关键词： Epilepsy   Migraine   Multiple sclerosis   Paraneoplastic disorders   Stroke   Systemic lupus erythematosis   Women  

摘要： Concern about what is best practice when caring for women with neurologic disease is a common clinical scenario. Therefore, knowledge about women's health issues and their intersection with neurologic disorders is imperative. This review will discuss the appropriate gender-based considerations in epilepsy, multiple sclerosis, migraine, autoimmune disease, sleep disorders, stroke, and paraneoplastic disorders. (C) 2018 Elsevier Inc. All rights reserved.

关键词： Neurological Impairments   Speech Impairments   Physical Disabilities   Speech Language Pathology   Educational Games   Verbal Communication   Speech Skills   Educational Technology   Technology Uses in Education   Comparative Analysis   Computer Games   Program Effectiveness   Satisfaction   Preferences   Pronunciation   Comprehension   Older Adults  

摘要： The majority of patients with neurological impairment like Parkinson's Disease (PD) or stroke are affected by dysarthria. Dysarthria is a motor speech impairment which negatively affects speech dimensions such as articulation and loudness. This leads to reduced intelligibility, often hindering daily life communication. Intensive and prolonged speech training can increase patients' speech intelligibility. Unfortunately, interventions by speech therapists are generally provided only for a short period of time, while continuing practice is needed to maintain or improve intelligibility. eHealth applications might provide a solution. In our research, we explored whether it is possible to develop a game that is suitable for providing speech training in elderly patients with dysarthria due to PD or stroke. In the game, we developed, called Treasure Hunters, two players interact verbally to find the way to the treasure, while receiving automatic feedback on voice loudness and pitch. Participants played with our game in several sessions and generally appreciated it, hinting at our game's potential for speech training in elderly patients. In a within-subjects experiment with five dysarthric patients, our game was compared to a non-game computer-based speech training system: e-learning-based Speech Therapy (EST). We focussed on three variables: speech intelligibility, user satisfaction and user preference. Substantial variability between participants was observed, in the outcomes of these three variables and their relations. We conclude that one size that fits all does not apply to computer-based speech training, but a personalised approach is needed.

关键词： oxygen   Pilot Studies   Newton meter   Mechanical Ventilation   Nervous System Trauma   ROS1 gene   pulmonary complications   Oxidative Stress   blood drug concentration   Incidence   Hyperoxia  

摘要： BackgroundNormobaric hyperoxia is used to alleviate secondary brain ischaemia in patients with traumatic brain injury (TBI), but clinical evidence is limited and hyperoxia may cause adverse events. MethodsAn open label, randomised controlled pilot study comparing blood concentrations of reactive oxygen species (ROS), interleukin 6 (IL-6) and neuron-specific enolase (NSE) between two different fractions of inspired oxygen in severe TBI patients on mechanical ventilation. ResultsWe enrolled 27 patients in the Fi O-2 0.40 group and 38 in the Fi O-2 0.70 group;19 and 23 patients, respectively, completed biochemical analyses. In baseline, there were no differences between Fi O-2 0.40 and Fi O-2 0.70 groups, respectively, in ROS (64.8 nM [22.6-102.1] vs. 64.9 nM [26.8-96.3], P = 0.80), IL-6 (group 92.4 pg/ml [52.9-171.6] vs. 94.3 pg/ml [54.8-133.1], P = 0.52) or NSE (21.04 ug/l [14.0-30.7] vs. 17.8 ug/l [14.1-23.9], P = 0.35). ROS levels did not differ at Day 1 (24.2 nM [20.6-33.5] vs. 29.2 nM [22.7-69.2], P = 0.10) or at Day 2 (25.4 nM [21.7-37.4] vs. 47.3 nM [34.4-126.1], P = 0.95). IL-6 concentrations did not differ at Day 1 (112.7 pg/ml [65.9-168.9) vs. 83.9 pg/ml [51.8-144.3], P = 0.41) or at Day 3 (55.0 pg/ml [34.2-115.6] vs. 49.3 pg/ml [34.4-126.1], P = 0.95). NSE levels did not differ at Day 1 (15.9 ug/l [9.0-24.3] vs. 15.3 ug/l [12.2-26.3], P = 0.62). There were no differences between groups in the incidence of pulmonary complications. ConclusionHigher fraction of inspired oxygen did not increase blood concentrations of markers of oxidative stress, inflammation or neurological injury or the incidence of pulmonary complications in severe TBI patients on mechanical ventilation.

