148,956 results match your criteria Focal Dermal Hypoplasia Syndrome


Correlation of focal adhesion kinase expression with nodal metastasis in patients with head and neck cutaneous squamous cell carcinoma.

Head Neck 2018 Dec 10. Epub 2018 Dec 10.

Service of Dermatology, Hospital Universitario Central de Asturias, Universidad de Oviedo, Oviedo, Spain.

Background: Focal adhesion kinase (FAK) and cortactin overexpression is frequently detected in a variety of cancers, and has been associated with poor clinical outcome. However, there are no data in cutaneous squamous cell carcinoma (cSCC).

Objective: To investigate the relationship of FAK and cortactin expression with the clinicopathologic features and the impact on the prognosis of cSCC patients. Read More

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December 2018

LL-37 and its analog FF/CAP18 attenuate neutrophil migration in sepsis-induced acute lung injury.

J Cell Biochem 2018 Dec 9. Epub 2018 Dec 9.

Department of Pulmonary and Critical Care Medicine, Beijing Anzhen Hospital, Capital Medical University, Experimenter Center, Beijing Institute of Heart, Lung and Blood Vessel Diseases, Beijing, China.

Introduction: Sepsis can result in acute lung injury. LL-37 is a small cationic host defense peptide involved in anti-inflammatory. In the current study, it was hypothesized that antimicrobial peptide LL-37 could play a protective role in attenuating the progression of sepsis-induced acute lung injury. Read More

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December 2018

Immediate remote ischemic postconditioning reduces cerebral damage in ischemic stroke mice by enhancing leptomeningeal collateral circulation.

J Cell Physiol 2018 Dec 10. Epub 2018 Dec 10.

Beijing Key Laboratory of Hypoxia Conditioning Translational Medicine, Xuanwu Hospital, Capital Medical University, Beijing, China.

Remote ischemic postconditioning (RIPC) is a promising neuroprotective strategy for ischemic stroke. Here, we employed a focal ischemic stroke mouse model to test the hypothesis that poststroke collateral circulation as a potent mechanism of action underlying the therapeutic effects of immediate RIPC. During reperfusion of cerebral ischemia, the mice were randomly assigned to receive RIPC, granulocyte colony-stimulating factor (G-CSF) as a positive control, or no treatment. Read More

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December 2018

Voxel-wise correlation of PET/CT with multiparametric MRI and histology of the prostate using a sophisticated registration framework.

BJU Int 2018 Dec 10. Epub 2018 Dec 10.

School of Physics, The University of Sydney, Sydney, New South Wales, Australia.

Objectives: To develop a registration framework for correlating positron emission tomography/computed tomography (PET/CT) images with multiparametric MRI (mpMRI) and histology of the prostate, thereby enabling voxel-wise analysis of imaging parameters.

Patients And Methods: In this prospective proof-of-concept study, nine patients scheduled for radical prostatectomy underwent mpMRI and PET/CT imaging prior to surgery. One had PET imaging using F-fluoromethylcholine (FCH), five using Ga-labelled prostate-specific membrane antigen (PSMA)-HBED-CC (PMSA-11) and three using a trial Ga-labelled THP-PSMA tracer. Read More

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December 2018

Combined aclidinium bromide and long-acting beta2-agonist for chronic obstructive pulmonary disease (COPD).

Cochrane Database Syst Rev 2018 Dec 11;12:CD011594. Epub 2018 Dec 11.

Faculty of Medicine, SEGi University, Hospital Sibu, Jalan Ulu Oya, Sibu, Sarawak, Malaysia, 96000.

Background: Several dual bronchodilator combinations of long-acting beta-agonist (LABA) and long-acting muscarinic antagonist (LAMA) have been approved for treatment of stable chronic obstructive pulmonary disease (COPD). The current GOLD (Global Initiative for Chronic Obstructive Lung Disease) recommendations suggest the use of LABA/LAMA combinations in people with group B COPD with persistent symptoms, group C COPD with further exacerbations on LAMA therapy alone and group D COPD with or without inhaled corticosteroids (ICS). Fixed-dose combination (FDC) of aclidinium/formoterol is one of the approved LABA/LAMA therapies for people with stable COPD. Read More

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December 2018

[Cost-utility analysis of two formulations of botulinum toxin type A in the treatment of blepharospasm and cervical dystonia in Spain].

Rev Neurol 2018 Dec;67(12):465-472

Hospital Clinico San Carlos, 28040 Madrid, Espana.

