Advances in imaging studies of primary angiitis of the central nervous system / 中华神经科杂志

Primary angiitis of the central nervous system (PACNS) is a rare inflammatory disease of the central nervous system with unknown etiology affecting the brain and spinal cord. As the incidence of PACNS is low, the clinical manifestations are diverse, the cerebrospinal fluid examination lacks specificity, its diagnosis mainly depends on the "gold standard" pathological biopsy. However, due to the subjectivity of the sampling site, tissue staining and microscopic reading, some patients may be misdiagnosed. In addition, the potentially aggressive course of PACNS may reduce disability and mortality through appropriate immunosuppressive therapy, so the early diagnosis of PACNS is conducive to the prognosis of patients. Imaging examination is convenient, non-invasive, and can provide important information for the diagnosis and differential diagnosis of PACNS from various aspects. Therefore, it is very important to explore the imaging features of the disease. This paper reviews the current research status of imaging examination in PACNS and summarizes the different types of imaging findings in PACNS.

The central nervous system involvement of patients with microscopic polyangiitis / 中华风湿病学杂志

Objective:To explore the clinical features and prognosis of central nervous system involvement in patients with microscopic polyangiitis (MPA).Methods:We retrospectively investigated the clinical data of 138 MPA patients hospitalized with MPA in Tianjin Medical University General Hospital from January 1, 2010 to November 1, 2019. Patients were divided into two groups according to whether they had the central nervous system (CNS) involvement or not and then Kaplan-Meier survival curve was used to analyze the survival rate between the two groups, Logistic regression model analysis was adopted to analyze risk factors, and P<0.05 was considered statistically significant. Results:①29 patients (21.0%)among the 138 MPA had CNS-affected, including 13(44.8%) males and 16(55.2%) females. CNS involvement was present at the diagnosis of MPA in 20 cases (69.0%) and after the diagnosis of MPA in 9 cases (31.0%). ②The clinical manifestations were motor impairment in 14 cases (48.3%), sensory impairment in 10 cases (34.5%), speech loss in 9 cases (31.0%), headache in 8 cases (27.6%), consciousness disorder in 7 cases (24.1%), dysphagia and bucking in 4 cases (13.8%), cranial nerves involvement in 3 cases (10.3%). The imaging manifestations of the head included infarction, hemorrhage, infarction with hemorrhage and linear dural thickening. Five patients received lumbar puncture. One patient showed elevation of cerebrospinal fluid pressure, 1 patient had elevated protein and 5 patients showed elevation of LDH.③Eighteen patients received glucocortoid combined with cyclophosphamide. CNS symptoms recurred in 6 patients, four patients had recurrent cerebral infarction. ④Median survival time was 55 months in the CNS affected group [95% CI=(14.215, 95.785)] and 86 months in the N-CNS group [95% CI=(24.378, 147.622)]. Kaplan-Meier survival curve showed that there was no significant difference in survival rate between the two groups ( χ2=0.07, P=0.794) . Conclusion:The central nervous system involvement of microscopic polyangiitis is not uncommon. The clinical manifestations are various, with motor impairment the most. The most common imaging manifestation is cerebral infarction and the patients mainly presenteas multiple cerebral infarction. However, the CNS involvement of microscopic polyangiitis is not associated with mortality.

A systemic lupus erythematosus patient with sudden dyspnea and disordered consciousness / 中华风湿病学杂志

Objective:To improve the ability of identification and differential diagnosis of severe systemic lupus erythematosus (SLE).Methods:A severe SLE patient with lupus myocarditis, neuropsychiatric lupus, thrombotic microangiopathy (TMA) and other multiple system involvement was reported and discussed.Results:A young female patient developed albuminuria 5 months ago, edema of both lower limbs 3 months ago, change of consciousness 1 month ago and two convulsions attack 2 days ago. She experienced life threatening manifestations such as neuropsychiatric lupus, myocardial involvement of lupus, and TMA. During the course, her condition was generally improved after glucocorticoid pulse therapy and plasma exchange.Conclusion:Various complicated clinical manifestations related to SLE need to be recognized earlier and intervened as soon as possible.

