摘要： Aim To assess how co-occurring conditions influence recovery after hip reconstruction surgery in children with neurological complex chronic conditions (CCCs). Method This was a retrospective analysis of 4058 children age 4 years or older with neurological CCCs who underwent hip reconstructive surgery between 1st January 2015 and 31st December 2018 in 49 children's hospitals. The presence of co-occurring chronic conditions was assessed using the Agency for Healthcare Research Chronic Condition Indicator system. Multivariable, hierarchical regression was used to assess the relationship between co-existing conditions and postoperative hospital length of stay (LOS), cost, and 30-day readmission rate. Results The most common co-occurring conditions were digestive (60.1%) and respiratory (37.9%). As the number of co-existing conditions increased from one to four or more, median LOS increased 67% (3d [interquartile range {IQR} 2-4d] to 5d [IQR 3-8d]);median hospital cost increased 41% ($20 248 [IQR $14 921-$27 842] to $28 692 [IQR $19 236-$45 887]);and readmission rates increased 250% (5.5-13.9%), p0.001 for all. Of all specific co-existing chronic conditions, malnutrition was associated with the greatest increase in postoperative hospital resource use. Interpretation Co-occurring conditions, and malnutrition in particular, are a significant risk factor for prolonged, in-hospital recovery after hip reconstruction surgery in children with a neurological CCC. Further investigation is necessary to assess how improved preoperative optimization of multiple co-occurring conditions may improve postoperative outcomes and resource utilization.

关键词： rTMS   Biomarkers   Depression   Pain   Stroke   BDNF  

摘要： Objective: Repetitive transcranial magnetic stimulation (rTMS) is an evidenced based treatment for depression and an emerging treatment for several other neuropsychiatric disorders. The objective of this systematic review was to assess molecular changes produced by rTMS or molecular markers that predict treatment response in neuropsychiatric disorders. Methods: PubMed, PsycINFO, and Embase were searched through July 2019 for studies published in peerreviewed journals. Eighty-nine studies were identified examining healthy adults and patients with neuropsychiatric disorders including depression, chronic pain, post-stroke deficits, and movement disorders. Results: Our ability to synthesize the information was limited by the large variability in treatment parameters and a limited number of placebo-controlled studies. While few findings were replicated by multiple strong studies, brain derived neurotrophic factor (BDNF) and gamma aminobutyric acid (GABA) in depression, BDNF in post-stroke deficits, and 0-endorphin in chronic pain may be altered by rTMS. Conclusion: BDNF, GABA and 0-endorphin were identified as potential molecular markers of rTMS and warrant further exploration. Significance: This study, which is the first systematic review to examine molecular markers of rTMS in both neurological and psychiatric disorders, provides an updated review of this subject and highlight the need for more placebo-controlled and adequately powered studies to identify biomarkers of rTMS. (c) 2020 Published by Elsevier B.V. on behalf of International Federation of Clinical Neurophysiology.

关键词： Wilson's disease   biomarkers   neurofilament light  

摘要： Background Outcomes are unpredictable for neurological presentations of Wilson's disease (WD). Dosing regimens for chelation therapy vary and monitoring depends on copper indices, which do not reflect end-organ damage. Objective To identify a biomarker for neurological involvement in WD. Methods Neuronal and glial-specific proteins were measured in plasma samples from 40 patients and 38 age-matched controls. Patients were divided into neurological or hepatic presentations and those with recent neurological presentations or deterioration associated with non-adherence were subcategorized as having active neurological disease. Unified WD Rating Scale scores and copper indices were recorded. Results Unlike copper indices, neurofilament light (NfL) concentrations were higher in neurological than hepatic presentations. They were also higher in those with active neurological disease when controlling for severity and correlated with neurological examination subscores in stable patients. Conclusion NfL is a biomarker of neurological involvement with potential use in guiding chelation therapy and clinical trials for novel treatments. (c) 2020 University College *** Disorderspublished by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society

关键词： Neurological conditions   Patient experience   Qualitative research   Self-management   Self-care   TEDSS   Chronic conditions  

摘要： Objective: This paper uses the Taxonomy of Everyday Self-management Strategies (TEDSS) to provide insight and understanding into the complex and interdependent self-management strategies people with neurological conditions use to manage everyday life. Methods: As part of a national Canadian study, structured telephone interviews were conducted monthly for eleven months, with 117 people living with one or more neurological conditions. Answers to five open-ended questions were analyzed using qualitative content analysis. A total of 7236 statements were analyzed. Results: Findings are presented in two overarching patterns: 1) self-management pervades all aspects of life, and 2) self-management is a chain of decisions and behaviours. Participants emphasized management of daily activities and social relationships as important to maintaining meaning in their lives. Conclusion: Managing everyday life with a neurological condition includes a wide range of diverse strategies that often interact and complement each other. Some people need to intentionally manage every aspect of everyday life. Practice implications: For people living with neurological conditions, there is a need for health providers and systems to go beyond standard advice for self-management. Self-management support is best tailored to each individual, their life context and the realities of their illness trajectory. (C) 2020 The Authors. Published by Elsevier B.V.