Introduction: Studies on focal dystonia showed that the formulations of botulinum toxin type A, incobotulinumtoxin-A (Inco-BTA) and onabotulinumtoxin-A (Ona-BTA), have equivalent efficacy and safety.

Aim: To carry-out a cost-utility analysis of Inco-BTA administered on flexible intervals vs. Ona-BTA on a fixed interval, in the treatment of blepharospasm and cervical dystonia. Read More

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December 2018

[Pathogenesis of multiple myeloma].

Authors:
L Rasche N Weinhold

Internist (Berl) 2018 Dec 10. Epub 2018 Dec 10.

Myeloma Center, University of Arkansas for Medical Sciences, Little Rock, AR, USA.

Background: Multiple myeloma (MM) is a hematologic malignancy characterized by monoclonal plasma cells infiltrating the bone marrow thereby causing anemia and lytic bone lesions. Despite significant improvement in overall survival, most MM patients inevitably, yet unpredictably, develop refractory disease and MM remains largely incurable.

Objective: This article describes the stages of progression and presents current insights into the pathogenesis of MM. Read More

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December 2018

Immunohistochemical and ultrastructural analysis of sporadic inclusion body myositis: a case series.

Rheumatol Int 2018 Dec 8. Epub 2018 Dec 8.

Division of Histology and Embryology, Department of Human Morphology and Embryology, Wroclaw Medical University, Chałubińskiego Street 6a, 50-368, Wrocław, Poland.

Sporadic inclusion body myositis (s-IBM) is a progressive, skeletal muscle disease with poor prognosis. However, establishing the final diagnosis is difficult because of the lack of clear biomarkers in the blood serum and very slow development of clinical symptoms. Moreover, most other organs function normally without any disturbance. Read More

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December 2018

Differential impact of ventromedial prefrontal cortex damage on "hot" and "cold" decisions under risk.

Cogn Affect Behav Neurosci 2018 Dec 7. Epub 2018 Dec 7.

Dipartimento di Psicologia, Università di Bologna, Bologna, Italy.

The ventromedial prefrontal cortex (vmPFC) is known to play a key role in reward processing and decision making. However, its relative contribution to affect-rich ("hot") and affect-poor ("cold") decisions is not fully understood. Damage to vmPFC is associated with impaired performance on laboratory tasks of decision making under ambiguity and risk. Read More

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December 2018

Tropomyosin Tpm 2.1 loss induces glioblastoma spreading in soft brain-like environments.

J Neurooncol 2018 Dec 9. Epub 2018 Dec 9.

Children's Cancer Research Unit, Kids Research Institute, The Children's Hospital at Westmead, Locked Bag 4001, Westmead, NSW, 2145, Australia.

Introduction: The brain is a very soft tissue. Glioblastoma (GBM) brain tumours are highly infiltrative into the surrounding healthy brain tissue and invasion mechanisms that have been defined using rigid substrates therefore may not apply to GBM dissemination. GBMs characteristically lose expression of the high molecular weight tropomyosins, a class of actin-associating proteins and essential regulators of the actin stress fibres and focal adhesions that underpin cell migration on rigid substrates. Read More

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December 2018

Persistent upregulation of the β-tubulin tubb6, linked to muscle regeneration, is a source of microtubule disorganization in dystrophic muscle.

Hum Mol Genet 2018 Dec 6. Epub 2018 Dec 6.

Light Imaging Section, Office of Science and Technology, National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, USA.

In healthy adult skeletal muscle fibers microtubules form a three-dimensional grid-like network. In the mdx mouse, a model of Duchenne muscular dystrophy (DMD), microtubules are mostly disordered, without periodicity. These microtubule defects have been linked to the mdx mouse pathology. Read More

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December 2018

Lesions to the Fronto-Parietal Network Impact Alpha-Band Phase Synchrony and Cognitive Control.

Cereb Cortex 2018 Dec 7. Epub 2018 Dec 7.

Department of Psychology, Helen Wills Neuroscience Institute, University of California, Berkeley, CA, USA.

Long-range phase synchrony in the α-oscillation band (near 10 Hz) has been proposed to facilitate information integration across anatomically segregated regions. Which areas may top-down regulate such cross-regional integration is largely unknown. We previously found that the moment-to-moment strength of high-α band (10-12 Hz) phase synchrony co-varies with activity in a fronto-parietal (FP) network. Read More

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December 2018

The importance of the histopathological examination in lethal acute intoxication with ethylene glycol. Case report.

