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https://www.readbyqxmd.com/read/29045954/-pulmonary-arterial-hypertension-as-leading-manifestation-of-methylmalonic-aciduria-clinical-characteristics-and-gene-testing-in-15-cases
#1
X Q Liu, H Yan, J X Qiu, C Y Zhang, J G Qi, X Zhang, H J Xiao, Y L Yang, Y H Chen, J B DU
OBJECTIVE: To deepen our understanding of Methylmalonic aciduria (MMA) associated pulmonary hypertension (PH) by analyzing the characteristics of clinical presentation, pulmonary high resolusion CT(HRCT), treatment response and gene mutation. METHODS: This study includes 15 cases of pediatric patients with MMA associated PH diagnosed and treated in Peking University First Hospital pediatric department between May 2012 and May 2016 with symptoms of PH as their leading presentation...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29045812/facial-emotion-perception-in-patients-with-epilepsy-a-systematic-review-with-meta-analysis
#2
REVIEW
Michelle Edwards, Elizabeth Stewart, Romina Palermo, Suncica Lah
Facial emotion perception is a fundamental social competency relying on a specialised, yet distributed, neural network. This review aimed to determine whether patients with epilepsy have facial emotion perception accuracy impairments overall, or for a subset of emotions (anger, disgust, happiness, sadness, fear, and surprise), and the relationship to epilepsy type, demographic/treatment variables, and brain organisation. Database searches used PRISMA guidelines with strict inclusion/exclusion criteria. Thirty included studies assessed patients with temporal lobe (TLE; n=709), frontocentral (FCE; n=22), and genetic generalised (GGE; n=48) epilepsy...
October 15, 2017: Neuroscience and Biobehavioral Reviews
https://www.readbyqxmd.com/read/29045710/radiation-induced-cognitive-toxicity-pathophysiology-and-interventions-to-reduce-toxicity-in-adults
#3
Christopher Wilke, David Grosshans, Joseph Duman, Paul Brown, Jing Li
Radiotherapy is ubiquitous in the treatment of patients with both primary brain tumors as well as disease which is metastatic to the brain. This therapy is not without cost, however, as cognitive decline is frequently associated with cranial radiation, particularly with whole brain radiotherapy (WBRT). The precise mechanisms responsible for radiation-induced morbidity remain incompletely understood and continue to be an active area of ongoing research. In this article, we review the hypothetical means by which cranial radiation induces cognitive decline as well as potential therapeutic approaches to prevent, minimize or reverse treatment-induced cognitive deterioration...
October 14, 2017: Neuro-oncology
https://www.readbyqxmd.com/read/29045549/emerging-treatment-paradigms-in-brain-metastasis-in-non-small-cell-lung-cancer-an-overview-of-the-current-landscape-and-challenges-ahead
#4
D Ulahannan, J Khalifa, C Faivre-Finn, S M Lee
Advances in the last decade in genomic profiling and the identification of druggable targets amenable to biological agents, has transformed the management and survival of a subgroup of patients with brain metastasis in non-small cell lung cancer. In parallel, clinicians have re-evaluated the role of whole brain radiotherapy in selected patients with brain metastases to reduce neurocognitive toxicity. Continual progress in this understudied field is required: optimisation of the sequence of schedules for therapies in patients with brain metastases of differing genomic profiles, focusing on new strategies to overcome mechanisms of biological resistance and increasing drug penetrability into the central nervous system...
October 17, 2017: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/29045524/combined-irradiation-and-targeted-therapy-or-immune-checkpoint-blockade-in-brain-metastases-toxicities-and-efficacy
#5
A V Tallet, F Dhermain, E Le Rhun, G Noël, Y M Kirova
Background: Targeted therapies (TT) and immune checkpoint inhibitors (ICI) are currently modifying the landscape of metastatic cancer management and are increasingly used over the course of many cancers treatment. They allow long-term survival with controlled extra-cerebral disease, contributing to the increasing incidence of brain metastases (BMs). Radiation therapy remains the cornerstone of BMs treatment (either whole brain irradiation or stereotactic radiosurgery), and investigating the safety profile of radiation therapy combined with TT or ICI is of high interest...
