关键词： Mitochondrial dysfunction   Alzheimer's disease   Parkinson's disease   Progressive supranuclear palsy   Mitochondria-based medicine   Neurological diseases  

摘要： The mitochondria, apart from being known as the cell's "powerhouse," are crucial in the viability of nerve cells. Any damage to these cellular organelles can result in their cellular level dysfunction which includes rapidly multiplying reactive oxygen species (ROS) from the mitochondrial membrane, impaired calcium ion homeostasis, and disturbed mitochondrial dynamics by the formation of permeability transition pore in mitochondria. All these impaired biochemical changes lead to various neurological disorders such as progressive supranuclear palsy (PSP), Parkinson's disease (PD), and Alzheimer's disease (AD). Moreover, impaired mitochondrial functions are particularly prone to damage owing to prolonged lifespan and stretched length of the neurons. At the same time, neurons are highly dependent on ATP, and thus, the mitochondria play a central role in the pathogenesis pertaining to neuronal disorders. Dysfunction in the mitochondria is an early pathological hallmark of neurological disorders, and its early detection with the help of suitable biomarkers can lead to promising treatment in this area. Thus, the drugs which are targeting mitochondrial dysfunctions are the emerging area of research in connection with neurological disorders. This can be evidenced by the great opportunities for mitigation, diagnosis, and treatment of numerous human disorders that entail mitochondrial dysfunction at the nexus of their pathogenesis. Here, we throw light at the mitochondrial pathologies and indications of dysfunctional mitochondria in PD, AD, and PSP. There is also an insight into the possible therapeutic strategies highlighting the need for mitochondria-based medicine and made an attempt for claiming the prerequisite for the therapy of neurological diseases.

关键词： Art   Dissociative seizures   Functional seizures   Paintings   Psychogenic nonepileptic seizures  

摘要： Purpose: The purpose of this study was to explore how neurology specialists with interest in epilepsy depict psychogenic nonepileptic seizures (PNES) and their experience of diagnosing and treating the disorder. Methods: An art project was organized as part of the curriculum of the 14th Latin American Summer School on Epilepsy (LASSE) in Sao Paulo (Brazil) from February 27th to March 6th, 2020. Willing neurologists were invited to draw or paint PNES and present their professional perspective of this disorder. Grouped by common themes, the artworks were analyzed in the context of the existing literature on PNES. Results: The participants created 31 paintings and drawings, 13 of which were selected to represent the recurring themes. Throughout the artworks, neurologists often portrayed PNES as masking the underlying psychological issues, but there was little emphasis on the genesis of PNES itself. The manifestation of PNES was more attributed to just emotional suppression rather than to the influence of different situational factors on a pre-established seizure scaffold. Considering the management of PNES, neurology specialists used the symbol of a labyrinth to represent the difficulties of effectively providing diagnosis and multidisciplinary care for the patients. The general outlook on PNES was associated with darkness, possibly due to limited understanding of the disorder's nature and the unavailability of effective treatment options. Conclusion: Our study shows that art projects can be useful to better grasp neurology specialists' opinions about a certain disorder. Further research is needed to elucidate the mechanisms of PNES and help establish multidisciplinary care.

关键词： SARS-CoV-2   neuroinflammation   vertical transmission   neuropsychiatric complications   anosmia  

摘要： Although COVID-19 is predominantly a respiratory disease, it is known to affect multiple organ systems. In this article, we highlight the impact of SARS-CoV-2 (the coronavirus causing COVID-19) on the central nervous system as there is an urgent need to understand the longitudinal impacts of COVID-19 on brain function, behaviour and cognition. Furthermore, we address the possibility of intergenerational impacts of COVID-19 on the brain, potentially via both maternal and paternal routes. Evidence from preclinical models of earlier coronaviruses has shown direct viral infiltration across the blood-brain barrier and indirect secondary effects due to other organ pathology and inflammation. In the most severely ill patients with pneumonia requiring intensive care, there appears to be additional severe inflammatory response and associated thrombophilia with widespread organ damage, including the brain. Maternal viral (and other) infections during pregnancy can affect the offspring, with greater incidence of neurodevelopmental disorders, such as autism, schizophrenia and epilepsy. Available reports suggest possible vertical transmission of SARS-CoV-2, although longitudinal cohort studies of such offspring are needed. The impact of paternal infection on the offspring and intergenerational effects should also be considered. Research targeted at mechanistic insights into all aspects of pathogenesis, including neurological, neuropsychiatric and haematological systems alongside pulmonary pathology, will be critical in informing future therapeutic approaches. With these future challenges in mind, we highlight the importance of national and international collaborative efforts to gather the required clinical and preclinical data to effectively address the possible long-term sequelae of this global pandemic, particularly with respect to the brain and mental health.

