Clinical and radiological characteristics in five cases of neuromyelitisoptica with tumefactive-like demyelinating lesions
10.3760/cma.j.issn.1006-7876.2013.04.005
- VernacularTitle:视神经脊髓炎脑部瘤样脱髓鞘病变五例临床及影像学特征分析
- Author:
Chen CHENG
;
Ying JIANG
;
Jian BAO
;
Zhuang KANG
;
Xueqiang HU
- Publication Type:Journal Article
- Keywords:
Neuromyelitis optica;
Demyelinating diseases;
Brain edema;
Brain diseases;
Magnetic resonance imaging
- From:
Chinese Journal of Neurology
2013;(4):233-237
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze clinical and radiological characteristics in 5 cases of neuromyelitisoptica (NMO) with tumefactive-like demyelinating lesions (TDLs).Methods Five NMO patients with TDLs hospitalized during November 2007 and March 2012 were retrospectively analyzed for clinical and magnetic resonance imaging (MRI) features,as well as therapeutic efficiency and prognoses.Results The male-to-female ratio is 1∶4 and the range of onset ages is 15-44 years old.The median time when TDLs was developed was 36 months after the onset of NMO.The anti-aquaporin 4 was seropositive in all NMO patients with TDLs.Four patients had clinical manifestations of encephalopathy symptoms including coma (n =2),seizures (n =1),narcolepsy (n =1),homonymous hemianopia (n =1).All TDLs grew unilaterally.Two TDLs were located at parietal-occipital,1 at parietal,I at the cerebellum and 1 at the temporal.On MRI,there was no enhanced signal in 4 TDLs,and there was open-ring-like enhanced signals in 1 case.Three TDLs revealed mixed signals intensities on diffusion-weighted images and an increase in the apparent diffusion coeficient values.On the subsequent MRIs,the sizes of 5 TDLs were markedly reduced in 3 cases,disappeared in 1 and another 1 case remained the same.Meanwhile,3 lesions subsequently developed cavity forming.Five cases improved after treatment of high-dose corticosteroid and intravenous immune globulin.Conclusions TDLs is not uncommon in NMO.Encephalopathysymptoms are more frequently affected in NMO with TDLs.TDLs often accompany with extensive vasogenic edema,but without enhancement in acute stage.NMO-IgG antibody testing and brain MRI are important in the diagnosis of TDLs.The therapy of corticosteroid and intravenous immune globulin is recommended.
