;McKeon A;Lennon VA;Pittock SJ
通讯作者：
Pittock SJ
影响因子：
6
作者：
Seewann, A.;Enzinger, C.;Fazekas, F.
通讯作者：
Fazekas, F.