Relapsing-remitting Course Research Articles

Behçet syndrome in children and adults: discovering similarities and differences by a comparative study.

Behçet's syndrome (BS) is a rare disorder with a relapsing-remitting course. Clinical variance across geographical regions and different age groups has been observed. This study matched the demographic, clinical and treatment features of adult- and juvenile-onset BS in the Italian population. Two clinical databases of BS patients were compared. The paediatric BS database was collected at the Meyer Children's Hospital, Florence, while the adult BS database was collected at the Careggi University Hospital, Florence. A familiar predisposition for BS was significantly more frequent in the paediatric cohort (3/33 vs 1/165, P = 0.015). No difference emerged in terms of prevalence of HLA-B51 positivity. The proportion of patients meeting the revised ICBD and/or the ISG criteria at BS diagnosis was comparable in the two cohorts. No significant difference emerged between the two cohorts in terms of muco-cutaneous, ocular and neurological involvement, and gastrointestinal symptoms. Articular manifestations resulted as more common in the paediatric cohort, whereas venous vascular events were more frequent in the adult cohort. Regarding treatment strategy, paediatric patients more frequently received no treatment or corticosteroid monotherapy. Conversely, the use of DMARDs, both traditional and biologic, was significantly higher in the adult cohort. Remarkable differences between juvenile-onset and adult-onset BS, both in terms of gender, familiar predisposition and clinical manifestations have been observed and a different therapeutic approach in the real clinical practice of the two settings emerged. Prospective, comparison studies with a longer follow-up are encouraged to provide further data about the disease course for juvenile- and adult-onset BS.

Early Successful Eye Movement Desensitization and Reprocessing (EMDR) Therapy for Verbal Memory Impairment in an Adjustment Disorder: A Case Report in a Newly-Diagnosed Multiple Sclerosis Patient

Multiple sclerosis (MS) is a chronic inflammatory disease of the immune system affecting the central nervous system. Several phenotypes are possible, and cases usually present with a relapsing-remitting (RR) course with disease onset at a young age. MS diagnosis can represent a traumatic event for the patient, possibly evolving into adjustment disorder (AD). AD is defined by the presence of emotional or behavioral symptoms in response to identifiable stress occurring within the prior three months and similarly to post-traumatic stress disorder (PTSD) can significantly affect quality of life. Usually, neuropsychological disorders are not associated with AD. Several treatments are available for AD, and among them, eye movement desensitization and reprocessing (EMDR) is one of the most effective in relieving depression and anxiety. However, little is known about AD and PTSD in the MS population and no data are available on the effectiveness of EMDR for cognitive impairment associated with AD. We describe a 25-year-old patient with RR MS developing an AD with a verbal memory deficit after being diagnosed. Both the psychological and cognitive deficits were diagnosed using an extensive neuropsychological battery. Considering the high impact of the verbal memory deficit, on the patient’s quality of life, an EMDR intervention was planned. After a six-month EMDR intervention performed by two trained neuropsychologists, the patient was retested. There was an improvement in verbal memory tests and depression anxiety scales and the Dissociative Experiences Scale. It is recognized that emotional changes and psychiatric disorders, frequently affect MS patients at diagnosis. It is imperative to recognize this and promptly set a neuropsychological treatment. Moreover, we suggest checking cognition along with depression and anxiety. Finally, to our knowledge, this is the first report of AD with an isolated neuropsychological deficit (verbal memory) developed after the MS diagnosis and treated beneficially with e EMDR. More studies are needed to confirm the efficacy of EMDR in treating cognitive impairment associated with AD in MS patients.

