Systemic lupus erythematosus associated with sarcoidosis: Case report

BackgroundThe association between systemic lupus erythematosus (SLE) and sarcoidosis has been considered as extremely rare. Most often, sarcoidosis coexists with Sjögren's syndrome. Researching the literature, it seems that the association of SLE and sarcoidosis is much more frequent than previously thought.Aim of the workWe present a case of a Serbian woman who was diagnosed with coexisting sarcoidosis and long-standing SLE.Case presentationThe 40 years old SLE patient was in long-standing remission on oral prednisolone (10 mg/day) and hydroxychloroquine (HCQ) (400 mg/day). She presented with fatigue, chest pain, and dry cough. Chest computerized tomography (CT) showed hilar and mediastinal lymphadenopathy. The biopsy had been performed and results showed sarcoidosis. Diagnosis has been confirmed: eosinophilic granulomas without central necrosis morphologically corresponding to chronic granulomatous lymphadenitis like sarcoidosis. Thereafter, the patient was hospitalized. No new symptoms appeared, and the physical examination was unremarkable. Serum calcium was elevated (2.75 mmol/l), anti-nuclear antibody (ANA), anti-double stranded deoxyribonucleic acid (anti-dsDNA) and anti-Ro antibodies were positive. Angiotensin converting enzyme (ACE) level was high normal (51 IU/L) and QuantiFERON-TB Gold test negative. The dose of prednisolone was increased to 40 mg/day with HCQ and the patient was discharged from hospital. On follow-up the patient improved with reduction of the steroid dose.ConclusionThe association of SLE and sarcoidosis should be considered even though it is reported to be extremely rare. May be the real incidence of these combinations is underestimated. More research about genetics and pathogenesis is needed to completely understand these conditions.     

肌萎缩侧索硬化伴认知功能障碍的研究进展

认知功能障碍在肌萎缩侧索硬化（ALS）患者中是普遍存在的，ALS伴认知功能障碍常提示预后不良。近年来对ALS患者认知功能障碍的研究取得了一定的进展，本文对ALS伴认知功能障碍的临床特点、生物标志物、遗传学特征和最新治疗进展进行综述，以期为ALS伴认知功能障碍的临床诊疗提供新思路。     

General vaccination willingness and current vaccination status in relation to clinical and psychological variables in patients with multiple sclerosis

BackgroundInfections can have a significant impact on morbidity and mortality in multiple sclerosis (MS) patients. Therefore, vaccinations are of immense importance. If vaccination willingness is to be increased, possible influencing factors should be identified. The aim of the present study was to investigate the status of active immunisation in MS patients in association with sociodemographic, clinical-neurological, psychopathological and personality variables using the NEO-Five Factor Inventory, the Temperament and Character Inventory-Revised and the Hospital Anxiety and Depression Scale.MethodFour hundred and four MS patients from two German neurological hospitals were examined for their vaccination attitudes, in detail, the general willingness to vaccinate and the current vaccination status of mumps, measles and rubella (MMR) as well as tetanus and influenza. We also looked at the current level of disability in relation to the current vaccination status, as well as possible associated personality and psychopathological variables.ResultsPatients with a complete MMR vaccination status were significantly younger and those with a complete influenza vaccination status were significantly older than those with related incomplete vaccination status. Tetanus vaccination status completeness did not differ depending on age and did not show substantial association with personality scores. However, influenza vaccination completeness was associated with differences in personality and psychopathological variables; extraversion, openness, novelty seeking, harm avoidance and anxiety. A reported general vaccination willingness was significantly correlated with the current completeness of tetanus and influenza vaccinations. Novelty seeking, persistence, extraversion, agreeableness, conscientiousness and neuroticism were found associated with an increased vaccination willingness. Anxiety and depression were not related to general vaccination willingness.ConclusionsNo specific personality trait could be defined on its own in relation to general vaccination willingness or complete vaccination status. Younger patients should be made more aware of influenza vaccination. Reasons for rather low vaccination rates need to be further investigated.     

Evaluation of unsupervised 30-second chair stand test performance assessed by wearable sensors to predict fall status in multiple sclerosis

BackgroundOne in two people with multiple sclerosis (PwMS) will fall in a three-month period. Predicting which patients will fall remains a challenge for clinicians. Standardized functional assessments provide insight into balance deficits and fall risk but their use has been limited to supervised visits.Research questionThe study aim was to characterize unsupervised 30-second chair stand test (30CST) performance using accelerometer-derived metrics and assess its ability to classify fall status in PwMS compared to supervised 30CST.MethodsThirty-seven PwMS (21 fallers) performed instrumented supervised and unsupervised 30CSTs with a single wearable sensor on the thigh. In unsupervised conditions, participants performed bi-hourly 30CSTs and rated their balance confidence and fatigue over 48-hours. ROC analysis was used to classify fall status for 30CST performance.ResultsNon-fallers (p = 0.02) but not fallers (p = 0.23) differed in their average unsupervised 30CST performance (repetitions) compared to their supervised performance. The unsupervised maximum number of 30CST repetitions performed optimized ROC classification AUC (0.79), accuracy (78.4%) and specificity (90.0%) for fall status with an optimal cutoff of 17 repetitions.SignificanceBrief durations of instrumented unsupervised monitoring as an adjunct to routine clinical assessments could improve the ability for predicting fall risk and fluctuations in functional mobility in PwMS.     