August 2, 2017: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/29045030/drug-delivery-to-the-brain-across-the-blood-brain-barrier-using-nanomaterials
#6
REVIEW
Yung-Hao Tsou, Xue-Qing Zhang, He Zhu, Sahla Syed, Xiaoyang Xu
A major obstacle facing brain diseases such as Alzheimer's disease, multiple sclerosis, brain tumors, and strokes is the blood-brain barrier (BBB). The BBB prevents the passage of certain molecules and pathogens from the circulatory system into the brain. Therefore, it is nearly impossible for therapeutic drugs to target the diseased cells without the assistance of carriers. Nanotechnology is an area of growing public interest; nanocarriers, such as polymer-based, lipid-based, and inorganic-based nanoparticles can be engineered in different sizes, shapes, and surface charges, and they can be modified with functional groups to enhance their penetration and targeting capabilities...
October 16, 2017: Small
https://www.readbyqxmd.com/read/29044823/the-many-characters-of-visual-alpha-oscillations
#7
Michael S Clayton, Nick Yeung, Roi Cohen Kadosh
A central feature of human brain activity is the alpha rhythm: a 7 - 13 Hz oscillation observed most notably over occipitoparietal brain regions during periods of eyes-closed rest. Alpha oscillations covary with changes in visual processing, and have been associated with a broad range of neurocognitive functions. In this article, we review these associations and suggest that alpha oscillations can be thought to exhibit at least five distinct 'characters': those of the inhibitor, perceiver, predictor, communicator, and stabiliser...
October 17, 2017: European Journal of Neuroscience
https://www.readbyqxmd.com/read/29044802/differential-and-complementary-roles-of-medial-and-lateral-septum-in-the-orchestration-of-limbic-oscillations-and-signal-integration
#8
Marian Tsanov
Anatomical differences between the medial and lateral septum have associated these nuclei with dissimilar functional roles and behaviors. While the medial septum has been implicated, predominantly, in theta rhythm generation along the septo-hippocampal axis, the lateral septum has mainly been investigated in the context of septo-hypothalamic dialogue. Recent advances suggest that medial and lateral septum are more closely functionally-related than previously appreciated. Here, we explore the hypothesis that the medial septum mediates ascending septo-hippocampal theta propagation, while the lateral septum processes a descending hippocampo-septal and septo-hypothalamic reinforcement signal that mediates navigation during motivated behavior...
October 17, 2017: European Journal of Neuroscience
https://www.readbyqxmd.com/read/29044772/rd-lawrence-lecture-2017-incretins-the-intelligent-hormones-in-diabetes
#9
V A Gault
The incretin hormones glucose-dependent insulinotropic polypeptide (GIP) and glucagon-like peptide-1 (GLP-1) have attracted considerable scientific and clinical interest due largely to their insulin-releasing and glucose-lowering properties. Indeed, GLP-1-based therapies are now key treatment options for many people with diabetes worldwide. In contrast, GIP-based agents have yet to reach the clinic based primarily on the impaired insulinotropic action of GIP observed in people with diabetes. Nevertheless, GIP is a key physiological regulator of insulin secretion and stable forms of GIP show much promise in rodent models to alleviate diabetes-obesity...
October 16, 2017: Diabetic Medicine: a Journal of the British Diabetic Association
https://www.readbyqxmd.com/read/29044654/animal-models-of-neuroinflammation-secondary-to-acute-insults-originated-outside-the-brain
#10
REVIEW
Mike Yoshio Hamasaki, Marcel Cerqueira César Machado, Fabiano Pinheiro da Silva
The term "neuroinflammation" has been widely used to describe a series of acute or chronic conditions that cause inflammation in the central nervous system (CNS). Neurological damage can be a consequence of direct local injury or, secondary, of systemic or even distant inflammatory processes. In this respect, animal models have been developed to better understand the pathophysiology and, possibly, to evaluate more effective methods of treatment for these disorders. Animal models that promote alterations in blood-brain barrier permeability-the activation of microglia or astrocytes, modifications in neuropeptide expression, oxidative stress, increased apoptosis, release of inflammatory mediators, leukocyte infiltration, and brain edema-are likely to involve neuroinflammation and therefore can serve as useful models for human inflammatory CNS injury...