Rom J Morphol Embryol 2018 ;59(3):965-969

Department of Morphological Disciplines, Faculty of Medicine and Pharmacy, University of Oradea, Romania;

Ethylene glycol is a toxic alcohol that is mainly introduced into an organism through the digestive pathway. Its priority toxic metabolites are glycolic acid and oxalic acid. We present the case of a young person, of the male persuasion, without any personal pathological history, found unconscious and presenting signs of violence. Read More

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January 2018

The importance of pancreatic inflammation in endosonographic diagnostics of solid pancreatic masses.

Med Ultrason 2018 Dec;20(4):427-435

Department of Internal Medicine 1, Friedrich-Alexander-University, Erlangen- Nuremberg, Germany.

Aims: Endosonography (EUS) is one of the main diagnostic tools for the differential diagnosis of pancreatic masses. The aim of our study was to describe the value of this technique in the work-up of solid pancreatic lesions, considering the influence of the morphological evidence of pancreatic inflammation in the diagnostic process.

Material And Methods: Retrospective analysis of prospectively collected data in our tertiary University center. Read More

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December 2018

Stereotactic Lesioning of the Thalamic Vo Nucleus for the Treatment of Writer's Cramp (Focal Hand Dystonia).

Front Neurol 2018 26;9:1008. Epub 2018 Nov 26.

Department of Neurodegenerative Disorders Research, Graduate School of Medical Sciences, Institute of Biomedical Sciences, Tokushima University, Tokushima, Japan.

Writer's cramp (focal hand dystonia) is a sporadic focal dystonia that affects a specific part of the upper limb causing excessive co-contraction of antagonistic muscles. It usually presents as a task-specific dystonia, including, among others, writing of a character or playing a musical instrument. Although treatments for writer's cramp exist, medical therapy often results in unsatisfactory outcomes in patients with this type of dystonia. Read More

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November 2018

Unknown primary large-cell neuroendocrine tumor.

Autops Case Rep 2018 Apr-Jun;8(2):e2018025. Epub 2018 Jun 8.

a Centro Hospitalar de São João, Medical Oncology Department. Porto, Portugal.

Large-cell neuroendocrine tumors (NETs) are poorly differentiated malignancies of rare incidence and aggressive nature. NETs mostly arise in the lung followed by the gastrointestinal tract, although they are potentially ubiquitous throughout the body. Primary unknown NET has a worse prognosis and shorter survival comparing with other NETs, with limited available data in the literature concerning this subgroup. Read More

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Intraosseous Bioplasty for a Chondral Cyst in the Lateral Tibial Plateau.

Arthrosc Tech 2018 Nov 15;7(11):e1149-e1156. Epub 2018 Oct 15.

Advanced Orthopaedics & Sports Medicine, San Francisco, California, U.S.A.

Subchondral lesions are the result of osseous modifications seen in a different array of pathologies such as osteoarthritis, rheumatoid arthritis, calcium pyrophosphate deposition, and osteonecrosis. The physiopathologic changes in all of the aforementioned pathologies are not clear yet. What is clear is that the development of a cystic change in the subchondral bone can cause pain and can lead to modification of the activity of daily life. Read More

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November 2018

A Case of Sjögren's Syndrome Mimicking Inflammatory Myopathy.

Cureus 2018 Oct 1;10(10):e3393. Epub 2018 Oct 1.

Neurology, Keck School of Medicine of the University of Southern California, Los Angeles, USA.

Sjögren's syndrome (SS) is a chronic autoimmune disorder, characterized by lymphocytic infiltration of exocrine glands and causing the decreased function of lacrimal and salivary glands. We describe a case of a 34-year-old male who presented with Sjögren's syndrome presenting as myopathy and sensorimotor neuropathy. His creatinine kinase levels were elevated with positive anti-Sjögren's syndrome-related antigen A autoantibodies and anti-Sjögren's syndrome Type B autoantibodies. Read More

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October 2018

Identification of key candidate genes and biological pathways in bladder cancer.

PeerJ 2018 4;6:e6036. Epub 2018 Dec 4.

Central Laboratory, Central South University Xiangya School of Medicine Affiliated Haikou Hospital, Haikou, China.

Background: Bladder cancer is a malignant tumor in the urinary system with high mortality and recurrence rates. However, the causes and recurrence mechanism of bladder cancer are not fully understood. In this study, we used integrated bioinformatics to screen for key genes associated with the development of bladder cancer and reveal their potential molecular mechanisms. Read More

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December 2018

Inappropriate Laughter and Behaviours: How, What, and Why? Case of an Adult with Undiagnosed Gelastic Seizure with Hypothalamic Hamartoma.

