关键词： Drop foot   Early decompression   Lumbar degenerative diseases   Neurological recovery   Manual muscle test  

摘要： The purpose of this study was to investigate the effectiveness of early (<72 h) versus late (>= 72 h) decompression surgery after the onset of drop foot caused by root disorder in lumbar degenerative diseases (LDDs). Data were included from 60 patients who underwent decompression surgery for drop foot caused by LDDs, including lumbar disk herniation or lumbar spinal stenosis. The primary outcome was ordinal change in the manual muscle test (MMT) at 2 years follow-up. Secondary outcomes included changes in the Japanese Orthopedic Association's (JOA) score. The early- and late-stage surgery groups included 20 and 40 patients with mean durations from the onset of drop foot to operation of 0.8 days (range, 0-3 days) and 117.1 days (range, 10-891 days), respectively. There was no significant difference (p = 0.33) between the early- and late-stage surgery groups in the improvement of MMT scores to >4 (90% versus 80%, respectively). However, more patients in the early-stage group achieved an MMT score >5 compared with those in the late-stage surgery group (80% versus 45%;p = 0.03). Furthermore, the recovery rate of JOA scores was significantly higher in the early-stage (89.1%) compared with the late-stage surgery group (68.6%;p < 0.001). Early decompression surgery produced better neurological recovery;however, an improvement of >4 in the MMT score was achieved in 80% of cases with late decompression. (C) 2020 Elsevier Ltd. All rights reserved.

关键词： Nitrous oxide   Laughing gas   Neuropathy   Vitamin B12   Neurological disorders  

摘要： Introduction In recent years, there has been a sharp increase in the number of patients with neurological disorders associated with recreational use of nitrous oxide (N2O) in China. Here, we summarize the clinical characteristics of patients with neurological disorders associated with N2O abuse diagnosed in our Hospital. Further, we conducted a literature search on recent cases reported in mainland China to improve the awareness of the outbreak of neurological disorders associated with N2O abuse. Methods We retrospectively collected data of patients diagnosed with neurological disorders associated with recreational use of N2O in Shengjing Hospital of China Medical University from January 2018 to June 2019, and performed a literature search using the "nitrous oxide" and "neurological disorder" as keywords in the Chinese literature databases of WANFANG and CNKI and the English literature databases of Pubmed and Web of Science Results We enrolled 43 patients (average age: 21.9 +/- 3.3 years). The main clinical manifestations were weakness and paresthesia in the four extremities and unsteady gait. Further, most patients showed significantly lower levels of serum vitamin B12 (169.4 +/- 79.1 pg/mL) and increased homocysteine levels (78.1 +/- 32.2 mu mol/L). MRI of the spinal cord showed longitudinal high T2 signal lesions in the dorsal spinal cord in some patients. Moreover, electromyography showed sensory and motor nerve axonal damage combined with demyelination, which was relatively more severe in the lower limbs. There was rapid improvement of the symptoms after treatment with intramuscular injections of vitamin B12 and the overall prognosis was good. The literature search indicated that the number of published papers and related patients showed a rapid annual increase since the first Chinese case reported in 2016 Conclusion Recreational use of N2O is an emerging public health problem in China that needs prompt action from the society and government. Early diagnosis an

关键词： Adult phenylketonuria   Neurological symptoms   Brain MRI   Leucopathy  

摘要： Objective Phenylketonuria (PKU) is a rare autosomal recessive disease characterised by high plasma phenylalanine levels inducing, if untreated, serious neurological manifestations in children but also, rarely, in adults who stopped their diet. The objective of the study was to describe the neurological manifestations observed in adults with PKU. Methods We analysed cases reported in French reference centres for inborn errors of metabolism and cases already reported in the literature. Results We report 8 new cases of neurological manifestations and 22 cases in the literature, which occurred in adult PKU patients, associated with chronic or rapid increase of phenylalanine levels, mostly when strict low-phenylalanine diet was stopped early in life. Neurological symptoms consisted in cerebellar ataxia, tremor, brisk reflexes, visual loss, sensory manifestations, and/or headaches. Visual loss was more frequent in the new cases (4/8) of the present series than in the literature (4/22). These neurological complications were associated with leucopathy on brain magnetic resonance imaging (27/29). The start of a low-phenylalanine diet improved or fully reversed neurological manifestations, even in patients with late diagnosis during adulthood. Conclusion Neurological manifestations can complicate PKU in adult patients with elevated phenylalanine levels, after long or short period of diet discontinuation. Neurologists should be aware of this diagnosis, and measure phenylalaninemia in case of neurological symptoms associated with non-specific leucopathy on brain MRI. PKU patients should be systematically encouraged to continue their diet and their medical follow-up to avoid neurological complications.

关键词： bacterial meningitis   dexamethasone   long-term sequelae   neurological impairment   risk factors  

摘要： Aim To examine the incidence, clinical presentation and risk factors for neurological sequelae following childhood community-acquired bacterial meningitis (CABM). Methods We included all children aged 1 month to 15 years old with CABM in North Denmark Region, 1998-2016. Using medical records, we registered baseline demographics, signs and symptoms at admission, laboratory investigations, and outcome assessed by the Glasgow Outcome Scale (GOS). A GOS score of 1-4 was considered an unfavourable outcome. We used modified Poisson regression to examine predefined risk factors for neurological sequelae among survivors. Results We identified 88 cases of CABM in 86 patients (45 female) with a median age of 1.4 years (interquartile range 0.7-4.6). Neisseria meningitidis was the most common pathogen (48/88). Neurological sequelae occurred in 23 (27%) as hearing deficits in 13 (15%), cognitive impairment in 10 (12%) and motor or sensory nerve deficits in 8 (9%). Unfavourable outcome was observed in 16 (18%) patients and three (3%) patients died. Abnormalities on cranial imaging remained the only independent risk factor for developing neurological sequelae in adjusted analysis. Conclusion Neurological sequelae following CABM in children remain frequent and abnormal cranial imaging may be an independent risk factor.

