Imaging And Pathology Of Primary Central Nervous System Vasculitis

Part I MRI Features of Primary Angiitis of the Central Nervous SystemObjective To analyze MRI features of Primary Angiitis of the Central Nervous System and to evaluate its diagnostic value in this disease.Methods The Institutional Review Boards of Nanjing First Hospital(Nanjing,China)as well as the Nanjing Medical University local ethics committees approved this investigation.We retrospectively analyzed the clinical data of nineteen patients with PACNS.All cases were performed unenhanced and enhanced MRI.The MRI scan protocol included:T1-weighted imaging(T1 WI),T2-weighted imaging(T2WI),diffusion weighted imaging(DWI),apparent diffusion coefficient(ADC)mapping,susceptibility weighted imaging(SWI),fluid attenuated inversion recovery(FLAIR).All enhanced scanning procedures used T1-weighted images,with gadopentate dimeglumine as contrast medium,0.1mmol/kg.After diagnosis,the patients underwent treatment with steroids or steroids plus immunosuppressive agents,which resulted in improvement or disappearance of symptoms,lesions partly or completely disappeared.The lesions of brain were assessed in terms of location,size,shape,signal intensity,enhancement pattern,and changes on the follow-up studies.Results MRI findings showed seven cases of unilateral lesion and twelve cases of bilateral lesions involving grey and white matter.On all sequences,indistinct margins characterised most of the lesions,and certain lesions were oedematous.Nine cases showed bilateral asymmetrical involvement of both frontal,temporal and parietal lobes;three cases showed bilateral asymmetrical basal ganglia involvement.One case of brainstem,one case of left cerebellar hemisphere,two cases of right temporal lobe,two cases of left frontal lobe,and one case of left parietal lobe involvement were also identified.All lesions were slightly hypointense on T1WI,slightly hyperintense on T2WI,markedly hyperintense on FLAIR,iso-or slightly hyperintense on DWI,and hyperintense on ADC mapping.On SWI,we found distortion and thickening of vessels.After contrast injection,all lesions showed focal,cord-like,or goral enhancement.Nineteen PACNS patients,less severely patients were treated with prednisone,1-2 mg/kg/d,eight patients in severe condition were administered intravenous pulse therapy of lg methylprednisolone for three days plus intravenous cyclophosphamide(CTX),CTX(10mg/kg)was given every two weeks for three months.Seventeen patients had aparently improved in their clinical symptoms and imaging performance,only two patients relapsed.Conclusion PACNS has characteristic MRI features,DWI?ADC map and enhanced MRI may be very important for the differentiation and therapy in early stage.Part II Angiographic Features of Primary Angiitis of the Central Nervous SystemPurpose:To analyze cerebral angiography for the diagnosis of primary angiitis of the central nervous system and to evaluate its diagnostic value in this disease.Methdos:The Institutional Review Boards of Nanjing First Hospital(Nanjing,China)as well as the Nanjing Medical University local ethics committees approved this investigation.A total of 40 patients consisted of 14 men and 26 women.Their ages ranged from 29 to 58 years old(45 years on average).During 2-year period,these patients undergone biopsy.In the following,they undergone cerebral angiography.We evaluated cerebral angiographic findings and analyzed the morphology changes and distribution of involved vesssels systematically.Angiographic findings were divided into three types,namely,typical vasculitis,normal,and other.We assessed the value of cerebral angiography for the diagnosis of PACNS.Results:Sixteen patients had angiographic findings with typical vasculitis.None had PACNS at brain biopsy(40%specificity).Brain biopsy showed that seven patients had other diseases.Findings of brain biopsy showed the remaining nine cases were diagnosed as nonspecific disease.Results of cerebral angiography in twelve cases were normal.Angiographic findings of nine cases were atherosclerotic disease.The remaining three patients were other disease.PACNS was found at biopsy in two of the twenty-four patients(0%sensitivity).Conclusion:In this group,Sensitivity and specificity of cerebral angiography for the diagnosis of PACNS were limited.Patients suspected of having PACNS on the basis of clinical and angiographic findings did not have PACNS at biopsy.Typical angiographic findings of PACNS are often associated with other disease,which emphasizes the importance of brain biopsy.Part? Pathological Features of Primary Angiitis of the Central Nervous SystemPurpose:To analyze pathological features of primary angiitis of the central nervous system.Methdos:The Institutional Review Boards of Nanjing First Hospital(Nanjing,China)as well as the Nanjing Medical University local ethics committees approved this investigation.We retrospectively analyzed surgical biopsies from sixteen patients with PACNS.A total of sixteen PACNS patients(19 biopsies)consecutively hospitalized at our hospital(6 males and 10 females;mean age,45 years;age range,25-54years)were included.Results:The biopsy site for nineteen of parenchymal lesions included twelve cases diagnostic vasculitis and four cases nondiagnostic vasculitis.Three morphologic patterns of vasculitis were observed:granulomatous in seven cases,Lymphocytic in three cases,Necrotizing in two cases.Biopsies not diagnostic of PACNS in four cases showed nonspecific gliosis in two cases,mild perivascular mononuclear inflammation in one case,and parenchymal infarction in one case.Conclusion:All positive biopsies were based on the abnormal imaging and biopsies including leptomeninges were more often positive than those that did not.Thus,where possible,a targeted biopsy that includes leptomeninges is recommended to maximize diagnostic potential.In the three different pathological pattern PACNS,as well as biopsy positive versus biopsy negative PACNS groups comparison,there was no significant difference in statistics.