Hawaii J Med Public Health 2018 Dec;77(12):319-324

John A. Burns School of Medicine, University of Hawai'i, Honolulu, HI (NLB,JCK,AS).

Gelastic seizures (GS) are a rare form of epilepsy characterized by inappropriate, uncontrolled laughter. They are highly associated with abnormal cognitive development and behavioral problems in patients. Research has shown that GS can originate from hypothalamic hamartomas (HH), non- neoplastic masses consisting of gray matter with large and small neurons interspersed with glial nuclei. Read More

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December 2018

Lateral position and utility of navigation for posterior fixation of unstable cervical fracture with ankylosing spondylitis.

Surg Neurol Int 2018 2;9:225. Epub 2018 Nov 2.

Grant Medical Center, Department of Neurosurgery, Ohio Health, Columbus, Ohio, USA.

Background: Ankylosing spondylitis (AS) is a seronegative spondyloarthropathy within the spectrum of rheumatologic diseases. The systemic inflammation that characterizes AS leads to bone resorption and reformation. Pathologic remodeling may include kyphosis, osteoporosis, and multi-segment auto-fusion. Read More

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November 2018

An elective entrustable professional activity-based thematic final medical school year: an appreciative inquiry study among students, graduates, and supervisors.

Adv Med Educ Pract 2018 15;9:837-845. Epub 2018 Nov 15.

Department of Anesthesiology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands,

Purpose: The transition from undergraduate to postgraduate training can be facilitated by offering electives that focus on increased patient care responsibilities. This transitional year model in the Netherlands has recently been expanded to offer packages of electives geared to specific residencies and was called "dedicated transitional year" (DTY). At University Medical Center Utrecht, an entrustable professional activity (EPA)-based multidisciplinary DTY in acute care (DTY-AC), rooted in self-determination theory (SDT), has been implemented. Read More

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November 2018

Type 2 Diabetes Mellitus is Associated with Carotid Artery Plaques in Patients with Premature Coronary Heart Disease.

Rev Invest Clin 2018 ;70(6):301-309

Department of Nephrology, Laboratory of Renal Physiopathology. Instituto Nacional de Cardiología Ignacio Chávez, Mexico City, Mexico.

Background: In subjects without a history of coronary heart disease (CHD), type 2 diabetes mellitus (T2DM) is associated with carotid artery plaques (CAP), which is a better marker than high carotid intima-media thickness (hCIMT) for predicting first or recurrent cardiovascular events.

Objective: The objective of this study is to analyze the association of T2DM with CAP and hCIMT in premature CHD patients.

Methods: Premature CHD was considered before the age of 55 years in men and before 65 in women. Read More

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January 2018

CASP4 gene silencing in epithelial cancer cells leads to impairment of cell migration, cell-matrix adhesion and tissue invasion.

Sci Rep 2018 Dec 7;8(1):17705. Epub 2018 Dec 7.

National Research Council, Institute of Cell Biology and Neurobiology - Campus Adriano Buzzati-Traverso Via E. Ramarini, 32 00015, Monterotondo (Rome), Italy.

Inflammatory caspases, including human caspase-4 (CASP4), play key roles in innate immune responses to promote fusion of phagosomes harboring pathogenic bacteria with lysosomes, halt intracellular replication of pathogens, maturation and secretion of pro-inflammatory cytokines. The role of inflammatory caspases in cancer cells remains poorly investigated. Here, we explored the consequences of modulating CASP4 expression levels on the migratory behavior of epithelial cancer cell lines. Read More

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December 2018

Intranasal Calcitonin Gene-Related Peptide Protects Against Focal Cerebral Ischemic Injury in Rats Through the Wnt/β-Catenin Pathway.

Med Sci Monit 2018 Dec 7;24:8860-8869. Epub 2018 Dec 7.

Key Lab of Cerebral Microcirculation in Universities of Shandong, Taishan Medical University, Taian, Shandong, China (mainland).

BACKGROUND Intranasal calcitonin gene-related peptide (CGRP) delivery offers a noninvasive method of bypassing the blood-brain barrier for the delivery of CGRP to the brain. Here, we first reported the therapeutic benefits of intranasal CGRP delivery in rats following middle cerebral artery occlusion (MCAO). MATERIAL AND METHODS Real-time quantitative polymerase chain reaction (RT-qPCR) assay, enzyme-linked immunosorbent assay (ELISA), rat MCAO model, TTC (2, 3, 5-triphenyltetrazolium chloride) staining, hematoxylin and eosin (H & E) staining, Morris water maze test, TUNEL assay, immunofluorescence, and western blot assay were used to investigate the role of CGRP in rats. Read More

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December 2018

Novel Use of Responsive Neurostimulation (RNS System) in the Treatment of Super Refractory Status Epilepticus.