Key Drivers and Facilitators of the Choice to Use mHealth Technology in People With Neurological Conditions: Observational Study

BackgroundThere is increasing interest in the potential uses of mobile health (mHealth) technologies, such as wearable biosensors, as supplements for the care of people with neurological conditions. However, adherence is low, especially over long periods. If people are to benefit from these resources, we need a better long-term understanding of what influences patient engagement. Previous research suggests that engagement is moderated by several barriers and facilitators, but their relative importance is unknown.ObjectiveTo determine preferences and the relative importance of user-generated factors influencing engagement with mHealth technologies for 2 common neurological conditions with a relapsing-remitting course: multiple sclerosis (MS) and epilepsy.MethodsIn a discrete choice experiment, people with a diagnosis of MS (n=141) or epilepsy (n=175) were asked to select their preferred technology from a series of 8 vignettes with 4 characteristics: privacy, clinical support, established benefit, and device accuracy; each of these characteristics was greater or lower in each vignette. These characteristics had previously been emphasized by people with MS and or epilepsy as influencing engagement with technology. Mixed multinomial logistic regression models were used to establish which characteristics were most likely to affect engagement. Subgroup analyses explored the effects of demographic factors (such as age, gender, and education), acceptance of and familiarity with mobile technology, neurological diagnosis (MS or epilepsy), and symptoms that could influence motivation (such as depression).ResultsAnalysis of the responses to the discrete choice experiment validated previous qualitative findings that a higher level of privacy, greater clinical support, increased perceived benefit, and better device accuracy are important to people with a neurological condition. Accuracy was perceived as the most important factor, followed by privacy. Clinical support was the least valued of the attributes. People were prepared to trade a modest amount of accuracy to achieve an improvement in privacy, but less likely to make this compromise for other factors. The type of neurological condition (epilepsy or MS) did not influence these preferences, nor did the age, gender, or mental health status of the participants. Those who were less accepting of technology were the most concerned about privacy and those with a lower level of education were prepared to trade accuracy for more clinical support.ConclusionsFor people with neurological conditions such as epilepsy and MS, accuracy (ie, the ability to detect symptoms) is of the greatest interest. However, there are individual differences, and people who are less accepting of technology may need far greater reassurance about data privacy. People with lower levels of education value greater clinician involvement. These patient preferences should be considered when designing mHealth technologies.

SARS-CoV-2 infection in patients with neuroimmunological disorders in a tertiary referral centre from the north of Portugal

IntroductionThe impact of COVID-19 in patients with neuroimmunological disorders is not fully established. There is some evidence suggesting an increased risk of more severe infection associated with the use of immunosuppressors in this population. ObjectiveTo characterize SARS-CoV-2 infection in patients followed in the neuroimmunology outpatient clinic of a tertiary centre from the north of Portugal. MethodsRetrospective analysis of neuroimmunological patients with PCR-proven SARS-CoV-2 infection during the observational period of 20 months. ResultsNinety-one patients were infected, 68.1% female, with a mean age of 48.9±16.7 years. The median disease duration was 11.0 (IQR 6.0-19.0) years. Sixty-one patients (67.0%) had Multiple Sclerosis, of which 50 with relapsing-remitting course, 12 (13.2%) Myasthenia Gravis (MG), 6 (6.6%) Autoimmune Encephalitis and 6 (6.6%) Chronic Inflammatory Demyelinating Polyneuropathy. Seventy-six patients (83.5%) were taking disease-modifying therapy, 77.6% of which were on immunosuppressants, including anti-CD20 in 12 (13.2%). Most patients had mild COVID-19 (84.6%), with 3 cases (3.3%) of severe disease and, 7 cases (7.7%) of critical disease being reported. In total, 13 patients were hospitalized and 4 died. Patients with severe to critical disease were significantly older than patients with milder forms (69.4±21.0 versus 46.5±14.4 years, p<0.01). MG was also associated with more severe disease (p=0.02). There was no association between comorbidities or use of immunosuppressors (including anti-CD20) and COVID-19 severity. ConclusionsGreater age and MG were associated with severe or critical COVID-19. We found no association between a specific DMT, including anti-CD20, and outcome. Clinical recovery was achieved by 93.4%.

Progression is independent of relapse activity in early multiple sclerosis: a real-life cohort study.

