关键词： child   prognosis   risk factor   stroke   therapeutics  

摘要： Background The aim of this study was to retrospectively assess short-term neurological outcomes in pediatric stroke with regard to patient characteristics. Methods Children aged 28 days-18 years with arterial ischemic stroke (AIS), cerebral sinovenous thrombosis (CSVT), and hemorrhagic stroke (HS) between 2007 and 2013 were evaluated. Neurological findings in the first 3 months were accepted as short-term prognosis, and modified Rankin scale was used. Results A total of 33 patients (62%) with AIS, 12 (23%) with HS, and eight (15%) with CSVT were included. Moya moya syndrome was the most common new diagnosis in AIS. Stroke recurred in five (15%);and one AIS patient with posterior circulation infarct died (3%). Prognosis in AIS was favorable for 20 patients (61%) and poor for 13 patients (39%). Forty-two percent of HS were of vascular origin. Seven patients (70%) with HS had good prognosis and three (30%) had poor prognosis with no death. Homocysteine-related hypercoagulability was most frequently noted in the etiology of CSVT. Synchronous systemic thrombosis was observed in three CSVT patients (37.5%) and death occurred in two (25%). Prognosis was evaluated as favorable for three CSVT patients (37.5%) and poor for five (62.5%). For thrombophilia, thrombosis panel was performed fully in 83% of AIS and CSVT patients. Conclusions Pediatric stroke is associated with a poor prognosis in a substantial number of patients in the short term, with CSVT having the worst prognosis. Detailed patient characteristics are listed not only for ischemic but also for hemorrhagic stroke;and a full thrombosis panel was achieved for most ischemic stroke patients.

关键词： wilderness   austere   medicine   neurology   evacuation   extended treatment  

摘要： The study of wilderness medicine is within the scope of medical care in the austere environment and addresses medicine as practiced in the setting of delayed access to definitive medical care, hostile environment, limited equipment, and inherent risks to the patient and/or rescuers. Part of this topic includes the care of patients with neurologic illness and/or injury. We will address the five most important skills of a wilderness medicine professional: decision making, prevention, preparation, protocol development, and education by applying the principles to select common neurologic problems that occur in the extended environment: traumatic brain injury, dehydration, hyponatremia, heat illness, hypothermia, spine injury, and lightning injury. We will focus on the most pertinent aspects of wilderness medicine: signs and symptoms, initial stabilization and treatment, evacuation, and extended care. An astute wilderness medicine specialist brings environmental and medical skill sets together to know when it is better to treat in the field and when evacuation, with its inherent risks to the patient and rescuers, is unavoidable.

关键词： Autoimmune diseases   Paraneoplastic neurological syndromes   Cancer   Small cell lung cancer   Autoantibodies   Autoimmunity  

摘要： Background: Paraneoplastic neurological syndromes (PNS) are a group of syndromes that affect the central and peripheral neuromuscular system in association with cancer. Specific antibodies may assist in the diagnosis of PNS. The antibodies tested can be classified into those directed against intracellular neuronal proteins ("well characterized" PNS: Hu, Yo, RI, CV2, amphiphysin, Ma1, Ma2) and those directed against neural surface antigens (autoimmune encephalitis syndromes: NMDA, AMPA, LGI1, CASPR2, GABAR). We aimed to characterize patients with unexplained neuropsychiatric symptoms, in whom positive PNS antibodies were detected in the Sheba medical center, a large referral hospital. Methods: Clinical and demographic data of patients with positive PNS antibodies were collected during the years 2002-2016. Antibodies were tested by either Line immunoassay or by cell-based indirect immunofluorscent assay. Results: During the follow up of 14 years, 4010 PNS tests were performed in patients with unexplained neuropsychiatric symptoms. Seventy-two were found to be positive;among them we had full clinical data access to 44. The most frequent antibodies were anti-Hu (31.8%), anti-Yo (18.2%), anti-CV2 (13.6%), and anti-NMDA (9.1%), and the most common cancers were small-cell lung (SCLC) and ovarian cancers. In the well characterized paraneoplastic group, cancer was diagnosed in 55.9% of the patients, and in the autoimmune encephalitis group, 40.0% were diagnosed with cancer. A positive correlation between antibody titer and the presence of cancer was found. Ninety percent of the tests in patients who were found positive were ordered by a neurologist or neuro-oncologist. Conclusions: The titers of PNS auto-antibodies can predict cancer in patients whom anti-PNS antibodies are tested. In addition, consultation with a specialist should be considered before this test is ordered.

