摘要： Background: Various pathological elbow lesions are often complicated with ulnar neuropathy at the elbow (UNE), although the precise pathology, incidence, and clinical and neurological features of these lesions have not been identified. We therefore investigated elbow pathology and neurological severity in Japanese patients with UNE. Methods: The medical records of 457 Japanese UNE patients who were surgically treated among 6 hospitals were retrospectively examined. Eligible patients had UNE diagnosed by physical findings and nerve conduction studies according to the criteria of the American Association of Electrodiagnostic Medicine. The elbows were analyzed with regard to age, gender, occupation, pathology at the elbow, and severity of nerve palsy. Results: A total of 398 patients with 413 UNE elbows of a mean age of 63 years (range: 15-87) met the inclusion criteria. UNE elbows were predominantly in male patients (69.0%). Overall, 310 elbows (75.1%) had 1 or more elbow lesions: 238 elbows (76.8%) had a single lesion and 72 elbows (23.3%) had 2 or more lesions. The most common lesion was primary elbow osteoarthritis (EOA) occurring in 54.5% of elbows, followed next by medial elbow ganglion in 8.5% and cubitus valgus in 6.5%. Most elbows with medial elbow ganglion or cubitus valgus were associated with EOA. Entrapment sites were at the cubital tunnel in 84.5%-91.3% of UNE elbows, regardless of an association with elbow lesion. The incidence of McGowan grade III lesion was 50.8% in elbows with primary EOA, which was higher than the 35.0% in elbows with no lesion. Conclusions: This study revealed that UNE had various isolated or combined elbow lesions. In Japanese UNE, primary or secondary EOA was found in 62.2% of cases and severe motor weakness was noted in 47.2%. The incidences of EOA and severe ulnar nerve palsy in the Japanese UNE are higher than those in Caucasians. (C) 2019 The Japanese Orthopaedic Association. Published by Elsevier B.V. All rights reserved.

关键词： Perfluoroalkyl substances   PFAA   PFAS   Brain   Neuroendocrinology   Animal models  

摘要： Perfluoroalkyl acids (PFAAs) are man-made organic pollutants that are found ubiquitously in the environment and may impact human health. Here, we review the published literature concerning PFAA impacts on neurobiological, neuroendocrine, and neurobehavioral outcomes. We find that there are many mechanisms through which PFAAs may enter the brain and interact with biochemical endpoints to impact neurological function. These results are supported by epidemiological evidence in humans and experimental evidence in animals that demonstrate numerous and varied PFAA impacts on the nervous system. However, the methods commonly used in animal models of PFAA exposure result in durations of exposure and serum PFAA concentrations in blood that may not appropriately mimic human absorption, distribution, metabolism, and excretion. If animal models lack validity, confidence in mechanistic inferences regarding PFAA exposure and brain function is reduced, limiting these studies' utility. Finally, we end by suggesting some potential impacts of PFAA exposure in human neurological health and disease states whose associations may not readily present themselves in the epidemiological literature.

关键词： Lead   Di (n-butyl) phthalate   Co-exposure   Developmental neurological toxicity   Synergistic effect  

摘要： In this study, the immature mice were taken to assess the potential neurological toxicity of lead (Pb) and di (n-butyl) phthalates (DBP) combination exposure. Mouse administration with DBP combination with Pb exhibited longer escape latency and lower average number of crossing of the platform. Pb content in the tissues was increased, especially in the brain, after Pb exposure as compared to those without Pb exposure. The alterations of oxidative damages in tissues (MDA and SOD) and biochemical indicators in the brain (AChE, TNOS, and iNOS) were observed, as well as the synergistic effect of joint exposure. Expressions of apoptosis-related protein (bax/bcl-2 ratio and caspase-3) were significantly increased in the hippocampus, while the bcl-2 was remarkably decreased and no significant differences were observed on the bax. The results suggested that the possible mechanisms for the learning and memory ability impairments were as follows: Firstly, the combination exposure induced the occurrence of lipid peroxidation in the brain, leading to damage to the brain cells. Secondly, it destroyed the normal metabolic balance of ACh, causing nerve damage in mice. Thirdly, it induced apoptosis in mouse hippocampal cells. The overall findings revealed that Pb and DBP co-exposure greatly influenced the developmental nervous system and accompanied with synergistic toxic effect.