Disability accrual in multiple sclerosis may occur as relapse-associated worsening or progression independent of relapse activity. The role of progression independent of relapse activity in early multiple sclerosis is yet to be established. The objective of this multicentre, observational, retrospective cohort study was to investigate the contribution of relapse-associated worsening and progression independent of relapse activity to confirmed disability accumulation in patients with clinically isolated syndrome and early relapsing-remitting multiple sclerosis, assessed within one year from onset and with follow-up ≥5 years (n = 5169). Data were extracted from the Italian Multiple Sclerosis Register. Confirmed disability accumulation was defined by an increase in Expanded Disability Status Scale score confirmed at 6 months, and classified per temporal association with relapses. Factors associated with progression independent of relapse activity and relapse-associated worsening were assessed using multivariable Cox regression models. Over a follow-up period of 11.5 ± 5.5 years, progression independent of relapse activity occurred in 1427 (27.6%) and relapse-associated worsening in 922 (17.8%) patients. Progression independent of relapse activity was associated with older age at baseline [hazard ratio (HR) = 1.19; 95% confidence interval (CI) 1.13-1.25, P < 0.001], having a relapsing-remitting course at baseline (HR = 1.44; 95% CI 1.28-1.61, P < 0.001), longer disease duration at baseline (HR = 1.56; 95% CI 1.28-1.90, P < 0.001), lower Expanded Disability Status Scale at baseline (HR = 0.92; 95% CI 0.88-0.96, P < 0.001) and lower number of relapses before the event (HR = 0.76; 95% CI 0.73-0.80, P < 0.001). Relapse-associated worsening was associated with younger age at baseline (HR = 0.87; 95% CI 0.81-0.93, P < 0.001), having a relapsing-remitting course at baseline (HR = 1.55; 95% CI 1.35-1.79, P < 0.001), lower Expanded Disability Status Scale at baseline (HR = 0.94; 95% CI 0.89-0.99, P = 0.017) and a higher number of relapses before the event (HR = 1.04; 95% CI 1.01-1.07, P < 0.001). Longer exposure to disease-modifying drugs was associated with a lower risk of both progression independent of relapse activity and relapse-associated worsening (P < 0.001). This study provides evidence that in an early relapsing-onset multiple sclerosis cohort, progression independent of relapse activity was an important contributor to confirmed disability accumulation. Our findings indicate that insidious progression appears even in the earliest phases of the disease, suggesting that inflammation and neurodegeneration can represent a single disease continuum, in which age is one of the main determinants of disease phenomenology.

Saving the kidneys in the lupus patient: Beyond immunosuppression, the need to collaborate across multiple disciplines

Systemic lupus erythematosus (SLE) is the classical systemic autoimmune disease with heterogeneous manifestations and unpredictable behaviour in which almost 70% may have a relapsing-remitting course, while 30% are divided equally between prolonged remission and persistently active disease [ [1] Tselios K. Gladman D.D. Touma Z. Su J. Anderson N. Urowitz M.B. Disease course patterns in systemic lupus erythematosus. Lupus. 2019; 28 ([Internet]JanAvailable from:): 114-122http://www.ncbi.nlm.nih.gov/pubmed/30526328 Crossref PubMed Scopus (28) Google Scholar ]. According to the mosaic of autoimmunity [ [2] Shoenfeld Y. Isenberg D.A. The mosaic of autoimmunity. Immunol Today. 1989; 10 ([Internet]AprAvailable from:): 123-126http://www.ncbi.nlm.nih.gov/pubmed/2665774 Abstract Full Text PDF PubMed Scopus (157) Google Scholar ], immune responses (innate and adaptative), as well as interaction of genes with environmental factors (ultraviolet radiation, smoking and drugs, such as, procainamide, hydralazine and anti-tumour necrosis factor agents) lead to numerous immunologic abnormalities that culminate in persistent immune responses against autologous nucleic acids. In this scenario, lupus nephritis (LN), the most frequent severe complication appears in 35% to 50% of patients in the first ten years of disease and contributes as a major cause for morbidity, rising medical expenses and mortality in SLE.

