神经领域-神经系统自身免疫性疾病方向

METHODS:INTRODUCTION:The Duke Myasthenia Gravis (MG) Clinic Registry is a disease-specific database containing physician-derived data from patients seen in the Duke MG Clinic since 1980. METHODS:Data from 1060 MG patients initially seen between 1980 and 2008 were reviewed. RESULTS:Fifty-four percent were male. Symptoms began after age 50 in 66% of males and 42% of females. Peak onset age in males was in their 60's; females had no predominant onset age. Onset age for both sexes increased from 1980 to 2008. Thymoma was present in 8.5%. Weakness was limited to ocular muscles for at least 2 y in 22% and became generalized later in 8.3% of these. Acetylcholine receptor antibodies were present in 78% overall, 82% with generalized MG and 52% with ocular MG (OMG). The distribution of MG disease class was similar in males and females, except that a greater proportion of women experienced myasthenic crisis and men were more likely to have OMG. DISCUSSION:Data in the Registry permit comprehensive and longitudinal analysis of a validated MG population. Analysis of Registry data shows that the frequency of AChR antibody negative MG, ocular MG, and thymoma are similar to other reports, but the onset age and proportion of males have progressively increased compared to studies published more than 20 y ago. These observations demonstrate the value of collecting comprehensive clinical information and comparing historic and contemporary populations. Other potential uses of Registry data include comparison of outcome measures in different disease subgroups and the response to specific treatments.

METHODS:OBJECTIVE:Myasthenia gravis (MG) is characterized by fluctuating muscle weakness due to immune mediated damage of acetylcholine receptor (AchR). COVID-19 infection, mental stress and non-availability of drugs following lockdown may worsen myasthenic symptoms. We report the impact of COVID 19, and lockdown on the physical and mental health, and quality of sleep in a cohort of MG. METHODS:Thirty-eight MG patients were telephonically interviewed 2months after the declaration of lockdown in India. The difficulty in procuring drugs, complications, and worsening in the MG Foundation of America (MGFA) stage were noted. The patients were enquired about MG Quality of Life 15 (MGQOL15), MG Activity of Daily Living (MGADL), Hospital Anxiety and Depression Scale (HADS) and Pittsburgh Sleep Quality Index (PSQI) using a prefixed questionnaire. Their pre-COVID parameters were retrieved from our earlier trial data, which was completed 4months back. The scores of the above mentioned parameters before and after COVID were compared. RESULTS:Their median age was 45 years, and the median duration of treatment for MG was 4.5years. Eleven (28.9 %) patients were hypertensive and 3(7.9 %) diabetic. All were on prednisolone and 18(47.4 %) received azathioprine. None developed COVID, but three had other infections. Two patients needed hospitalization because of wrong medication in one and severe anxiety-insomnia in another. Following COVID19 and lockdown, MG patients had worsening in MGQOL15, MGADL, HADS and PSQI scores. Pittsburgh Sleep Quality Index score correlated with MGQOL15 and dose of acetylcholine esterase inhibitors. CONCLUSION:COVID-19 and lockdown were associated with anxiety, depression, poor MGQOL and sleep especially in severe MG patients.

METHODS::A working group convened by the Section of Multiple Sclerosis and Neuroimmunology of the Polish Neurological Society has developed a statement with regard to the currently available mRNA vaccines (Pfizer-BioNTech and Moderna) preventing novel coronavirus (severe acute respiratory syndrome coronavirus 2, SARS-CoV-2) infection, which causes Coronavirus disease 2019 (COVID-19), in patients with multiple sclerosis (MS). This statement has been based on the literature available as of 15 January, 2021. The guidance will be updated as new data emerges. All data regarding the above-mentioned vaccines comes from clinical trials which have been reviewed, published and approved by the regulatory authorities [1, 2]. In the current manuscript, whenever a SARS-CoV-2 vaccine is discussed, it refers to mRNA vaccines only.