关键词： Risk factors   Signs and symptoms  

摘要： Study design Retrospective cohort study. Objective To assess the rate, injury site, aetiology and outcomes in elective spinal surgery patients who sustained a spinal cord injury (SCI). Setting SCI national centre Toledo, Spain. Methods The study sample included patients who sustained an SCI after elective spinal surgery from 2013 to 2017. Oncological patients and patients receiving interventional therapies were excluded. Data collected included: demographics, aetiology, precipitating cause, injury mechanism, injury site, neurological status (AIS), SCIMIII at admission and discharge, hypertension, diabetes mellitus, obesity, dyslipidemia, depression and hospital length of stay. Results One thousand two hundred and eighty-two patients were admitted in this period of whom 114 met the inclusion criteria with a median (IQR) age of 58 (45-69) years;46% female. The prevalence of SCI as a complication following spinal surgery in the total number of patients admitted to our centre was 9%. In 43%, the injury was to the dorsal spine with T12 being the most common neurological level of injury (20% of cases following laminectomy secondary to spinal canal stenosis). The most frequent precipitating cause was epidural haematoma (38% of cases). The median (IQR) SCIMIII scores at admission and discharge were (31) points (20-54) and (67) points (34-81), respectively. General AIS at admission were C (35%) and D at discharge (54%). The presence of hypertension, diabetes mellitus, obesity and dyslipidemia adjusted by age was not linked to a higher complication rate. The median (IQR) hospital length of stay was 120 days (60-189). Conclusion In total 8.9% of patients admitted with SCI were the result of elective spinal surgery.

关键词： Neurological conditions   depression   behavioural activation   behavioural therapy   activity scheduling  

摘要： Objective: To evaluate the effectiveness of behavioural activation interventions for people with neurological conditions with comorbid depression, and explore content and adaptations. Data sources: PsycINFO, MEDLINE, CINAHL, AMED, and EMBASE databases were searched on the 19 November 2019. Reference lists of selected full-texts were screened by title. Review methods: We included peer-reviewed studies published in English that used behavioural activation for treatment of depression in adults with a neurological condition. Single-case reports, reviews, and grey literature were excluded. Methodological quality was assessed by two authors independently, and quality was appraised using Critical Appraisal Skills Programme checklists. Results: From 2714 citations, 10 articles were included comprising 590 participants. Behavioural activation was used to treat depression in people with dementia (n = 4), stroke (n = 3), epilepsy (n = 1), Parkinson's disease (n = 1), and brain injury (n = 1). Sample size ranged from 4 to 105 participants. There were seven randomized controlled studies;however, no studies compared behavioural activation to an alternative psychological therapy. The effect sizes varied between small and large in the studies where effect size could be calculated (d = 0.24-1.7). Methodological quality of the included studies was variable. Intervention components were identifying and engaging in pleasurable activities, psychoeducation, and problem-solving. Adaptations included delivering sessions via telephone, delivering interventions via primary caregivers, and giving psychoeducation to caregivers. Conclusion: The effectiveness of behavioural activation in randomized controlled trials varied from small to large (d = 0.24-1.7) in reducing depression. The content of behavioural activation was comparable to established treatment manuals. Adaptations appeared to support individuals to engage in therapy. Review registration: PROSPERO 2018, CRD42018102604.

关键词： Atlantoaxial joint   Conservation   Craniovertebral junction   Surgery   Treatment  

摘要： BACKGROUND: Tuberculosis (TB) in the atlantoaxial joint is a rare disease. However, the treatment of atlantoaxial TB with neurologic impairment is controversial. The aim of this review is to provide clinical outcomes of surgical and nonsurgical management of atlantoaxial TB. METHODS: Databases including PubMed, Embase, and the Cochrane Central Register of Controlled Trials were searched for English literature describing the treatment of atlantoaxial TB with neurologic deficits. The outcomes of conservative and surgical treatment approaches, including treatment failure, death, changes in neurologic impairment, and complications, were compared by performing odds ratio (OR) analysis. RESULTS: Overall, 24 studies (247 patients) meeting the inclusion criteria were analyzed. Ninety-four patients (38%) were treated conservatively and 153 (62%) patients were treated surgically. The rate of poor outcomes was greater in the conservative group (14.89%) than in the surgery group (1.3%) (OR, 0.081;95% confidence interval [CI], 0.016-0.39).There was no significant difference in mortality between the conservative (1.06%) and surgery (3.27%) groups (OR, 3.28;95% CI, 0.494-27.381). There was no significant difference in muscle power improvement between the 2 treatments (conservative, 95.7%;surgery:, 94.8%;OR, 1.353;95% CI, 0.291-4.925). CONCLUSIONS: Conservative and surgical treatments both significantly improved neurologic deficits in most patients. Compared with conservative treatment, surgical treatment reduced treatment failures without significantly increasing the rates of neurologic deficit improvement or mortality.

关键词： network approach   neurodiagnostic marker specific neurodiagnostic biomarker   neurological disorders   non-specific neurodiagnostic biomarker   personalized medication  

摘要： An exponential increase in the prevalence of neurological disorders requires substantial steps to be taken for their prevention and treatment. Neurodiagnostic biomarkers are gaining momentum presently in order to enhance the diagnostic accuracy of neurodegenerative disorders, to precisely assess their advancement and to monitor the efficiency of therapeutic interventions. Therefore, the primary focus of the present review is the recent development in this field of neurodiagnostic biomarkers, and the current state of biomarker exploration in the context of various neurodegenerative diseases. This review encompasses an updated and detailed account of specific (beta-Amyloid, Tau and Phospho-tau 181, Tar-DNA binding protein-43, Progranulin, a-synuclein, Clusterin, etc) and non-specific (genetic, synaptic, inflammatory and coagulation) neurodiagnostic biomarkers and the recent advances in this growing field. This comprehensive review also suggests the utilization of neurodiagnostic markers in network approaches and personalized medication that will eventually improvise the existing diagnostic and therapeutic complexities of neurodiagnostic biomarkers.