Association of Viral Infection With the Development and Pathogenesis of Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disease that causes multiple organ damage in women of childbearing age and has a relapsing-remitting course. SLE is caused by the interaction between genetic and environmental factors, however, its underlying triggers remain unknown. Among the environmental factors, the involvement of infections as a trigger for SLE, especially those of viral etiology, has been widely reported. Human endogenous retroviruses (HERVs) may put patients at a genetic predisposition to SLE, while the Epstein-Barr virus (EBV) may play a role as an environmental factor that triggers the development of SLE. It has been suggested that EBV-infected B-cells may become resistant to apoptosis, resulting in the activation, proliferation, and antibody production of autoreactive B-cells, which cause tissue damage in SLE. However, the interaction between the virus and immune cells, as well as the impact of the virus on the differentiation and dysfunction of immune cells, remain unclear. In this review, we focus on the relationship between the development and pathogenesis of SLE and viral infections, as well as the mechanism of SLE exacerbation via activation of immune cells, such as B-cells, based on the latest findings.

Association Between Short-term Exposure to Environmental Air Pollution and Psoriasis Flare

Psoriasis is a chronic inflammatory disease with a relapsing-remitting course. Selected environmental factors such as infections, stressful life events, or drugs may trigger disease flares. Whether air pollution could trigger psoriasis flares is still unknown. To investigate whether short-term exposure to environmental air pollution is associated with psoriasis flares. This observational study with both case-crossover and cross-sectional design retrospectively analyzed longitudinal data from September 2013 to January 2020 from patients with chronic plaque psoriasis consecutively attending the outpatient dermatologic clinic of the University Hospital of Verona. For the case-crossover analysis, patients were included who had at least 1 disease flare, defined as Psoriasis Area and Severity Index (PASI) increase of 5 or greater between 2 consecutive assessments in a time frame of 3 to 4 months. For the cross-sectional analysis, patients were included who received any systemic treatment for 6 or more months, with grade 2 or higher consecutive PASI assessment. We compared the mean and cumulative (area under the curve) concentrations of several air pollutants (carbon monoxide, nitrogen dioxide, other nitrogen oxides, benzene, coarse particulate matter [PM; 2.5-10.0 μm in diameter, PM10] and fine PM [<2.5 μm in diameter, PM2.5]) in the 60 days preceding the psoriasis flare and the control visits. A total of 957 patients with plaque psoriasis with 4398 follow-up visits were included in the study. Patients had a mean (SD) age of 61 (15) years and 602 (62.9%) were men. More than 15 000 measurements of air pollutant concentration from the official, open-source bulletin of the Italian Institute for Environmental Protection and Research (ISPRA) were retrieved. Among the overall cohort, 369 (38.6%) patients with psoriasis flare were included in the case-crossover study. We found that concentrations of all pollutants were significantly higher in the 60 days before psoriasis flare (median PASI at the flare 12; IQR, 9-18) compared with the control visit (median PASI 1; IQR, 1-3, P < .001). In the cross-sectional analysis, exposure to mean PM10 over 20 μg/m3 and mean PM2.5 over 15 μg/m3 in the 60 days before assessment were associated with a higher risk of PASI 5 or greater point worsening (adjusted odds ratio [aOR], 1.55; 95% CI, 1.21-1.99; and aOR, 1.25; 95% CI, 1.0-1.57, respectively). Sensitivity analyses that stratified for trimester of evaluation, with various lag of exposure and adjusting for type of treatment, yielded similar results. The findings of this case-crossover and cross-sectional study suggest that air pollution may be a trigger factor for psoriasis flare.

