关键词： guideline   management   trigeminal neuralgia  

摘要： Background and purposeTrigeminal neuralgia (TN) is an extremely painful condition which can be difficult to diagnose and treat. In Europe, TN patients are managed by many different specialities. Therefore, there is a great need for comprehensive European guidelines for the management of TN. The European Academy of Neurology asked an expert panel to develop recommendations for a series of questions that are essential for daily clinical management of patients with TN. MethodsA systematic review of the literature was performed and recommendations was developed based on GRADE, where feasible;if not, a good practice statement was given. ResultsThe use of the most recent classification system is recommended, which diagnoses TN as primary TN, either classical or idiopathic depending on the degree of neurovascular contact, or as secondary TN caused by pathology other than neurovascular contact. Magnetic resonance imaging (MRI), using a combination of three high-resolution sequences, should be performed as part of the work-up in TN patients, because no clinical characteristics can exclude secondary TN. If MRI is not possible, trigeminal reflexes can be used. Neurovascular contact plays an important role in primary TN, but demonstration of a neurovascular contact should not be used to confirm the diagnosis of TN. Rather, it may help to decide if and when a patient should be referred for microvascular decompression. In acute exacerbations of pain, intravenous infusion of fosphenytoin or lidocaine can be used. For long-term treatment, carbamazepine or oxcarbazepine are recommended as drugs of first choice. Lamotrigine, gabapentin, botulinum toxin type A, pregabalin, baclofen and phenytoin may be used either alone or as add-on therapy. It is recommended that patients should be offered surgery if pain is not sufficiently controlled medically or if medical treatment is poorly tolerated. Microvascular decompression is recommended as first-line surgery in patients with classical TN.

关键词： alirocumab   asfotase alfa   evolocumab   hypolipidemic agent   hypophosphatasia   sarilumab  

摘要： Every year, lower-profile medical fields benefit from the arrival of new drugs on the market. A brand-new concept for the treatment of high cholesterol has been developed, while other recent specialities in allergology, rheumatology and neurology are the fruit of innovations first initiated a few years ago. (C) 2019 Elsevier Masson SAS. All rights reserved

关键词： Subarachnoid hemorrhage   Unruptured intracranial aneurysms   Common data elements   Clinical trials   Observational studies   Outcomes  

摘要： ObjectivesThe goal for this project was to develop a comprehensive set of common data elements (CDEs), data definitions, case report forms and guidelines for use in unruptured intracranial aneurysm (UIA) and subarachnoid hemorrhage (SAH) clinical research, as part of a new joint effort between the National Institute of Neurological Disorders and Stroke (NINDS) and the National Library of Medicine of the US National Institutes of Health. These UIA and SAH CDEs will join several other neurological disease-specific CDEs that have already been developed and are available for use by research *** Working Group (WG) divided into eight sub-groups and a Steering Committee comprised of international UIA and SAH experts reviewed existing NINDS CDEs and instruments, created new elements when needed and provided recommendations for UIA and SAH clinical research. The recommendations were compiled, internally reviewed by the entire UIA and SAH WG and posted online for 6weeks for external public comments. The UIA and SAH WG and the NINDS CDE team reviewed the final version before posting the SAH Version 1.0 CDE *** NINDS UIA and SAH CDEs and supporting documents are publicly available on the NINDS CDE (https://***/#page=Default) and NIH Repository (https://***/home) websites. The recommendations are organized into domains including Participant Characteristics and Outcomes and *** and widespread use of CDEs can facilitate UIA and SAH clinical research and clinical trial design, data sharing, and analyses of observational retrospective and prospective data. It is vital to maintain an international and multidisciplinary collaboration to ensure that these CDEs are implemented and updated when new information becomes available.

