关键词： design   EEG   filter   montage   quality  

摘要： Aims. The aim of this study was to investigate the pitfalls in performing EEG in neurology centres in Iran, in order to identify any mistakes and provide appropriate advice for the neurology community. Methods. In this cross-sectional study, all patients who referred to the outpatient epilepsy clinic at Shiraz University of Medical Sciences, from April through to June 2018, were asked to provide any EEGs they had from other neurology centres. We reviewed the following information from their EEGs: number of channels, the design of the montages, and the setting of the filters. Results. Eighty EEGs from different neurology centres were studied. With respect to the number of channels, 27 centres (34%) recorded 16 or more channels. With regards to the montage design, 34 centres (43%) had an appropriate design(s). With respect to high-frequency filter, three centres (4%) recorded their EEGs with 70-Hz high filter. With regards to the low-frequency filter, 43 centres (54%) recorded their EEGs with 1-Hz or lower filter. Just one centre performed EEGs in a standard fashion considering all variables. Conclusion. In this study, we observed that most neurology centres in Iran did not record EEGs in a standard and acceptable fashion. Provision of a well-designed educational programme that ensures neurology residents are competent in the fundamentals of EEG recording and interpretation is of paramount significance.

关键词： Neuroinfection   Neuroinflammation   Bacterial meningitis   Metalloproteinase   MMP   Multiple sclerosis  

摘要： Metalloproteinases-such as matrix metalloproteinases (MMPs) and a disintegrin and metalloproteinases (ADAMs)-are involved in various diseases of the nervous system but also contribute to nervous system development, synaptic plasticity and neuroregeneration upon injury. MMPs and ADAMs proteolytically cleave many substrates including extracellular matrix components but also signaling molecules and receptors. During neuroinfectious disease with associated neuroinflammation, MMPs and ADAMs regulate blood-brain barrier breakdown, bacterial invasion, neutrophil infiltration and cytokine signaling. Specific and broad-spectrum inhibitors for MMPs and ADAMs have experimentally been shown to decrease neuroinflammation and brain damage in diseases with excessive neuroinflammation as a common denominator, such as pneumococcal meningitis and multiple sclerosis, thereby improving the disease outcome. Timing of metalloproteinase inhibition appears to be critical to effectively target the cascade of pathophysiological processes leading to brain damage without inhibiting the neuroregenerative effects of metalloproteinases. As the critical role of metalloproteinases in neuronal repair mechanisms and regeneration was only lately recognized, the original idea of chronic MMP inhibition needs to be conceptually revised. Recently accumulated research urges for a second chance of metalloproteinase inhibitors, which-when correctly applied and dosed-harbor the potential to improve the outcome of different neuroinflammatory diseases.

关键词： Obstructive sleep apnea   acute ischemic stroke   infarction volume   neurological recovery  

摘要： Background: Obstructive sleep apnea (OSA) is a probable risk factor with speculative roles in the induction or aggravation of acute ischemic stroke (AIS). Methods: The association between OSA and AIS severity was retrospectively analyzed using clinical data of first-onset AIS patients, admitted to our hospital between January 2013 and September 2016. Eligible patients were categorized based on the presence of OSA prior to stroke. Stroke severity and functional outcomes were evaluated using the National Institute of Health Stroke Severity Scale (NIHSS) and the modified Rankin scale (mRS), respectively. Results: No significant differences were observed among OSA and non-OSA groups for infarction volume, NIHSS at admission and discharge, or mRS at discharge and at the 3-month follow-up (all P >.05). OSA prior to stroke negatively correlated with infarction volume (P =.008), NIHSS at discharge (P =.006), and the 3-month mRS (P =.015). In addition to OSA, it was also found that infarction volume significantly correlated with large artery occlusion (LAO), anterior circulation involvement, neutrophil count, and fibrinogen level;NIHSS at discharge significantly correlated with LAO, transient ischemia attack (TIA), neutrophil count, and thrombolysis;and the 3-month mRS significantly correlated with LAO, TIA, age, neutrophil count, and thrombolysis. Conclusions: OSA before AIS does not increase the severity of stroke. The negative association between OSA and infarction volume, stroke severity, and clinical outcomes suggests an endogenous neuroprotective effect.