关键词： Autonomic nervous system diseases   Denervation   Autonomic   Diabetic autonomic neuropathy   Valsalva Maneuver   Tilt table test   Hypotension   Orthostatic  

摘要： Evaluation of disorders of the autonomic nervous system is both an art and a science, calling upon the physician's most astute clinical skills as well as knowledge of autonomic neurology and physiology. Over the last three decades, the development of noninvasive clinical tests that assess the function of autonomic nerves, the validation and standardization of these tests, and the growth of a large body of literature characterizing test results in patients with autonomic disorders have equipped clinical practice further with a valuable set of objective tools to assist diagnosis and prognosis. This review, based on current evidence, outlines an international expert consensus set of recommendations to guide clinical electrodiagnostic autonomic testing. Grading and localization of autonomic deficits incorporates scores from sympathetic cardiovascular adrenergic, parasympathetic cardiovagal, and sudomotor testing, as no single test alone is sufficient to diagnose the degree or distribution of autonomic failure. The composite autonomic severity score (CASS) is a useful score of autonomic failure that is normalized for age and gender. Valid indications for autonomic testing include generalized autonomic failure, regional or selective system syndromes of autonomic impairment, peripheral autonomic neuropathy and ganglionopathy, small fiber neuropathy, orthostatic hypotension, orthostatic intolerance, syncope, neurodegenerative disorders, autonomic hyperactivity, and anhidrosis. (c) 2020 International Federation of Clinical Neurophysiology. Published by Elsevier B.V.

关键词： Caregivers   Critical care   Neurology   Qualitative research  

摘要： Background Family of patients hospitalized in an intensive care unit (ICU) often immediately assume the role of caregiver to an individual with significant health care needs. The transition into this caregiver role may be sudden and unexpected;their experiences are not well understood. The purpose of this qualitative study was to explore experiences of family caregivers in the neurocritical care unit in order to identify areas for enhancing patient- and family-centered care. Methods This single-center ethnographic study explored the use of systems theory to investigate the perceptions, experiences, and attitudes of family/caregivers regarding their relationships and interactions between the patient, other family, members of the healthcare provider team, and health system after an acute neurological event in Argentina. Field notes from 9 weeks of direct observation together with transcripts from nine semi-structured interviews (transcribed verbatim and translated from Spanish to English) were analyzed using a grounded theory approach. Results Nine themes emerged based on iterative thematic analysis, including: adjusting to a changed life, managing emotions, changing role, relying on faith, redefining recovery, participating in patient care, depending on clinical experts, el tratohumano, and finding unity in purpose. In the neurocritical care environment, an important intermediary role exists for family/caregivers and the patient, other family, and healthcare providers. Conclusions The results demonstrate the potential for family, providers, and the health system to influence family/caregivers' experience with neurocritical care. Involving families as part of the care team could have implications for patient- and family-centered care.

关键词： cerebral ischemia   thrombolysis   thrombectomy  

摘要： Background and purpose Better understanding the incidence, predictors and mechanisms of early neurological deterioration (END) following intravenous thrombolysis (IVT) for acute stroke with mild symptoms and isolated internal carotid artery occlusion (iICAo) may inform therapeutic decisions. Methods From a multicenter retrospective database, we extracted all patients with both National Institutes of Health Stroke Scale (NIHSS) score <6 and iICAo (i.e. not involving the Willis circle) on admission imaging, intended for IVT alone. END was defined as >= 4 NIHSS points increase within 24 h. END and no-END patients were compared for (i) pre-treatment clinical and imaging variables and (ii) occurrence of intracranial occlusion, carotid recanalization and parenchymal hemorrhage on follow-up imaging. Results Seventy-four patients were included, amongst whom 22 (30%) patients experienced END. Amongst pre-treatment variables, suprabulbar carotid occlusion was the only admission predictor of END following stepwise variable selection (odds ratio = 4.0, 95% confidence interval: 1.3-12.2;P = 0.015). On follow-up imaging, there was no instance of parenchymal hemorrhage, but an intracranial occlusion was now present in 76% vs. 0% of END and no-END patients, respectively (P < 0.001), and there was a trend toward higher carotid recanalization rate in END patients (29% vs. 9%,P = 0.07). As compared to no-END, END was strongly associated with a poor 3-month outcome. Conclusions Early neurological deterioration is a frequent and highly deleterious event after IVT for minor stroke with iICAo, and is of thromboembolic origin in three out of four patients. The strong association with iICAo site-largely a function of underlying stroke etiology-may point to a different response of the thrombus to IVT. These findings suggest END may be preventable in this setting.