关键词： Pregnancy   neurology  

关键词： Canadian Longitudinal Study on Aging   comorbidities   healthcare utilization   neurology  

摘要： Background and purpose Our objective was to study the association between the presence of a neurological disease and the comorbidity burden as well as healthcare utilization (HCU). Methods Using baseline data from the Canadian Longitudinal Study on Aging (CLSA), we examined the burden of five neurological conditions. The CLSA is a population-based study of approximately 50 000 individuals, aged 45-85 years at baseline. We used multivariable Poisson regression to identify correlates of comorbidity burden and HCU. Results The lifetime prevalence of five neurological diseases is presented: epilepsy, Parkinson's disease/parkinsonism, stroke/transient ischaemic attack, multiple sclerosis and migraine. We found the somatic and psychiatric comorbidity burden to be higher in those individuals with a neurological disease (an 18-45% mean increase in the number of chronic conditions) as compared with the comparison group without a neurological disease, except for Parkinson's disease/parkinsonism. The presence of a neurological disease was associated with only a modest increase in the probability of visiting a general practitioner but was associated with a greatly increased probability of visiting a medical specialist (up to 68% more likely) or an emergency department (up to 79% more likely) and an overnight hospitalization (up to 108% more likely). Conclusions We found striking associations between our neurological diseases and increased comorbidity burdens and HCU. These findings are important for informing public policy planning as well as driving avenues for future research. The present study established the CLSA as an important research platform for the study of neurological conditions in an aging general population.

关键词： Ewing sarcoma   Ewing's sarcoma   Spine   Oncology   Surgery   Radiation treatment  

摘要： Object: Many patients with Ewing sarcoma (ES) of the mobile spine present with neurologic symptoms leading to emergency decompressive surgery. Only rarely is optimal treatment involving neo-adjuvant chemotherapy followed by en bloc excision possible. The purpose of this study was to study treatment, neurologic and oncologic outcome in patients with ES of the mobile spine. Methods: Twenty-four patients diagnosed between 1986 and 2012 were identified through the Scandinavian Sarcoma Group registry. Charts were reviewed in order to assess details in patient characteristics, neurologic status, treatment and outcome. Prognostic factors were analyzed with respect to local control, disease-free survival and overall survival. Results: Neurologic symptoms were frequently observed at presentation, being present in 19/23 patients with documented neurologic status. Most (13/19) patients had a complete neurologic recovery regardless of whether or not emergency decompressive surgery was performed. The majority (18/24) of patients were treated with definitive radiotherapy. However, only 9/17 received the recommended dose of >= 50.4 Gy. The disease-free and overall survival rates at 10 years were 48% and 57%, respectively. The local recurrence rates were 19% and 27% at 5 and 10 years, respectively. Only year of diagnosis, categorized into periods with significant changes in chemotherapy protocols, was a significant factor for local recurrence, but there was a trend (p = 0.06) for an increased risk of a local recurrence if emergency decompressive surgery was performed. Conclusion: Patients with ES of the mobile have a relatively favorable prognosis. Nonetheless, local recurrence rate is high for this group of patients for which local treatment mainly relies on definitive radiotherapy. Emergency decompressive surgery may increase the risk for local recurrence.

关键词： Cardiomyopathies   Ventricular dysfunction   Takotsubo cardiomyopathy   Nervous system diseases   Critical care   Subarachnoid hemorrhage   Status epilepticus  

摘要： Background Case series have reported reversible left ventricular dysfunction, also known as stress cardiomyopathy or Takotsubo cardiomyopathy (TCM), in the setting of acute neurological diseases such as subarachnoid hemorrhage. The relative associations between various neurological diseases and Takotsubo remain incompletely *** performed a cross-sectional study of all adults in the National Inpatient Sample, a nationally representative sample of US hospitalizations, from 2006 to 2014. Our exposures of interest were primary diagnoses of acute neurological disease, defined by ICD-9-CM diagnosis codes. Our outcome was a diagnosis of TCM. Binary logistic regression models were used to examine the associations between our pre-specified neurological diagnoses and TCM after adjustment for *** acute neurological diagnoses, the strongest associations were seen with subarachnoid hemorrhage (odds ratio [OR] 11.7;95% confidence interval [CI] 10.2-13.4), status epilepticus (OR 4.9;95% CI 3.7-6.3), and seizures (OR 1.3;95% CI 1.1-1.5). In a sensitivity analysis including secondary diagnoses of acute neurological diagnoses, associations were also seen with transient global amnesia (OR 2.3;95% CI 1.5-3.6), meningoencephalitis (OR 2.1;95% CI 1.7-2.5), migraine (OR 1.7;95% CI 1.5-1.8), intracerebral hemorrhage (OR 1.3;95% CI 1.1-1.5), and ischemic stroke (OR 1.2;95% CI 1.1-1.3). In addition, female sex was strongly associated with Takotsubo (OR 5.1;95% CI 4.9-5.4).ConclusionTCM appears to be associated with varying degrees with several acute neurological diseases besides subarachnoid hemorrhage.