关键词： Carotid artery   Carotid stenosis   Stroke   NR   Endarterectomy   Recurrent event  

摘要： Objective: The risk of ipsilateral neurological recurrence (NR) was assessed in patients awaiting carotid endarterectomy (CEA) due to symptomatic carotid artery stenosis and whether current national guidelines of performing CEA within 14 days are adequate in present day practice. Methods: This was a retrospective multicentre observational cohort study. Patients scheduled for CEA due to symptomatic carotid artery stenosis in a five year period, 1 January 2014 to 31 December 2018, from four centres were included. Data from the Danish Vascular Registry (***), operative managing systems, and electronic medical records were reviewed. Results: In total, 1 125 patients scheduled for CEA were included and 1 095 (97%) underwent the planned surgery. During a median delay from index event to CEA of 11 days (interquartile range 8-16 days), 40 patients (3.6%;95% confidence interval [CI] 2.5%-5%) experienced a NR. One third were minor strokes (n = 12, 30%);half were transient ischaemic attacks (TIA) (n = 22, 55%);and amaurosis fugax accounted for 15% (n = 6). Twenty-six (2%) CEA procedures was cancelled, of which one was due to a disabling recurrent ischaemic event (aphasia). There were no deaths or major strokes in the waiting time for CEA. Best medical treatment (BMT) with platelet inhibitory or anticoagulation drugs and a statin was initiated in nearly all patients (98%) at first assessment. The overall 30 day risk of a post-operative major event (death or stroke) was (Kaplan-Meier [KM] estimate) 2.7% (95% CI 1.8-3.8), and not significantly correlated with the timing of surgery. Most (69%) occurred within the first three days. One, two, and three year mortality rate for CEA patients was (KM estimate) 4.8%, 7.8%, and 11.5% respectively. Conclusion: In symptomatic carotid artery stenosis patients awaiting CEA, very few NRs occurred within 14 days. Institution of immediate BMT in specialised TIA/stroke units followed by early, but not necessarily urgent, CEA is a reaso

关键词： Certification   Education   Evaluation   Psychiatry   Interrater reliability   Test-retest reliability  

摘要： Objective The American Board of Psychiatry and Neurology (ABPN) requires applicants to successfully complete at least 3 Clinical Skills Evaluations (CSE) as part of the credentialing requirements to sit for the psychiatry certification examination. The authors examined a database of CSE assessments conducted at a single site to determine the interrater and test-retest reliability of the evaluation. Methods The authors examined 159 CSE assessments of 51 practicing psychiatrists who completed residency prior to the implementation of CSEs, but did not previously sit for the ABPN examination and later required CSEs to do so. Of these, 36 were simultaneously observed by 2 evaluators, at least 1 of whom had extensive experience with the CSE and 8 of whom were novice evaluators. At least 3 CSEs were done on the same day by each candidate. Results The intraclass correlations for CSEs observed by 2 evaluators for the 3 clinical skills assessed (patient-physician relationship, conduct of the interview, and case presentation), were in the low-moderate "good" range for interrater reliability. Test-retest reliability for all candidates was in the moderate-high "good" range. Significant learning took place between the 1st and 3rd CSEs, with a mean improvement of 0.42-0.49 points on an 8-point scale (p< 0.01). Conclusions These data demonstrate good interrater reliability for the CSE even when conducted by novice faculty evaluators and good test-retest reliability despite significant learning that occurred over the series of assessments. The study supports both the educational and assessment value of the CSE.

关键词： evaluation   international congress   quality   transition   virtual congress  

摘要： Background and purpose Due to the COVID-19 pandemic, scientific congresses are increasingly being organized as virtual congresses (VCs). In May 2020, the European Academy of Neurology (EAN) held a VC, free of charge. In the absence of systematic studies on this topic, the aim of this study is to evaluate the attendance and perceived quality of the 2020 EAN VC compared to the 2019 EAN face-to-face congress (FFC). Methods An analysis of the demographic data of participants obtained from the online registration was done. A comparison of the two congresses based on a survey with questions on the perception of speakers' performance, quality of networking and other aspects was made. Results Of 43,596 registered participants, 20,694 active participants attended the VC. Compared to 2019, the number of participants tripled (6916 in 2019) and the cumulated number of participants attending the sessions was five times higher (169,334 in 2020 vs. 33,024 in 2019). Out of active participants 55% were from outside Europe, 42% were board-certified neurologists (FFC 80%) and 21% were students (FFC 0.6%). The content of the congress was evaluated as 'above expectation' by 56% of the attendees (FFC 41%). Of the respondents who had been exposed to earlier EAN congresses 73% preferred the FFC compared to the VC (17%). Conclusion The VC fulfilled the main mission of organizing high quality EAN congresses despite the restrictions of the impersonal format. The geographical distribution of the participants proves the expected higher inclusivity of a VC. The large participation of students and neurologists in training opens new educational potentials for the EAN.