METHODS::The novel coronavirus disease 2019 (COVID-19) pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), remains a global challenge. Currently, there is some information on the consequences of COVID-19 infection in multiple sclerosis (MS) patients, as it is a newly discovered coronavirus, but its far-reaching effects on participation in neurodegenerative diseases seem to be significant. Recent cases reports showed that SARS-CoV-2 may be responsible for initiating the demyelination process in people who previously had no symptoms associated with any nervous system disorders. It is presently known that infection of SARS-CoV-2 evokes cytokine storm syndrome, which may be one of the factors leading to the acute cerebrovascular disease. One of the substantial problems is the coexistence of cerebrovascular disease and MS in an individual's life span. Epidemiological studies showed an enhanced risk of death rate from vascular disabilities in MS patients of approximately 30%. It has been demonstrated that patients with severe SARS-CoV-2 infection usually show increased levels of D-dimer, fibrinogen, C-reactive protein (CRP), and overactivation of blood platelets, which are essential elements of prothrombotic events. In this review, the latest knowledge gathered during an ongoing pandemic of SARS-CoV-2 infection on the neurodegeneration processes in MS is discussed.

METHODS:BACKGROUND:Some epidemiologic studies have reported a sharp increase in multiple sclerosis (MS) incidence in different provinces in Iran. This report aimed to investigate more closely the increasing trend of MS incidence in the past 10 years in Iran. METHODS:In this longitudinal study, the data for all MS patients meeting the McDonald criteria were obtained from a national registry, coordinated by the Ministry of Health (MOH). Joinpoint (JP) regression was used for time trend analysis of MS incidence and determine the optimal number of significant joinpoints. Finally, an annual percentage change (APC) in MS incidence for each segment of the trend line was estimated with 95% confidence interval. RESULTS:The mean age of the patients and the mean annual incidence rate of MS were 30.9 ± 1.1 and 5.3 ± 1.9 per 100,000 population, respectively. The overall incidence rate of MS had increased significantly from 2.14 in 2006 to its peak (7.5) in 2014, per 100,000 population (APC = 12%, P < 0.001). The first JP was observed in 2011 in both male and female groups. The overall APC in the first segment was 22.6% (17.2-28.2%, p < 0.01). Besides, the corresponding APC values for males and females were 22.1% (14.7-30%, p < 0.01) and 22.5% (17.5-27.8%, p < 0.01), respectively. After 2011, the MS incidence underwent a more or less decreasing trend in both genders. CONCLUSION:Contrary to previous studies, the MS incidence trend in Iran was rising just before 2011, and in the recent decade, Iran has a stable rate of MS cases.

METHODS:BACKGROUND:Currently, myelin oligodendrocyte glycoprotein (MOG)-IgG-associated encephalomyelitis (MOG-EM) is regarded as an independent inflammatory demyelinating disease. Magnetic resonance imaging (MRI) abnormalities occur in 44.4% of patients with MOG-EM. However, symmetrical deep gray matter involvement with leptomeningeal enhancement is rarely described in the literature. CASE PRESENTATION:A 3-year-old boy was admitted to our hospital because of acute onset fever, headache, vomiting and disturbance of consciousness. Neurological examination showed somnolence, neck stiffness and positive Kernig's sign. Brain MRI demonstrated bilateral symmetrical lesions in the basal ganglia and thalamus as well as diffuse leptomeningeal enhancement along the sulci of bilateral hemisphere. Cerebrospinal fluid analysis demonstrated increased cell count (7 cells/mm3, mononuclear cells dominant) and protein (1.17 g/L) without glucose and chloride abnormality. Work-up for infectious and autoimmune causes, serum MOG IgG was positive by cell based assay. Therefore, a diagnosis of MOG-EM was established according to the international recommendatory criteria in 2018. He was administrated with intravenous methylprednisolone followed by oral corticosteroids and had recovered completely within 1 week. CONCLUSIONS:In the setting of meningoencephalitis-like clinical presentation with bilateral symmetrical deep gray matter involvement, MOG-EM should be distinguished from other infectious and autoimmune disorders, such as Epstein-Barr virus (EBV) encephalitis, Japanese encephalitis and Anti-NMDA receptor (NMDAR) encephalitis. Besides, aseptic meningitis associated with leptomeningeal enhancement may be an atypical phenotype of MOG-EM.

METHODS::Acute necrotizing encephalopathy (ANE) is a recently identified, uncommon encephalopathy affecting children. ANE is characterized by a preceding viral illness followed by seizures and rapid progressive neurologic deterioration. The diagnosis of ANE is made based on clinical presentation and characteristic multifocal brain lesions seen on computed tomography (CT). We report a previously healthy two-year-old boy who presented to our emergency department (ED) after a seizure in the setting of fever and diarrhea. He was ultimately diagnosed with ANE and treated with steroids and IVIG. Early identification of this high morbidity condition by its typical clinical picture and characteristic radiologic findings is key to allow for optimal treatment.

