关键词： Neonatal seizures   Preterm infants   Newborn   Epilepsy   Outcome  

摘要： Introduction: Neonatal seizures (NS) are associated with increased mortality and risk of cerebral palsy, epilepsy and intellectual disability. We performed a systematic review with the primary objective to delineate the rate of these outcomes following NS in preterm infants from studies published in the 2000s and the secondary objective to identify risk factors. Methods: Inclusion criteria: original articles published between 1/1/2000 and 12/31/2018, written in English, evaluating newborns <= 37 weeks of gestational age and suffering from NS, in which at least one of these was evaluated: epilepsy, cerebral palsy, intellectual disability/developmental delay, normal outcome, death. Results: Twenty-two papers were selected and all were observational, with a retrospective design in 15. Three were population-based and twenty-one have a comparison. It has been found a 22-80 % of mortality, 11.3-38.9 % of epilepsy, 12-84.6 % of cerebral palsy, and 20-42.7 % of intellectual disability/developmental delay rate. An increased risk for all outcomes considered was reported. Risk factors for specific outcomes were provided by a minority of studies. However, inclusion criteria, definition of NS and measured outcomes, follow-up lengths differed considerably between studies. Discussion: Results of the selected studies are only partially comparable or generalizable because of differences in study design. They have a risk for potential biases, although they provide (if analyzed) readily available prognostic factors, easy to apply in clinical practice. Prospective, population-based studies with EEG-defined NS are warranted in order to produce evidence-based guidance for management of preterm newborns with seizures.

关键词： Medical devices and equipment   Measurement equipment   Rehabilitation medicine   Physical activity   Inpatients   Virtual reality   Neurorehabilitation   Video games  

摘要： Background Digitally enabled rehabilitation may lead to better outcomes but has not been tested in large pragmatic trials. We aimed to evaluate a tailored prescription of affordable digital devices in addition to usual care for people with mobility limitations admitted to aged care and neurological rehabilitation. Methods and findings We conducted a pragmatic, outcome-assessor-blinded, parallel-group randomised trial in 3 Australian hospitals in Sydney and Adelaide recruiting adults 18 to 101 years old with mobility limitations undertaking aged care and neurological inpatient rehabilitation. Both the intervention and control groups received usual multidisciplinary inpatient and post-hospital rehabilitation care as determined by the treating rehabilitation clinicians. In addition to usual care, the intervention group used devices to target mobility and physical activity problems, individually prescribed by a physiotherapist according to an intervention protocol, including virtual reality video games, activity monitors, and handheld computer devices for 6 months in hospital and at home. Co-primary outcomes were mobility (performance-based Short Physical Performance Battery [SPPB];continuous version;range 0 to 3;higher score indicates better mobility) and upright time as a proxy measure of physical activity (proportion of the day upright measured with activPAL) at 6 months. The dataset was analysed using intention-to-treat principles. The trial was prospectively registered with the Australian New Zealand Clinical Trials Registry (ACTRN12614000936628). Between 22 September 2014 and 10 November 2016, 300 patients (mean age 74 years, SD 14;50% female;54% neurological condition causing activity limitation) were randomly assigned to intervention (n = 149) or control (n = 151) using a secure online database (REDCap) to achieve allocation concealment. Six-month assessments were completed by 258 participants (129 intervention, 129 control). Intervention participants received o

关键词： Bone marrow transplantation   Peripheral neuropathies  

摘要： These updated EBMT guidelines review the clinical evidence, registry activity and mechanisms of action of haematopoietic stem cell transplantation (HSCT) in multiple sclerosis (MS) and other immune-mediated neurological diseases and provide recommendations for patient selection, transplant technique, follow-up and future development. The major focus is on autologous HSCT (aHSCT), used in MS for over two decades and currently the fastest growing indication for this treatment in Europe, with increasing evidence to support its use in highly active relapsing remitting MS failing to respond to disease modifying therapies. aHSCT may have a potential role in the treatment of the progressive forms of MS with a significant inflammatory component and other immune-mediated neurological diseases, including chronic inflammatory demyelinating polyneuropathy, neuromyelitis optica, myasthenia gravis and stiff person syndrome. Allogeneic HSCT should only be considered where potential risks are justified. Compared with other immunomodulatory treatments, HSCT is associated with greater short-term risks and requires close interspeciality collaboration between transplant physicians and neurologists with a special interest in these neurological conditions before, during and after treatment in accredited HSCT centres. Other experimental cell therapies are developmental for these diseases and patients should only be treated on clinical trials.