Clinical immunologic analysis of 19 children with neuropsychiatric systemic lupus erythematosus / 中国医师杂志

Objective:To explore the clinical immune manifestations and high risk factors of children with neuropsychiatric systemic lupus erythematosus (NPSLE), so as to find the basis for early clinical differential diagnosis, prevention and treatment of NPSLE.Methods:Ninety-four children with systemic lupus erythematosus (SLE) admitted to Hunan Children′s Hospital from January 1, 2015 to April 30, 2022 were retrospectively selected. Among them, 19 children with NPSLE were in the NPSLE group, and 75 children without NPSLE were in the SLE group. The disease activity, immune system, lymphocyte subsets, inflammatory factors and autoantibodies were compared between the two groups.Results:Among 94 children with SLE, the incidence of NPSLE was 20.21%(19/94). The most common symptoms of 19 children with NPSLE were headache, epileptic seizure and cerebrovascular disease. The Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)-2000 score was (19.89±8.33)points. Most of them had a good prognosis through hormone intervention. Compared with the SLE group, NPSLE group had higher SLEDAI-2000 score, white blood cell count, urine protein, erythrocyte sedimentation rate (ESR), interferon (IFN)-α, positive rate of anti-Sm antibody and anticardiolipin antibody, lower platelet count, interleukin-2 (IL-2), C 3, C 4 level (all P<0.05), while there was no significant difference in positive rate of anti-dsDNA antibody and anti-SSA antibody between the two group (both P>0.05). Conclusions:Compared with SLE children, children with NPSLE are often complicated with many kinds of abnormal immune function, mainly abnormal humoral immune function and many kinds of positive antibody, with increased inflammatory factors.

Vertebrobasilar circulation hemorrhages in childhood primary angiitis of the central nervous system

ABSTRACT Childhood primary angiitis of the CNS (cPACNS) is a poorly understood, rare, and diagnostically challenging neurologic disease. We describe an unusual and autopsy-confirmed case of cPACNS presenting as vertebrobasilar circulation hemorrhagic strokes in a 4-year-old girl. The presentation and clinical features were inconsistent with primary CNS vasculitis and skewed the diagnosis. Autopsy and histopathological analyses revealed a progressive lymphocytic vasculitis affecting the medium to large vessels of vertebrobasilar circulation and sparing the anterior circulation. It is imperative to raise the index of suspicion for cPACNS in any case of unusual or unexplained neurological presentation, especially in the absence of cerebrovascular risk factors and/or coagulation disorders.

Herpes simplex virus-1 triggered hemophagocytic lymphohistiocytosis in a patient with granulomatosis with polyangiitis

ABSTRACT Hemophagocytic lymphohistiocytosis (HLH) is a rare, aggressive hyperinflammatory syndrome in which an inciting event triggers massive, uninhibited activation of T lymphocytes and macrophages. Although viral infections are the most common trigger of HLH, cases of HSV-1 induced HLH are rare in adults. We present the case and postmortem findings of a 27-year-old woman diagnosed with HLH in the setting of immunosuppression for the treatment of granulomatosis with polyangiitis (GPA). Autopsy revealed evidence of herpes simplex virus-1 (HSV-1) infection and no findings suggestive of GPA recurrence.

Arteriopathy in pediatric stroke: an underestimated clinical entity / Arteriopatia em crianças com acidente vascular cerebral: uma entidade clínica subestimada

ABSTRACT Background: Pediatric arterial ischemic stroke (AIS), which was thought to be a rare disorder, is being increasingly recognized as an important cause of neurological morbidity, thanks to new advances in neuroimaging. Objective: The aim of this study was to review the main etiologies of stroke due to arteriopathy in children. Methods: Using a series of cases from our institution, we addressed its epidemiological aspects, physiopathology, imaging findings from CT, MR angiography, MR conventional sequences and MR DWI, and nuclear medicine findings. Results: Through discussion of the most recent classification for childhood AIS (Childhood AIS Standardized Classification and Diagnostic Evaluation, CASCADE), we propose a modified classification based on the anatomical site of disease, which includes vasculitis, varicella, arterial dissection, moyamoya, fibromuscular dysplasia, Takayasu's arteritis and genetic causes (such as ACTA-2 mutation, PHACE syndrome and ADA-2 deficiency). We have detailed each of these separately. Conclusions: Prompt recognition of AIS and thorough investigation for potential risk factors are crucial for a better outcome. In this scenario, neurovascular imaging plays an important role in diagnosing AIS and identifying children at high risk of recurrent stroke.

