Clinical, Radiological And Pathologicalstudy In Patients With Tumefactive Demyelinating Disease Or Primary Central Nervous System Vasculitis

Objective To summarize the radiological characteristics in patients with tumefactive demyelinating disease.Methods Patients who underwent surgery and had a pathological diagnosis of tumefactive demyelination at PLA General Hospital from January 2006 through January 2014 were enrolled in the present study. Those patients underwent surgeries because of misdiagnosed as brain tumefactive disease or needing differentiation from brain tumors. Their radiological features on MRIs, including lesion number, size, presense and degree of mass effect and edema, as well as enhancement patterns were analysed. In addition, lesion number were compared between pre-and post-biopsy.Results Among 17 cases in our cohort, multifocal lesions were present in 52.9% of cases on their MRIs at disease onset in various locations, predominantly including subcortical or deep white matter (52.9%), periventricular white matter (47.1%), and juxtacortical or cortical gray matter (35.3%). The median largest lesion size on pre-biopsy MRI was 4.8 cm (IQR 3.5,5.9; range 2.6-11.5 cm) and 3.4 cm (IQR 2.7,3.7; range 1.5-5.1 cm) when the perilesional edema was excluded. Perilesional edema and、 or mass effect was observed in 82.4% of our cases. Enhancement was present in 82.4% of our cases, including 50% rim or ring-like,35.7% patchy, and 21.4% venular-like. During a median follow-up period of 4.2 years,87.5% of cases who had a single lesion on their pre-biopsy MRIs had no new lesions post surgery. In nine cases with multiple lesions prebiopsy, lesion number increased in four cases at last follow-up.Conclusion Tumefactive demyelinating lesions (TDLs) could be unifocal or multifocal at disease onset. Lesion size were always more than 2 cm in diameter. Most of the lesions were with perilesional edema and varialble mass effect. TDLs were predominantly located in white matter, while the cortical gray matter could sometimes be involved. Ring-like enhancement, pathy enhancement and venular-like enhancement were often observed in tumefactive demyelinating lesions. TDLs could be reduced or resolved with steroids or without any specific therapy post-surgery, a few of them increased in size or number eventhough applying steroid treatment.Objective To analyze the histopathological features in patients with tumefactive demyelinating disease.Methods Patients who underwent surgery and had a pathological diagnosis of tumefactive demyelination at PLA General Hospital from January 2006 through January 2014 were enrolled. Those patients underwent surgeries because of misdiagnosed as brain tumefactive diseases or needing differentiation from brain tumors. In each case, sections or tissue samples (biopsies or excisions) were collected and the following conditions, including the presence and type of inflammatory infiltration, astrocytes reaction, tissue break-down or necrosis, perivacular lymphocytic infiltration, vascular hyperplasia, as well as degree of myelin loss and of axonal preservation, were assessed.Results All 17 cases share the characteristics features of antive inflammatory demyelianting process which includes parenchymal inflammatory infiltration and confluent demyelination with relative axonal preservation. Macrophages infiltration were present in 100% of cases,52.9% of which contains myelin debris. Reactive astracytes were observed in 88.2% of cases, among which,29.4% contains Creutzfeldt astrocytes and 1 case shows mitotic figure. Perevascular lymphocytes infiltration were present in 76.5% of cases.47.1% of cases contained vascular hyperplasia, and a intramural inflammation were obsrved in 17.6% of cases.35.3% of cases had a sharp lesion border from perilesional tissue. Tissue breakdown were present in 35.3% of cases and necrosis in 17.6%. In six cases who underwent craniotomy, all were misdiagnosed with low-grade glioma.Conclusion pathological changes were always nonspecific in TDLs. Tissue break-down and intramural inflammation could be present in TDLs. Even with histolocial materials, the diagnosis of TDLs were often misleading, usually with astracytes neoplasms. Therefore, the following stains, such as LFB-PAS and CD68, which could detect macrophages and myelin, shoud be used as a conventional pathological approach when differenciating the diagnosis of demyelinating disease and astrocytoma.Objective To analyze the clinical and radiological features in 16 Chinese patients with biopsy confirmed primary central nervous system vasculitis (PCNSV), and to compare them with that in patients with pathogy confirmed tumefactive demyelinating disease.Methods Patients who visited and underwent surgeries at the PLA General Hospital from January 2004 through January 2014 because of being misdianosed as brain tumors/abscesses or other mass-like diseases were enrolled. The clinical, radiological, and pathological data were evaluated and compared with those in patiens with pathology confirmed tumefactive demyelinating disease.Results 16 patients were identified, median