关键词： Randomized controlled trials   Trauma  

摘要： An amendment to this paper has been published and can be accessed via a link at the top of the paper.

关键词： Sandifer syndrome   Gastro-oesophageal reflux disease   Misdiagnosis   Intractable neurological symptoms  

摘要： Sandifer syndrome is a rare complication of gastro-oesophageal reflux disease (GERD) when a patient presents with extraoesophageal symptoms, typically neurological. The aim of this study was to review the existing literature and describe a typical presentation and most appropriate investigations and management for the Sandifer syndrome. A comprehensive literature search was performed via PubMed, Cochrane Library and NHS Evidence databases. Twenty-seven cases and observational studies were identified. The literature demonstrates that presenting symptoms of Sandifer's may include any combination of abnormal movements and/or positioning of head, neck, trunk and upper limbs, seizure-like episodes, ocular symptoms, irritability, developmental and growth delay and iron-deficiency anaemia. A 24-h oesophageal pH monitoring was positive in all the cases of Sandifer's where it was performed, while upper GI endoscopy +/- biopsy and barium swallow were diagnostic only in a subset of cases. Successful treatment of the underlying gastro-oesophageal pathology led to a complete or near-complete resolution of the neurological symptoms in all of the cases. Conclusion: It is evident from the literature that many patients with Sandifer syndrome were originally misdiagnosed with various neuropsychiatric diagnoses that led to unnecessary testing and ineffective medications with significant side effects. Earlier diagnosis of Sandifer's would have allowed to avoid *** is Known:center dot Sandifer syndrome is a rare complication of gastro-oesophageal reflux disease (GERD) when a patient presents with extraoesophageal symptoms, typically *** dot It may be difficult to recognise due to its non-specific presentation and lack of gastrointestinal *** is New:center dot Based on the review of 44 clinical cases of suspected Sandifer syndrome, the clinical picture was clarified: the presenting symptoms of Sandifer's may include any combination of abnormal movements an

关键词： Neurodegeneration   Mitochondria   Mitophagy   Calcium   Reactive oxygen species  

摘要： The brain is highly dependent on mitochondrial energy metabolism. As a result, mitochondrial dysfunction is a central aspect of many adult-onset neurological diseases, including stroke, ALS, Alzheimer's, Huntington's, and Parkinson's diseases. We review here how different mitochondrial functions, including oxidative phosphorylation, mitochondrial dynamics, oxidant generation, cell death regulation, Ca2+ homeostasis, and proteostasis are involved in these disorders.

关键词： Platelets   platelet biogenesis   platelet activation   neurological disorders  

摘要： Platelets are anucleated blood constituents, vital for hemostasis and involved in the pathophysiology of several cardiovascular, neurovascular diseases as well as inflammatory processes and metastasis. Over the past few years, the molecular processes that regulate the function of platelets in hemostasis and thrombosis have emerged revealing platelets to be perhaps more complex than may have been expected. The most understood part of platelets is to respond to a blood vessel injury by altering shape, secreting granule contents, and aggregating. These responses, while advantageous for hemostasis, can become detrimental when they root ischemia or infarction. Only a few transcription and signaling factors involved in platelet biogenesis have been identified till date. Platelets encompass an astonishingly complete array of organelles and storage granules including mitochondria, lysosomes, alpha granules, dense granules, a dense tubular system (analogous to the endoplasmic reticulum of nucleated cells);a highly invaginated plasma membrane system known as the open canalicular system (OCS) and large fields of glycogen. Platelets as a model cells to study neurological disorders have been recommended by several researchers since several counterparts exist between platelets and the brain, which make them interesting for studying the neurobiology of various neurological disorders. This review has been compiled with an aim to integrate the latest research on platelet biogenesis, activation and aggregation focusing on the molecular pathways that power and regulate these processes. The dysregulation of important molecular players affecting fluctuating platelet biology and thereby resulting in neurovascular diseases has also been discussed.

关键词： adverse reactions   metabolic disturbances   psychotropic medication   second-generation antipsychotics   weight gain  

摘要： Aim This study examined the use and adverse reactions of second-generation antipsychotics (SGAs), alone or combined with other psychotropic medication, to identify areas for standardising prescribing and monitoring practices. Methods We conducted a retrospective study at Tampere University Hospital, Finland, involving 128 patients (81% boys) who were under 13 years old at SGA initiation and had SGA treatment between October 2013 and October 2014. Results The median age at baseline was 9.4 years. Weight gain was reported as an adverse reaction in 33%, but an increase in standardised body mass index, adjusted for age and sex (BMI z-score), was detected in 75% of patients with sufficient data. The statistically significant median changes during the study were an increase of 0.46 in BMI z-score, a reduction of 0.25 mmol/L in fasting plasma high-density lipoprotein and an increase of 0.28 mmol/L in triglyceride values. The weight gain was most apparent in patients treated with just an SGA or SGA plus melatonin. Patients treated with an SGA plus medication for attention deficit hyperactivity disorder were less likely to gain weight. Conclusion SGA-induced metabolic disturbances remained partly unrecognised in children under 13 years of age and more systematic monitoring is needed.