关键词： Young carers   Young caregivers   ALS   Huntington's disease   Family caregiving   Caregiver training  

摘要： BackgroundCaregiver skill training and support programs are traditionally offered to adult caregivers, leaving out the over 1 million children and youth who provide care (young caregivers). Skill building and support programs are critical to caregiver and patient well-being and can be informed by the knowledge of current caregiver skill and support *** data from three studies of neurological disorders, this paper provides initial data on how young caregivers acquire caregiver skills and training needed to inform young caregiver *** method data drawn from three studies of young caregivers in neurological disorders, Huntington's disease (HD) and amyotrophic lateral sclerosis (ALS).ResultsYouth (N=96), ranged in age from 8 to 20, care for a family member with HD or ALS, and involved in tasks ranging from assistance with walking (76%), toileting (32%) and administering medications (37%). The majority (N=59;61%), received no training or guidance. When asked how they knew what to do, six main themes arose: (1) patient tells me what to do, (2) watching and observing, (3) common sense, (4) treating patient like child or self, (5) process of figuring it out, and (6) don't *** caregivers in neurological disorders engage in numerous caregiving tasks with little formal guidance, despite a desire for training and education by adults and professionals. Reliance on various skill methods and lack of formal guidance, points to the need for health care professionals to develop education and training programs targeting this isolated and underserved population, improving the well-being of both caregiver and care recipient.

关键词： Cervical   Clinical correlation   Hoffmann sign   Magnetic resonance imaging  

摘要： BACKGROUND: The Hoffmann sign is usually used as an indicator of upper motor neuron lesion, but its clinical effect remains controversial in previous reports. METHODS: A retrospective case control study including 107 patients with cervical complaints was carried out. According to the presence of Hoffmann sign, patients were divided into 2 groups. The radiographic results were assessed and the sensitivity, specificity, positive and negative predictive values, and false positive and false negative values of Hoffmann sign for cervical pathology, segment, cervical spine canal ratio, and S-index were calculated. RESULTS: There were 56 patients in the positive Hoffmann group and 51 patients in negative group. The sensitivity, specificity, positive and negative predictive values, and false positive and false negative values of Hoffmann sign for cervical pathology were found to be 61.6%, 85.7%, 94.6%, 35.3%, 14.3%, 38.4% and 60.5%, 81.0%, 92.9%, 33.3%, 19.0%, 39.5%, respectively. The ratio of cervical spine canal was lower in the positive Hoffmann group than in control group. CONCLUSIONS: Although the Hoffmann sign is not foolproof in the diagnosis of cervical spinal cord compression, it can be used to assess symptomatic patients. The narrower the cervical spine canal or the higher the cervical segment compression, the higher of the incidence of positive Hoffmann sign.

关键词： Epilepsy   Inflammation   Migraine   Stroke  

摘要： Inflammatory processes contribute to neurological disorders, and many therapeutic breakthroughs in neurological disease have been immune-targeted. The choice of neuroinflammation as the theme for the 5th European Academy of Neurology Congress in 2019 and of this Focus issue highlights its importance to neurologists across the discipline. [ABSTRACT FROM AUTHOR]

关键词： JTB gene   Visible Light   Pathway   Nervous System Diseases   Post-Translational Protein Processing   Neurodegenerative Diseases   Proteins   Frontotemporal degeneration  

摘要： A central and causative feature of age-related neurodegenerative disease is the deposition of misfolded proteins in the brain. To devise novel approaches to treatment, regulatory pathways that modulate these aggregation-prone proteins must be defined. One such pathway is post-translational modification by the addition of poly(ADP-ribose) (PAR), which promotes protein recruitment and localization in several cellular contexts. Mounting evidence implicates PAR in seeding the abnormal localization and accumulation of proteins that are causative of neurodegenerative disease. Inhibitors of PAR polymerase (PARP) activity have been developed as cancer therapeutics, raising the possibility that they could be used to treat neurodegenerative disease. We focus on pathways regulated by PAR in neurodegenerative disease, with emphasis on amyotrophic lateral sclerosis (ALS) and frontotemporal degeneration (FTD).

