关键词： Myelin oligodendrocyteglycoprotein (MOG) antibodies   Multiple sclerosis (MS)   Neuromyelitis optica (NMO)   Optic neuritis(ON)   Cerebrospinal fluid (CSF)   MRI  

摘要： Myelin oligodendrocyte glycoprotein (MOG) antibodydisease (MOG-AD) is now recognised as a nosological entity with specific clinical and paraclinical features to aid early diagnosis. Although no age group is exempt, median age of onset is within the fourth decade of life, with optic neuritis being the most frequent presenting phenotype. Disease course can be either monophasic or relapsing, with subsequent relapses most commonly involving the optic nerve. Residual disability develops in 50-80% of patients, with transverse myelitis at onset being the most significant predictor of long-term outcome. Recent advances in MOG antibody testing offer improved sensitivity and specificity. To avoid misdiagnosis, MOG antibody testing should be undertaken in selected cases presenting clinical and paraclinical features that are felt to be in keeping with MOG-AD, using a validated cell-based assay. MRI characteristics can help in differentiating MOG-AD from other neuroinflammatory disorders, including multiple sclerosis and neuromyelitis optica. Cerebrospinal fluid oligoclonal bands are uncommon. Randomised control trials are limited, but observational open-label experience suggests a role for high-dose steroids and plasma exchange in the treatment of acute attacks, and for immunosuppressive therapies, such as steroids, oral immunosuppressants and rituximab as maintenance treatment.

关键词： CENTRAL nervous system injuries   NEUROLOGICAL disorders   ALZHEIMER'S disease   GLOBAL analysis (Mathematics)   MOTOR neuron diseases   MULTIPLE sclerosis  

摘要： Background Neurological disorders are increasingly recognised as major causes of death and disability worldwide. The aim of this analysis from the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2016 is to provide the most comprehensive and up-to-date estimates of the global, regional, and national burden from neurological disorders. Methods We estimated prevalence, incidence, deaths, and disability-adjusted life-years (DALYs;the sum of years of life lost [YLLs] and years lived with disability [YLDs]) by age and sex for 15 neurological disorder categories (tetanus, meningitis, encephalitis, stroke, brain and other CNS cancers, traumatic brain injury, spinal cord injury, Alzheimer's disease and other dementias, Parkinson's disease, multiple sclerosis, motor neuron diseases, idiopathic epilepsy, migraine, tension-type headache, and a residual category for other less common neurological disorders) in 195 countries from 1990 to 2016. DisMod-MR 2.1, a Bayesian meta-regression tool, was the main method of estimation of prevalence and incidence, and the Cause of Death Ensemble model (CODEm) was used for mortality estimation. We quantified the contribution of 84 risks and combinations of risk to the disease estimates for the 15 neurological disorder categories using the GBD comparative risk assessment approach. Findings Globally, in 2016, neurological disorders were the leading cause of DALYs (276 million [95% UI 247-308]) and second leading cause of deaths (9.0 million [8.8-9.4]). The absolute number of deaths and DALYs from all neurological disorders combined increased (deaths by 39% [34-44] and DALYs by 15% [9-21]) whereas their age-standardised rates decreased (deaths by 28% [26-30] and DALYs by 27% [24-31]) between 1990 and 2016. The only neurological disorders that had a decrease in rates and absolute numbers of deaths and DALYs were tetanus, meningitis, and encephalitis. The four largest contributors of neurological DALYs were stroke (42.2% [38.6-46

关键词： Cardiac arrest   Neurological outcome   Survival   Targeted temperature management   Transcranial direct current stimulation  

