神经领域-神经系统自身免疫性疾病方向

METHODS:OBJECTIVES:Ultrasound of temporal and axillary arteries may reveal vessel wall inflammation in patients with giant cell arteritis (GCA). We developed a ultrasound scoring system to quantify the extent of vascular inflammation and investigated its diagnostic accuracy and association with clinical factors in GCA. METHODS:This is a prospective study including 89 patients suspected of having GCA, of whom 58 had a confirmed clinical diagnosis of GCA after 6 months follow-up. All patients underwent bilateral ultrasound examination of the three temporal artery (TA) segments and axillary arteries, prior to TA biopsy. The extent of vascular inflammation was quantified by (1) counting the number of TA segments and axillary arteries with a halo and (2) calculating a composite Halo Score that also incorporated the thickness of each halo. RESULTS:Halo counts and Halo Scores showed moderate diagnostic accuracy for a clinical diagnosis of GCA. They correlated positively with systemic inflammation. When compared with the halo count, the Halo Score correlated better with C-reactive protein (CRP) levels and allowed to firmly establish the diagnosis of GCA in more patients. Higher halo counts and Halo Scores were associated with a higher risk of ocular ischaemia. They allowed to identify subgroups of patients with low risk (≤5%) and high risk of ocular ischaemia (>30%). CONCLUSIONS:Ultrasound halo scoring allows to quantify the extent of vascular inflammation in GCA. Extensive vascular inflammation on ultrasound may provide strong diagnostic confirmation and associates with ocular ischaemia in GCA.

METHODS:BACKGROUND:Meningitis is a very rare atypical presenting feature of anti-NMDA receptor encephalitis. In our case report, we describe an unusual clinical presentation of anti-NMDA receptor encephalitis with a biphasic pattern of meningitis followed by encephalitis and discuss potential mechanisms underlying this presentation. We aim to widen the differential diagnosis to be considered in a patient presenting with clinical meningitis and pyrexia. CASE PRESENTATION:This is a case of a 33-year old Caucasian woman who initially presented with a lymphocytic meningitis attributed to a viral infection. She subsequently developed fluctuating consciousness, agitation, visual hallucinations, dyskinetic movements, a generalized tonic-clonic seizure, and autonomic instability. Investigations revealed a diagnosis of anti-NMDA receptor encephalitis secondary to a previously unidentified ovarian teratoma. She made an excellent recovery with immunotherapy and removal of the teratoma. CONCLUSION:Clinicians should consider autoimmune encephalitides in individuals with meningitis, particularly where extensive investigations fail to identify a causative pathogen and there is rapid development of an encephalitic phenotype.

METHODS:BACKGROUND:Guillain-Barré syndrome (GBS) is the most common acute paralytic neuropathy. Many clinical trials indicate acupuncture provides a good effect as a complementary therapy of Western medicine for GBS. The objective of this systematic review protocol is to provide the evidence to evaluate the effectiveness and safety of acupuncture on the treatment of GBS. METHODS:We will search relevant randomized controlled trials investigating the effect of acupuncture for GBS in following databases from start to October 2019: PubMed, Embase, the Cochrane Library, CINAHL Complete, National Digital Science Library, China National Knowledge Infrastructure, and Wanfang Database without language restriction. For articles that meet our inclusion criteria, 2 researchers will extract the data information independently, and assess the risk of bias and trial quality by the Cochrane collaboration's tool. All data will be analyzed by RevMan V.5.3.3 statistical software. RESULTS:According to the Barthel index of Activities of Daily Living (ADL) and the Medical Research Council (MRC) muscle scale, the efficacy and safety of acupuncture for GBS will be determined in this study. CONCLUSION:This systemic review will provide high quality evidence to judging whether acupuncture provides benefits to treat GBS.Prospero registration number: CRD42019158710.

METHODS:AIMS:Although immune-mediated inflammatory diseases (IMID) are associated with multiple mental health conditions, there is a paucity of literature assessing personality disorders (PDs) in these populations. We aimed to estimate and compare the incidence of any PD in IMID and matched cohorts over time, and identify sociodemographic characteristics associated with the incidence of PD. METHODS:We used population-based administrative data from Manitoba, Canada to identify persons with incident inflammatory bowel disease (IBD), multiple sclerosis (MS) and rheumatoid arthritis (RA) using validated case definitions. Unaffected controls were matched 5:1 on sex, age and region of residence. PDs were identified using hospitalisation or physician claims. We used unadjusted and covariate-adjusted negative binomial regression to compare the incidence of PDs between the IMID and matched cohorts. RESULTS:We identified 19 572 incident cases of IMID (IBD n = 6,119, MS n = 3,514, RA n = 10 206) and 97 727 matches overall. After covariate adjustment, the IMID cohort had an increased incidence of PDs (incidence rate ratio [IRR] 1.72; 95%CI: 1.47-2.01) as compared to the matched cohort, which remained consistent over time. The incidence of PDs was similarly elevated in IBD (IRR 2.19; 95%CI: 1.69-2.84), MS (IRR 1.79; 95%CI: 1.29-2.50) and RA (IRR 1.61; 95%CI: 1.29-1.99). Lower socioeconomic status and urban residence were associated with an increased incidence of PDs, whereas mid to older adulthood (age 45-64) was associated with overall decreased incidence. In a restricted sample with 5 years of data before and after IMID diagnosis, the incidence of PDs was also elevated before IMID diagnosis among all IMID groups relative to matched controls. CONCLUSIONS:IMID are associated with an increased incidence of PDs both before and after an IMID diagnosis. These results support the relevance of shared risk factors in the co-occurrence of PDs and IMID conditions.