Benign paroxysmal vertigo of childhood

Background: Benign paroxysmal vertigo (BPV), one of the most common causes of pediatric vertigo, is characterized by recurrent sudden short-lived attacks of vertigo, which resolve spontaneously without residual disability in otherwise healthy children. In this report, we present a literature review on BPV in childhood.Current Concepts: A child with BPV appears frightened and experiences a spinning sensation with occasional nystagmus and vomiting. Episodes usually last less than a minute and are not associated with hearing loss and altered consciousness. Most patients present with a relapsing-remitting clinical course for weeks to years, after which they usually resolve spontaneously. Based on current evidence, BPV of childhood is considered a variant of migraine, and vasospasm observed in patients with migraine plays an important role in the pathogenesis of this disorder. It is important to differentiate BPV from other diseases that present with vertigo, including basilar migraine, closed head injury, psychiatric dizziness, and acute labyrinthitis.Discussion and Conclusion: BPV is a common but important disorder observed in children and should be considered in the differential diagnosis in children with the aforementioned clinical presentation. It is important to reassure patients and parents regarding the benign course of BPV.

Patient-centered approaches for patients with systemic autoimmune rheumatic diseases: development and evolution

ABSTRACT Introduction European Reference Networks (ERNs) are dedicated to rare complex diseases. Systemic autoimmune rheumatic diseases (SARDs) comprise a group of disorders some of which are rare, complex and chronic, characterized by relapsing-remitting course and requiring targeted treatments for long periods; SARDs are also associated with various co-morbidities and therefore health care infrastructures, at the highest level of expertise are required. Areas covered For the current work, literature on the basic characteristics of a center of excellence dedicated to SARDs, its advantages over the existing health infrastructures in order to improve health and social care, its contribution to the education of health care workers, and the related research opportunities are presented. In addition, our experience, vision, and initiatives as a new member of the ERNs are reported. Expert opinion A restructure in healthcare policy and resource allocation, based on centers of expertise, is necessary to improve the medical care of patients with SARDs.

Значення часових етапів при різних типах перебігу розсіяного склерозу для формування прогнозу

Мета дослідження: розробити систему оцінки характеру прогнозу на основі клініко-математичного аналізу показників, що виникають на різних етапах при різних типах перебігу розсіяного склерозу. Матеріали та методи. Використані клінічні (клініко-неврологічний і метод анкетування — за анкетою, розробленою у відділі аутоімунної і дегенеративної патології нервової системи ДУ «Інститут неврології, психіатрії та наркології Національної академії медичних наук України») і математико-статистичні (метод пермутації) методи. За допомогою методу пермутації (перестановочного тесту) в групах хворих з різними типами перебігу обчислювалися відмінності в середніх значеннях клінічних показників, що характеризують перебіг розсіяного склерозу на різних часових етапах: доклінічному етапі, етапі дебюту, рецидивному етапі для рецидивуючого й вторинно-прогредієнтного перебігу, етапі прогресування — для вторинно- і первинно-прогредієнтного. На цій підставі було виділено клінічні показники, які з високою вірогідністю (рівень зв’язку 0,95) на кожному часовому етапі перебігу розсіяного склерозу визначають кінцевий варіант прогнозу захворювання. Результати. Обстежено 280 хворих, серед них: 80 (50 жінок і 30 чоловіків) — з рецидивуючим перебігом, 140 (80 жінок і 60 чоловіків) — із вторинно-прогредієнтним і 60 (30 жінок та 30 чоловіків) — із первинно-прогредієнтним. Характер прогнозу (сприятливий і невизначений при рецидивному перебігу, невизначений і несприятливий при прогредієнтних типах перебігу) оцінювався на основі клініко-діагностичних критеріїв, розроблених з урахуванням особливостей перебігу захворювання в цілому. Проведені дослідження показали, що сприятливий прогноз при рецидивуючому перебігу з високою вірогідністю зустрічався при поєднанні таких клінічних показників, як легка тяжкість дебюту, повна ремісія після дебюту, легкі рецидиви, що розвиваються швидкими темпами, і тривалі ремісії між рецидивами на рецидивному етапі; невизначений прогноз — за наявності дебютів середньої тяжкості, стовбурової симптоматики в дебюті, тяжких і середньої тяжкості рецидивів і тенденції до обтяження й подовження рецидивів на рецидивному етапі. Несприятливий прогноз при вторинно-прогредієнтному перебігу вірогідно пов’язаний з перенесеною вітряною віспою на доклінічному етапі в преморбідному анамнезі, блискавичними темпами розвитку дебюту, неухильним варіантом прогресування, що перебігає без клінічно окреслених періодів стабілізації; невизначений прогноз — із швидкими темпами розвитку дебюту. Несприятливий прогноз при первинно-прогредієнтному перебігу був тісно асоційований з перенесеною тяжкою черепно-мозковою травмою на доклінічному етапі в преморбідному анамнезі, мозочковою симптоматикою в дебюті, формуванням етапу прогресування безпосередньо після дебюту, минаючи етап стабілізації, неухильним варіантом прогресування на етапі прогресування; невизначений прогноз — із перенесеними герпетичними інфекціями на доклінічному етапі в преморбідному анамнезі, легкою тяжкістю дебюту, формуванням етапу прогресування після періоду стабілізації, що настала слідом за дебютом, поступальним варіантом прогресування на етапі прогресування, який перебігає у вигляді чергування періодів повільного накопичення неврологічного дефіциту, що має, як правило, локальний акцент, і етапів стабілізації різної тривалості. Висновки. Отже, за допомогою клініко-математичного аналізу було показано, що формування альтернативних варіантів прогнозу при різних типах перебігу розсіяного склерозу відбувається шляхом вибіркового залучення до єдиного патерну клінічних показників, які мають діагностичне значення на різних часових етапах перебігу захворювання.

