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Neurology

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55 papers 0 to 25 followers
https://www.readbyqxmd.com/read/28033622/reversible-hypertensive-myelopathy-the-spinal-cord-variant-of-posterior-reversible-encephalopathy-syndrome
#1
Rahsan Gocmen, Didem Ardicli, Yasin Erarslan, Ali Duzova, Banu Anlar
The posterior reversible encephalopathy syndrome (PRES) is a well-known clinical and radiologic entity mainly affecting the territory of the posterior cerebral circulation. Spinal cord involvement is extremely rare, and as of yet, only a few cases have been reported in the literature. The present case describes a reversible, longitudinal spinal cord lesion in a patient with high blood pressure. We discuss the differential diagnosis of longitudinal myelopathy and focus on the clinical presentation, diagnosis, and management of the "spinal cord variant of PRES...
December 29, 2016: Neuropediatrics
https://www.readbyqxmd.com/read/28052304/is-tadpole-pupil-in-an-adolescent-girl-caused-by-denervation-hypersensitivity
#2
Jonas Kjeldbjerg Hansen, Hans Ulrik Møller
Tadpole pupil is a rarely encountered phenomenon caused by episodic, segmental iris dilator muscle spasm of short duration (2-15 minutes), occurring in clusters without a known precipitating factor. It has most commonly been described in women aged 28 to 48 years. A few hypotheses on pathogenesis have been discussed but none has been proved. Here, we present an adolescent girl with bilateral tadpole pupil that appeared during physical exercise. This is the first pediatric case of tadpole pupil, not caused by preceding surgery, to be published...
January 4, 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28025405/teaching-video-neuroimages-minimal-anomalies-of-dorsal-midbrain-syndrome-parinaud-syndrome
#3
Pilar Rojas, Philippe Maeder, François-Xavier Borruat
No abstract text is available yet for this article.
January 3, 2017: Neurology
https://www.readbyqxmd.com/read/28055131/subthalamic-nuclei-stimulation-in-patients-with-pantothenate-kinase-associated-neurodegeneration-pkan
#4
Ziyuan Liu, Yang Liu, Yingmai Yang, Lin Wang, Wanchen Dou, Jinzhu Guo, Yu Wang, Yi Guo, Xinhua Wan, Wenbin Ma, Renzhi Wang
INTRODUCTION: Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal recessive genetic disease that leads to extrapyramidal symptoms, such as dystonia, ataxia, dysarthria, and involuntary movements. Treatment of PKAN with deep brain stimulation (DBS) has been reported, but mainly focuses on targeting the globus pallidus internus (GPi). Subthalamic nuclei (STN) may also be a potential target for treatment of PKAN. METHODS: In this study, we reviewed three patients with PKAN (two with typical PKAN and one with atypical PKAN) treated by bilateral STN stimulation and present a review of the literature...
January 5, 2017: Neuromodulation: Journal of the International Neuromodulation Society
https://www.readbyqxmd.com/read/28019640/semantic-dementia-a-mini-review
#5
Blanka Klimova, Michal Novotny, Kamil Kuca
BACKGROUND: At present there are about 47.5 million people suffering from different types of dementia and by 2030 this number should reach 75.6 million. This obviously brings about serious social and economic burden for people suffering from any kind of dementia. OBJECTIVE: The purpose of this article is to explore only semantic dementia (SD) as one of the forms of frontotemporal dementia (FTD) and provide the latest information on its diagnosis and treatment which play a significant role in the maintenance of quality of life of both patients and their caregivers...
December 23, 2016: Mini Reviews in Medicinal Chemistry
https://www.readbyqxmd.com/read/27036225/-adult-onset-opsoclonus-myoclonus-ataxia-syndrome-revealing-rubella-meningoencephalitis
#6
A Nasri, M Mansour, M Messelmani, A Riahi, H Derbali, I Bedoui, J Zaouali, R Mrissa
INTRODUCTION: Opsoclonus-myoclonus-ataxia (OMS) is a rare clinical syndrome, of paraneoplastic infectious, post-infectious, post-vaccinal or idiopathic origin. CASE REPORT: We report a 24-year-old young man who presented with gait disorder preceded by a febrile rash and retroauricular lymph nodes. Three days before admission, he had headache, vertigo, nausea and vomiting followed by gait unsteadiness and movement disorders of limbs and eyes. On examination, he had OMS syndrome...
December 2016: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/27989333/inherited-pain-syndromes-and-ion-channels
#7
REVIEW
Francis J DiMario
Individuals rely on the perception of pain to avoid injury, to signal disease, and to warn about tissue inflammation and damage. However, the inheritance of inappropriate, extreme, or inadequate pain production is a source of significant human suffering. Substantial progress has been made in our understanding of the genetics and pathophysiology of pain through the study of individuals and families with several specific inherited pain syndromes. These studies have led to the discovery of a number of gene mutations associated with specific ion channel disturbances that produce familial inherited pain sensitivity and insensitivity syndromes...
August 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27872231/teaching-neuroimages-giant-neurocysticercosis-with-unusual-imaging-manifestations
#8
Dekang Nie, Liang Xia, Jian Chen, Wei Shi, Guan Sun, Jun Guo
No abstract text is available yet for this article.