October 16, 2017: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/29044552/dynamics-of-cognitive-control-theoretical-bases-paradigms-and-a-view-for-the-future
#11
REVIEW
Gabriele Gratton, Patrick Cooper, Monica Fabiani, Cameron S Carter, Frini Karayanidis
Cognitive control (with the closely related concepts of attention control and executive function) encompasses the collection of processes that are involved in generating and maintaining appropriate task goals and suppressing task goals that are no longer relevant, as well as the way in which current goal representations are used to modify attentional biases to improve task performance. Here, we provide a comprehensive but nonexhaustive review of this complex literature, with an emphasis on the contributions made by techniques for studying human brain function...
October 17, 2017: Psychophysiology
https://www.readbyqxmd.com/read/29044349/high-risk-of-brain-tumors-in-farmers-a-mini-review-of-the-literature-and-report-of-the-results-of-a-case-control-study
#12
P Fallahi, R Foddis, A Cristaudo, A Antonelli
Even though a relationship between farmer work and the development of brain tumors was suggest by the scientific literature, a small number of italian studies investigate on the impact of this job on the cancer morbidity of farmer population. The informations about this case-control study were obtained from patients recruited in the Neurosurgical Department of the University-Hospital of Pisa, Italy, from 1990 to 2000. One hundred and seventy-four, newly diagnosed cases of brain tumors (glioma and meningiomas, histologically confirmed), were recruited, such as 522 controls (with other non tumoral neurologic diseases: trauma, etc), by matching cases and controls (1:3), for age (± 5 years) and gender...
September 2017: La Clinica Terapeutica
https://www.readbyqxmd.com/read/29044015/-argentina-enacts-first-law-on-medical-use-of-the-cannabis-plant-history-and-perspectives
#13
Eduardo L De Vito
In the last few years, great expectations have risen in the society concerning the eventual therapeutic usefulness of compounds derived from the cannabis plant. It is well known that these compounds are being used in treating certain health conditions, either through medical prescription or self-administration. Extreme opinions range from believing that it is a harmful and dangerous drug to sustaining that it is a panacea. However, the sheer existence of an endocannabinoid system in the brain compels us to study its dimensions and derivations thoroughly...
2017: Medicina
https://www.readbyqxmd.com/read/29043964/accuracy-of-diagnosis-criteria-in-patients-with-suspected-diagnosis-of-sporadic-creutzfeldt-jakob-disease-and-detection-of-14-3-3-protein-france-1992-to-2009
#14
Laurene Peckeu, Nicole Delasnerie-Lauprètre, Jean-Philippe Brandel, Dominique Salomon, Véronique Sazdovitch, Jean-Louis Laplanche, Charles Duyckaerts, Danielle Seilhean, Stéphane Haïk, Jean-Jacques Hauw
Diagnostic criteria of Creutzfeldt-Jakob disease (CJD), a rare and fatal transmissible nervous system disease with public health implications, are determined by clinical data, electroencephalogram (EEG), detection of 14-3-3 protein in cerebrospinal fluid (CSF), brain magnetic resonance imaging and prion protein gene examination. The specificity of protein 14-3-3 has been questioned. We reviewed data from 1,572 autopsied patients collected over an 18-year period (1992-2009) and assessed whether and how 14-3-3 detection impacted the diagnosis of sporadic CJD in France, and whether this led to the misdiagnosis of treatable disorders...
October 2017: Euro Surveillance: Bulletin Européen sur les Maladies Transmissibles, European Communicable Disease Bulletin
https://www.readbyqxmd.com/read/29043491/the-multifactorial-origin-of-posterior-reversible-encephalopathy-syndrome-in-cyclophosphamide-treated-lupus-patients
#15
REVIEW
Tatjana Zekić, Mirjana Stanić Benić, Ronald Antulov, Igor Antončić, Srđan Novak
The cyclophosphamide as a predisposing factor for Posterior Reversible Encephalopathy Syndrome (PRES) and therapeutic option for systemic lupus erythematosus (SLE) is still confusing. The first and only case of PRES, probably induced by cyclophosphamide, in Croatia followed by the findings of 36 SLE patients diagnosed with PRES after treatment with cyclophosphamide worldwide are described. An 18-year-old Caucasian female patient with a 1-year history of SLE was admitted to the hospital due to lupus nephritis and acute arthritis...
