关键词： Childhood cancer   Neurology   Chemotherapy   Immunotherapy  

摘要： Both the onset of various malignancies as well as the treatment of cancer can lead to neurologic symptoms which can be difficult to diagnose. In this review, we highlight the varied ways in which neurologic sequelae of cancer and its treatment manifest in children. Initial neurologic presentation may be secondary to mass effect or to immune-mediated paraneoplastic syndromes. Treatment effects on the nervous system may arise from surgery, chemotherapy, radiation, or bone marrow transplantation. In addition, the rapidly expanding field of immunotherapies for cancer has generated numerous new approaches to eradicating cancer including monoclonal antibodies, checkpoint inhibitors, and chimeric antigen receptor T cells (CAR-T cells), which have neurologic side effects mediated by immune responses that are also being recognized. Here we review common consult questions to the neurologist and our general approach to these scenarios including altered mental status, headaches, seizures, and sensorimotor complaints, considering the multifactorial nature of each.

关键词： Neurological diseases   Respiratory failure   Hypercapnia   Hypoxemia   Invasive mechanical ventilation   Noninvasive ventilation  

摘要： Many neurological diseases may cause acute respiratory failure (ARF) due to involvement of bulbar respiratory center, spinal cord, motoneurons, peripheral nerves, neuromuscular junction, or skeletal muscles. In this context, respiratory emergencies are often a challenge at home, in a neurology ward, or even in an intensive care unit, influencing morbidity and mortality. More commonly, patients develop primarily ventilatory impairment causing hypercapnia. Moreover, inadequate bulbar and expiratory muscle function may cause retained secretions, frequently complicated by pneumonia, atelectasis, and, ultimately, hypoxemic ARF. On the basis of the clinical onset, two main categories of ARF can be identified: (i) acute exacerbation of chronic respiratory failure, which is common in slowly progressive neurological diseases, such as movement disorders and most neuromuscular diseases, and (ii) sudden-onset respiratory failure which may develop in rapidly progressive neurological disorders including stroke, convulsive status epilepticus, traumatic brain injury, spinal cord injury, phrenic neuropathy, myasthenia gravis, and Guillain-Barre syndrome. A tailored assistance may include manual and mechanical cough assistance, noninvasive ventilation, endotracheal intubation, invasive mechanical ventilation, or tracheotomy. This review provides practical recommendations for prevention, recognition, management, and treatment of respiratory emergencies in neurological diseases, mostly in teenagers and adults, according to type and severity of baseline disease.

关键词： Palliative care   pediatric   patient-centered outcomes research   qualitative research   caregiver   family   parents  

摘要： Background: The interest in outcome measurement in pediatric palliative care is rising. To date, the majority of studies investigating relevant outcomes of pediatric palliative care focus on children with cancer. Insight is lacking, however, about relevant outcome domains for children with severe neurological impairment and their families. Aim: The aim of this study was to identify meaningful outcome domains of pediatric palliative care for children with severe neurological impairment and their families. Design: A qualitative research design following a constructivist research paradigm was employed. Guided interviews were conducted with parents of children with life-limiting conditions and severe neurological impairment and professional caregivers. The data were analyzed using qualitative content analysis. Setting: Overall, 10 cooperating pediatric palliative care institutions across Germany (outpatient and inpatient settings) aided in the recruitment of eligible parents and professional caregivers. A total of 11 interviews with 14 parents and 17 interviews with 20 professional caregivers were conducted. Results: Six core outcome domains of pediatric palliative care for children with severe neurological impairment and their families were identified, namely (1) symptom control, (2) respite and support, (3) normalcy, (4) security, (5) empowerment, and (6) coping with the disease, each consisting of 1 to 13 individual aspects. Conclusion: As for other diagnostic groups, symptom control is a relevant outcome domain for children with severe neurological impairment. However, other outcome domains which focus on the whole family and take into account the long disease trajectory, such as respite and support, security, empowerment, and coping with the disease, are also crucial.

