关键词： Moyamoya diease   Transient neurological events   Combined bypass   Vascular disorders  

摘要： Objective: Transient neurological events (TNEs) are frequently observed after revascularization surgery for moyamoya disease (MMD). However, clinical features and pathophysiology of TNEs in MMD are still unclear. This study was aimed to clarify the incidence and time course of TNEs and to determine the independent predictors of TNEs in MMD. Patients and methods: A total of 195 hemispheres in 171 consecutive patients with MMD who had undergone combined direct and indirect bypass surgery were analyzed. Preoperative clinical characteristics and radiographic features were recorded. The incidence and clinical feature of postoperative TNEs were evaluated. Multivariate logistic regression analyses were performed to identify the risk factors for postoperative TNEs. Outcomes were compared between patients who had TNEs with those without TNEs at the time of discharge. Results: Postoperative TNEs were detected in 40 (20.5%) of 195 operated hemispheres, including 17 (42.5%) aphasia, 9 numbness of the extremities (22.5%), 6 seizures (15%), 5 motor weakness (12.5%), 4 dysarthria (10%) and 6 others (15%). The incidence of TNEs was significantly higher in adult patients than in pediatric ones. Multivariate analysis revealed that female, left-sided surgery and the presence of the edematous lesion was an independent predictor of TNEs after surgery in MMD (OR, 3.0;95% CI, 1.1-8.2;P = 0.03, OR, 2.9;95% CI, 1.2-7.0;P = 0.02 and OR, 17.4;95% CI, 5.7-53.0;P < 0.01, respectively). DSA stage (OR 0.05, 95% CI 0.0-0.5, p = 0.005;OR 0.08, 95% CI 0.0-0.4, p = 0.008), PCA involvement (OR 2.75, 95% CI 1.0-7.4, p = 0.046), left-sided surgery (OR 2.73, 95% CI 1.2-6.5, p = 0.022) and edematous lesion (OR 21.2, 95% CI 7.6-59.7, < 0.001) were significantly associated with TNE severity. Compared with patients without postoperative TNEs, no significant differences in mRS score between the two groups were detected. Conclusions: Female, left-sided surgery and edematous lesion were independent risk factors

关键词： Myelomeningocele   Scoliosis   Tethered cord release   Prophylactic untethering  

摘要： Purpose Children with myelomeningocele (MMC) are at increased risk of developing neuromuscular scoliosis and spinal cord re-tethering (Childs Nerv Syst 12:748-754, 1996;Neurosurg Focus 16:2, 2004;Neurosurg Focus 29:1, 2010). Some centers perform prophylactic untethering on asymptomatic MMC patients prior to scoliosis surgery because of concern that additional traction on the cord may place the patient at greater risk of neurologic deterioration peri-operatively. However, prophylactic untethering may not be justified if it carries increased surgical risks. The purpose of this study was to determine if prophylactic untethering is necessary in asymptomatic children with MMC undergoing scoliosis surgery. Methods A multidisciplinary, retrospective cohort study from seven children's hospitals was performed including asymptomatic children with MMC < 21 years old, managed with or without prophylactic untethering prior to scoliosis surgery. Patients were divided into three groups for analysis: (1) untethering at the time of scoliosis surgery (concomitant untethering), (2) untethering within 3 months of scoliosis surgery (prior untethering), and (3) no prophylactic untethering. Baseline data, intra-operative reports, and 90-day post-operative outcomes were analyzed to assess for differences in neurologic outcomes, surgical complications, and overall length of stay. Results A total of 208 patients were included for analysis (mean age 9.4 years, 52% girls). No patient in any of the groups exhibited worsened motor or sensory function at 90 days post-operatively. However, comparing the prophylactic untethering groups with the group that was not untethered, there was an increased risk of surgical site infection (SSI) (31.3% concomitant, 28.6% prior untethering vs. 12.3% no untethering;p = 0.0104), return to the OR (43.8% concomitant, 23.8% prior untethering vs. 17.4% no untethering;p = 0.0047), need for blood transfusion (51.6% concomitant, 57.1% prior untethering vs. 33.8% no unt

关键词： Anti-epileptic drugs   epilepsy   headache   migraine   neurological disease   neuropathy   pre-pregnancy counselling   pregnancy   stroke  

摘要： Neurological disease encompasses a broad spectrum of conditions which may be affected by pregnancy, present de novo in pregnancy, or are caused by the pregnancy itself. In the UK, 9.8 women in 100,000 died during pregnancy or up to 42 days after delivery (Mothers and Babies Reducing Risk through Confidential Enquiry (MBRRACE) report 2018). Neurological diseases, including epilepsy and stroke, continues to be the second leading indirect cause of maternal mortality and the numbers have not changed since reporting began in 1985, despite the availability of easily accessible Green-top Guidelines on the management of epilepsy through the Royal College of Obstetricians and Gynaecologists (RCOG). It is important than any women of child-bearing age with a neurological condition receive appropriate pre-pregnancy counselling and that during pregnancy they are managed by an experienced multi-disciplinary team including a neurologist, specialist nurse or midwife, maternal medicine obstetrician or obstetric physician and obstetric anaesthetist. Additional benefits in care will come with improving awareness in the general public and community doctors so that appropriate support is provided to enable the safest possible pregnancy. © 2019 Elsevier Ltd

摘要： We present a case of a 54-year-old man with primary angiitis of the central nervous system (PACNS) who was initially admitted to a psychiatric clinic with a diagnosis of delirium. We discuss the difficulty in establishing the diagnosis of PACNS and provide the reader with some recommendations on how to promptly and correctly diagnose this disease in order to avoid potentially lethal outcomes.