关键词： Zika virus   Pregnancy   Neurological symptoms   Urine   Amniotic fluid  

摘要： Paired maternal and newborn urine and amniotic fluid from 138 subjects collected during a Zika virus (ZIKV) outbreak was analyzed for ZIKV by gene amplification (RT-qPCR), and the findings were correlated with clinical symptoms and neurological anomalies in the babies. ZIKV was detected in 1 of 9 symptomatic women (11.1%) and in 19 of 129 asymptomatic women (14.7%). Neurological manifestations were present in 19 babies (13.7%), 10 of 20 (50%) positive and 9 of 119 (7.6%) negative (p < 0.001) for ZIKV. Twelve (8.6%) urines collected during gestation were ZIKV-positive;only 2 remained positive for ZIKV postpartum. Six (4.1%) newborn urines collected within 1 day of delivery were ZIKV-positive cases. In 3 of these cases, ZIKV was detected in mother's urine pre- and postpartum and in both mother's urine and babies' urine. Four of the amniotic fluid samples (2.9%) were ZIKV-positive. Among ZIKV-negative babies with neurological sequel, 87.5% were female;in contrast, 72.7% ZIKV-positive babies with neurological abnormalities were male (p = 0.019). We conclude that during a ZIKV outbreak, clinical symptoms and ZIKV detection in biological fluids are poor predictors of infection and adverse neurologic sequel in newborns.

关键词： Drop foot   Early decompression   Lumbar degenerative diseases   Neurological recovery   Manual muscle test  

摘要： The purpose of this study was to investigate the effectiveness of early (<72 h) versus late (>= 72 h) decompression surgery after the onset of drop foot caused by root disorder in lumbar degenerative diseases (LDDs). Data were included from 60 patients who underwent decompression surgery for drop foot caused by LDDs, including lumbar disk herniation or lumbar spinal stenosis. The primary outcome was ordinal change in the manual muscle test (MMT) at 2 years follow-up. Secondary outcomes included changes in the Japanese Orthopedic Association's (JOA) score. The early- and late-stage surgery groups included 20 and 40 patients with mean durations from the onset of drop foot to operation of 0.8 days (range, 0-3 days) and 117.1 days (range, 10-891 days), respectively. There was no significant difference (p = 0.33) between the early- and late-stage surgery groups in the improvement of MMT scores to >4 (90% versus 80%, respectively). However, more patients in the early-stage group achieved an MMT score >5 compared with those in the late-stage surgery group (80% versus 45%;p = 0.03). Furthermore, the recovery rate of JOA scores was significantly higher in the early-stage (89.1%) compared with the late-stage surgery group (68.6%;p < 0.001). Early decompression surgery produced better neurological recovery;however, an improvement of >4 in the MMT score was achieved in 80% of cases with late decompression. (C) 2020 Elsevier Ltd. All rights reserved.

关键词： Nitrous oxide   Laughing gas   Neuropathy   Vitamin B12   Neurological disorders  

