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https://www.readbyqxmd.com/read/29151092/conversion-factitious-disorder-and-malingering-a-distinct-pattern-or-a-continuum
#1
Silvio Galli, Laurent Tatu, Julien Bogousslavsky, Selma Aybek
This chapter is aimed at highlighting the recent findings concerning physiopathology, diagnosis, and management of conversion, factitious disorder, and malingering. Conversion disorder is the unintentional production of neurological symptom, whereas malingering and factitious disorder represent the voluntary production of symptoms with internal or external incentives. They have a close history and this has been frequently confounded. Practitioners are often confronted to medically unexplained symptoms; they represent almost 30% of neurologist's consultation...
2018: Frontiers of Neurology and Neuroscience
https://www.readbyqxmd.com/read/29151060/genetics-of-parkinson-s-disease-and-related-disorders
#2
REVIEW
Pei-Lan Zhang, Yan Chen, Chen-Hao Zhang, Yu-Xin Wang, Pedro Fernandez-Funez
Parkinson's disease (PD) is a complex and heterogeneous neurological condition characterised mainly by bradykinesia, resting tremor, rigidity and postural instability, symptoms that together comprise the parkinsonian syndrome. Non-motor symptoms preceding and following clinical onset are also helpful diagnostic markers revealing a widespread and progressive pathology. Many other neurological conditions also include parkinsonism as primary or secondary symptom, confounding their diagnosis and treatment. Although overall disease course and end-stage pathological examination single out these conditions, the significant overlaps suggest that they are part of a continuous disease spectrum...
November 18, 2017: Journal of Medical Genetics
https://www.readbyqxmd.com/read/29150919/a-focus-on-the-beneficial-effects-of-alpha-synuclein-and-a-re-appraisal-of-synucleinopathies
#3
Larisa Ryskalin, Carla L Busceti, Fiona Limanaqi, Francesca Biagioni, Stefano Gambardella, Francesco Fornai
Alpha synuclein (α-syn) belongs to a class of proteins which are commonly considered to play a detrimental role in neuronal survival. This assumption is based on the occurrence of a severe neuronal degeneration in patients carrying a multiplication of the α-syn gene (SNCA) and in a variety of experimental models, where overexpression of α-syn leads to cell death and neurological impairment. In these conditions, a higher amount of normally structured α-syn produces a damage, which is even worse compared with that produced by α-syn owing an abnormal structure (as occurring following point gene mutations)...
November 16, 2017: Current Protein & Peptide Science
https://www.readbyqxmd.com/read/29150899/p-arg69trp-in-rnaseh2c-is-a-founder-variant-in-three-indian-families-with-aicardi-gouti%C3%A3-res-syndrome
#4
Malavika Hebbar, Anil Kanthi, Aroor Shrikiran, Snehal Patil, Mamta Muranjan, Febi Francis, Vishnu Bhat B, Katta M Girisha, Anju Shukla
Aicardi-Goutières syndrome is an early-onset severe neurological disorder characterized by intracranial calcification, white matter abnormalities, hepatosplenomegaly, cerebrospinal fluid lymphocytosis, and elevated interferon-α levels, thus mimicking congenital viral infections. It is a genetically heterogeneous condition and autosomal recessive and autosomal dominant forms with variations in seven genes known till date. Variations in RNASEH2C cause an autosomal recessive form of AGS. Here we report three Indian families with variant, c...
November 17, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29149593/notch2-hajdu-cheney-mutations-escape-scf-fbw7-dependent-proteolysis-to-promote-osteoporosis
#5
Hidefumi Fukushima, Kouhei Shimizu, Asami Watahiki, Seira Hoshikawa, Tomoki Kosho, Daiju Oba, Seiji Sakano, Makiko Arakaki, Aya Yamada, Katsuyuki Nagashima, Koji Okabe, Satoshi Fukumoto, Eijiro Jimi, Anna Bigas, Keiichi I Nakayama, Keiko Nakayama, Yoko Aoki, Wenyi Wei, Hiroyuki Inuzuka
Hajdu-Cheney syndrome (HCS), a rare autosomal disorder caused by heterozygous mutations in NOTCH2, is clinically characterized by acro-osteolysis, severe osteoporosis, short stature, neurological symptoms, cardiovascular defects, and polycystic kidneys. Recent studies identified that aberrant NOTCH2 signaling and consequent osteoclast hyperactivity are closely associated with the bone-related disorder pathogenesis, but the exact molecular mechanisms remain unclear. Here, we demonstrate that sustained osteoclast activity is largely due to accumulation of NOTCH2 carrying a truncated C terminus that escapes FBW7-mediated ubiquitination and degradation...