RESUMO Introdução: O acidente vascular cerebral (AVC) pediátrico, considerado um distúrbio raro, está sendo cada vez mais reconhecido como importante causa de morbidade neurológica, graças aos novos avanços na neuroimagem. Objetivo: Revisar as principais etiologias do AVC por arteriopatia em crianças. Métodos: Utilizando-se de uma série de casos de nossa instituição, abordamos seus aspectos epidemiológicos, fisiopatológicos e de imagem na angiotomografia computadorizada e angiorressonância magnética, sequências convencionais e avançadas de ressonância magnética e medicina nuclear. Resultados: Com base na classificação mais recente de AVC na infância (Classificação Padronizada e Avaliação Diagnóstica do AVC na Infância - CASCADE) propusemos uma classificação modificada com base no local anatômico da doença, que inclui vasculite, varicela, dissecção arterial, Moyamoya, displasia fibromuscular, arterite de Takayasu e causas genéticas (como mutação ACTA-2, síndrome PHACE e deficiência de ADA-2), detalhando cada uma separadamente. Conclusões: O reconhecimento imediato do AVC na infância e a investigação minuciosa de possíveis fatores de risco são cruciais para um melhor resultado. Nesse cenário, a imagem neurovascular desempenha papel importante no diagnóstico de AVC e na identificação de crianças com alto risco de recorrência.

MRI features of primary angiitis of central nervous system / 中国医学影像技术

Objective: To explore MRI features of primary angiitis of central nervous system (PACNS). Methods Clinical and imaging data of 16 patients with PACNS were retrospectively analyzed. MRI features of PACNS were observed. Results Among 16 patients with PACNS, 9 patients were found with single lesion and 7 with multiple lesions. A total of 45 lesions were detected with MRI in all 16 patients, including 38 lesions (38/45, 84.44%) on the supratentorial, 4 lesions (4/45, 8.89%) in the brainstem, 2 lesions (2/45, 4.44%) in the spinal cord and 1 lesion (1/45, 2.22%) in cerebellum. Lesions of 14 patients were demonstrated large or patchy shaped on MRI, while multiple masses or single mass like lesions were found in the other 2 patients. For MR plain scan, the lesions were mainly hypointense on T1WI, hyperintense on T2WI and FLAIR images. Hemorrhage lesions were found in 6 patients. For DWI, hyperintense lesions were found in all of 16 patients, including 13 with hyperintense and 3 with hypointense in ADC map. Various degrees of edema were shown around the lesions. For enhanced MRI, enhanced lesions were found in 14 patients, while lesions without obvious enhancement were found in the other 2 patients. Only 1 patient underwent MRA, with A4 segment of the left anterior cerebral artery stenosis. Conclusion: MRI manifestations of PACNS are complex and diverse. Comprehensive analysis of MRI features is needed, but mass-like PACNS is still difficult to be distinguished from brain tumors.

Small vessel-childhood primary angiitis of the central nervous system: a case report and literature review / 中华儿科杂志