Dose-dependent effect of myelin oligodendrocyte glycoprotein on visual function and optic nerve damage in experimental autoimmune encephalomyelitis.

Female Dark Agouti rats were immunized with increasing doses of myelin oligodendrocyte glycoprotein (MOG) to develop experimental autoimmune encephalomyelitis (EAE), a preclinical model of multiple sclerosis. Typical EAE motor impairments were assessed daily and noninvasive visual evoked potentials (VEPs) were recorded at baseline and 5weeks after immunization, with final histopathology of optic nerves (ONs). Immunized rats exhibited a relapsing-remitting clinical course. Both VEP and histological abnormalities were detected in a MOG dose-dependent gradient. Increasing MOG dosage augmented visual function impairment in EAE, which could be monitored with VEP recording to assess demyelination and axonal loss along ONs.

Anti-Phosholipid Syndrome Misdiagnosed As Multiple Sclerosis: A Case Report.

Multiple sclerosis (MS) and anti-phospholipid syndrome (APS) are chronic, immune-mediated, relapsing-remitting disorders affecting young adults. APS is characterized by thrombosis and pregnancy mortality associated with anti-phospholipid antibodies. The diagnosis of APS may be difficult, and it is not uncommon for patients with APS to be misdiagnosed with multiple sclerosis. In this case report, we describe a patient who was diagnosed as having multiple sclerosis for eleven-year. Initially, the patient had neurological deficits with relapsing-remitting courses, like MS but he had not classic magnetic resonance imaging appearance of MS and absence of oligoclonal bands in the cerebrospinal fluid. He did not meet the 'dissemination criteria in time and place' specified in the MS diagnostic criteria. Also, he had an acute myocardial infarction and ischemic stroke at different times. The diagnosis of primary APS was made after the patient had thrombotic attacks, seizure and was positive for anti-phospholipid antibodies (aPLs) twice. Our observations raise awareness about the importance of the early and correct diagnosis of APS. When assessing MS patients, clinicians should consider APS, if the MS has atypical features.

The Immunogenetics of Behcet's Disease.

Behcet's disease (BD) is an autoimmune disorder that affects the blood vessels and thus could entangle virtually every organ of the body. Oral ulceration, genital aphthous lesions, and ocular inflammation are the main manifestations of the disease that tend to have a chronic, relapsing-remitting course. The disease comes from an association between environmental and genetic backgrounds. The clustering of cases in families and the high rate of co-occurrence of the disease in siblings were the initial findings that proposed a genetic basis for BD. Later on, multiple case-control studies and genome-wide association studies were able to clarify particular genes included in the etiopathogenesis of BD. The major gene polymorphisms include HLA and HLA-related genes, interleukins, and other genes involved in inflammation and transcription activation. Herein we have summarized the susceptibility genes that are associated with BD. Investigations on the genetics of BD could potentially clarify the disease pathogenesis and provide insights for the development of better treatments.