J Clin Neurophysiol 2018 Dec 6. Epub 2018 Dec 6.

Departments of Neurology and.

There are very few randomized controlled trials studying treatment of super refractory status epilepticus (SE), despite estimated occurrence in about 15% of SE cases and its association with high morbidity and mortality rates. Small case series and case reports have described use of neurostimulation, including vagal nerve stimulation, transcranial magnetic stimulation, and deep brain stimulation, to treat super refractory SE when medical interventions have failed. To our knowledge, this is the first reported case of responsive neurostimulation being used to successfully treat a case of super refractory SE. Read More

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December 2018

Methotrexate-induced senescence of human colon cancer cells depends on p53 acetylation, but not genomic aberrations.

Anticancer Drugs 2018 Dec 6. Epub 2018 Dec 6.

Nencki Institute of Experimental Biology.

Human colon cancer C85 cell response to methotrexate has been documented previously to take on a form of reversible premature senescence. Seeking genomic aberrations encompassing candidate genes whose functional impairment could determine such a response to the drug, an array Comparative Genomic Hybridization method was applied, complemented by expression microarray data set searching. In the C85 cell genome, only short aberrations were identified, classified as focal chromosomal aberrations. Read More

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December 2018

[DEPDC5, a new key to understand various epilepsies].

Authors:
Saeko Ishida

Nihon Yakurigaku Zasshi 2018 ;152(6):281-285

Laboratory of Molecular Neuroscience, Medical Research Institute (MRI), Tokyo Medical and Dental University (TMDU).

Epilepsy is one of the most frequent neurological disorders characterized by spontaneous and recurrent seizures. Most seizures last for the lifetime and the patients require long term therapies. However, about 30% of the patients are refractory to antiepileptic drugs. Read More

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January 2018

[Function of synaptic vesicle protein 2A (SV2A) as a novel therapeutic target for epilepsy].

Nihon Yakurigaku Zasshi 2018 ;152(6):275-280

Department of Pharmacology, Osaka University of Pharmaceutical Sciences.

Epilepsy is a chronic neurologic disease characterized by recurrent seizures, affecting nearly 1% of the population. Synaptic vesicle protein 2A (SV2A) is a membrane protein specifically expressed in synaptic vesicles and is now implicated in the pathogenesis of epileptic disorders. This is because 1) Sv2a-knockout mice exhibit severe seizures, 2) SV2A serves as a specific binding site for certain antiepileptics (e. Read More

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January 2018

Hemiconvulsion-Hemiplegia-Epilepsy in a girl with cobalamin C deficiency.

Epileptic Disord 2018 Dec 10. Epub 2018 Dec 10.

Division of Neurology, Department of Pediatrics, Montreal Children's Hospital, McGill University,, Department of Neurology and Neurosurgery, McGill University,, Research Institute of the McGill University Health Centre.

Hemiconvulsion-Hemiplegia-Epilepsy initially involves an infantile presentation of febrile focal motor status epilepticus, with subsequent hemiplegia of the initially affected side. Months to years later, affected children go on to develop a chronic epilepsy with recurrent focal seizures which are often refractory to treatment. This uncommon paediatric epilepsy syndrome is poorly understood, with only a very small minority of cases associated with an underlying genetic or metabolic abnormality. Read More

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December 2018

KCNQ2 mutation in an infant with encephalopathy of infancy with migrating focal seizures.

Epileptic Disord 2018 Dec 10. Epub 2018 Dec 10.

Department of Pediatrics (Neurology), McMaster University, 1280 Main St., W. Hamilton, Ontario L8S 4K1, Canada.

A male neonate presented with seizures at 18 hours of life, characterized by tonic posturing with eye deviation to the right, apnoea, bradycardia, and oxygen desaturation. Initial structural, metabolic, and infectious work-up was unremarkable. He continued to have seizures refractory to a variety of antiepileptic medications. Read More

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December 2018

Intractable apnoeic seizures in a child with a deletion typically associated with Williams syndrome.

Epileptic Disord 2018 Dec 10. Epub 2018 Dec 10.

Division of Pediatric Neurology, Department of Pediatrics, The Hospital for Sick Children, Toronto.