November 16, 2017: Molecular Cell
https://www.readbyqxmd.com/read/29149018/please-wait-processing-a-selective-literature-review-of-the-neurological-understanding-of-emotional-processing-in-asd-and-its-potential-contribution-to-neuroeducation
#6
REVIEW
Eric Shyman
Autism spectrum disorder (ASD) and its corresponding conditions have been investigated from a multitude of perspectives resulting in varying understandings of its origin, its outplay, its prognosis, and potential methods of intervention and education for individuals with the disorder. One area that has contributed significantly to providing a different type of understanding is that of neuroscience, and specifically neuroimaging. This paper will offer a selective literature review of research that investigates the role of emotional processing in ASD, and how a deepening of this line of understanding can be used to inform more comprehensive educational practices...
November 17, 2017: Brain Sciences
https://www.readbyqxmd.com/read/29148900/combined-interventional-and-surgical-treatment-of-tandem-middle-cerebral-artery-embolus-and-internal-carotid-artery-occlusion-case-report
#7
Mark B Frenkel, Jaclyn J Renfrow, Jasmeet Singh, Nitin Garg, Stacey Q Wolfe
Tandem internal carotid artery (ICA) origin occlusion and middle cerebral artery (MCA) thromboembolism is a life-threatening condition with poor neurological outcome. The authors report on a patient presenting with acute ischemic stroke from a tandem ICA and MCA occlusion with penumbra. Emergency MCA mechanical thrombectomy was performed through percutaneous cervical ICA access due to the inability to cross the cervical carotid occlusion. Emergency carotid endarterectomy to reperfuse the poorly collateralized hemisphere and repair the ICA access site was performed 2 hours after completion of tissue plasminogen activator (tPA) infusion...
November 17, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/29148267/detection-of-teschovirus-type-13-from-two-swine-herds-exhibiting-nervous-clinical-signs-in-growing-pigs
#8
J Carnero, C Prieto, L Polledo, F J Martínez-Lobo
Recently, the number of clinical reports of growing pigs showing neurological signs possibly related to viral infections has increased. The objective of this report was to describe two outbreaks of an atypical condition observed in 6- to 7-week-old pigs with a morbidity of 20% and a fatality rate of 60% in two unrelated farms of the same company. During the acute phase of the disease, fever, sudden death, neurological signs, ear necrosis and occasional corneal opacity were observed. Histopathological examination revealed interstitial pneumonia, lymphoid depletion and lymphocytic vasculitis in different organs and mild polioencephalomyelitis suggesting a potential viral infection...
November 16, 2017: Transboundary and Emerging Diseases
https://www.readbyqxmd.com/read/29148124/the-safety-of-live-attenuated-influenza-vaccine-in-children-and-adolescents-2-through-17%C3%A2-years-of-age-a-vaccine-safety-datalink-study
#9
Matthew F Daley, Christina L Clarke, Jason M Glanz, Stanley Xu, Simon J Hambidge, James G Donahue, James D Nordin, Nicola P Klein, Steven J Jacobsen, Allison L Naleway, Michael L Jackson, Grace Lee, Jonathan Duffy, Eric Weintraub
PURPOSE: To evaluate the safety of live attenuated influenza vaccine (LAIV) in children 2 through 17 years of age. METHODS: The study was conducted in 6 large integrated health care organizations participating in the Vaccine Safety Datalink (VSD). Trivalent LAIV safety was assessed in children who received LAIV between September 1, 2003 and March 31, 2013. Eighteen pre-specified adverse event groups were studied, including allergic, autoimmune, neurologic, respiratory, and infectious conditions...