Objective@#To summarize the clinical and pathological features of small vessel-childhood primary angiitis of the central nervous system (SV-cPACNS), discuss the immune therapy and increase the attention to brain biopsy in SV-cPACNS.@*Methods@#The clinical data, pathology of brain biopsy, treatment and outcome of an SV-cPACNS patient hospitalized in Beijing Children's Hospital in February 2016 were analyzed retrospectively. The cases reported at Pubmed, CNKI and Wanfang databases from 2007 to 2017 were searched, the clinical and pathological features, immunotherapy and prognosis of the disease were summarized according to the literature review.@*Results@#A 70 months old girl had 6 times relapses during 5 months' disease course. Symptoms included convulsions, limb paralysis, blurred vision and speech difficulty. Multiple cortical lesions were found successively in brain MRI but CT angiography was negative. The pathology of brain biopsy revealed thickening of small vessel walls together with lymphocytic infiltration. After the treatment with rituximab, remission was achieved and remained stable without recurrence in 1 year follow up. A total of 44 pathologically confirmed cases reported in nearly 10 years were retrieved. Male to female ratio was 1∶3.5. The average onset age was 9.8 years. Clinical manifestations included seizures (37/45, 82%), headache (35/45, 78%), cognitive decline (28/45, 62%), speech regression(20/45, 44%), paralysis (15/45, 33%), and so on; 70% (19/27) patients experienced relapses. Erythrocyte sedimentation rate and C-reactive protein slightly elevated, antinuclear antibody and other autoimmune antibodies were mostly negative. Mild lymphocytosis in cerebrospinal fluid was found in 67%(29/43) patients. 53%(23/43) patients had elevated CSF protein level, several had elevated IgG and positive oligoclonal band. Bilateral multifocal lesions were revealed in 80% (36/45) brain MRIs, meanwhile all angiographies were unremarkable. The pathology showed small angiitis and immunohistochemistry positive for CD3 and CD20. Twenty-seven patients had detailed therapeutic information; 25 of them received immunosuppressive agents, including cyclophosphamide, mycophenolate mofetil and rituximab. One patient died, 26 patients achieved remission with 54% (22/44) had neurological sequelae.@*Conclusions@#SV-cPACNS had varied clinical manifestations, there was no specificity in laboratory and imaging examination and angiography was often negative. The definite diagnosis relied on brain biopsy, which showed lymphocytic inflammation of small vessels. SV-cPACNS tended to relapse and induce neurologic deterioration. Treatment required long-term use of steroids and immunosuppressive agents. Rituximab could be an effective agent.

A clinical analysis of 5 patients with infratentorial primary angiitis of central nervous system / 中华内科杂志

Objective To explore the clinical characteristics of infratentorial primary angiitis in central nervous system (PACNS).Methods A total of 5 cases diagnosed as infratentorial PACNS in the neurology department of Navy General Hospital of PLA in 2015 were enrolled in the study.The clinical,imaging and pathological data were collected and analyzed.Results All the 5 cases were male with the median onset age of thirty-four.Five cases presented with dizziness,two with headache,three with walking unstable,two with facial numbness and one with dysarthria.Rising pressure of cerebrospinal fluid (CSF) (190-245 cmH2O,1 cmH2O =0.098 kPa) was found in 4 cases by the lumbar puncture,mildly increased number of leukocyte in 2 cases [(12-28) × 106/L],increased CSF protein in 3 cases(540-979 mg/L) and increased IgG index in 3 cases (0.84-1.45).Pons lesions were revealed by magnetic resonance imaging(MRI) in 4 cases,brachium pontis lesions in 2 cases,cerebellum lesions in 2 cases,one with midbrain lesion in 1 case,unilateral lesions in 4 cases and bilateral lesion in 1 case.Different degree of edema and mass effect were shown in all lesions by MRI.Patch like enhancement was found by contrast MRI in 5 cases and meningeal enhancement in 2 cases.Elevation of choline (Cho) peak was found by magnetic resonance spectroscopy (MRS) in 4 cases,reduction of N-acetyl aspartate (NAA) peak in 3 cases,appearance of lactate peak in 1 case and lipid peak in another case.Arterial spin labeling (ASL) was performed in 4 cases and no hyperperfusion was found.Susceptibility weighted imaging(SWI) was performed in 3 cases and microhemorrhage in the lesions was found in 2 cases and normal in 1 case.Magnetic resonance arteriography(MRA) was performed in 1 case and no stenosis was found.Digital subtraction arteriography(DSA) was performed in 1 case and multiple stenosis of the intracranial arteries was showed.Two cases had taken the stereotactic brain biopsy and the histopathologic diagnosis was angiitis.Five cases were treated with methylprednisolone and cyclophosphamide was added on in 1 case.Good prognosis was found in all cases.Conclusions Infratentorial PACNS mostly attacks middle-aged males.The lesions tend to locate in unilateral pons,brachium pontis,cerebellum and midbrain.Hemorrhage or microhemorrhage in lesions is often found by SWI and no hyperperfusion is shown by ASL,which would be useful to distinguish PACNS from malignant tumors.Given the limitations of brain biopsy in clinical practice,clinical and imaging features would be helpful to diagnose PACNS.