October 17, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29043230/advances-in-the-treatment-of-newly-diagnosed-primary-central-nervous-system-lymphomas
#16
REVIEW
Liren Qian, Ciprian Tomuleasa, Ioan-Alexandru Florian, Jianliang Shen, Ioan-Stefan Florian, Mihnea Zdrenghea, Delia Dima
Primary central nervous system lymphoma (PCNSL) is a type of highly invasive non-Hodgkin lymphoma. With a growing number of organ transplantation and immunosuppressant therapy, the incidence of PCNSL has been growing rapidly in recent years, which is attributed to the increased incidence of HIV/AIDS, a prominent risk factor for developing PCNSL. The rising rate of PCNSL incidence is the highest among the intracranial tumors. In the past 20 years, dozens of clinical trials related to PCNSL have been registered, but adequate therapeutics are still challenging...
September 2017: Blood Research
https://www.readbyqxmd.com/read/29043205/an-extremely-rare-case-of-concurrent-braf-v600e-mutation-driven-hairy-cell-leukemia-and-melanoma-case-report-and-review-of-literature
#17
Amir Ghorbani-Aghbolaghi, Mirna Lechpammer, Saba F Ali, Nam K Ku, Denis M Dwyre, Hooman H Rashidi
BRAF protein is a serine/threonine kinase with 766 amino acids. Approximately 15% of human cancers harbor BRAF mutations as well as other BRAF anomalies (amplifications, fusions). Somatic mutations mainly occur in the catalytic kinase domain (CR3), and the predominant mutation is p.V600E which is the substitution of glutamic acid (E) for valine (V) as result of a mutation at codon 600 of the kinase domain. To our knowledge, the vast majority of the cancers have non-germline BRAF mutations. Here we describe a case of a 60-year-old female with a history of hairy cell leukemia (HCL) who presented with aphasia and forgetfulness...
July 2017: Autopsy & case reports
https://www.readbyqxmd.com/read/29042939/aphasic-status-epilepticus-as-the-sole-symptom-of-epilepsy-a-case-report-and-literature-review
#18
Ji-Qing Qiu, Yu Cui, Li-Chao Sun, Zhan-Peng Zhu
Aphasia is a common symptom encountered by neurologists. However, the presence of aphasia as the sole manifestation of partial status epilepticus is rare. The present study reports a case of aphasic status epilepticus in a 27-year-old right-handed female who presented after the abrupt onset of aphasia, which had persisted for 1.5 days. The patient's medical history included head trauma followed by a craniectomy and cranioplasty. Computed tomography scans revealed a lesion in the patient's left parietal lobe, and an electroencephalogram showed a spike and slow wave pattern in the left hemisphere of the brain during aphasia...
October 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29042902/unusual-case-of-posterior-reversible-encephalopathy-syndrome-in-a-patient-with-anti-glomerular-basement-membrane-antibody-glomerulonephritis-a-case-report-and-review-of-the-literature
#19
Boram Cha, Dae Young Kim, Hyunil Jang, Seun Deuk Hwang, Huck Jei Choi, Moon-Jae Kim
Posterior reversible encephalopathy syndrome (PRES) is characterized by a clinical and radiological entity with the sudden onset of seizures, headache, altered consciousness, and visual disturbances in patients with the findings of reversible vasogenic subcortical edema without infarction. Hypertension, renal disease, and autoimmune disease are co-morbid conditions of PRES. Nevertheless, there have only been a few case reports of PRES in a patient with anti-glomerular basement membrane antibody glomerulonephritis (anti-GBM GN)...
September 2017: Electrolyte & Blood Pressure: E & BP
https://www.readbyqxmd.com/read/29042375/non-invasive-brain-stimulation-interventions-for-management-of-chronic-central-neuropathic-pain-a-scoping-review-protocol
#20
Mei Lin Chen, Lin Yao, Jennifer Boger, Kathryn Mercer, Benjamin Thompson, Ning Jiang
INTRODUCTION: Pain can affect people regardless of age, gender or ethnicity. Chronic central neuropathic pain (CCNP) is a debilitating condition that affects populations such as stroke survivors, amputees, spinal cord injury patients and patients with multiple sclerosis, with prevalence rates between 30% and 80%. This condition can be caused by a lesion or disease affecting the somatosensory system. CCNP is notoriously drug resistant, and few effective CCNP treatment or management strategies exist...
October 16, 2017: BMJ Open
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