关键词： Vocational rehabilitation   soft systems methodology   long-term neurological conditions   service mapping   occupational therapy  

摘要： Introduction This study aimed to understand existing vocational rehabilitation service provision in one locality in London (population 3.74 million), identify any gaps and explore reasons for this, to support service development. Method Using soft systems methodology to guide the research process, semi-structured interviews were completed with nine participants, who were clinicians and managers providing vocational rehabilitation within a National Health Service context. Data were analysed thematically to build a 'rich picture' and develop a conceptual model of vocational rehabilitation service delivery. Findings were then ratified with participants at an engagement event. Results The findings indicate a spectrum of vocational rehabilitation service provision for long-term neurological conditions with differing levels of funding in place. Vocational rehabilitation often takes place 'under the radar' and therefore the true vocational rehabilitation needs of this population, and the extent of service provision, is not known. There is inconsistency of understanding across the services as to what constitutes vocational rehabilitation and outcomes are not routinely measured. Conclusion For vocational rehabilitation services to develop they require appropriate funding, driven by government policy to commissioners. Clear definitions of vocational rehabilitation, collecting and sharing outcome data and effective communication across services are needed at a local level. This is expressed in a conceptual model of vocational rehabilitation service delivery.

关键词： brain edema   cell volume regulation   ischemic stroke   KCCs   NKCC1   WNK-SPAK/OSR1  

摘要： In recent years, cation-chloride cotransporters (CCCs) have drawn attention in the medical neuroscience research. CCCs include the family of Na+-coupled Cl- importers (NCC, NKCC1, and NKCC2), K+-coupled Cl- exporters (KCCs), and possibly poly amine transporters (CCC9) and CCC interacting protein (CIP1). For decades, CCCs have been the targets of several commonly used diuretic drugs, including hydrochlorothiazide, furosemide, and bumetanide. Genetic mutations of NCC and NKCC2 cause congenital renal tubular disorders and lead to renal salt-losing hypotension, secondary hyperreninemia, and hypokalemic metabolic alkalosis. New studies reveal that CCCs along with their regulatory WNK (Kinase with no lysine (K)), and SPAK (Ste20-related proline-alanine-rich kinase)/ OSR1(oxidativ e stress-responsive kinase-1) are essential for regulating cell volume and maintaining ionic homeostasis in the nervous system, especially roles of the WNKSPAK-NKCC1 signaling pathway in ischemic brain injury and hypersecretion of cerebrospinal fluid in posthemorrhagic hydrocephalus. In addition, disruption of Cl- exporter KCC2 has an effect on synaptic inhibition, which may be involved in developing pain, epilepsy, and possibly some neuropsychiatric disorders. Interference with KCC3 leads to peripheral nervous system neuropathy as well as axon and nerve fiber swelling and psychosis. The WNK-SPAK/OSR1-CCCs complex emerges as therapeutic targets for multiple neurological diseases. This review will highlight these new findings.

关键词： Neurological impairment   POMES syndrome   Cervical disc herniation   Cervical spondylosis  

摘要： PurposePOEMS syndrome is a rare clonal plasma cell disease characterized by polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. We report a rare case of neurological impairment in patients with concurrent cervical disc herniation and POEMS syndrome. MethodsA patient presented to a local hospital with C3/4 and C4/5 disc herniation, apparent spinal cord compression concomitant with neurological signs, and concurrent POEMS syndrome. Anterior cervical discectomy and fusion was *** limb numbness was only slightly alleviated, and 10days postoperatively the patient complained of muscle weakness of the extremities and was referred to our hospital. The patients exhibited non-typical neurological signs and an enlarged liver and spleen that could not be explained. Electroneuromyography and immunofixation electrophoresis produced abnormal results. We diagnosed concurrent POEMS syndrome, for which drug therapy was prescribed. The patient's symptoms *** presenting with cervical spondylopathy and non-typical neurological signs and symptoms or other systemic problems should be evaluated for the presence of concurrent disease and ruled out differential diagnoses.