摘要： Introduction In recent years, there has been a sharp increase in the number of patients with neurological disorders associated with recreational use of nitrous oxide (N2O) in China. Here, we summarize the clinical characteristics of patients with neurological disorders associated with N2O abuse diagnosed in our Hospital. Further, we conducted a literature search on recent cases reported in mainland China to improve the awareness of the outbreak of neurological disorders associated with N2O abuse. Methods We retrospectively collected data of patients diagnosed with neurological disorders associated with recreational use of N2O in Shengjing Hospital of China Medical University from January 2018 to June 2019, and performed a literature search using the "nitrous oxide" and "neurological disorder" as keywords in the Chinese literature databases of WANFANG and CNKI and the English literature databases of Pubmed and Web of Science Results We enrolled 43 patients (average age: 21.9 +/- 3.3 years). The main clinical manifestations were weakness and paresthesia in the four extremities and unsteady gait. Further, most patients showed significantly lower levels of serum vitamin B12 (169.4 +/- 79.1 pg/mL) and increased homocysteine levels (78.1 +/- 32.2 mu mol/L). MRI of the spinal cord showed longitudinal high T2 signal lesions in the dorsal spinal cord in some patients. Moreover, electromyography showed sensory and motor nerve axonal damage combined with demyelination, which was relatively more severe in the lower limbs. There was rapid improvement of the symptoms after treatment with intramuscular injections of vitamin B12 and the overall prognosis was good. The literature search indicated that the number of published papers and related patients showed a rapid annual increase since the first Chinese case reported in 2016 Conclusion Recreational use of N2O is an emerging public health problem in China that needs prompt action from the society and government. Early diagnosis an

关键词： Adult phenylketonuria   Neurological symptoms   Brain MRI   Leucopathy  

摘要： Objective Phenylketonuria (PKU) is a rare autosomal recessive disease characterised by high plasma phenylalanine levels inducing, if untreated, serious neurological manifestations in children but also, rarely, in adults who stopped their diet. The objective of the study was to describe the neurological manifestations observed in adults with PKU. Methods We analysed cases reported in French reference centres for inborn errors of metabolism and cases already reported in the literature. Results We report 8 new cases of neurological manifestations and 22 cases in the literature, which occurred in adult PKU patients, associated with chronic or rapid increase of phenylalanine levels, mostly when strict low-phenylalanine diet was stopped early in life. Neurological symptoms consisted in cerebellar ataxia, tremor, brisk reflexes, visual loss, sensory manifestations, and/or headaches. Visual loss was more frequent in the new cases (4/8) of the present series than in the literature (4/22). These neurological complications were associated with leucopathy on brain magnetic resonance imaging (27/29). The start of a low-phenylalanine diet improved or fully reversed neurological manifestations, even in patients with late diagnosis during adulthood. Conclusion Neurological manifestations can complicate PKU in adult patients with elevated phenylalanine levels, after long or short period of diet discontinuation. Neurologists should be aware of this diagnosis, and measure phenylalaninemia in case of neurological symptoms associated with non-specific leucopathy on brain MRI. PKU patients should be systematically encouraged to continue their diet and their medical follow-up to avoid neurological complications.

关键词： bacterial meningitis   dexamethasone   long-term sequelae   neurological impairment   risk factors  

摘要： Aim To examine the incidence, clinical presentation and risk factors for neurological sequelae following childhood community-acquired bacterial meningitis (CABM). Methods We included all children aged 1 month to 15 years old with CABM in North Denmark Region, 1998-2016. Using medical records, we registered baseline demographics, signs and symptoms at admission, laboratory investigations, and outcome assessed by the Glasgow Outcome Scale (GOS). A GOS score of 1-4 was considered an unfavourable outcome. We used modified Poisson regression to examine predefined risk factors for neurological sequelae among survivors. Results We identified 88 cases of CABM in 86 patients (45 female) with a median age of 1.4 years (interquartile range 0.7-4.6). Neisseria meningitidis was the most common pathogen (48/88). Neurological sequelae occurred in 23 (27%) as hearing deficits in 13 (15%), cognitive impairment in 10 (12%) and motor or sensory nerve deficits in 8 (9%). Unfavourable outcome was observed in 16 (18%) patients and three (3%) patients died. Abnormalities on cranial imaging remained the only independent risk factor for developing neurological sequelae in adjusted analysis. Conclusion Neurological sequelae following CABM in children remain frequent and abnormal cranial imaging may be an independent risk factor.