关键词： copy number variation sequencing   diagnostic yield   neurological disorders   targeted next-generation sequencing panels   whole-exome sequencing  

摘要： This retrospective study aims to investigate the diagnostic yields of multiple strategies of next-generation sequencing (NGS) for children with rare neurological disorders (NDs). A total of 220 pediatric patients with NDs who visited our hospital between Jan 2017 and Dec 2018 and had undergone NGS were included. Most patients were 5 years old or younger, and the number of patients visiting the hospital decreased with age. Seizures were the most common symptom in this cohort. The positive rates for targeted NGS panels (Panel), whole-exome sequencing (WES), and copy number variation sequencing (CNVseq) were 26.5% (9/34), 36.6% (63/172), and 16.7% (22/132), respectively. The positive rate for patients undergoing a combination of WES and CNVseq (WES + CNVseq) was 47.8% (54/113), which was significantly better than the positive rate for patients who underwent WES alone (32.7%, 37/113). A total of 83 variants were found in 42 genes, and SCN1A was the most frequently mutanted gene. Twenty-four CNVs were identified in 22 patients: two CNVs were inherited from the mother;12 CNVs were de novo;and the CNV origins could not be determined in 10 patients. WES + CNVseq may potentially be the mostly effective NGS approach for diagnosis of rare NDs in pediatric patients.

关键词： Cognitive functioning   Cognitive impairment   Transient neurological attacks   Heart failure  

摘要： Introduction Nonfocal transient neurological attacks (TNAs) are associated with an increased risk of future dementia, but it is unclear whether TNAs are also associated with concurrent cognitive impairment. We hypothesized that recent TNAs are related to worse cognitive functioning. We tested our hypothesis in patients with heart failure, as these patients are at risk of cerebral hypoperfusion, which might play a role in the etiology of TNAs. Methods We performed neuropsychological testing in all patients with heart failure enrolled in the Heart Brain Connection study. We assessed global cognition, attention-psychomotor speed, executive functioning, memory and language. All patients were interviewed with a standardized questionnaire on the occurrence of TNAs in the preceding 6 months. We studied associations between TNAs and cognitive functioning with linear and logistic regression analyses, adjusted for age, sex and education. We performed additional analyses in patients without previous stroke or TIA and in patients without brain infarction on MRI. Results Thirty-seven (23%) of 158 patients (mean age 70 years, 67% men) experienced one or more TNAs. Patients with a recent TNA were more likely to be impaired on >= 1 cognitive domains than patients without TNAs [41% vs. 18%, adjusted odds ratio 4.6, 95% confidence interval (CI) 1.8-11.8]. Patients with TNAs performed worse than patients without TNAs on global cognition (mean difference in z scores -0.36, 95% CI -0.54 to -0.18), and on the cognitive domains attention-psychomotor speed (mean difference -0.40, 95% CI -0.66 to -0.14), memory (mean difference -0.57, 95% CI -0.98 to -0.15) and language (mean difference -0.47, 95% CI -0.79 to -0.16). These associations were independent of cardiac output and volume of white matter hyperintensities. Subgroup analyses in patients without previous stroke or TIA or brain infarction on MRI (n=78) yielded comparable results, with the exception of the cognitive domain language, whi

关键词： cognition   evolution   neurogenesis   reptiles  

摘要： Unlike birds and mammals, reptiles are commonly thought to possess only the most rudimentary means of interacting with their environments, reflexively responding to sensory information to the near exclusion of higher cognitive function. However, reptilian brains, though structurally somewhat different from those of mammals and birds, use many of the same cellular and molecular processes to support complex behaviors in homologous brain regions. Here, the neurological mechanisms supporting reptilian cognition are reviewed, focusing specifically on spatial cognition and the hippocampus. These processes are compared to those seen in mammals and birds within an ecologically and evolutionarily relevant context. By viewing reptilian cognition through an integrative framework, a more robust understanding of reptile cognition is gleaned. Doing so yields a broader view of the evolutionarily conserved molecular and cellular mechanisms that underlie cognitive function and a better understanding of the factors that led to the evolution of complex cognition.