Neuropsychiatric Lupus in clinical practice / Lúpus neuropsiquiátrico na prática clínica

ABSTRACT Systemic lupus erythematosus (SLE) is a chronic autoimmune disease involving multiple organs, characterized by the production of autoantibodies and the development of tissue injury. The etiology of SLE is partially known, involving multiple genetic and environmental factors. As many as 50% of patients with SLE have neurological involvement during the course of their disease. Neurological manifestations are associated with impaired quality of life, and high morbidity and mortality rates. Nineteen neuropsychiatric syndromes have been identified associated with SLE, and can be divided into central and peripheral manifestations. This article reviews major neuropsychiatric manifestations in patients with SLE and discusses their clinical features, radiological findings and treatment options.

RESUMO Lúpus eritematoso sistêmico (LES) é uma doença autoimune crônica que envolve múltiplos órgãos e sistemas, caracterizada pela produção de auto anticorpos e lesão tecidual. A etiologia do LES é parcialmente conhecida e envolve interação entre fatores genéticos e ambientais. Até 50% dos pacientes com LES apresentam envolvimento neurológico no decorrer da doença. Manifestações neurológicas estão associadas a prejuízo na qualidade de vida e altas taxas de mortalidade e morbidade. Foram identificadas 19 síndromes neuropsiquiátricas em pacientes com LES, divididas entre manifestações do sistema nervoso central e periférico. O objetivo deste artigo é revisar as manifestações neuropsiquiátricas mais importantes. Serão abordadas as características clínicas, os aspectos radiológicos e opções de tratamento dos eventos neuropsiquiátricos.

Quantitative analysis of brain metabolites in systemic lupus erythematosus patients using multivoxel MR spectroscopy / 中华风湿病学杂志

Objective To investigate the metabolite changes in systemic lupus erythematosus (SLE) patients with and without neuropsychiatric symptoms using magnetic resonance spectroscopy (MRS) and explore the associations between image findings and clinical variables. Methods Twenty-two SLE patients with neuropsychiatric symptoms (NPSLE), twenty-one SLE patients without neuropsychiatric symptoms (non-NPSLE) and twenty healthy controls (HCs) underwent routine MRI scan and multivoxel magnetic reson-ance spectroscopy (MVS). The absolute metabolite concentrations were measured bilaterally in the posterior cingulate gyrus (PCG), dorsal thalamus (DT), lentiform nucleus (LN) and posterior paratrigonal white matter (PWM) using LCModel and SAGE software. The relationships between metabolite con-centrations and cognitive function scores were analyzed by Spearman rank correlation. Single-factor Chi-square analysis and t-test were used for analysis. Results ① Compared to control subjects, NPSLE patients had significantly lower N-acetylaspartate (NAA) values in bilateral PCG and DT, with the mean differences of -1.504 [95% confidence interval ( CI) (-2.335, -0.672), P=0.001], -1.460 [95%CI (-2.349, -0.570), P=0.002], -1.259 [95%CI (-1.894, -0.625), P=0.000] and -1.022[95%CI (-1.688, -0.356), P=0.003] for RPCG, LPCG, RDT and LDT, respectively. The concentration of total creatinine were observed to decline in RPCG and RDT, with the mean differences of-1.094 [95%CI (-1.845, -0.342), P=0.003], -0.955 [95%CI (-1.630, -0.280), P=0.006], -1.259 [95%CI (-1.894,-0.625), P=0.006] respectively. Glutamine and glutamate-values decreased significantly in RDT [mean difference=-2.586, 95%CI (-4.139, -1.033), P=0.002]. ② Compared to non-NPSLE patients, NPSLE patients had a lower NAA level in LPCG [mean difference=-1.256, 95%CI (-2.146, -0.367), P=0.006]. Positive correlations between mini-mental state examination scores [RPCG: rs=0.312, P<0.05; LPCG: rs=0.355, P<0.01], Montreal cognitive assessment scores (RPCG: rs=0.362, P<0.01; LPCG: rs=0.285, P<0.05) and NAA values in bilateral PCG were detected. Conclusion Both NPSLE and non-NPSLE patients may have metabolite dysfun-ctions in different brain regions. The cognitive disorder in SLE patients may be interpreted by neuronic damage of PCG.

