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.

关键词： Onchocerca volvulus   Epilepsy   Neurological features  

摘要： Human onchocerciasis, caused by infection by the filarial nematode Onchocerca volvulus, is a major neglected public health problem that affects millions of people in the endemic regions of sub-Saharan Africa and Latin America. Onchocerciasis is known to be associated with skin and eye disease and more recently, neurological features have been recognized as a major manifestation. Especially the latter poses a severe burden on affected individuals and their families. Although definite studies are awaited, preliminary evidence suggests that neurological disease may include the nodding syndrome, Nakalanga syndrome and epilepsy but to date, the exact pathophysiological mechanisms remain unclear. Currently, the only way to prevent Onchocera volvulus associated disease is through interventions that target the elimination of onchocerciasis through community distribution of ivermectin and larviciding the breeding sites of the Similium or blackfly vector in rivers. In this review, we discuss the epidemiology, potential pathological mechanisms as well as prevention and treatment strategies of onchocerciasis, focusing on the neurological disease.

关键词： Karenia brevis (K. brevis)   brevetoxin   headache   exposure-response   illness cost  

摘要： Human respiratory and gastrointestinal illnesses can result from exposures to brevetoxins originating from coastal Florida red tide blooms, comprising the marine alga Karenia brevis (K. brevis). Only limited research on the extent of human health risks and illness costs due to K. brevis blooms has been undertaken to date. Because brevetoxins are known neurotoxins that are able to cross the blood-brain barrier, it is possible that exposure to brevetoxins may be associated with neurological illnesses. This study explored whether K. brevis blooms may be associated with increases in the numbers of emergency department visits for neurological illness. An exposure-response framework was applied to test the effects of K. brevis blooms on human health, using secondary data from diverse sources. After controlling for resident population, seasonal and annual effects, significant increases in emergency department visits were found specifically for headache (ICD-9 784.0) as a primary diagnosis during proximate coastal K. brevis blooms. In particular, an increased risk for older residents (>= 55 years) was identified in the coastal communities of six southwest Florida counties during K brevis bloom events. The incidence of headache associated with K. brevis blooms showed a small but increasing association with K. brevis cell densities. Rough estimates of the costs of this illness were developed for hypothetical bloom occurrences.

关键词： MRS   Wilson's disease   Neurological changes   Children  

摘要： Purpose: Although brain magnetic resonance spectroscopy (MRS) imaging findings in adult Wilson disease (WD) have been explained in extensive details, a paucity of information currently exists regarding brain MRS imaging findings in pediatric WD. The purpose of this study was to clarify the role of brain MRS in detecting early metabolite abnormalities in children with WD. Patient and methods: A case-controlled prospective study included 26 children with WD and 26 healthy controls. All children were subjected to examination on a 1.5 T MRI scanner. The spectra of N-acetyl aspartate (NAA), choline (Cho), and creatine (Cr), as well as the metabolite ratios of NAA/Cho, NAA/Cr, and Cho/Cr, were measured and compared between two groups. Results: Eight patients revealed increased signal intensity in the basal ganglia at T1-weighted images. When compared with healthy controls, WD patients showed a significant decrease (p < 0.05) in NAA (63.8 +/- 9.6 vs 97.6 +/- 3.8), Cho (46.7 +/- 8.9 vs 87.3 +/- 4.7), Cr (44 +/- 10.1 vs 81.9 +/- 4.05), NAA/Cho (1.92 +/- 1.2 vs 3.34 +/- 0.55), NAA/Cr (1.29 +/- 0.7 vs 2.46 +/- 0.34), and Cho/Cr (0.78 +/- 0.4 vs 2 +/- 0.13). Patients complicated with liver cell failure showed a significant decrease in all previous parameters (p < 0.05) than patients without complications. Patients with mixed neurological and hepatic diseases showed a severe reduction in NAA, NAA/Cr, and NAA/Cho compared with patients with hepatic disease only. Conclusion: MRS in pediatric WD detects early neurological changes even with normal MRI.