Enlarged high frequency oscillations of the median nerve somatosensory evoked potential and survival in amyotrophic lateral sclerosis

ObjectiveA large N20 and P25 of the median nerve somatosensory evoked potential (SEP) predicts short survival in amyotrophic lateral sclerosis (ALS). We investigated whether high frequency oscillations (HFOs) over N20 are enlarged and associated with survival in ALS.MethodsA total of 145 patients with ALS and 57 healthy subjects were studied. We recorded the median nerve SEP and measured the onset-to-peak amplitude of N20 (N20o-p), and peak-to-peak amplitude between N20 and P25 (N20p-P25p). We obtained early and late HFO potentials by filtering SEP between 500 and 1 kHz, and measured the peak-to-peak amplitude. We followed up patients until endpoints (death or tracheostomy) and analyzed the relationship between SEP or HFO amplitudes and survival using a Cox analysis.ResultsPatients showed larger N20o-p, N20p-P25p, and early and late HFO amplitudes than the control values. N20p-P25p was associated with survival periods (p = 0.0004), while early and late HFO amplitudes showed no significant association with survival (p = 0.4307, and p = 0.6858, respectively).ConclusionsThe HFO amplitude in ALS is increased, but does not predict survival.SignificanceThe enlarged HFOs in ALS might be a compensatory phenomenon to the hyperexcitability of the sensory cortex pyramidal neurons.     

Methodological Quality Assessment of Meta-analyses and Systematic Reviews of the Relationship Between Periodontal and Systemic Diseases

ObjectivesThe aims of this article are to identify all the published systematic reviews (SRs) and meta-analyses (MAs) that studied the relationship between periodontal and systemic diseases and to assess their quality using 2 scales (the Overview Quality Assessment Questionnaire [OQAQ] and A Measurement Tool to Assess Systematic Reviews [AMSTAR] checklist).MethodsFor SRs and MAs to be included, they should have investigated one of the following systemic diseases: pulmonary conditions, cardiac conditions, endocrine conditions, cancer, blood disorders, psychological conditions, anxiety, depression, mood disorders, and several other diseases. Two investigators screened MEDLINE via PubMed, Embase, Scopus, Web of Science, and the Cochrane Database of Systematic Reviews. The tools used to evaluate quality were the AMSTAR scale and OQAQ. The protocol was prospectively registered in PROSPERO (CRD42018102208).ResultsThe search strategy found 691 unique articles, 42 of which met the eligibility criteria and were included in this review. Diabetes mellitus was the most investigated disease (14 out of 42 studies), followed by obesity (11 studies) and cardiovascular diseases (5 studies). A total of 40 reviews reported on the characteristics of included studies, and, as per the AMSTAR scale, 39 reviews had an a priori design. The number of reviews that fulfilled the status of publication criterion was the lowest (7 reviews only), followed by the number used in the assessment of publication bias (11 reviews). The number of high-quality reviews was higher with the OQAQ than with the AMSTAR checklist (33 vs 25 studies), but the AMSTAR showed a higher number of medium-quality reviews than the OQAQ (14 vs 6 studies). Both showed the same number of low-quality reviews.ConclusionsHigh-quality SRs and MAs are crucial to understanding the relationship between systemic and periodontal diseases. Medical practitioners must be able to inform patients about oral health and specific periodontal health concerns.     

Effectiveness of cognitive rehabilitation for people with multiple sclerosis: a meta-synthesis of patient perspectives

While previous randomised controlled trials and meta-analyses offer only limited evidence for the effectiveness of cognitive rehabilitation, qualitative studies examining patient perspectives report more positive outcomes. This meta-synthesis of qualitative studies examined patient perspectives of cognitive rehabilitation for memory, attention, and executive function problems in people with multiple sclerosis. Using set eligibility criteria, we screened electronic databases, reference lists, and academic networks for relevant papers. Seven papers (195 participants) were selected. Two independent researchers conducted quality appraisals of papers. Data analysis, guided by the thematic synthesis approach, yielded six main themes. These suggested that patients benefitted from the group environment in rehabilitation. Cognitive rehabilitation facilitated the participants’ reflection and awareness of their cognitive deficits, and was associated with increased knowledge and understanding of their illness. Increased strategy use was reported and associated with improvements in cognitive functioning and greater confidence and perseverance. Participants reported emotional and social improvements, and felt more optimistic. Overall, these changes had a positive impact on participants’ quality of life. This synthesis of qualitative studies indicates that people with multiple sclerosis who experience cognitive deficits benefit from cognitive rehabilitation programmes. This finding must, however, be viewed in light of the limitations of this meta-synthesis. The meta-synthesis was registered in the PROSPERO database under CRD42017040148.     

Relationship between EMG-detected and ultrasound-detected fasciculations in amyotrophic lateral sclerosis: A prospective cohort study

ObjectivesFasciculation potentials (FP) are an important consideration in the electrophysiological diagnosis of ALS. Muscle ultrasonography (MUS) has a higher sensitivity in detecting fasciculations than electromyography (EMG), while in some cases, it is unable to detect EMG-detected fasciculations. We aimed to investigate the differences of FP between the muscles with and without MUS-detected fasciculations (MUS-fas).MethodsThirty-one consecutive patients with sporadic ALS were prospectively recruited and in those, both needle EMG and MUS were performed. Analyses of the amplitude, duration, and number of phases of EMG-detected FPs were performed for seven muscles per patient, and results were compared between the muscles with and without MUS-fas in the total cohort.ResultsThe mean amplitude and phase number of FP were significantly lower in patients with EMG-detected FP alone (0.39 ± 0.25 mV and 3.21 ± 0.88, respectively) than in those with both FP and MUS-fas (1.22 ± 0.92 mV and 3.74 ± 1.39, respectively; p < 0.0001 and p = 0.017, Welch’s t-test).ConclusionSmall FP may be undetectable with MUS. MUS cannot replace EMG in the diagnostic approach for ALS.SignificanceClinicians should use a combination of EMG and MUS for the detection and quantitative analysis of fasciculation in ALS.