Clinical analysis of 5 cases of children with primary angiitis of the central nervous system / 中华风湿病学杂志

Objective The purpose of the study was to investigate clinical manifestation and the characters of diagnosis and treatment among children with primary angiitis of the central nervous system (cPACNS) in order to improve awareness of the disease.Methods Clinical data of 5 children with cPACNS in the First People's Hospital of Yunnan Province from January 2009 to December 2013 were collected,and the clinical manifestations and laboratory test results were analyzed and summarized.Results Five cases of children with cPACNS were misdiagnosed at the first clinic visit,and were confirmed a clear diagnosis on the average of (4±6) months; clinical manifestations of five cases of varying degrees of headache,one case with severe headache,2 patients with decreased visual acuity,a cases with hearing were loss,two cases with secondarily generalized seizures; five cases with mild abnormal cerebrospinal fluid examination; 1 case with elevated ESR and CRP level,1 case with elevated immunoglobulin IgG level; 5 cases with abnomal MRI examinations,which showed multiple bilateral lesions,diffuse,lesions,involving the cortex and deep white matter; 4 cases had vascular abnormalities on MRA,treated with corticosteroids alone or in combination with cyclophosphamide and achieved good results.Conclusion Children of primary central nervous system vasculitis is ar are autoimmmune disease primarily involving the central nervous system.It is difficult for the clinical diagnosis.Children need to be wary of the major manifestation of headache associated with vision loss,hearing loss,seizures and other focal neurological system damage.

Parálisis general sifilítica: presentación de 5 casos / Paretic neurosyphilis: report of five patients

We report five male patients, aged 35 to 63 years who suffered from paretic neurosyphilis. The clinical course was that of a subacute dementia with a frontal syndrome, with more apathy than euphoria. All were HIV negative and four were heterosexual. In all, the cerebrospinal fluid had a mononuclear pleocytosis and a positive VDRL. EEG was abnormal in the 3 cases in whom it was performed. One patient in whom a brain angiography was performed, had images of vasculitis. Treatment with 18-24 million units of penicillin per day during two weeks or more, was partially effective.

MRI study of cerebral lesions and analysis of laboratory examination and prognosis of patients with neuropsychiatric systemic lupus erythematosus / 中华风湿病学杂志

Objective To investigate the magnetic resonance imaging (MRI) characteristics of cerebral lesions,laboratory data and prognosis of patients with neuropsychiatric systemic lupus erythematosus (NPSLE) and evaluate the correlation of MRI findings,autoantibodies and prognosis of NPSLE.Methods Thirty-one patients with NPSLE admitted during Jan 1998 to Oct 2011 were enrolled into this study,including 2 males and 29 females.MRI characteristics of cerebral lesions,laboratory data and clinical outcomes were retrospectively analyzed.x2-test and Spearman's correlation were used for statistical analysis.Results Cranial MRI scans on admission were abnormal in 27 (87％) of the 31 patients,among which 17 cases showed diffuse manifestations,9 cases showed focal lesions in brain and 1 case showed brain atrophy.The percentage of cases with focal lesions in the dead group (86％) was significantly higher than that of the survival group (13％) (P＜0.01),while the percentage of diffuse lesion cases was significantly lower in dead group (P＜0.01).Although the mean lesion numbers in the dead group were fewer compared with the survival group,lesion numbers in brainstem (18％) (P＜0.01) or basal ganglia (29％) (P＜0.05) were much more in the dead group.The positive rate of anti-ribosomal P protein antibodies shown in the dead group (43％) was markedly higher than that in the survival group (29％) (P＜0.05).The positive rates of ACL in cases with diffuse lesions and cases with focal lesion were higher compared with cases showing normal MR images (P＜0.01).Furthermore,the positive rate of anticardiolipin antibodies (ACL) in cases with focal lesions (56％) were higher than cases with diffuse lesion (35％) (P＜0.05).There was no correlation between the seven types of autoantibodies and lesion locations such as basal ganglia,subcortical white matter,anterior and posterior horn of lateral ventricle,semiovale center,cerebral cortex,brainstem and cerebellum.Conclusion MRI findings and autoantibodies are correlated in patients with NPSLE and both of them may be used as prognostic markers for NPSLE.Focal lesions,location of brainstem and basal ganglia on cranial MR images,positive anti-ribosomal P protein antibody may predict an unfavorable clinical outcome of NPSLE; however,the correlation between ACL and prognosis could not be determined.