age at disease onset was 40 years, with female to male ratio of 1:2.2. In most of our cases, the clinical onset was acute or subacute, with a median duration from first onset to biopsy of 3.2 weeks. The clinical course prior to biopsy was a first neurological event in 75% cases, a relapsing-remitting episode in 12.5%, and a progressing course in 12.5%. During a median follow-up of 2.5 years,50% of our cases remained monophasic,25% remitting-relapsing, and 25% primary progressive.81.3% of cases were misdiagnosed pre-biopsy. Clinical symptoms were unspecific, predominantly including 56.3% of motor dysfunction,43.8% headache, 37.5% sensory dysfunction, and 31.3% dizziness.Multifocal lesions were present in 31.3% of cases on their MRIs at disease onset.81.3% of lesions were located at cortical and subcortical regions,43.8% at basal ganglion and internal capsule regions,37.5% at periventricular white matter,31.3% at thalamus, and 18.8% at infratentorial regions. The median largest lesion size on pre-biopsy MRI was 7.3 cm (IQR 5.4,7.6) and 3.3 cm (IQR 2.5,3.6) when the perilesional edema was excluded. Moderate to severe Edema and/or mass effect were observed in 81.3% of our cases. Enhancement was present in 100% of our cases, predominately including 50% rim or ring-like,43.8% leptomeningal enhancement, and 25% gyrus-like and nodular enhancement respectively. Necrosis, reactive macrophages and astrocytes could be observed in all 16 cases. Intramural inflammation were present in 100% of our cases, including 50% lymphocytic type,37.5% fibrinoid pattern and 12.5% granulomatous pattern. Perivascular inflammatiory infiltration were present in 87.5% of our cases, and demyelination in 62.5%.During a median follow-up period of 2.5 years, seven cases received glucocorticoid therapy, but only two of them had a positive response, four cases had a progressive course although applying steroid. One case was responsive in three who applied cyclophosphamide treatment. The median Rankin score was 2(1,3) pre-biopsy and 1.5 (0,4) at last follow up.Conclusion Clinical symptoms were variable in patients with PCNSV. Even with pathological materials, the differential diagnosis between PCNSV and tumefactive demyelinating disease is considerably challenging, as they share features both radiologically and pathologically. The following may help clinicians to raise the alertness of PCNSV. Gnerally, cortical gray matter were always involed in PCNSV, while TDLs were predominantly located in white matter. In addition, the perilesional edema and mass effect was more obvious in PCNSV than that in TDLs. A combination of at least angiography and biopsy was necessary for some cases who had a uncertain diagnosis post-biopsy with conventional MRI, and the biopsy sections should contain material from leptomeninges and underlying cortex inaddtion to radiologically identified lesion.Objective To obsrve the general and clinical features in patients with tumefactive demyelinating disease.Methods Patients who underwent surgery and had a pathological diagnosis of tumefactive demyelination at PLA General Hospital from January 2006 through January 2014 were enrolled. Those patients underwent surgeries because of misdiagnosed as tumefactive diseases or needing differentiation from brain tumor. Demographic and clinical data, including clinical course, symptoms, diagnosis pre-and post-biopsy, as well as therapy and prognosis were evaluated.Results 17 patients were identified, median age at disease onset was 47 years, with female to male ratio of 1.1:1.82.4% of our cases were thought to have brain tumors, abscess, parasites or other tumefactive diseases prior to biopsy and therefore underwent surgeries. The predominant clinical onset was acute or subacute, with a median duration from first onset to biopsy of 5.3 weeks. The clinical course prior to biopsy was a first neurological event in 82.4% cases, a relapsing-remitting episode in 11.8%, and a progressing course in 5.9%.70.6% of our cases remained monophasic post biopsy during a median follow-up of 4.2 years. Clinical symptoms varied in our cases, predominantly including 41.2% of patients with motor dysfunction,23.5% with optic neuritis, and 23.5% with brainstem symptoms such as dysphagia or diplopia. During a median 4.1-year follow-up period, eight cases received glucocorticoid therapy, but only four of them had a positive response. Nine cases had no specific therapy post surgery, while both clinical and radiological outcome spontaneously improved with no new episodes. Upon the last follow-up,64.7% of cases were diagnosed with clinical isolated syndrome,11.8% with MS, and 23.5% uncertain. The median EDSS score prior to biopsy was 2.0 and 0 at last follow-up.Conclusion Patients with tumefactive demyelinating disease typically had an acute or subacute clinical onset, with variable clinical symptoms. The clinical course were relatively bengin. Although some patients were responsive to steroids, some others had a relapsing or progressive course after steroids therapy. Most patients had a favourable prognosis regardless of whether applying specific treatment.