关键词： hnRNPs   Alzheimer's disease   Multiple sclerosis   ALS   FTD  

摘要： Heterogenous nuclear ribonucleoproteins (hnRNPs) are a complex and functionally diverse family of RNA binding proteins with multifarious roles. They are involved, directly or indirectly, in alternative splicing, transcriptional and translational regulation, stress granule formation, cell cycle regulation, and axonal transport. It is unsurprising, given their heavy involvement in maintaining functional integrity of the cell, that their dysfunction has neurological implications. However, compared to their more established roles in cancer, the evidence of hnRNP implication in neurological diseases is still in its infancy. This review aims to consolidate the evidences for hnRNP involvement in neurological diseases, with a focus on spinal muscular atrophy (SMA), Alzheimer's disease (AD), amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), multiple sclerosis (MS), congenital myasthenic syndrome (CMS), and fragile X-associated tremor/ataxia syndrome (FXTAS). Understanding more about hnRNP involvement in neurological diseases can further elucidate the pathomechanisms involved in these diseases and perhaps guide future therapeutic advances.

关键词： Neurological   palliative   end-of-life   carers   family  

摘要： Background: Family and friends are key providers of care for people living with a long-term neurological condition. Neurological conditions are a significant global contributor to disability and premature death. However, previous research suggests carers often struggle to access appropriate support at end of life. Aims: This review sought to synthesise qualitative studies discussing end-of-life and palliative issues for informal carers supporting people living with neurological conditions. Design: This was a meta-ethnographic synthesis of 38 qualitative studies discussing end-of-life and palliative issues for informal carers supporting people living with long-term neurological conditions. Data Sources: Qualitative articles published after January 2010 in English, addressing carers of people with long-term neurological conditions with regard to palliative care, end of life and/or bereavement. Papers were excluded if it was not possible to separately assess the views of carers. Quality appraisal was not undertaken, but consideration was given to research context. Results: Across the papers, five key themes were identified: the future (un)certainties in the progression of life-limiting neurological conditions;an information paradox of not receiving the right information at the right time;access to support;carers' roles in decision making around end of life;and maintaining continuity while facing change and disruption in day-to-day living. Conclusions: Given the broad agreement on the challenges faced by carers of people living with long-term neurological conditions, future research should consider opportunities to improve information and support for this group, and the development and evaluation of practical models of service delivery.

关键词： ACNS nomenclature   Cardiac arrest   EEG   Neurological prognosis   Prognostication  

摘要： Background: Electroencephalography (EEG) patterns are predictive of neurological prognosis in comatose survivors from cardiac arrest but intensive care clinicians are dependent of neurophysiologist reports to identify specific patterns. We hypothesized that the proportion of correct assessment of neurological prognosis would be higher from short statements confirming specific EEG patterns compared with descriptive plain text reports. Methods: Volunteering intensive care clinicians at two university hospitals were asked to assess the neurological prognosis of a fictional patient with high neuron specific enolase. They were presented with 17 authentic plain text reports and three short statements, confirming whether a "highly malignant", "malignant" or "benign" EEG pattern was present. Primary outcome was the proportion of clinicians who correctly identified poor neurological prognosis from reports consistent with highly malignant EEG patterns. Secondary outcomes were how the prognosis was assessed from reports consistent with malignant and benign patterns. Results: Out of 57 participants, poor prognosis was correctly identified by 61% from plain text reports and by 93% from the short statement "highly malignant" EEG patterns. Unaffected prognosis was correctly identified by 28% from plain text reports and by 40% from the short statement "malignant" patterns. Good prognosis was correctly identified by 64% from plain text reports and by 93% from the short statement "benign" pattern. Conclusion: Standardized short statement, "highly malignant EEG pattern present", as compared to plain text EEG descriptions in neurophysiologist reports, is associated with more accurate identification of poor neurological prognosis in comatose survivors of cardiac arrest.