title: Clinical and radiological characteristics of 17 Chinese patients with pathology confirmed tumefactive demyelinating diseases: follow-up study.
abstract: Tumefactive demyelinating disease is a rare inflammatory demyelinating disease (IDD) of the central nervous system (CNS). The literature lacks a clear and consistent description of the clinical and radiological spectrum of this disorder, and few Chinese cases have been studied. Here we report 17 Chinese patients, with pathology confirmed CNS IDD, who had distinct clinical and imaging features from those in previous reports. Median age at onset was 47 years, with a female to male ratio of 1.1:1. Multifocal lesions were present in nine cases (53%) on their pre-biopsy magnetic resonance imagings (MRIs), with locations predominantly involving periventricular white matter (41%), subcortical white matter (41%), juxtacortical regions (41%), and cortical gray matter (35%). Moderate to severe perilesional edema and/or mass effect were present in 35% of cases. A variety of enhancement patterns were observed; most were heterogeneous, including ring-like, patchy, venular-like, nodular, punctate, and diffuse in a decreased frequency. Perilesional restriction on diffusion-weighted images (DWI) were evident in 70% cases. Clinical course prior to biopsy was a first neurological event in 82% cases. During a median follow-up of 4.1 years, 76% of cases remained as isolated demyelinating syndrome, and 70% experienced a total or near-total recovery regardless of whether they received immunotherapy. Further studies are needed, especially concerning series with pathological confirmation and long-term follow-up information.
authors.affiliation: Department of Neurology, General Hospital of Chinese PLA, China.
authors.forename: Jiarui
authors.initials: J
authors.lastname: Yao
authors.affiliation: Department of Neurology, General Hospital of Chinese PLA, China.
authors.forename: Dehui
authors.initials: D
authors.lastname: Huang
authors.affiliation: Department of Pathology, General Hospital of Chinese PLA, China.
authors.forename: Qiuping
authors.initials: Q
authors.lastname: Gui
authors.affiliation: Department of Neurosurgery, General Hospital of Chinese PLA, China.
authors.forename: Xiaolei
authors.initials: X
authors.lastname: Chen
authors.affiliation: Department of Radiology, General Hospital of Chinese PLA, China.
authors.forename: Xin
authors.initials: X
authors.lastname: Lou
authors.affiliation: Department of Neurology, General Hospital of Chinese PLA, China.
authors.forename: Lei
authors.initials: L
authors.lastname: Wu
authors.affiliation: Department of Neurology, General Hospital of Chinese PLA, China.
authors.forename: Chen
authors.initials: C
authors.lastname: Cheng
authors.affiliation: Department of Neurology, General Hospital of Chinese PLA, China.
authors.forename: Jie
authors.initials: J
authors.lastname: Li
authors.affiliation: Department of Neurology, General Hospital of Chinese PLA, China. Electronic address: wpwu_301@hotmail.com.
authors.forename: Weiping
authors.initials: W
authors.lastname: Wu
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D002681:China; D003711:Demyelinating Diseases / Q000473:pathology* / Q000503:physiopathology; D018450:Disease Progression; D004660:Encephalitis / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D066128:Gray Matter / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D066127:White Matter / Q000473:pathology*; D055815:Young Adult
keywords: Chinese; Clinical; Follow-up; Pathology; Radiology; Tumefactive demyelinating disease