关键词： Epilepsy   Persons with epilepsy   Antiepileptic drugs   Depression   MINI scale   NDDI-E  

摘要： Purpose: The Neurological Disorders Depression Inventory for Epilepsy (NDDI-E) is an efficient tool for rapid detection of depression, an important comorbid condition in persons with epilepsy (PWE). Since social and cultural differences can potentially affect the cutoff score of NDDI-E, in this study, the reliability and validity of the Indian version of the NDDI-E in PWE was determined. Method: After ethical clearance, 217 PWE above 18 years of age, on antiepileptic drugs (AEDs), attending neurology outpatient department (OPD) of All India Institute of Medical Sciences (AIIMS), New Delhi, India, were evaluated for depression using the NDDI-E (Indian version) and Mini International Neuropsychiatric Interview (MINI-Module A, version 6.0.0) as reference standard. Informed consent was taken before recruitment. Receiver operating characteristic (ROC) analysis and Cronbach's alpha, a measure of the internal consistency and reliability, were carried out to validate cutoff and questionnaire, respectively. Results: Of the 217 PWE (112 males/105 females), mean age of 28.6 +/- 9.4 years, with generalized (69.1%) or focal seizures (30.9%), 41.5% and 10.6% were diagnosed with depression using MINI and NDDI-E Indian version (at cutoff > 15), respectively. However, at a cutoff score of >11, the Indian version of NDDI-E had a sensitivity of 96.67%, a specificity of 8425%, a positive predictive value of 81.31%, and a negative predictive value of 9727%. ROC analysis showed an area under the curve (AUC) of 0.9547 (confidence interval (CI) 95% = 0.929-0.979;standard error (SE): 0.0127). With the Indian version of NDDI-E, the Cronbach's a value was 0.877. Conclusion: A periodic assessment of PWE using a quickly administrable and reliable tool for screening depression is highly desirable given the high incidence. In the Indian population with a cutoff of >11, NDDI-E is a reliable and valid instrument to screen depression in PWE. (C) 2019 Elsevier Inc. All rights reserved.

关键词： Central nervous system demyelination   Immunomodulatory therapy   Peripheral nervous system demyelination   Southeast Asia   Therapeutic plasma exchange  

摘要： In conjunction with the third regional Southeast Asian (SEA) therapeutic plasma exchange (TPE) conference in Kuala Lumpur, Malaysia, 25 clinicians and researchers from SEA and South Asian countries attended the inaugural strategy meeting for the establishment of a regional TPE consortium for neurological disorders. The primary objective was to establish regional collaboration to improve delivery of TPE services in SEA. A pre-meeting survey was conducted to gather insights on disease spectrum, contextual practice challenges, and the need for a regional TPE consensus. Challenges identified include limited healthcare funding in support of diagnostic workup, TPE therapy, as well as development of clinical infrastructure and expertise capacity building. There was favorable interest in developing a working plan contextualized to this region. Strategies to overcome challenges were discussed. This included the need for a comprehensive referral system and network of regional TPE centers suited to local needs, supported by innovative TPE delivery programs.

关键词： Preeclampsia   Renal functions   Headache   Neuroimaging  

摘要： Background and purposeNeurological manifestations and neuroimaging abnormalities are common in patients with severe preeclampsia;however, the differences between these abnormal features occurring during early- and late-onset severe preeclampsia are unclear, and the factors associated with abnormal imaging changes in patients with neurological manifestations have not yet been fully *** and methodsA retrospective study was conducted on 172 patients with severe preeclampsia from January 2017 to June 2018 in the Department of Neurology and Obstetrics, Shengjing Hospital of China Medical University. The neurological manifestations, clinical parameters, laboratory, and neuroimaging findings were ***- and late-onset preeclampsia were diagnosed in 83 and 89 patients, respectively. Headache and dizziness were more common in patients with early-onset preeclampsia than in patients with late-onset preeclampsia (p=0.013, p=0.004, respectively). Serum uric acid, creatinine, and urea nitrogen were significantly elevated in the patients with early-onset preeclampsia (p<0.001, p=0.004, and p=0.005, respectively). Neuroimaging was performed in 81 patients, of which 57 were positive. Findings indicating cerebral edema were the most common neuroimaging abnormality. Gestational weeks (p=0.014), headache (p<0.001), and blood urea nitrogen level (p=0.027) may be associated with positive imaging findings. By multiple logistic regression, headache (OR=10.2, 95% CI, 2.4-42.7;p=0.002) proved to be an independent factor associated with neuroimaging *** symptoms such as headache and dizziness were more common in patients with early-onset preeclampsia. Renal dysfunction may also associate with early-onset severe preeclampsia. Cerebral edema was the most common neuroimaging abnormality, and headache might be independently associated with abnormal imagine changes.