关键词： Blood   Cerebrospinal fluid   Diagnosis   Equine   Neurobiomarker  

摘要： Neurological disorders (NDs) are often fatal to horses. Thus, symptoms of equine NDs commonly indicate euthanasia. Current diagnostic approaches for equine NDs is based on clinical signs, differential diagnoses, analysis of cerebrospinal fluid (CSF), assessment of histopathological lesions, and imaging. However, advances in biofluid biomarkers in the diagnosis of human neurological diseases can potentially be applied to equine NDs. In this review, we described the established human blood and CSF neurobiomarkers that could potentially be used to diagnose equine NDs. (C) 2019 Elsevier Inc. All rights reserved.

关键词： CLN7   functional studies   inherited retinal disease   locus resequencing of ABCA4   maculopathy   MFSD8 variants   neuronal ceroid lipofuscinosis   whole exome sequencing  

摘要： Biallelic MFSD8 variants are an established cause of severe late-infantile subtype of neuronal ceroid lipofuscinosis (v-LINCL), a severe lysosomal storage disorder, but have also been associated with nonsyndromic adult-onset maculopathy. Here, we functionally characterized two novel MFSD8 variants found in a child with juvenile isolated maculopathy, in order to establish a refined prognosis. ABCA4 locus resequencing was followed by the analysis of other inherited retinal disease genes by whole exome sequencing (WES). Minigene assays and cDNA sequencing were used to assess the effect of a novel MFSD8 splice variant. MFSD8 expression was quantified with qPCR and overexpression studies were analyzed by immunoblotting. Transmission electron microscopy (TEM) was performed on a skin biopsy and ophthalmological and neurological re-examinations were conducted. WES revealed two novel MFSD8 variants: c.[590del];[439+3A>C] p.[Gly197Valfs*2];[Ile67Glufs*3]. Characterization of the c.439+3A>C variant via splice assays showed exon-skipping (***67Glufs*3), while overexpression studies of the corresponding protein indicated expression of a truncated polypeptide. In addition, a significantly reduced MFSD8 RNA expression was noted in patient's lymphocytes. TEM of a skin biopsy revealed typical v-LINCL lipopigment inclusions while neurological imaging of the proband displayed subtle cerebellar atrophy. Functional characterization demonstrated the pathogenicity of two novel MFSD8 variants, found in a child with an initial diagnosis of juvenile isolated maculopathy but likely evolving to v-LINCL with a protracted disease course. Our study allowed a refined neurological prognosis in the proband and expands the natural history of MFSD8-associated disease.

关键词： Dioctyl phthalate (DOP)   Neurotoxicity   Heavy metals   Mixture exposure  

摘要： Dioctyl phthalate (DOP) (200, 500, and 1000 mg kg(-1) bw, i.g.), Pb (Ac)(2) (50 mg L-1, p.o.), and NaAsO2 (10 mg L-1, p.o.) were administered individually and as mixtures to weanling male mice for 8 weeks. It was observed that Pb, As, and DOP exposure could significantly inhibit the growth and development of mice. Compared with the Pb, As, and Pb + As groups, the activities of iNOS and TNOS were significantly increased, the levels of AChE and SOD were significantly decreased, and the level of MDA was significantly increased in the Pb + DOP-H, As + DOP-H, and Pb + As + DOP-H groups. The factorial analysis shows that the iNOS, TNOS, and AChE present synergistic effects on Pb, As, and DOP. A significant increase of escape latency and a significant decrease of original platform quadrant stops were observed between Pb + As + DOP-H and Pb + As groups. The factorial analysis shows that there was a synergistic effect on Pb, As, and DOP. Compared with that of the control group, the expression levels of caspase-3 and Bax expression in Pb + As, DOP-H, Pb + DOP-H, As + DOP-H, and Pb + As + DOP-H groups were significantly increased in the hippocampus. The expression levels of Bcl-2 expression decreased significantly and the Bax/Bcl-2 ratio increased significantly. Pathological alterations on the hippocampus were found in exposed groups. This result shows that combined exposure of Pb, As, and DOP could induce neurotoxicity, of which possible mechanism is hippocampal neuronal apoptosis. This study shows that there were three components with eigenvalues greater than 1, which together explained 89.40% of total variance. The first component (PC1) showed high loadings on B-SOD, L-SOD, B-MDA, L-MDA, K-MDA, iNOS, tNOS, and AChE and accounted for 46.55% of the total variance after Varimax rotation. PC2 accounted for 23.81% of the total variance with high loadings on B-As, L-As, K-As, and K-SOD, whereas PC3 showed high loadings on B-Pb, L-Pb, and K-Pb and accounted for 19.04% of the total