November 17, 2017: Pharmacoepidemiology and Drug Safety
https://www.readbyqxmd.com/read/29147637/subtotal-resection-of-a-thalamic-glioblastoma-multiforme-through-transsylvian-approach
#10
Rouzbeh Motiei-Langroudi, Homa Sadeghian, Alireza M Mohammadi
Glioblastoma multiforme (GBM) is a malignant brain tumor with an ominous prognosis. The standard treatment includes maximal safe resection plus adjuvant therapy. Thalamic GBMs, however, are unfavorable for microsurgical removal because of deep location and proximity to critical structures. We present a patient presenting with progressive hemiparesis and decreased consciousness with a large thalamic GBM who underwent subtotal resection through a transsylvian approach. His clinical and neurologic condition improved after surgery and he survived nine months after surgery...
September 7, 2017: Curēus
https://www.readbyqxmd.com/read/29146880/optogenetic-inhibition-of-striatal-gabaergic-neuronal-activity-improves-outcomes-after-ischemic-brain-injury
#11
Lu Jiang, Wanlu Li, Muyassar Mamtilahun, Yaying Song, Yuanyuan Ma, Meijie Qu, Yifan Lu, Xiaosong He, Jieyu Zheng, Zongjie Fu, Zhijun Zhang, Guo-Yuan Yang, Yongting Wang
BACKGROUND AND PURPOSE: Striatal GABAergic neuron is known as a key regulator in adult neurogenesis. However, the specific role of striatal GABAergic neuronal activity in the promotion of neurological recovery after ischemic stroke remains unknown. Here, we used optogenetic approach to investigate these effects and mechanism. METHODS: Laser stimulation was delivered via an implanted optical fiber to inhibit or activate the striatal GABAergic neurons in Gad2-Arch-GFP or Gad2-ChR2-tdTomato mice (n=80) 1 week after 60-minute transient middle cerebral artery occlusion...
November 16, 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/29146718/deep-brain-nuclei-t1-shortening-after-gadobenate-dimeglumine-in-children-influence-of-radiation-and-chemotherapy
#12
S Kinner, T B Schubert, R J Bruce, S L Rebsamen, C A Diamond, S B Reeder, H A Rowley
BACKGROUND AND PURPOSE: Intrinsic T1-hyperintense signal has recently been reported in the deep gray nuclei on brain MR imaging after multiple doses of gadolinium-based contrast agents. Most reports have included adult patients and excluded those undergoing radiation or chemotherapy. We investigated whether T1 shortening is also observed in children and tried to determine whether radiochemotherapy is a risk factor for this phenomenon. MATERIALS AND METHODS: In this single-center retrospective study, we reviewed clinical charts and images of all patients 18 years of age or younger with ≥4 gadobenate dimeglumine-enhanced MRIs for 6 years...
November 16, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/29146406/central-nervous-involvement-by-chronic-lymphocytic-leukaemia
#13
Dariusz Szczepanek, Ewa Wąsik-Szczepanek, Agnieszka Szymczyk, Małgorzata Wach, Maria Cioch, Monika Podhorecka, Ewelina Grywalska, Marek Hus
Inclusion of the central nervous system (CNS) in the course of chronic lymphocytic leukaemia (CLL) is rare. At the moment no risk factors or proven treatment methods are known. The disease is described both in its early phase and during its acceleration period, thus it has been suggested that there might be independent mechanisms influencing the development of this condition. As there are no unified diagnostic procedure algorithms each patient needs to be assessed individually. CLL can manifest mostly in elderly people, for whom a possibility of development of neurological disorders with their aetiology different from leukaemia, should also be taken into consideration...
November 1, 2017: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/29145187/diogenes-syndrome
#14
Frédéric Assal
Diogenes syndrome (DS) is not a specific disease but a real neurobehavioral syndrome, characterized by severe domestic squalor, pathological hoarding, lack of insight into the condition, and no need for help. DS can be secondary when associated to psychosis or bipolar disorder, or primary when it occurs as a single entity, usually in the elderly. DS is a clinically complex transnosographic syndrome for which multidimensional approaches need to be considered: medical, psychiatric, neurological, social, scientific, and ethical...
2018: Frontiers of Neurology and Neuroscience
https://www.readbyqxmd.com/read/29145183/catastrophe-reaction-and-emotionalism
#15
Antonio Carota, Julien Bogousslavsky
The catastrophic reaction (CR; a disruptive and uncontrolled behavior triggered by anger, irritability, and hostility) and emotionalism (a condition of uncontrolled crying or laughing) are disorders of the emotional regulation and expression, the prevalence of which is underestimated in neurology. Their occurrence is an additional factor of poor outcome for neurologic patients. Although they have been recognized and completely described in their clinical manifestations more than a century ago, many issues remain unsolved regarding their pathogenesis and the respective role of the brain damage and psychological factors...