关键词： traumatic brain injury   CD8+ T cells   adaptive immune cells   B cells   autoantibodies   neuroimmunology   granzyme B  

摘要： Traumatic brain injury (TBI) leaves many survivors with long-term disabilities. A prolonged immune response in the brain may cause neurodegeneration, resulting in chronic neurological disturbances. In this study, using a TBI mouse model, we correlate changes in the local immune response with neurodegeneration/neurological dysfunction over an 8-month period. Flow cytometric analysis reveals a protracted increase in effector/memory CD8(+) T cells (expressing granzyme B) in the injured brain. This precedes interleukin-17(+)CD4(+) T cell infiltration and is associated with progressive neurological/motor impairment, increased circulating brain-specific autoantibodies, and myelin-related pathology. Genetic deficiency or pharmacological depletion of CD8(+) T cells, but not depletion of CD4(+) T cells, improves neurological outcomes and produces a neuroprotective Th2/Th17 immunological shift, indicating a persistent detrimental role for cytotoxic T cells post-TBI. B cell deficiency results in severe neurological dysfunction and a heightened immune reaction. Targeting these adaptive immune cells offers a promising approach to improve recovery following TBI.

关键词： Gut microbiota   probiotics   prebiotics   Alzheimer's disease   autism   depression   anorexia nervosa  

摘要： Gut microbiota has significant effects on the structure and function of the enteric and central nervous system including human behaviour and brain regulation. Herein, we analyze the role of this intestinal ecosystem, the effects of dietary changes and the administration of nutritional supplements, such as probiotics, prebiotics, or fecal transplantation in neuropsychiatric disorders. Numerous factors have been highlighted to influence gut microbiota composition, including genetics, health status, mode of birth delivery and environment. However, diet composition and nutritional status has been repeatedly shown to be one of the most critical modifiable factors of this ecosystem. A comprehensively analysis of the microbiome-intestine-brain axis has been performed, including the impact of intestinal bacteria in alterations in the nervous, immune and endocrine systems and their metabolites. Finally, we discuss the latest literature examining the effects of diet composition, nutritional status and microbiota alterations in several neuropsychiatric disorders, such as autism, anxiety, depression, Alzheimer's disease and anorexia nervosa.

关键词： Sickle cell anemia   Stroke   Neurocognitive impairment   Transcranial doppler   Sub-Saharan Africa  

摘要： Background Children with sickle cell anemia (SCA) are highly susceptible to stroke and other manifestations of pediatric cerebral vasculopathy. Detailed evaluations in sub-Saharan Africa are limited. Methods We aimed to establish the frequency and types of pediatric brain injury in a cross-sectional study at a large SCA clinic in Kampala, Uganda in a randomly selected sample of 265 patients with HbSS ages 1-12 years. Brain injury was defined as one or more abnormality on standardized testing: neurocognitive impairment using an age-appropriate test battery, prior stroke by examination or transcranial Doppler (TCD) velocities associated with stroke risk in children with SCA (cerebral arterial time averaged mean maximum velocity >= 170 cm/second). Results Mean age was 5.5 +/- 2.9 years;52.3% were male. Mean hemoglobin was 7.3 +/- 1.01 g/dl;76.4% had hemoglobin < 8.0 g/dl. Using established international standards, 14.7% were malnourished, and was more common in children ages 5-12. Overall, 57 (21.5%) subjects had one to three abnormal primary testing. Neurocognitive dysfunction was found in 27, while prior stroke was detected in 15 (5.7%). The most frequent abnormality was elevated TCD velocity 43 (18.1%), of which five (2.1%) were in the highest velocity range of abnormal. Only impaired neurocognitive dysfunction increased with age (OR 1.44, 95%CI 1.23-1.68), p < 0.001). In univariate models, malnutrition defined as wasting (weight-for-height <= -2SD), but not sex or hemoglobin, was modestly related to elevated TCD (OR 1.37, 95%CI 1.01-1.86, p = 0.04). In adjusted models, neurocognitive dysfunction was strongly related to prior stroke (OR 6.88, 95%CI 1.95-24.3, p = .003) and to abnormal TCD (OR 4.37, 95%CI 1.30, p = 0.02). In a subset of 81 subjects who were enriched for other abnormal results, magnetic resonance imaging and angiography (MRI/MRA) detected infarcts and/or arterial stenosis in 52%. Thirteen subjects (25%) with abnormal imaging had no other abnormalities

关键词： Adaptation   Communication   Needs   Progressive neurology diseases   Quality of life  

摘要： Background Progressive neurological diseases, such as multiple sclerosis, Parkinson's disease, Huntington's disease, significantly interfere with patients' lives, and those of their families. The aim of the research was to establish whether the extent of the information on patients' health conditions, and the way patients learn this information from doctors affect their adaptation to chronic and progressive diseases. Methods Qualitative methodology was used for a total of 52 participants (patients with progressive neurological diseases, their family members, and health and social workers). Data were collected using individual, in-depth interviews and focus groups. Analysis of data for interpretation, conceptualization, and re-integration was performed by open, axial, and selective coding. Results It was determined that adequate information about patients' health status, and the use of coping strategies are related to their adaptation to their disease, and consequently, to their quality of life. The participants often considered the extent of the information provided, and the way they were informed to be inadequate. Receiving the diagnosis, the progression of the disease, and the end of life were found to be the most burdensome. Conclusion Our results show that Czech neurologist should develop better communication skills, particularly for informing patients with progressive neurological diseases. Open communication, emotional support, and support in selecting effective coping strategies can help patients adapt more readily to their disease, and improve their quality of life.