Surrogate biomarkers identification for neuropsychiatric lupus by proteomic fingerprint technology / 中华风湿病学杂志

Objective To identify biomarkers in cerebrospinal fluid (CSF) by proteomic technology and develop a diagnostic model for neuropsychiatric lupus (NPSLE).Methods CSF proteomic spectra of 27 patients with NPSLE before and after treatment,and 27 controls including 17 patients with scoliosis,and 10 SLE patients without neuropsychiatric manifestation (non-NPSLE) were generated by matrix assisted laser desorption/ionization time of flight mass spectrometry (MALDI-TOF-MS) combined with weak cationic exchange (WCX) magnetic beads.Data were analyzed with t test,non-parametric Kruskal-Wallis H test or Wilcoxon sign-rank test.A decision tree model for NPSLE classification was built based on the discriminating peaks.In addition,CSF samples of 12 patients with NPSLE,12 patients with lumbar disc herniation and 9 patients with other neurological conditions were employed as blind test group to verify the accuracy of the model.Results Twelve discriminating mass-to-charge (m/z) peaks were identified between NPSLE and controls.The diagnostic decision tree model,built with a panel of m/z peaks 8595,7170,7661,7740 and 5806,recognized NPSLE with the sensitivity and specificity of 92.6％ and 92.6％ based on training group samples,91.7％ and 85.7％ based on blind test group,respectively.Conclusion Potential CSF NPSLE biomarkers are identified by proteomic technology,the novel diagnostic model is sensitive and relatively specific for the diagnosis of NPSLE.

Clinical analysis of 100 patients with Wegener's granulomatosis / 中华风湿病学杂志

Objective To investigate the clinical features of 100 cases with Wegener's granulomatosis (WG). Methods One hundred patients with WG admitted to our center in recent 11 years were retrospectively analyzed. Results The ratio of male to female was 1.04:1. The average age was (39±17) years (ranging from 4 to 72 years). The upper respiratory tract (86%), lung (82%), kidney (70%) and ocular (53%) were the major affected organs, followed by neurological system ( 12% ) and cardiovascular system ( 11% ). cANCA was positive in 77% of patients, while ANCA was negative in 8%. Images mostly showed multiple nodules/mass with/without cavity in lung (59%) and sinusitis (57%). The pathologic features were necrotic granulomatosis and/or microvasculitis,which was 78% in nasal mucosa/mass biopsy and 75% in lung.Focal segmental necrotic glomerulonephritis ( 59% ) was an important feature for confirming the diagnosis. Of the WG groups, 49% of patients were in generalized subgroup followed by localized (22%), early systemic ( 15% ), severe renal (9%) and refractory group (5%). The patients were treated with corticosteroid and cyclophosphamide. The remission rate in the induction phase was 78%, while the mortality rate was 4%. The follow-up duration ranged from 1 to 145 months. Complications included infection (22%), chronic renal failure( 12% ), deep venous thrombosis( 11% ). Five patients died(8% ), in which 2 patients died of infection.Conclusion The clinical manifestations of Wegener's granulomatosis are complicate. ANCA testing, images of sinus and lung and histological biopsy have played important roles in early diagnosis, which is significant to initiate appropriate and prompt treatment in order to reduce complications and improve prognosis.

