关键词： cardiac surgery   cerebrovascular accident   postoperative cognitive dysfunction   postoperative delirium  

摘要： Brain injury from cardiac surgery is an important source of patient morbidity and mortality. The relationship between risk of brain injury and advanced age portends a rising frequency of these complications due to an increasing proportion of elderly patients undergoing cardiac surgery. This review will explore the aetiology and risk factors for peri-operative stroke, postoperative cognitive dysfunction and postoperative delirium. The prevention of each of these conditions will also be discussed, with a focus on brain protection strategies and the avoidance of cerebral embolism and hypoperfusion.

摘要： Here, we provide an in-depth literature and experience-based review of nonclinical models and data used to support orphan medicinal product designations (OMPDs) in rare neurodegenerative conditions. The Committee for Orphan Medicinal Products (COMP) of the European Medicines Agency updates its assessment processes based on scientific progress and aims to provide transparent criteria required in support of OMPDs. Thus, we also provide an updated analysis of existing nonclinical models in selected conditions and identify key features of nonclinical studies that are crucial for the support of OMPDs. This could not only inform future drug development in rare neurological conditions, but also indicate areas where the use of nonclinical models can be made more efficient.

关键词： Prenatal   Fetofetal transfusion   Polymycrogyria   Imaging  

摘要： Fetofetal transfusion syndrome is a dreaded cause of morbidity and mortality in monochorionic pregnancies. We present two pairs of twins one of which we have followed for more than 6 years. The donors suffer from cerebral palsy, orofacial, and motor problems, and both are significantly smaller than their recipient twins. Interestingly, cranial MRI revealed medial frontal lobe polymicrogyria, ventriculomegaly, and decreased thickness in both parietal lobes in both donors. We suggest this as a possible feature of fetofetal transfusion syndrome. A minireview of the literature on neuroimaging and neurodevelopmental outcome in fetofetal transfusion syndrome is presented. While the close resemblance of the imaging features of both cases is likely incidental further study of a connection between migration and gyration disorders and fetofetal transfusion syndrome is warranted.

关键词： GordonMorgan Holmes   Stewart-Holmes maneuver   Stewart-Holmes test   Gordon-Holmes syndrome   Holmes-Adie pupil  

摘要： Sir Gordon Morgan Holmes (1876-1965) was one of the most important founders of modern neurology and a great teacher and scientist. He was the first scientist to challenge the theory of the unitary function of the cerebellum and described cerebellar disorders. Holmes together with Thomas Grainger Stewart (1877-1957) described 40 cases of the rebound phenomenon in cerebellar disease (Stewart-Holmes maneuver or Stewart-Holmes test). He also described the symptoms of inherited neurodegenerative spinocerebellar ataxia involving the olivary nucleus (Gordon-Holmes syndrome). Independently from the Australian neurologist William John Adie (1886-1935), he described the partial iridoplegia (Holmes-Adie pupil or Holmes-Adie syndrome). His teaching skills became clearly visible in Goulstonian and Croonian lectures dedicated to spinal cord injuries.

关键词： Laparoscopic gastroesophageal dissociation (LGED)   Total esophagogastric dissociation (TEGD)   Esophagogastric disconnect   Gastroesophageal reflux disease (GERD)   Neurologically impaired children  

摘要： Purpose: Neurologically impaired children with severe gastroesophageal reflux disease (GERD) are a challenging group of patients. We theorized that a laparoscopic gastroesophageal dissociation (LGED) may decrease reflux-related readmissions and healthcare visits, and improve quality of life (QOL) for them and their caregivers. Methods: A retrospective review was performed on our pediatric patients that underwent an LGED along with a caregiver survey from 2013 to 2017. Results: Twenty-two neurologically impaired patients (14 months-17 years) with severe GERD underwent an LGED. Patients weighed 7.9-57 kg (avg = 23.8 kg), length of stay ranged from 5 to 20 days (avg = 12 days), estimated blood loss ranged from <5cm(3) to 450cm(3) (avg = 66 cm(3), median = 25 cm(3)), and duration of operation ranged from 299 to 641 min (avg = 462 min). One death occurred on postoperative day 19 from gram negative sepsis (30-day perioperative mortality of 4.5%). There were a modest number of minor and major complications (follow-up avg.=13.7 months, range=2-40 months). There was a decrease in healthcare visits for respiratory illnesses (rated 5/5 from all 13/19 survey respondents) as well as improvements in perceived QOL of the patient (avg=4.3/5) and caregiver (avg=4.6/5). Conclusions: Our cohort of patients had a reduction in readmissions and healthcare visits, and improved QOL after undergoing an LGED based on the perceptions of their caregivers. In neurologically impaired patients with severe GERD, an LGED may be a viable alternative to traditional treatments. Type of study: Retrospective case series review. (C) 2017 Elsevier Inc. All rights reserved.