关键词： Out-of-hospital cardiopulmonary arrest   Hypoxia-ischemia brain   Induced hypothermia   Phosphopyruvate hydratase   Prognosis   Survivors  

摘要： Introduction and objectives: Neuron-specific enolase (NSE) is a prognostic marker in out-of-hospital cardiopulmonary arrest (OHCA) survivors treated with mild therapeutic hypothermia (MTH). The objectives were to analyze the correlation between dynamic changes in NSE and outcomes and to determine the measurement timing that best predicts neurological status. Methods: Multicenter cohort study including patients admitted after shockable rhythm OHCA and treated with MTH. Serum NSE was sampled at 2 different times and Delta-NSE (%) was calculated as 100 x (NSE2-NSE1)/NSE1. In-hospital mortality and neurological outcome, as assessed by the Cerebral Performance Category (CPC) scale, were evaluated during admission and after a 6-month follow-up. Results: We included 166 patients admitted to 4 hospitals. In-hospital mortality was 31.9%. Almost 60% of patients had a good neurological recovery (CPC 1-2). On univariate and multivariate logistic regression analyses, an increase in NSE levels was associated with higher in-hospital mortality and worse CPC on discharge and after 6-months (P<.001). Positive Delta-NSE showed an OR = 9.28 (95% CI 4.40-19.57) for mortality, OR = 11.23 (95% CI 5.24-24.11) for CPC 3-5 at discharge and OR = 11.14 (95% CI 5.05-24.55) for CPC 3-5 after 6-months' follow-up (P<.001). The first NSE measurement, conducted at 18 to 24 hours, and the second measurement at 69 to 77 hours after OHCA showed a high area under the curve in predicting CPC at discharge (0.9389 and 0.9909, respectively;0.8096 for the whole cohort). Conclusions: Dynamic changes in NSE serum levels are good markers of hard clinical outcomes after an OHCA due to shockable rhythm in an MTH-treated cohort. NSE measurements at specific intervals after OHCA may predict events even more precisely. (C) 2019 Published by Elsevier Espana, S.L.U. on behalf of Sociedad Espanola de Cardiologia.

关键词： Neuron theory   Cerebral cortex   Neuronal amoeboidism   Glial amoeboidism   Santiago Ram6n y Cajal (1852-1934)   Pedro Lopez-PelSez y Villegas (1863-1903)  

摘要： The purpose of this article is two-fold: first, to preserve, in updated English translations, two theoretical papers written by Santiago Ramon y Cajal (1852-1934) in 1895 and 1896 under the titles, "Conjectures on the anatomical mechanism of ideation, association and attention" and "Conjectural interpretations of certain points in neurological histophysiology";and second, to set some of the ideas proposed by Cajal in a modern perspective. In his "Conjectures," Cajal ventured to explain the mechanisms of perception, association and attention in cellular terms. He introduced the term "impression unit," which would propagate, leading to conscious act via an "avalanche of conduction." Additionally, he attributed mental repose and sleep to morphological variations of neuroglia;at times of relaxation, astrocytes would grow appendices that penetrated among nerve cell connections and blocked the conduction of the "nervous current";in energetic contraction, such glial "pseudopodia" would shrink, allowing neuronal processes to come into contact again. In the sequel to the "Conjectures," Cajal presented strong arguments defending the neuron theory against the reticular theory. Moreover, he discussed the functional differentiation of spinal motor neurons and cortical pyramidal cells, which respectively subserve movement and consciousness, despite their morphological similarity.

关键词： Myxoma   Stellate cells   Fusiform aneurysm   CRP   Radiotherapy  

摘要： Cardiac myxoma can embolize and cause early and delayed sequelae including stroke, growth into intracranial fusiform aneurysms and cerebral tumors with risk of hemorrhage and mass effect. Here, we report the rare coincidence of all these manifestations in a 63-year-old man who presented with cognitive and behavioral changes, and seizures 9 months after an embolic stroke from the heart tumor. C-reactive protein (CRP) was elevated at the time of stroke and cardiac myxoma diagnosis but was normal at late neurologic manifestation with isolated myxoma-related intracranial tumors and aneurysms. Low-dose whole-brain radiotherapy can be helpful to diminish cerebral myxoma tumors and fusiform aneurysms despite reported increased risk of aneurysm rupture.

关键词： Stroke   C-reactive protein   Early neurological deterioration   NIHSS   Atrial fibrillation  