The usefulness of visual evoked potentials in the assessment of the pediatric multiple sclerosis.

To evaluate the significance of visual evoked potentials (VEP) in the early diagnosis of optic neuritis (ON) and detecting clinically silent lesions in pediatric multiple sclerosis (PedMS). This study represents one of the largest series of PedMS which evaluated characteristics of VEP in PedMS patients. This was a retrospective study on 52 PedMS patients, aged 7-17 years. VEP analysis were done for all patients, after the first attack of disease and were compared to control subjects according to the pattern-reversal VEP findings. The mean age of patients was 15.65±1.89 years with male to female ratio of 16 (30.8%): 36 (69.2%). All of the patients had a relapsing-remitting course of the disease. ON was discovered on the initial attack in 18 (34.6%) patients, while 30 (57.7%) patients had ON in the second attack. Pathological VEP findings were present in 40 (76.9%) patients, of which 22 (42.3%) PedMS patients had clinically silent lesions. Prolonged latency of P100 waves in the PedMS group was statistically significant when compared to control subjects. The amplitude N1P1 showed a correlation with residual visual deficit. Our results show that ON is a common initial manifestation of PedMS in the Serbian PedMS population. The prolonged P100 latency is the main indicator of ON. VEP is an objective, fast and accessible diagnostic method for detecting clinical and subclinical lesions. Thus, VEP deserves evaluation to be considered as an additional criterion for PedMS diagnosis.

Autoimmune Encephalitis Presenting as Dystonia-Parkinsonism

Autoimmune encephalitis refers to a variety of conditions in which the body's immune system attacks the brain. The disease may present acutely but can also persist through a chronic progressive or relapsing-remitting time course.[1][1] The pathology of autoimmune encephalitis is either antibody- or

Population-based incidence and clinico-radiological characteristics of tumefactive demyelination in Olmsted County, Minnesota, United States.

Tumefactive demyelination (TD) presents with large inflammatory lesions mimicking tumors or other space-occupying lesions. Limited epidemiological, clinical and radiological data exist for TD. We aimed to report the incidence rate, and clinical and radiological features of TD in Olmsted County, Minnesota. We retrospectively reviewed patients with central nervous system inflammatory demyelination-related diagnostic codes (January 1, 1998 to December 31, 2018) in the Rochester Epidemiology Project database, and adjusted incidence rates by age and sex to the 2010 US total population. We used the Expanded Disability Status Scale (EDSS) to assess outcomes (index attack and last follow-up). Of 792 multiple sclerosis (MS) patients, 15 (eight males, seven females) had tumefactive MS, representing 1.9% of the MS population. The median (range) age at attack onset was 34.2(2-61) years. Tumefactive lesion was the first clinical MS attack in 8/16 patients. Cerebrospinal fluid oligoclonal bands (OCBs) were present in 8/12 patients and 11/16 patients met the Barkhof criteria for dissemination in space. Most patients remained fully ambulatory (EDSS score ≤4 in 13/16 patients [81%]) after a median (range) follow-up duration of 10.5(1-20.5) years. Age-adjusted annual incidence rates were 0.46/100,000 (95% confidence interval [CI] 0.12-0.81) for female patients, 0.66/100,000 (95% CI 0.23-1.02) for male patients, and 0.56/100,000 [95% CI 0.28-0.83] overall. When age- and sex-adjusted to the 2010 US total population, the overall annual incidence rate was 0.57 (95% CI 0.28-0.84). Despite aggressive clinical presentation at disease onset, most patients remained fully ambulatory (EDSS score ≤4 in 13/16 patients) with a relapsing-remitting course. Although incidence is rare, TD should be suspected in patients presenting with subacutely progressive neurological deficits associated with magnetic resonance imaging findings of ring enhancement, apparent diffusion coefficient restriction, and OCB on spinal fluid analysis.