关键词： Aqueduct stenosis   iNPH scale   Symptoms and signs   Outcome  

摘要： Background: Comprehensively describe and compare (pre/postoperatively) the clinical symptomatology in adult non-communicated hydrocephalus. Associated hydrocephalus signs were analyzed with the idiopathic Normal Pressure Hydrocephalus Scale (iNPH Scale). A standardized clinical scale for non communicated hydrocephalus is currently not in use. Methods: Ten patients with hydrocephalus occlusus (HO) were analyzed. Hydrocephalus signs were examined with the iNPH Scale in gait, neuropsychology, continence, and balance before and three months after treatment with shunt operation or third endoscopic ventriculostomy. Results: Patients significantly improved in iNPH total score (25.8%) and gait score (35.4%) three months after neurosurgical intervention. Domain scores in neuropsychology, continence, and balance reached statistical trends (p < 0.066). Most clinical symptoms and signs at baseline improved after surgery (dizziness, lapse of concentration, gait instability, and headache). Conclusion: Patients with non-communicated HO also showed classical hydrocephalus symptoms as communicated in iNPH patients. The iNPH Scale allows a structured neurological assessment over the disease's progress and surgical intervention. Further studies with a larger patient samples are necessary to support our results. (c) 2021 Published by Elsevier Ltd.

摘要： Aim To study the association between the Hammersmith Infant Neurological Examination (HINE) at age 2 years and neurocognition at age 11 years in children born very preterm. We hypothesized that the HINE at 2 years would be associated with neurocognition, that is, neurological, motor, and cognitive outcomes at 11 years. Method A total of 174 children (mean gestational age 29.0wks, SD 2.7;minimum 23.0, maximum 35.9;95 [55%] males, 79 [45%] females) born very preterm (birthweight <= 1500g/gestational age <32wks), were included in a prospective cohort recruited from 2001 to 2006 in Turku, Finland. The HINE was performed at 2 years' corrected age. Neurocognition at 11 years was assessed with the Touwen Infant Neurological Examination (TINE) , Movement Assessment Battery for Children, Second Edition (MABC-2), and full-scale IQ (Wechsler Intelligence Scale for Children, Fourth Edition). Results The HINE global score was associated with the results of the TINE (odds ratio [OR]=0.9, 95% confidence interval [CI] 0.8-0.9, p=0.001), MABC-2 (beta=1.4, 95% CI 0.7-2.2, p<0.001), and full-scale IQ (beta=1.2, 95% CI 0.8-1.7, p<0.001), even when adjusted. When children with cerebral palsy (CP) were excluded, the HINE was still associated with full-scale IQ (unadjusted beta=1.2, 95% CI 0.3-2.1, p=0.01). Interpretation A higher HINE global score at 2 years was associated with better general intelligence at 11 years even in children without CP. The HINE may be a useful tool to detect children at risk for later cognitive impairment.

关键词： Child neurology clinical research   Child neurology residency training   Antisense oligonucleotide   Enzyme replacement   Gene therapy  

摘要： The molecular understanding of the pathogenic mechanisms responsible for neurologic diseases of children has led to a remarkable period of research that addresses the root causes of diseases. The promise of this research has been realized with cures and treatments that correct underlying deficiencies. The breakneck rate at which new research is being proposed promises to usher in a transformation of child neurology from a diagnostic and supportive field into an interventional one. Training child neurology residents in clinical research and therapeutic intervention is increasingly important to assure the ongoing ability to support research discoveries and treatment.

关键词： Longitudinally extensive transverse myelitis   Childhood   Treatment   Prognosis  

摘要： Aim: Longitudinally extensive transvers myelitis (LETM) is a rare and disabling condition in childhood. The aim of the present study was to share experiences from our center regarding the treatment features and clinical and radiologic course in our LETM patients in light of the literature data. Material and method: The study was designed as cross-sectional and included children who followed for LETM at our pediatric neurology clinic between 2010 and 2019. ATM was diagnosed according to the diagnostic criteria report from the Transverse Myelitis Consortium Working Group. LETM was defined as the presence of spinal cord lesions spanning a length of 3 or more consecutive vertebral segments. The patients' medical records were examined in terms of demographic characteristics, presenting symptoms, history of infection prior to and during LETM, prodromal history, neurological examination, laboratory and radiological findings, clinical course, and treatment. The Barthel Index was used to assess the physical independence in activities of daily living of patients with LETM who were followed for at least one year. Results: A total of 15 (8 girl) patients were included in the study. The patients were between 1 and 17 years of age. Presenting symptoms included inability to walk in 12 patients, incontinence in 9 patients, low back pain in 4 patients, abdominal pain in 2 patients, and inability to use the arms in 2 patients. In Barthel Index assessment of physical independence in activities of daily living, 8 patients were evaluated as completely independent, 3 patients as moderately dependent, and 2 patients as slightly dependent. When the 4 patients with motor area impairment and moderate dependency according to the Barthel Index were examined, it was noted that all of them had been admitted 4 days after the onset of symptoms and that 2 (13.3%) had cervicothoracic involvement and 2 (13.3%) had involvement of the entire cord. Conclusion: Shorter delay from symptom onset to initiat