摘要： Background and purpose: The association of high-sensitivity C-reactive protein (hsCRP) with early neurological deterioration (END) is unclear, especially in stroke patients with atrial fibrillation (AF). In this study, we aimed to assess the association of baseline hsCRP levels with END in acute ischemic stroke with and without AF. Methods: Consecutive acute ischemic stroke patients prospectively recruited from the Affiliated Hospital of Yangzhou University were analyzed and divided into two groups: AF related stroke (AF-S) and non-AF related stroke (Non-AF-S) groups. Plasma hsCRP levels on admission were categorized into three risk groups: low (<1.0 mg/L), average (1-3 mg/L) and high (>3 mg/L). Results: A total of 655 consecutive patients diagnosed acute ischemic stroke were prospectively registered from our department in 2015-2018, which included 168 AF-S and 487 Non-AF-S cases. After standard therapy, 62 AF-S and 155 Non-AF-S cases developed END within 72 h of hospitalization. In AF-S cases, statistical differences between END and Non-END patients were found in age, gender, baseline National Institute of Health Stroke Scale (NIHSS) score, fasting blood glucose, responsible artery occlusion, CHA(2)DS(2)-VASc score and hsCRP level (p < 0.05). When variates showing p <= 0.1 in univariate analysis were adjusted, logistics regression analysis revealed following indexes as independent risk factors for END in AF-S patients: female (OR = 2.396, 95%CI:1.062-5.405, P = 0.035), fasting blood glucose (OR = 1.192, 95%CI:1.026-1.385, P = 0.022), responsible artery occlusion (OR = 3.589, 95%CI 1.425-9.036, P = 0.007), and high risk hsCRP (OR = 2.780, 95%CI 1.067-7.240, P = 0.036). In the Non-AF group, any level of hsCRP was not independently related to END after adjustment for age, sex, diabetes mellitus, smoking, baseline NIHSS, lesion size and responsible artery occlusion. Conclusion: High hsCRP level was independently correlated with END in patients with AF-S. (C) 2019 Elsev

关键词： Bone morphogenetic proteins   Oligodendrocytes   Demyelination   Remyelination   Smads   Inhibitory signaling factors  

摘要： During demyelinating disease such as multiple sclerosis and stroke, myelin is destroyed and along with it, the oligodendrocytes that synthesize the myelin. Thus, recovery is limited due to both interruptions in neuronal transmission as well as lack of support for neurons. Although oligodendrocyte progenitor cells remain abundant in the central nervous system, they rarely mature and form new functional myelin in the diseased CNS. In cell culture and in experimental models of demyelinating disease, inhibitory signaling factors decrease myelination and remyelination. One of the most potent of these are the bone morphogenetic proteins (BMPs), a family of proteins that strongly inhibits oligodendrocyte progenitor differentiation and myelination in culture. BMPs are highly expressed in the dorsal CNS during pre-natal development and serve to regulate dorsal ventral patterning. Their expression decreases after birth but is significantly increased in rodent demyelination models such as experimental autoimmune encephalomyelitis, cuprizone ingestion and spinal cord injury. However, until recently, evidence for BMP upregulation in human disease has been scarce. This review discusses new human studies showing that in multiple sclerosis and other demyelinating diseases, BMPs are expressed by immune cells invading the CNS as well as resident CNS cell types, mostly astrocytes and microglia. Expression of endogenous BMP antagonists is also regulated. Identification of BMPs in the CNS is correlated with areas of demyelination and inflammation. These studies further support BMP as a potential therapeutic target.

关键词： Wake-up stroke   Thrombolysis   Ischemic volume lesion   Neuroimaging   Decision-making  

摘要： BackgroundsWake-Up Stroke (WUS) patients are generally excluded from thrombolytic therapy (rTPA) due to the unknown time of stroke onset. This study aimed to investigate the effects of rTPA in WUS patients during every day clinical scenarios, by measuring ischemic lesion volume and functional outcomes compared to non-treated WUS *** retrospectively analyzed clinical and imaging data of 149 (75 rTPA;74 non-rTPA) patients with acute ischemic WUS. Ischemic volume was calculated on follow-up CT and functional outcomes were the NIHSS and mRS comparing rTPA and non-rTPA WUS. Patients were selected using ASPECTS>6 on CT and/or ischemic penumbra>50% of hypoperfused tissue on *** reduced volume was measured on the follow-up CT for rTPA (1 mL, 0-8) compared to the non-rTPA patients (10 mL, 0-40;p=0.000). NIHSS at 7 days from admission was significantly lower in the rTPA (1, 0-4) compared to non-rTPA group (3, 1-9;p=0.015), as was the percentage of improvement (Delta NIHSS) (70% vs 50%;p=0.002). A higher prevalence of mRS 0-2 was observed in the rTPA compared to the non-rTPA (54% vs 39%;p=0.060). Multivariate analysis showed that NIHSS at baseline and rTPA treatment are significant predictors of good outcome both in terms of NIHSS at 7 days and ischemic lesion volume on follow-up CT (p<0.05).ConclusionsrTPA in WUS patients selected with CT and/or CTP resulted in reduced ischemic infarct volume on follow-up CT and better functional outcome without increment of intracranial hemorrhages and in-hospital mortality.