Williams-Beuren syndrome is rarely associated with epilepsy. One previously reported case showed an association with apnoeic seizures while a few other cases showed an association with infantile epileptic spasms and generalized and focal seizures. We report the case of a 13-month-old boy with a deletion typically associated with Williams-Beuren syndrome, who presented with isolated apnoeic seizures which were refractory to multiple antiepileptic drugs but partially responsive to the ketogenic diet. Read More

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December 2018

Cortical surface intracranial electrodes identify clinically relevant seizures missed on scalp EEG after traumatic intracranial hemorrhage.

Epileptic Disord 2018 Dec 10. Epub 2018 Dec 10.

Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.

Seizures and other electrophysiological disturbances are an under-recognized cause of coma, focal deficits, and prolonged encephalopathy following subdural hematoma evacuation. In these patients, it is possible that seizures remain unrecognized on scalp EEG. It has been shown that a high burden of seizures and other electrophysiological disturbances exist following surgical evacuation and underlie the encephalopathy commonly seen in this patient population, predisposing them to medical complications and confounding estimates of prognosis. Read More

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December 2018

Faciobrachial dystonic seizures expressed as epileptic spasms, followed by focal seizures in anti-LGI1 encephalitis: a video-polygraphic study.

Epileptic Disord 2018 Dec 10. Epub 2018 Dec 10.

Epilepsy Centre - Clinic of Nervous System Diseases, Riuniti Hospital, Foggia, Italy.

The origin of faciobrachial dystonic seizures in anti-LGI1 encephalitis is controversial due to a lack of neurophysiological characterization. We report a 68-year-old man with subacute anterograde memory loss and involuntary faciobrachial movements. Video-polygraphic recordings disclosed repetitive events characterized by sudden, short contraction of the upper limbs and ipsilateral hemiface. Read More

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December 2018

Epileptology of the first tonic-clonic seizure in adults and prediction of seizure recurrence.

Epileptic Disord 2018 Dec 10. Epub 2018 Dec 10.

Department of Clinical Neurophysiology and Epilepsy, Guy's & St Thomas' NHS Foundation Trust and Institute of Psychiatry, Psychology & Neuroscience, Division of Neuroscience, King's College London, UK.

The risk of seizure recurrence after a first unprovoked seizure is influenced by certain risk factors. To understand their effect in people with early diagnosed new epilepsy, we assessed the risk of recurrence of focal to bilateral tonic-clonic or generalized tonic-clonic seizures and the associated factors in a clinically well-characterized cohort of adults with a first unprovoked tonic-clonic seizure. We prospectively studied 150 consecutive adults with a first unprovoked tonic-clonic seizure and full clinical, EEG, and brain imaging assessment within the first four weeks. Read More

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December 2018

Early and long-term electroclinical features of patients with epilepsy and PCDH19 mutation.

Epileptic Disord 2018 Dec 10. Epub 2018 Dec 10.

Pediatric Neurology Department, Centre de Référence des Épilepsies Rares, Necker Enfants Malades Hospital, Paris, Inserm U1129, Paris; University Paris Descartes; CEA, Gif sur Yvette.

Protocadherin 19 (PCDH19) mutations have been identified in epilepsy in females with mental retardation as well as patients with a "Dravet-like" phenotype. We aimed to elucidate the electroclinical phenotype associated with PCDH19 mutation, which is currently difficult to identify at onset leading to a delay in diagnosis. We retrospectively reviewed clinical and EEG data for 13 consecutive patients with PCDH19 mutations or deletions diagnosed at our centers from 2009 to 2011, and followed these patients into adolescence and adulthood. Read More

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December 2018

Rasmussen syndrome: an atypical presentation in ten patients.

Epileptic Disord 2018 Dec 10. Epub 2018 Dec 10.

Department of Neurosurgery, Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina.

The aim of this study was to analyse the electroclinical and imaging findings and outcome of patients with Rasmussen syndrome (RS) with atypical manifestations. We conducted a retrospective, descriptive study of 10 of 44 consecutive patients with RS with atypical features, followed between 1999 and 2017. Six patients were boys and four were girls. Read More

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December 2018

Simultaneous Axial Multifocal Imaging using a Single Acoustical Transmission: a Practical Implementation.

IEEE Trans Ultrason Ferroelectr Freq Control 2018 Dec 5. Epub 2018 Dec 5.

Standard ultrasound imaging techniques rely on sweeping a focused beam across a field of view; however, outside the transmission focal depth, image resolution and contrast are degraded. High-quality deep tissue in vivo imaging requires focusing the emitted field at multiple depths, yielding high resolution and high contrast ultrasound images but at the expense of a loss in frame rate. Recent developments in ultrasound technologies have led to user-programmable systems, which enable real-time dynamic control over the phase and apodization of each individual element in the imaging array. Read More

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December 2018

Considerations for Choosing Sensitive Element Size for Needle and Fiber-Optic Hydrophones II: Experimental Validation of Spatial Averaging Model.