METHODS:OBJECTIVES:Using a reversible multistate model, we prospectively examined neuropsychiatric (NP) events for attribution, outcome and association with health-related quality of life (HRQoL), in an international, inception cohort of systemic lupus erythematosus (SLE) patients. METHODS:Annual assessments for 19 NP events attributed to SLE and non-SLE causes, physician determination of outcome and patient HRQoL (short-form (SF)-36 scores) were measured. Time-to-event analysis and multistate modelling examined the onset, recurrence and transition between NP states. RESULTS:NP events occurred in 955/1827 (52.3%) patients and 592/1910 (31.0%) unique events were attributed to SLE. In the first 2 years of follow-up the relative risk (95% CI) for SLE NP events was 6.16 (4.96, 7.66) and non-SLE events was 4.66 (4.01, 5.43) compared with thereafter. Patients without SLE NP events at initial assessment had a 74% probability of being event free at 10 years. For non-SLE NP events the estimate was 48%. The majority of NP events resolved over 10 years but mortality was higher in patients with NP events attributed to SLE (16%) versus patients with no NPSLE events (6%) while the rate was comparable in patients with non-SLE NP events (7%) compared with patients with no non-SLE events (6%). Patients with NP events had lower SF-36 summary scores compared with those without NP events and resolved NP states (p<0.001). CONCLUSIONS:NP events occur most frequently around the diagnosis of SLE. Although the majority of events resolve they are associated with reduced HRQoL and excess mortality. Multistate modelling is well suited for the assessment of NP events in SLE.

METHODS:BACKGROUND:For older adults with relapsing-onset multiple sclerosis (MS), limited information is available to inform if, or when, disease-modifying drugs (DMDs) may be safely discontinued. OBJECTIVE:The aim of this study was to project the outcomes of DMD discontinuation among older adults with relapsing-onset MS. METHODS:We projected the 10-year outcomes of discontinuation of a DMD (interferon-β, fingolimod, or natalizumab) among older adults (aged 55 or 70 years) who were relapse-free for 5 or more years and had not reached an Expanded Disability Status Scale (EDSS) score of 6. Outcomes included the percentage of people who had at least one relapse or reached EDSS 6, and quality-adjusted life-years (QALYs), which incorporated both relapses and disability. We used a simulation modeling approach. With increased age, relapses decreased and the effectiveness of DMDs for disability outcomes also decreased. RESULTS:We found lower projected benefits for DMD continuation at 70 years of age than at 55 years of age. Compared with discontinuation, the projected benefit of DMD continuation ranged from 0.007 to 0.017 QALYs at 55 years of age and dropped to 0.002-0.006 at 70 years of age. The annual projected benefits of DMD continuation (0.1-3.0 quality-adjusted life-days) were very low compared with typical patient preferences regarding treatment burden. CONCLUSION:The benefits of DMDs may not be substantial among older adults with relapsing-onset MS. Direct clinical evidence remains limited and the decision of whether to discontinue a DMD should also take into account patient preferences. It is important to gain a better understanding of how age-related changes in the trajectory of relapsing-onset MS affect treatment effectiveness among older adults.

METHODS:OBJECTIVE:We compared the prevalence of physical and mental conditions and rate of health care utilization in mothers of children with multiple sclerosis (MS) (MS-mothers) with that of mothers of children without MS (non-MS-mothers). METHODS:Using population-based administrative data from Ontario, Canada, we identified MS-mothers and non-MS-mothers, matched on maternal age at childbirth, region, and the child's age and sex at the time of MS diagnosis. We compared the prevalence of any physical condition or any mood or anxiety disorder between the cohorts, in the child's MS diagnosis year, and 5 years before and after diagnosis. We compared rates of physician visits before diagnosis, during the diagnosis year, and after the child's diagnosis using multivariable regression models with generalized estimating equations adjusting for maternal age, socioeconomic status, region of residence, and index year. RESULTS:We identified 156 MS-mothers and 624 non-MS-mothers. MS-mothers had a higher prevalence of any physical condition and of any mood or anxiety disorder than non-MS-mothers, before, during, and after their child's diagnosis. On multivariable analysis, MS-mothers did not have higher rates of primary care visits (rate ratio [RR] 1.04; 95% confidence interval [CI] 0.90-1.20), but exhibited increased odds of having any psychiatry visits (odds ratio 1.60; 95% CI 1.10-2.31); the rate of visits did not differ when they did occur (RR 0.66; 95% CI 0.33-1.30). CONCLUSION:Mothers of children with MS use more mental health services before and after their child's MS diagnosis than mothers of children without MS. Pediatric health care providers should consider the mental well-being of mothers and their children with MS.