摘要： Background: Transcranial direct current stimulation (tDCS) modulates neuronal activity and is a potential therapeutic tool for many neurological diseases. However, its beneficial effects on post cardiac arrest syndrome remains uncertain. Objective/hypothesis: We investigated the effects of repetitive anodal tDCS on neurological outcome and survival in a ventricular fibrillation (VF) cardiac arrest rat model. Methods: Cardiopulmonary resuscitation was initiated after 6 min of VF in 36 Sprague-Dawley rats. The animals were randomized into three groups immediately after resuscitation (n = 12 each): no-treatment control (NTC) group, targeted temperature management (TTM) group, and tDCS group. For tDCS, 1 mA anodal tDCS was applied on the dorsal scalp for 0.5 h. The stimulation was repeated for four sessions with 1-h resting interval under normothermia. Post-resuscitation hemodynamic, cerebral, and myocardial injuries, 96-h neurological outcome, and survival were evaluated. Results: Compared with the NTC group, post-resuscitation serum astroglial protein S100 beta and cardiac troponin T levels and 96-h neuronal and myocardial damage scores were markedly reduced in the tDCS and TTM groups. Myocardial ejection fraction, neurological deficit score, and 96-h survival rate were also significantly better for the tDCS and TTM groups. The period of post-resuscitation arrhythmia with hemodynamic instability was considerably shorter in the tDCS group, but no differences were observed in neurological outcome and survival between the tDCS and TTM groups. Conclusions: In this cardiac arrest rat model, repeated anodal tDCS commenced after resuscitation improves 96-h neurological outcome and survival to an extent comparable to TTM by attenuating post-resuscitation cerebral and cardiac injuries. (C) 2018 Published by Elsevier Inc.

关键词： Metabolic disorders   Neurological manifestations  

摘要： Organic acidurias (OADs) are inherited neurometabolic diseases largely caused by deficiencies in enzymes involved in amino acid degradation, which result in accumulation of organic acids in the brain and other tissues. Disease presentation usually occurs in infancy, although late-onset variants can emerge during childhood or adulthood. Patients predominantly manifest with acute encephalopathy with life-threatening systemic manifestations (classical OADs) or progressive neurological symptoms (cerebral OADs), leading to permanent cerebral abnormalities. Some OADs are treatable, and early diagnosis and treatment implementation have substantially decreased the mortality and overall morbidity from OADs. However, long-term irreversible cerebral and systemic complications are frequent because the therapeutic options are currently limited. The pathophysiology of brain dysfunction is still unclear in most OADs, and further investigation is needed to enable the development of novel therapeutic strategies. This Review focuses on current knowledge of the OADs, including epidemiology, short-term and long-term neurological and systemic features, diagnosis and prognosis, and recent advances in therapy and pathophysiology. The goal of the article is to alert neurologists and related health professionals to the existence and importance of these neurometabolic diseases and to stimulate research into the damaging factors that contribute to their neurodegenerative sequelae.

关键词： nerve growth factor   fulranumab   cynomolgus monkey   sympathetic ganglia  

摘要： Fulranumab, an anti-human nerve growth factor antibody, was evaluated in a series of nonclinical toxicology studies. No treatment effects were observed in adolescent cynomolgus monkeys in standard design, repeat-dose toxicology studies of up to 6 months. Adverse effects on the developing nervous system were observed in offspring of pregnant cynomolgus monkeys treated with fulranumab. Subsequent studies including detailed morphologic investigations of the nervous system did reveal fulranumab-related changes in adult cynomolgus monkeys;this article is focused on those findings. A single dose of >= 1 mg/kg fulranumab administered subcutaneously (SC) caused a decrease in neuron and sympathetic ganglion size (superior cervical ganglion), observed morphologically and stereologically, with a resulting appearance of increased glial cell density. Similar results were observed in repeat-dose (15 to 52 weeks) toxicity studies at <= 50 mg/kg/wk fulranumab SC. These effects recovered after a 3-month treatment-free period. Fulranumab did not cause any neuronal death, necrosis, apoptosis, or any apparent decrease in function of sympathetic neurons/ganglia at any time point examined. A no observed effect level (NOEL) was established at 0.25 mg/kg fulranumab SC every 4 weeks for 28 weeks.