METHODS::Reports of Guillain-Barré syndrome (GBS) have emerged during the Coronavirus disease 2019 (COVID-19) pandemic. This epidemiological and cohort study sought to investigate any causative association between COVID-19 infection and GBS. The epidemiology of GBS cases reported to the UK National Immunoglobulin Database was studied from 2016 to 2019 and compared to cases reported during the COVID-19 pandemic. Data were stratified by hospital trust and region, with numbers of reported cases per month. UK population data for COVID-19 infection were collated from UK public health bodies. In parallel, but separately, members of the British Peripheral Nerve Society prospectively reported incident cases of GBS during the pandemic at their hospitals to a central register. The clinical features, investigation findings and outcomes of COVID-19 (definite or probable) and non-COVID-19 associated GBS cases in this cohort were compared. The incidence of GBS treated in UK hospitals from 2016 to 2019 was 1.65-1.88 per 100 000 individuals per year. GBS incidence fell between March and May 2020 compared to the same months of 2016-19. GBS and COVID-19 incidences during the pandemic also varied between regions and did not correlate with one another (r = 0.06, 95% confidence interval: -0.56 to 0.63, P = 0.86). In the independent cohort study, 47 GBS cases were reported (COVID-19 status: 13 definite, 12 probable, 22 non-COVID-19). There were no significant differences in the pattern of weakness, time to nadir, neurophysiology, CSF findings or outcome between these groups. Intubation was more frequent in the COVID-19 affected cohort (7/13, 54% versus 5/22, 23% in COVID-19-negative) attributed to COVID-19 pulmonary involvement. Although it is not possible to entirely rule out the possibility of a link, this study finds no epidemiological or phenotypic clues of SARS-CoV-2 being causative of GBS. GBS incidence has fallen during the pandemic, which may be the influence of lockdown measures reducing transmission of GBS inducing pathogens such as Campylobacter jejuni and respiratory viruses.

METHODS::The effects of motor imagery (MI) on functional recovery of patients with neurological pathologies, such as stroke, has been recently proven. The aim of this study is to evaluate the effectiveness of MI on motor recovery and quality of life (QOL) in patients with multiple sclerosis (pwMS). A search was carried out in the following scientific databases: PubMed, CINAHL, PEDro, Scopus, Cochrane and Web of Science, up to November 2020. The grey literature and reference lists of potentially relevant articles were also searched. The Checklist for Measuring Quality and The Cochrane collaboration's tool were used to assess the methodological quality and risk of bias of the studies. Five studies were included in the systematic review. Findings showed that pwMS using MI had significant improvements in walking speed and distance, fatigue and QOL. In addition, several benefits were also found in dynamic balance and perceived walking ability. Although the evidence is limited, rehabilitation using MI with the application of musical and verbal guides (compared to non-intervention or other interventions), can produce benefits on gait, fatigue and QOL in pwMS with a low score in the Expanded Disability Status Scale.

METHODS::Declared as a global public health emergency, coronavirus disease 2019 (COVID-19) is presented as a disease of the respiratory tract, although severe cases can affect the entire organism. Several studies have shown neurological symptoms, ranging from dizziness and loss of consciousness to cerebrovascular and neurodegenerative diseases. In this context, Guillain-Barré syndrome, an immune-mediated inflammatory neuropathy, has been closely associated with critical cases of infection with "severe acute respiratory syndrome of coronavirus 2" (SARS-CoV-2), the etiological agent of COVID-19. Its pathophysiology is related to a generalized inflammation that affects the nervous system, but neurotropism was also revealed by the new coronavirus, which may increase the risk of neurological sequel, as well as the mortality of the disease. Thus, considering the comorbidities that SARS-CoV-2 infection can promote, the modulation of purinergic signaling can be applied as a potential therapy. In this perspective, given the role of adenosine triphosphate (ATP) in neural intercommunication, the P2X7 receptor (P2X7R) acts on microglia cells and its inhibition may be able to reduce the inflammatory condition of neurodegenerative diseases. Finally, alternative measures to circumvent the reality of the COVID-19 pandemic need to be considered, given the severity of critical cases and the viral involvement of multiple organs.