关键词： dystonia   torsinA   gene dosage   arthrogryposis   congenital movement disorder  

关键词： Biomarkers of cerebral injury   Neuron-specific enolase   S100b   Paediatric cardiac arrest   Neurological outcome  

摘要： Aim: To investigate if the serum biomarkers of cerebral injury, neuron-specific enolase and S100b protein, may classify unfavourable neurological outcome after paediatric cardiac arrest. Methods: We performed a retrospective study of neuron-specific enolase and S100b measurements from 95 children treated in our paediatric cardiac intensive care unit after cardiac arrest. Neurological outcome at discharge was evaluated using the paediatric cerebral performance category scale, with unfavourable outcome defined as a change of >1 compared to pre-arrest status or death. Results: Fifty-eight patients (61.1%) survived to discharge with 48 (50.5%) having a favourable neurological outcome. We observed significantly higher levels of both biomarkers in the unfavourable outcome group at designated time points (neuron-specific enolase at 24, 48, and 72 h and S100b at 12, 24, and 48 h after cardiac arrest, p < 0.05). Receiver operating characteristic areas under the curve for neuron-specific enolase were 0.83, 0.80, and 0.73 at time points 24, 48, and 72 h and 0.87, 0.81, and 0.82 for S100b at 12, 24, and 48 h after cardiac arrest, respectively. Neuron-specific enolase measurement at 24 h after cardiac arrest was an independent predictor of unfavourable outcome in a multivariable analysis. Conclusions: Neuron-specific enolase and S100b classify unfavourable neurological outcome in this large paediatric cardiac arrest cohort. Further multi-institutional prospective studies to comprehensively evaluate the diagnostic accuracy of these biomarkers under various clinical conditions and to determine reliable cut-off values in children are warranted. (c) 2017 Elsevier B.V. All rights reserved.

关键词： Non-steroidal anti-inflammatory drugs   Cyclooxygenase inhibition   Prostaglandins   Neuroinflammation   Neurodegeneration   Neuroinflammation  

摘要： Non-steroidal anti-inflammatory drugs (NSAIDs) have been in use for many years and constitute a large part of prescriptions issued in daily practice. Although NSAIDs are used for many diseases in neurology, they have also been tested as a new therapeutic option for various other diseases. While their effects on headache and cerebrovascular diseases are well known, little is known about their impact on neurodegenerative diseases. This review discusses the use, effects and safety of NSAIDs in neurological diseases. (C) 2017 Published by Elsevier B.V.

关键词： Cauda equina   Conus medullaris   Schwannoma   Neurofibroma   Thoracolumbar junction   Unilateral laminectomy  

摘要： The objective of this retrospective case analysis was to evaluate the surgical impact on neurological functional recovery in cases of solitary spinal nerve sheath tumors (NSTs) originating around the epiconus, conus medullaris, or cauda equina and not associated with neurofibromatosis. This 10-year surgical study included 30 cases of spinal NSTs originating around the spine level of T12-L1 (epiconus-to-conus medullaris: Epi-CM group), and 15 cases of spinal NSTs originating below the spine level of L2 (cauda equina: CE group). A minimally invasive posterior unilateral approach was applied in 22 of 30 subjects in the Epi-CM group and in 14 of 15 subjects in the CE group. Total removal of the tumor was achieved in all cases except in two cases of subpial growth and foraminal extension in the Epi-CM group at the initial surgery. Functional assessment revealed satisfactory or acceptable neurological recovery in both groups. Twelve of 30 patients (40%) in the Epi-CM group demonstrated significant motor weakness of the ipsilateral leg, predominantly manifested as drop foot before surgery, and only 4 of 12 patients demonstrated complete recovery. Ten of 30 patients (33%) in the Epi-CM group demonstrated a significant disturbance of urination before surgery, and only 6 of 10 demonstrated complete recovery. No patients in the CE group demonstrated unsatisfactory recovery of leg motor weakness or urinary disturbance. The present study suggests that surgeons should be aware that spinal NSTs originating around the epiconus or conus medullaris may result in delayed or inadequate functional recovery, even after successful surgical resection of tumors.

关键词： BAVMs supplied by deep perforating arteries   Predominant supply   Lesion-to-eloquent fibre distance   Neurological deficits  

摘要： The treatment of brain arteriovenous malformations supplied by deep perforating arteries (PA) (P-BAVM) remains challenging. The aims of this study were to determine the outcomes after surgical treatment in patients with P-BAVMs and to identify the risk factors associated with postoperative neurological deficits. We retrospectively reviewed the medical charts and imaging records of 228 consecutive patients with BAVMs who underwent microsurgical resection of their BAVMs at Beijing Tiantan Hospital between September 2012 and March 2016. Patients were included if the BAVMs were totally or partially supplied by PA. All patients had undergone preoperative diffusion tensor imaging (DTI), MRI, 3D time-of-flight MRA (3D TOF-MRA) and digital subtraction angiography (DSA) followed by resection. Both functional and angioarchitectural factors were analysed with respect to the postoperative neurological deficits, including motor deficits, visual field deficits and aphasia. Statistical analysis was performed using the statistical package SPSS (version 20.0.0, IBM Corp.). Fifty-nine patients with P-BAVMs were enrolled. Radical obliteration was achieved in all P-BAVMs according to postoperative DSA. Forty-five (76.3%) patients obtained neurological deficits 1 week after surgery. At a mean follow-up of 14.7 +/- 9.4 (3-30) months after surgery, 34 patients (57.6%) had long-term neurological deficits. Multivariable logistic regression analyses showed that a shorter lesion-to-eloquent fibre tracts distance (LFD) was an independent risk factor for short- (P = 0.014) and long-term (P = 0.013) neurological deficits. The cut-off point of LFD for long-term neurological deficits was 5.20 mm. The predominant supply of the PA (P = 0.008) was an independent risk factor for long-term neurological deficits. This study identified a high risk of surgical morbidity for P-BAVMs. The predominant supply of the PA and a shorter LFD are crucial risk factors for postoperative neurological deficits in patie