关键词： Epilepsy   Suicide   Depression   NDDIE  

摘要： Objective: The objective of this study was to validate the Chinese version of the Neurological Disorders Depression Inventory for Epilepsy (NDDIE) as a suicidality screening tool in Chinese people with epilepsy (PWE). Methods: A consecutive cohort of PWE was recruited from West China Hospital and 363 Hospital. Each patient received a psychiatric evaluation with the Mini International Neuropsychiatric Interview (MINI) and the Chinese version of the NDDIE (C-NDDIE). Demographic and clinical characteristics were collected. Receiver operating characteristic (ROC) curve analysis was conducted. The best possible cutoff was identified with the highest Youden index. Specificity, sensitivity, positive, and negative predictive values were calculated. Results: Among a total of 355 participants, 41 (11.5%) had a moderate to high suicide risk according to the Suicidality Module (SM) of the MINI. Receiver operating characteristic (ROC) curve analysis showed that item 4 ("I'd be better off dead") of the NDDIE had an area under the curve (AUC) of 0.930 (95% confidence interval [CI] = 0.884-0.977), a sensitivity of 80.5%, a specificity of 94.9%, a positive predictive value (PPV) of 68.0%, a negative predictive value (NPV) of 97.7%, and the largest Youden index of 0.754 for a cutoff score of >2. Conclusion: Item 4 of the NDDIE is a valuable tool for screening suicidality in Chinese PWE. (C) 2019 Elsevier Inc. All rights reserved.

摘要： Because the resources and the ability of health-care systems facing this increasing demand vary by nation and the neurological diseases GBD provides the most detailed picture of burden faced by every nation, the national and regional needs can be more realistically matched.[...]the need to understand and develop disease-modifying strategies is of paramount importance.[...]as noted by the UN General Assembly report of December, 2017, progress up to now in reducing the burden from NCDs, including neurological disorders, is insufficient to meet the UN Sustainable Development Goals targets by 2030.1 Global, national, and regional neurological and disease-specific organisations such as the World Federation of Neurology, the World Stroke Organisation, and Alzheimer's International must join with WHO to attain the necessary momentum to meet this growing challenge.4 Brain health is one of humankind's most precious resources at all ages and at all levels of human activity.

关键词： functional neurological disorder   psychogenic   speech   dysarthria   dysphasia   dysphagia  

摘要： Background Functional neurological disorder (FND) is common across healthcare settings. The Diagnostic and Statistical Manual of Mental Disorders states that speech and swallowing symptoms can be present in FND. Despite this, there is a dearth of guidelines for speech and language therapists (SLTs) for this client group. Aims To address the following question in order to identify gaps for further research: What is known about speech, language and swallowing symptoms in patients with FND? Methods & Procedures A scoping review was conducted. Six healthcare databases were searched for relevant literature: CINAHL PLUS, MEDLINE, ProQuest Nursing and Allied Health Professionals, Science Citation Index, Scopus, and PsychINFO. The following symptoms were excluded from the review: dysphonia, globus pharyngeus, dysfluency, foreign accent syndrome and oesophageal dysphagia. Main Contribution A total of 63 papers were included in the final review;they ranged in date from 1953 to 2018. Case studies were the most frequent research method (n = 23, 37%). 'Psychogenic' was the term used most frequently (n = 24, 38%), followed by 'functional' (n = 21, 33%). Speech symptoms were reported most frequently (n = 41, 65%), followed by language impairments (n = 35, 56%) and dysphagia (n = 13, 21%). Only 11 publications comment on the involvement of SLTs. Eight papers report direct speech and language therapy input;however, none studied the effectiveness of speech and language therapy. Conclusions & Implications Speech, language and swallowing symptoms do occur in patients with FND, yet it is a highly under-researched area. Further research is required to create a set of positive diagnostic criteria, gather accurate data on numbers of patients with FND and speech, language or swallowing symptoms, and to evaluate the effectiveness of direct speech and language therapy involvement.

关键词： Malaria   Post-malaria neurological syndrome   Plasmodium falciparum   Autoimmune encephalitis   Anti VGKC antibody  

摘要： Post-malaria neurological syndrome (PMNS) is a complication that occurs after recovery from a severe Plasmodium falciparum attack. Over the past two decades, the description of several imported cases has confirmed that this syndrome is a clearly distinct entity, different from other post malarial neurological disorders. However, the underlying mechanisms are not yet elucidated. Herein, we present five imported PMNS cases managed in Marseille, France. The detection of neuronal surface antibodies to an encephalitic syndrome of unknown origin allowed us to reveal positivity of anti Voltage-Gated-Potassium Channel antibodies (anti VGKC) in one of them. Using treatment options from other autoimmune encephalitis has to be explored in patients with PMNS.