IEEE Trans Ultrason Ferroelectr Freq Control 2018 Dec 10. Epub 2018 Dec 10.

Acoustic pressure can be measured with a hydrophone. Hydrophone measurements can underestimate incident acoustic pressure due to spatial averaging effects across the hydrophone sensitive element. The spatial averaging filter for a nonlinear focused beam is a low-pass filter that decreases monotonically from 1 to 0 as frequency increases from 0 to infinity. Read More

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December 2018

Analytical Minimization-Based Regularized Sub-Pixel Shear Wave Tracking for Ultrasound Elastography.

IEEE Trans Ultrason Ferroelectr Freq Control 2018 Dec 7. Epub 2018 Dec 7.

Ultrasound elastography is a convenient and affordable method for imaging mechanical properties of tissue, which are often correlated with pathologies. An emerging novel elastography technique applies an external acoustic radiation force (ARF) to generate shear wave in the tissue, and uses ultrasound imaging to track the shear wave. Accurate tracking of the small tissue motion is a critical step in shear wave elastography, but is challenging due to various sources of noise in the ultrasound data. Read More

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December 2018

Self-scanned HIFU ablation of moving tissue using real-time hybrid US-MR imaging.

IEEE Trans Biomed Eng 2018 Dec 5. Epub 2018 Dec 5.

Objective: High Intensity Focused Ultrasound (HIFU) treatment in the abdominal cavity is challenging due to the respiratory motion. In the self-scanning HIFU ablation method the focal spot is kept static and the heating pattern is obtained through natural tissue motion. This paper describes a novel approach for modulating the HIFU power during self-scanning in order to compensate for the effect of tissue motion on thermal build-up. Read More

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December 2018

High fat diet exacerbates intestinal barrier dysfunction and changes gut microbiota in intestinal-specific ACF7 knockout mice.

Biomed Pharmacother 2018 Dec 7;110:537-545. Epub 2018 Dec 7.

Department of General Surgery, Shanghai Tenth People's Hospital Affiliated to Tongji University, No. 301, Yan-chang Road, Shanghai 200072, China; Department of Oncology, Affiliated Hospital of Yangzhou University, No. 368, Han-jiang Road, Yangzhou 225000, China. Electronic address:

Microtubule-actin cross-linking factor-1 (ACF7, or MACF1) regulates cytoskeletal focal adhesion dynamics and migration in various tissues. High fat diet (HFD) induces gut microbiota dysbiosis and metabolic disorders, and increases intestinal permeability and inflammatory response. Here we investigated the synergistic effects of intestinal ACF7 conditional knockout (ACF7 cKO) and HFD on metabolism phenotypes, gut microbiota and intestinal barrier function in mice. Read More

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December 2018

Brivaracetam in adults with drug-resistant epilepsy and psychiatric comorbidities.

Epilepsy Behav 2018 Dec 7;90:129-131. Epub 2018 Dec 7.

Atkinson Morley Regional Neuroscience Centre, St George's University Hospitals NHS Foundation Trust, London, UK; Institute of Medical and Biomedical Education, St George's University of London, UK. Electronic address:

This is a case series of 25 patients with drug-resistant epilepsy and psychiatric comorbidities who started on brivaracetam (BRV) at St George's University Hospitals and Frimley Health in London. Median BRV dose was 150 mg for a median follow-up period of 8 months. Twenty had focal epilepsy, four had generalized epilepsies, and one had unclassified epilepsy; 76% had mood disorders (either depression or bipolar disorder), 12% intellectual disabilities with autism spectrum disorder and challenging behavior, and 12% psychoses. Read More

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December 2018

Clinicopathological study of role of CD34 expressions in the stroma of premalignant and malignant lesions of uterine cervix.

Ann Diagn Pathol 2018 Nov 29;38:87-92. Epub 2018 Nov 29.

Department of Pathology, Jawahar Lal Nehru Medical College, Aligarh Muslim University, Aligarh, India.

CD34 is a transmembrane glycoprotein that is thought to be involved in the modulation of cell adhesion and signal transduction. The connective tissue stroma of virtually all human organs contain large amounts of resident CD34+ fibrocytes, which are involved in multiple functions such as wound healing, secretion of cytokines and also participate in stromal remodeling. It has been seen in various studies that absence of CD34+ fibrocytes within the stroma is associated with invasive carcinomas. Read More

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November 2018

Substitution has better efficacy than add-on therapy for patients with focal epilepsy after their first antiepileptic drug treatments fail.