METHODS:BACKGROUND:Transverse myelitis (TM) is due to inflammatory spinal cord injury with bilateral neurologic involvement, which is sensory, motor, or autonomic in nature. It may be associated with autoimmune disease, vaccination, intoxication and infections. The most common infection cause of TM is Coxsackie virus and Mycoplasma pneumoniae. The cryptococcosis is rare. We present the case of disseminated cryptococcosis revealed by transverse myelitis in an immunocompetent 55-year-old male patient. The literature review is also stated. CASE PRESENTATION:The 55-year-old man suffered from gradual numbness, weakness in both lower limbs and finally paralyzed in the bed. The thoracic spine Computed tomography (CT) was normal, but multiple nodules in the lung were accidentally discovered. Thoracic Magnetic Resonance Imaging (MRI) showed diffused thoracic spinal cord thickening and extensively intramedullary T2 hyper intensity areas. Gadolinium contrast enhanced T1WI showed an intramedullary circle-enhanced nodule at 9th thoracic level. Diagnosis was made by histological examination of the bilateral lung biopsy. The patient was treated successfully with systemic amphotericin B liposome and fluconazole and intrathecal dexamethasone and amphotericin B liposome. CONCLUSIONS:This is a patient with disseminated cryptococcosis involving the lung, spinal cord and adrenal glands, which is rare in the absence of immunodeficiency.

METHODS:OBJECTIVE:To gain a better understanding of the complex patterns of causes that contribute to death due to multiple sclerosis (MS) by assessing the relationship between MS and other causes of death listed on death certificates. METHODS:Multiple cause of death data for all adult deaths (aged ≥18 years) in British Columbia, Canada, between 1986 and 2013 were accessed. All causes, as listed on the death certificate, whether underlying or contributing, were considered "any mention" causes. The associations between mention of MS on the death certificate and mention of other causes of death were examined by logistic regression, adjusted for age, sex, and calendar year (Bonferroni-corrected α level = 0.002). Findings were also sex-stratified. RESULTS:Among 771,288 deaths, MS was mentioned on 2,153 certificates. If MS was mentioned (versus not mentioned), there was a greater chance that specific conditions contributed to the death: respiratory infection (adjusted odds ratio [aOR], 3.03 [95% confidence interval (CI), 2.73-3.36]), aspiration pneumonia (aOR, 7.15 [95% CI, 6.23-8.22]), urinary tract infection (UTI) (aOR, 10.2 [95% CI, 8.7-12.0]), other infection including sepsis (aOR, 1.34 [95% CI, 1.15-1.56]), and skin disease (aOR, 5.06 [95% CI, 3.96-6.46]). Sex differences existed for urinary tract infection (men: aOR, 14.9 [95% CI, 11.5-19.3]; women: aOR, 8.00 [95% CI, 6.53-9.81]) and chronic respiratory disease (men = aOR, 1.36 [95% CI, 1.14-1.63]; women = aOR, 0.97 [95% CI, 0.84-1.13]). CONCLUSIONS:Deaths attributed to MS were commonly caused by infection (especially respiratory and urinary tract-related); conditions associated with advanced disability and immobility, such as aspiration pneumonia; and chronic respiratory disease in men. All are potentially modifiable; interventions that reduce the frequency or severity of these complications could improve survival in MS.

METHODS::Multiple sclerosis (MS) is characterized by central nervous system lesions that lead to neurological dysfunctions including fatigue, depression and anxiety. MS is affecting almost 2.3 million people around the world, with the significant highest prevalence in the North America. MS also affects different cognitive abilities, such as attention, memory and executive functions. Furthermore, a significant impairment in verbal fluency and naming abilities in patients with MS has been reported. RehaCom, is a software that has improvement effects on cognitive functions. The goal of this research is to investigate the effect of treatment with RehaCom on verbal performance in patients with MS. To select the participants, 60 patients with MS who referred to our clinic were chosen randomly and divided into Control (n = 30) and Experimental (n = 30) groups. The participants in the experimental group were treated by RehaCom software for 10 sessions during 5 weeks (2 sessions per week and each session was 1 h). Controlled Oral Word Association Test (COWAT) and California Verbal Learning Test - Second Edition (CVLT-II), were used to assess verbal performance (verbal fluency, and verbal learning and memory) at weeks 0 (baseline), 5 (post-test) and 10 (follow-up). The results showed that, treatment with RehaCom improved verbal performance in patient with MS, at both post-test and follow-up stages. In conclusion, treatment with RehaCom cognitive rehabilitation software can improve verbal fluency, and verbal learning and memory in patient with MS, possibly by affecting the brain regions involved in language performance.