2018: Frontiers of Neurology and Neuroscience
https://www.readbyqxmd.com/read/29144407/the-effects-of-various-weather-conditions-as-a-potential-ischemic-stroke-trigger-in-dogs
#16
Kristy L Meadows, Gena M Silver
Stroke is the fifth leading cause of death in the United States, and is the leading cause of serious, long-term disability worldwide. There are at least 795,000 new or recurrent strokes each year, and approximately 85% of all stroke occurrences are ischemic. Unfortunately, companion animals are also at risk for ischemic stroke. Although the exact incidence of ischemic stroke in companion animals is unknown, some studies, and the veterinary information network (VIN), report that approximately 3% of neurological case referrals are due to a stroke...
November 16, 2017: Veterinary Sciences
https://www.readbyqxmd.com/read/29144185/rest-activity-circadian-rhythm-and-sleep-quality-in-patients-with-binge-eating-disorder
#17
E Roveda, A Montaruli, L Galasso, C Pesenti, E Bruno, P Pasanisi, M Cortellini, S Rampichini, S Erzegovesi, A Caumo, F Esposito
Recent findings suggest that altered rest-activity circadian rhythms (RARs) are associated with a compromised health status. RARs abnormalities have been observed also in several pathological conditions, such as cardiovascular, neurological, and cancer diseases. Binge eating disorder (BED) is the most common eating disorder, with a prevalence of 3.5% in women and 2% in men. BED and its associate obesity and motor inactivity could induce RARs disruption and have negative consequences on health-related quality of life...
November 16, 2017: Chronobiology International
https://www.readbyqxmd.com/read/29142086/shortage-of-generic-neurologic-therapeutics-an-escalating-threat-to-patient-care
#18
Jacklyn O Omorodion, Rami M Algahtani, Mark S Zocchi, Erin R Fox, Jesse M Pines, Henry J Kaminski
OBJECTIVE: To assess longitudinal trends in shortages of generic drugs used for neurologic conditions over a 15-year period in the United States. METHODS: Drug shortage data from the University of Utah Drug Information Service (UUDIS) from 2001 to 2015 were analyzed. Medications were included that were likely to be prescribed by a neurologist to treat a primary neurologic condition or critical for care of a patient with a neurologic condition. Trends in shortage length were assessed using standard descriptive statistics...
November 15, 2017: Neurology
https://www.readbyqxmd.com/read/29142081/genetic-and-molecular-regulation-of-extrasynaptic-gaba-a-receptors-in-the-brain-therapeutic-insights-for-epilepsy
#19
Shu-Hui Chuang, Doodipala Samba Reddy
GABA-A receptors play a pivotal role in many brain diseases. Epilepsy is caused by acquired conditions and genetic defects in GABA receptor channels regulating neuronal excitability in the brain. The latter is referred to as GABA channelopathies. In the last two decades, major advances have been made in the genetics of epilepsy. The presence of specific GABAergic genetic abnormalities leading to some of the classical epileptic syndromes has been identified. Advances in molecular cloning and recombinant systems have helped characterize mutations in GABA-A receptor subunit genes in clinical neurology...
November 15, 2017: Journal of Pharmacology and Experimental Therapeutics
https://www.readbyqxmd.com/read/29141990/rna-sequencing-of-drosophila-melanogaster-head-tissue-on-high-sugar-and-high-fat-diets
#20
Wayne Hemphill, Osvaldo Rivera, Matthew Talbert
Obesity has been shown to increase risk for cardiovascular disease and type-2 diabetes. In addition, it has been implicated in aggravation of neurological conditions such as Alzheimer's. In the model organism Drosophila melanogaster, a physiological state mimicking diet-induced obesity can be induced by subjecting fruit flies to a solid medium disproportionately higher in sugar than protein or that has been supplemented with a rich source of saturated fat. These flies can exhibit increased circulating glucose levels, increased triglyceride content, insulin-like peptide resistance, and behavior indicative of neurological decline...
November 15, 2017: G3: Genes—Genomes—Genetics
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