Seizure 2018 Dec 1;64:23-28. Epub 2018 Dec 1.

Department of Neurology, the First Affiliated Hospital of Wenzhou Medical University, Wenzhou, 325015, PR China. Electronic address:

Purpose: This study is to compare the efficacy of substitution with add-on therapy in patients with focal epilepsy, whose first monotherapy has failed after receiving usual treatments.

Methods: This is a prospective, long-term, non-randomized observational cohort study. Data were collected from Wenzhou Epilepsy Follow Up Registry Database. Read More

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December 2018

Age-dependent semiology of frontal lobe seizures.

Epilepsy Res 2018 Oct 23;149:83-87. Epub 2018 Oct 23.

Epilepsy Center, University Medical Center, University of Freiburg, Germany; Faculty of Medicine, University of Freiburg, Germany. Electronic address:

Introduction: Frontal lobe epilepsy is the second most frequent origin of focal epilepsy. Various studies have discussed localizing aspects of ictal signs in frontal lobe epilepsy; the effect of age on semiological manifestations has, however, not been analyzed so far.

Material And Methods: We retrospectively analyzed video-documented semiological signs in a cohort of 122 consecutive patients aged 0-70 years (mean age: 24. Read More

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October 2018

Pathological and molecular investigation of porcine sapelovirus infection in naturally affected Indian pigs.

Microb Pathog 2018 Dec 5. Epub 2018 Dec 5.

Division of Pathology, ICAR- Indian Veterinary Research Institute, Bareilly, Izatnagar, 243 122, U.P, India. Electronic address:

The aim of the present study was to pathological and molecular investigation of porcine sapelovirus (PSV) in naturally infected Indian pigs of various age groups. Eight samples (16%) out of 49 necropsied animals were positive for PSV on the basis of pathological and molecular investigation. Major lesions of PSV positive cases were thickening and clouding of meninges, congestion in brain, severe to moderate congestion in lungs along with froathy exudates in trachea, thickening of intestinal mucosa, especially mucosal folds of ileum. Read More

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December 2018

Tonic thermonociceptive stimulation selectively modulates ongoing neural oscillations in the human posterior insula: Evidence from intracerebral EEG.

Neuroimage 2018 Dec 5. Epub 2018 Dec 5.

Institute of Neuroscience, Université catholique de Louvain, 1200, Brussels, Belgium.

The human insula is an important target for spinothalamic input, but there is still no consensus on its role in pain perception and nociception. In this study, we show that the human insula exhibits activity preferential for sustained thermonociception. Using intracerebral EEG recorded from the insula of 8 patients (2 females) undergoing a presurgical evaluation of focal epilepsy (53 contacts: 27 anterior, 26 posterior), we "frequency-tagged" the insular activity elicited by sustained thermonociceptive and vibrotactile stimuli, by periodically modulating stimulation intensity at a fixed frequency of 0. Read More

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December 2018

Neuroprotective Effect of Dichloromethane Extraction From Piper nigrum L. and Piper longum L. on Permanent Focal Cerebral Ischemia Injury in Rats.

J Stroke Cerebrovasc Dis 2018 Dec 7. Epub 2018 Dec 7.

School of Pharmacy, Ningxia Medical University, Yinchuan, China; Key Laboratory of Hui Ethnic Medicine Modernization, Ministry of Education, Yinchuan, China; Ningxia Engineering and Technology Research Center for Modernization of Hui Medicine, Yinchuan, China. Electronic address:

Background: Piper nigrum L. and Piper longum L. consist a classic formula in traditional Chinese Hui medicine and are widely used in treatment of stroke. Read More

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December 2018

Micro-scaled topographies direct differentiation of human epidermal stem cells.

Acta Biomater 2018 Dec 5. Epub 2018 Dec 5.

Centre for Stem Cells and Regenerative Medicine, King's College London, 28th Floor, Tower Wing, Guy's Hospital, Great Maze Pond, London SE1 9RT, United Kingdom. Electronic address:

Human epidermal stem cells initiate terminal differentiation when spreading is restricted on ECM-coated micropatterned islands, soft hydrogels or hydrogel-nanoparticle composites with high nanoparticle spacing. The effect of substrate topography, however, is incompletely understood. To explore this, primary human keratinocytes enriched for stem cells were seeded on a topographical library with over 2000 different topographies in the micrometre range. Read More

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December 2018