关键词： Cassava   Cognition   Cyanogen   Konzo   Motor neuron disease  

摘要： Epidemics of neurodegenerative diseases putatively caused by food toxins have been reported in the tropics with no clear understanding of their pathogenetic mechanisms. These diseases include the disease named Konzo that has been well documented in sub-Sahara Africa, mostly among children and women of childbearing age. Outbreaks of Konzo have occurred in the Democratic Republic of Congo, Mozambique, Tanzania, Central African Republic, Angola, Cameroun, and most recently in Zambia. The main clinical picture consists of a symmetrical, permanent and irreversible spastic paraparesis (motor neuron disease) with no signs of sensory or genitourinary impairments. Recently, cognitive impairments and neurodevelopmental delays have been reported among school-aged and very young children. The exact pathogenetic mechanisms of the disease remain unknown. Epidemiological studies consistently show an association between outbreaks of the disease and chronic dietary reliance on insufficiently processed cyanogenic cassava (manioc or tapioca). Biochemical and toxicological studies suggest that the metabolites of linamarin (a-Hydroxyisobutyronitrile jl.D-glucopyranoside, the main cassava cyanogen), notably cyanide (mitochondria] toxin), thiocyanate (AMPA chaotropic agent), and cyanate (protein carbamoylating agent) may play an important role in the pathogenesis of Konzo. Experimental data suggest that thiol-redox and protein- folding mechanisms may also be perturbed. Factors of susceptibility including genetics, poor nutrition, poverty and dietary cyanogen exposure, or their interactions have been suggested. Serological studies have ruled out the role of retroviruses such as the human lymphotropic viruses HIV-I/II or HTLV-I/II. Because there is no cure for Konzo, prevention of the disease remains of paramount importance. Prospects for cognitive rehabilitation still need to be explored and tested.

关键词： gene editing   genome sequencing   macaques   marmosets   transgenesis   transgenic animals  

摘要： Research with non-human primates (NHP) has been essential and effective in increasing our ability to find cures for a large number of diseases that cause human suffering and death. Extending the availability and use of genetic engineering techniques to NHP will allow the creation and study of NHP models of human disease, as well as broaden our understanding of neural circuits in the primate brain. With the recent development of efficient genetic engineering techniques that can be used for NHP, there's increased hope that NHP will significantly accelerate our understanding of the etiology of human neurological and neuropsychiatric disorders. In this article, we review the present state of genetic engineering tools used in NHP, from the early efforts to induce exogeneous gene expression in macaques and marmosets, to the latest results in producing germline transmission of different transgenes and the establishment of knockout lines of specific genes. We conclude with future perspectives on the further development and employment of these tools to generate genetically engineered NHP.

关键词： calcaneal osteotomy   safe zone   foot   neurological damage   complications   outcomes  

摘要： Background. Calcaneal osteotomy is a commonly used surgical option for the correction of hindfoot malalignment. A previous cadaveric study described a neurological "safe zone" for calcaneal osteotomy. We performed a retrospective chart review to evaluate the presence of neurological injuries following calcaneal osteotomies and the location of the osteotomy in relation to the reported safe zone. Methods. In this retrospective study, we reviewed charts of patients who underwent calcaneal osteotomy at our institution from 2011 to 2015. All immediate postoperative radiographs were examined and the shortest distance between the calcaneal osteotomy line and a reference line connecting the posterior superior apex of the calcaneal tuberosity to the origin of the plantar fascia was measured. If the osteotomy line was positioned within an area 11.2 mm anterior to the reference line, it was considered to be inside the neurological safe zone. We correlated the positioning of the osteotomy with the presence of postoperative neurological complications. Results. We identified 179 calcaneal osteotomy cases. Of the 174 (97.2%) nerve injury-free cases, 62.6% (109/174) were performed inside the defined "safe zone" while 37.4% (65/174) outside. A total of 5 (2.8%) nerve complications were identified: 3 (60%) were inside the safe zone and 2 (40%) outside the safe zone. Osteotomies outside the safe zone had a 1.114 relative risk of nerve injury with a 95% CI of 0.191 to 6.500 and showed no statistically significant difference (P = .9042). Conclusion. Our findings suggest that the clinical "safe zone" in calcaneal osteotomies may not actually exist, likely because of wide anatomical variation of the implicated nerves, as described in prior studies. Patients should be properly counseled preoperatively on the low, but seemingly fixed, risk of nerve injury before undergoing calcaneal osteotomy.