关键词： Behcet's disease   Neurologic and ocular involvement   Glucocorticoids   Infliximab  

摘要： Beh double dagger et's disease is a chronic inflammatory condition of unknown origin characterized by multiple organ involvement. The most common symptoms of Beh double dagger et's disease are recurrent oral and/or genital ulcerations in combination with symptoms affecting eyes, skin, central and peripheral nervous system, blood vessels and gastrointestinal tract. We present a 43-year-old female patient with the history of recurrent episodes of genital and oral ulcerations, elevated acute phase reactants and skin lesions. The diagnosis of Beh double dagger et's disease has been delayed (for more than 10 years) and reached only after she developed neurological and ocular symptoms. Treatment with glucocorticoids and azathioprine was partially successful. High doses of glucocorticoids were needed to control the disease and cyclosporine A was nephrotoxic. Remission was reached after the introduction of infliximab (plus methotrexate) and glucocorticoids were stopped. In the recent years, infliximab has been accepted as a standard therapy for refractory cases of Beh double dagger et's disease (neurological, ocular or gastrointestinal). Our patient presented with refractory ocular and neurological symptoms and infliximab was effective for both manifestations. Long-term side-effects of glucocorticoids and other immunosuppressants can be avoided with TNF-alpha blockade. We emphasize the importance of a timely and accurate diagnosis and significance of excluding more common diseases in a work-up algorithm.

关键词： Chloride   Chloride regulation   GABA   GABAergic inhibition   GABA polarity   Chloride cotransporters   KCC2   NKCC1   Developmental disorders   Epilepsy   Stroke   Autism   E/I balance  

摘要： Neuronal chloride regulation is a determinant factor for the dynamic tuning of GABAergic inhibition during and beyond brain development. This regulation is mainly dependent on the two co-transporters K+/Cl- co-transporter KCC2 and Na+/K+/Cl- co-transporter NKCC1, whose activity can decrease or increase neuronal chloride concentrations respectively. Altered expression and/or activity of either of these co-transporters has been associated with a wide variety of brain disorders including developmental disorders, epilepsy, schizophrenia and stroke. Here, we review current knowledge on chloride transporter expression and activity regulation and highlight the intriguing potential for existing and future interventions to support chloride homeostasis across a wide range of mental disorders and neurological conditions.

关键词： Branched-chain amino acids   Branched-chain alpha-keto acid dehydrogenase   Protein metabolism   Glucose metabolism   Energy metabolism   Neurological development  

摘要： Branched-chain amino acids (BCAAs: leucine, isoleucine, and valine) are essential amino acids for humans and play an important role as the building blocks of proteins. Recent studies have disclosed that free BCAAs in the tissue amino acid pool function not only as substrates for protein synthesis, but also as regulators of protein and energy metabolism. Furthermore, BCAAs are actively used as an amino group donor to synthesize glutamate in the brain. These functions of BCAAs are closely related to human health. This review summarizes the recent findings concerning physiological and pathological roles of free BCAAs in the metabolism and neurological functions.

关键词： Schisandra chinensis Fructus   active ingredients   neurological diseases   molecular mechanism  

摘要： Neurological diseases (NDs) are a leading cause of death worldwide and tend to mainly affect people under the age of 50. High rates of premature death and disability caused by NDs undoubtedly constrain societal development. However, effective therapeutic drugs and methods are very limited. Schisandra chinensis Fructus (SCF) is the dry ripe fruit of Schisandra chinensis (Turcz.) Baill, which has been used in traditional Chinese medicine for thousands of years. Recent research has indicated that SCF and its active ingredients show a protective role in NDs, including cerebrovascular diseases, neurodegenerative diseases, or depression. The key neuroprotective mechanisms of SCF and its active ingredients have been demonstrated to include antioxidation, suppression of apoptosis, anti-inflammation, regulation of neurotransmitters, and modulation of brain-derived neurotrophic factor (BDNF) related pathways. This paper summarizes studies of the role of SCF and its active ingredients in protecting against NDs, and highlights them as promising resources for future treatment. Furthermore, novel insights on the future challenges of SCF and its active ingredients are offered.

关键词： Cerebrotendinous xanthomatosis   Neurodegenerative disorder   Genetic disorder   Bile acid synthesis disorder   Ataxia   Spasticity   Xanthomas   Cataracts  

摘要： Cerebrotendinous xanthomatosis (CTX) is a rare inherited neurodegenerative disorder in bile acid synthesis. The natural history of neurological abnormalities in CTX is not well understood. The object of this study was to determine neurological progression in CTX. A literature search on PubMed for "cerebrotendinous xanthomatosis" yielded 91 publications that reported cases of CTX patients. Two independent reviewers abstracted information about the presence and age of onset of neurological abnormalities in published CTX cases. For each neurological abnormality, we estimated the probability of its onset at any given age using cumulative incidence function analysis. We also present our own case series, in which five CTX patients were evaluated. The literature search yielded 194 CTX cases (ages ranging from newborn to 67 years old). The most common neurological abnormalities were corticospinal tract abnormalities including weakness, hyperreflexia, spasticity, Babinski sign (59.8%), ataxia (58.8%), cognitive decline (46.4%), and gait difficulty (38.1%);68 (35.0%) had baseline cognitive problems. Cumulative incidence function analysis revealed that ataxia, gait difficulties, and corticospinal tract abnormalities developed throughout life, while cognitive decline tended to develop later in life. Of the less common neurological abnormalities, seizures, psychiatric changes and speech changes developed throughout life, while parkinsonism and sensory changes tended to develop later in life. Our case series corroborated this temporal pattern of neurological abnormalities. We provide estimates for the neurological progression of CTX, categorizing neurological abnormalities according to time and probability of development. Our approach may be applicable to other rare disorders.