Characteristics of cognitive impairment in multiple sclerosis patients depending on different risk factors

Cognitive impairment (CI), along with motor deficit, is a crucial component of a disability aggravation in multiple sclerosis (MS) patients. The aim of this study was to asses characteristics of CI in separate cognitive domains depending on socio-demographic (age, sex, level of education), disease parameters (severity, course type and disease duration) and external factors (smoking). The current study enrolled 137 MS patients (102 women and 35 men) aged from 22 to 69 years. All participants were divided into two groups depending on the disease course: group А – patients with relapsing-remitting (RR-MS) type (n=106) and group B – participants with progressive forms of the disease (n=31). The following study discovered that disruption of separate cognitive domains was present even without the apparent CI according to MоCA (Montreal Cognitive Assessment): executive functions impairment (p=0,0013) was found most frequently in case of RR-MS, and memory (p=0,0233) decline in case of progressive forms. In the group A moderate CI were associated with decrease of memory (p&lt;0,0001), attention (p=0,0061), executive functions (p=0,0005), language (p=0,0080) and abstract thinking (p=0,0018); severe CI – with disorders of attention (p=0,0055), language (p&lt;0,0001) and abstract thinking (p=0,0144). As for the group B, moderate CI were associated with decline of abstract thinking (p&lt;0,0001), and severe CI – with impairment of memory and executive functions (p=0,0337). Level of physical disability and smoking impact CI independently of MS course, meanwhile, presence of higher education proves to be beneficial for preserving cognitive functions. In addition, disease duration, number of exacerbations and male gender (concerning attention decline) can affect cognition in relapsing-remitting course of MS.

MULTIPLE SCLEROSIS, MITOCHONDRIA AND DIET THERAPY: AN OVERVIEW

Multiple sclerosis (MS) is dened as one chronic disease of central nervous system with neurodegenerative and inammatory components, where most of the patients shown a relapsingremitting course dened by the acute inception of focal neurologic decits and consistent focal inammatory changes visible on MRI. The causal factor of this complicated autoimmune and neurodegenerative disease is still unknown. Mitochondrial dysfunction is the key contributor to the neurodegenerative process of this disease. The current review signies the possible potential role of mitochondria in MS and the different dietary approach as a disease modier with the special emphasis on mitochondrial function and neurodegenerations.Research regarding therapeutic implementation of different diet in MS is advancing day by day; but currently remains with limited data. Few studies have been intended with meticulously collected observations, and the very few clinical trials that have been executed with insufcient sample size or length to adequately assess efcacy. More epidemiological and observational studies on dietary implementations were required

Reduction in CD11c+ microglia correlates with clinical progression in chronic experimental autoimmune demyelination

Multiple sclerosis (MS) is a chronic autoimmune demyelinating disease with high variability of clinical symptoms. In most cases MS appears as a relapsing-remitting disease course that at a later stage transitions into irreversible progressive decline of neurologic function. The mechanisms underlying MS progression remain poorly understood. Experimental autoimmune encephalomyelitis (EAE) is an animal model of MS. Here we demonstrate that mice that develop mild EAE after immunization with myelin oligodendrocyte glycoprotein 35-55 are prone to undergo clinical progression around 30 days after EAE induction. EAE progression was associated with reduction in CD11c+ microglia and dispersed coalescent parenchymal infiltration. We found sex-dependent differences mediated by p38α signaling, a key regulator of inflammation. Selective reduction of CD11c+ microglia in female mice with CD11c-promoter driven p38α knockout correlated with increased rate of EAE progression. In protected animals, we found CD11c+ microglia forming contacts with astrocyte processes at the glia limitans and immune cells retained within perivascular spaces. Together, our study identified pathological hallmarks of chronic EAE progression and suggests that CD11c+ microglia may regulate immune cell parenchymal infiltration in autoimmune demyelination.