Retinal vasculitis in Susac syndrome: case report / Vasculite retiniana na síndrome de Susac: relato de caso

A 29 year old male was admitted at the emergency room suffering from gradually worsening headache followed by nausea. In the hospital patient presented with lethargy, reduction of consciousness level and bilateral hypoacusis. Ophthalmic examination and fluorescein angiography showed retinal vasculitis. This finding was crucial to the diagnosis of Susac syndrome, a rare disease characterized by vasculopathy of retina, encephalic tissues and cochlea. Patient was treated with immunosuppressors to prevent him from developing severe sequelae of this disease.

Paciente masculino de 29 anos foi admitido em pronto-socorro com quadro de cefaléia progressiva há 10 dias seguida de náuseas. Durante internação evoluiu com letargia, redução do nível de consciência e hipoacusia bilateral. Exame oftalmológico e angiofluoresceinografia evidenciaram vasculite retiniana. Este achado foi essencial para o diagnóstico de síndrome de Susac, uma patologia rara caracterizada por vasculopatia de retina, encéfalo e cóclea. Tratamento imunossupressor foi instituído, prevenindo sequelas graves da doença.

Clinical application of regionai cerebral blood flow SPECT imaging in systemic lupus erythematosus patients complicated with neuropsychiatric disorders / 中华风湿病学杂志

Objecfive To observe the changes of regional cerebral blood flow (rCBF) in systemic lupus erythematosus (SLE) patients with neuropsychiatrie disorders and evaluate rCBF SPECT imaging in the detection of neuropsychiatric problems in SLE patients.Methods Twenty neuropsychiatrie SLE patients were enrolled in the study and were performed SPECT rCBF imaging anti CT/MRI scans,respectively.Twenty SLE patients without neuropsychiatrie manifestatiuns (SLE) and 20 healthy volunteers also underwent SPECT rCBF imaging as controls.Semiquantitative analysis was conducted as designed by selecting 3 consecutive crosssections and delineating ROI,which generated HQ and the results were compared between with SLE and controls.Results SPECT rCBF findings were almormal in 20 NPSLE.in which 19 of the 20 with abnormal SPECT findings showed focal uptake defects the other patient showed increased foeal uptake.Four N PSLE patients had ahnormal CT/MRI scans findings.Compared with the methods of rCBF and CT/MRI imaging,the difference was significant (P＜0.01).Nineteen SLE patients without neuropsychiatrie manifestations had normal SPECT findings.the other 1 patient showed focal uptake defect.SPECT findings were all normal in 20 healthy volunteers.Temporal cortex was the most commonly involved region,followed by frontal cortex.Specific values generated from semiquantitative analysis (HQ) of temporal and frontal regions in neruopsychiatrie SLE patients were lower than that in SLE patients and healthy volunteers (P＜0.01 and P＜0.05).Conclusion SPECT rCBF imaging has the potential to be a sensitive tool to detect the neuropsyehiatric disordersin SLE patients,and has important value in helping for early diagnosis and therapy.Hypoperfusion on ECD-SPECT under the territory of the middle cerebral artery (MCA) is the most common observation,and which temporal and frontal regions are the most frequently involved regions in neruopsychiatrie SLE patients.

Neuropsychiatric symptoms in Chinese children with systemic lupus erythematosus / 中华风湿病学杂志

Objective To define the clinical characteristics of NPSLE in Chinese children by analyzing their demographic data,clinical manifestations,laboratory parameters and treatment.Methods Sixtytwo children with SLE who were diagnosed between 1990 and 2006 were retrospectively reviewed.The demographic data,clinical manifestations,laboratory parameters,imaging and treatment were analyzed.The syndromes were compared with those reported in other studies.Results Nineteen (31%) SLE patients with 21 NP events were identified.The most common NP manifestations were cognitive dysfunction (48%),seizure disorder (43%) and headache (29%).The abnormality percentage of SPECT was 80%.86% patients received MP pulses therapy.Most (74%) patients recovered without long-term neurological complications.Conclusi NP symptoms are common in Chinese children with SLE,however,the prese-ntations are different from that reported in other studies.SPECT and MR are sensitive tests for NPSLE.The prognosis of NPSLE in our series is favorable.