November 22, 2016: Neurology
https://www.readbyqxmd.com/read/27888415/magnetic-resonance-imaging-patterns-of-muscle-involvement-in-genetic-muscle-diseases-a-systematic-review
#9
REVIEW
Doris G Leung
A growing body of the literature supports the use of magnetic resonance imaging as a potential biomarker for disease severity in the hereditary myopathies. We performed a systematic review of the medical literature to evaluate patterns of fat infiltration observed in magnetic resonance imaging studies of muscular dystrophy and congenital myopathy. Searches were performed using MEDLINE, EMBASE, and grey literature databases. Studies that described fat infiltration of muscles in patients with muscular dystrophy or congenital myopathy were selected for full-length review...
November 25, 2016: Journal of Neurology
https://www.readbyqxmd.com/read/27955995/safety-and-immunogenicity-of-the-tau-vaccine-aadvac1-in-patients-with-alzheimer-s-disease-a-randomised-double-blind-placebo-controlled-phase-1-trial
#10
Petr Novak, Reinhold Schmidt, Eva Kontsekova, Norbert Zilka, Branislav Kovacech, Rostislav Skrabana, Zuzana Vince-Kazmerova, Stanislav Katina, Lubica Fialova, Michal Prcina, Vojtech Parrak, Peter Dal-Bianco, Martin Brunner, Wolfgang Staffen, Michael Rainer, Matej Ondrus, Stefan Ropele, Miroslav Smisek, Roman Sivak, Bengt Winblad, Michal Novak
BACKGROUND: Neurofibrillary pathology composed of tau protein is a main correlate of cognitive impairment in patients with Alzheimer's disease. Immunotherapy targeting pathological tau proteins is therefore a promising strategy for disease-modifying treatment of Alzheimer's disease. We have developed an active vaccine, AADvac1, against pathological tau proteins and assessed it in a phase 1 trial. METHODS: We did a first-in-man, phase 1, 12 week, randomised, double-blind, placebo-controlled study of AADvac1 with a 12 week open-label extension in patients aged 50-85 years with mild-to-moderate Alzheimer's disease at four centres in Austria...
December 9, 2016: Lancet Neurology
https://www.readbyqxmd.com/read/27956573/teaching-neuroimages-intracranial-vertebral-dissection-in-a-15-year-old-boy-with-sickle-cell-disease
#11
James E Siegler, Brenda Banwell, Rebecca N Ichord
No abstract text is available yet for this article.
December 13, 2016: Neurology
https://www.readbyqxmd.com/read/27920289/clinical-reasoning-an-unusual-cause-of-indeterminate-spells
#12
Anteneh M Feyissa, Daniel L Kenney-Jung, Cheolsu Shin, John C Cheville, Elson L So
No abstract text is available yet for this article.
December 6, 2016: Neurology
https://www.readbyqxmd.com/read/27880878/neurocysticercosis-infection-and-disease-a-review
#13
REVIEW
Lucy B Gripper, Susan C Welburn
Neurocysticercosis (NCC) is the most common parasitic disease of the human central nervous system (CNS), a pleomorphic disease with a diverse array of clinical manifestations. The infection is pleomorphic and dependent on a complex range of interconnecting factors, including number and size of the cysticerci, their stage of development and localisation within the brain with resulting difficulties in accurate diagnosis and staging of the disease. This review examines the factors that contribute to the accurate assessment of NCC distribution and transmission that are critical to achieving robust disease burden calculations...
February 2017: Acta Tropica
https://www.readbyqxmd.com/read/27872228/child-neurology-diencephalic-syndrome-like-presentation-of-a-cervicomedullary-brainstem-tumor
#14
Melanie Conway, Resham Ejaz, Elizabeth Kouzmitcheva, Deena Savlov, James T Rutka, Mahendranath Moharir
Diencephalic syndrome is a rare clinical entity, traditionally encompassing severe failure to thrive, nystagmus, and hyperkinesis, secondary to an intracranial neoplasm that is classically located in the hypothalamic region and its vicinity. However, the presenting features can be variable, often resulting in delayed diagnosis, which may worsen prognosis. This case report describes the atypical presentation of a posterior fossa tumor with features reminiscent of diencephalic syndrome that have not previously been reported in the literature...
November 22, 2016: Neurology
https://www.readbyqxmd.com/read/27720246/extra-central-nervous-system-target-for-assessment-and-treatment-in-refractory-anti-n-methyl-d-aspartate-receptor-encephalitis
#15
David W Nauen
Anti-N-methyl-d-aspartate-type glutamate receptor autoimmune encephalitis can arise in the setting of ovarian teratoma and often responds to resection. When it occurs in the absence of tumor, failure to respond to treatment may be more likely, and affected patients often require intensive care. To further understand the mechanisms and potential management, we present findings from an autopsy conducted on a young woman who died of refractory autoimmune encephalitis of this type. Rituximab was administered 70 days before death, and both 37 and 14 days before death, CD19(+) lymphocytes were only 0...
February 2017: Journal of Critical Care
https://www.readbyqxmd.com/read/25469247/reversible-posterior-leukoencephalopathy-syndrome-secondary-to-systemic-onset-juvenile-idiopathic-arthritis-a-case-report-and-review-of-the-literature
#16
Pingping Zhang, Xiaofeng Li, Yating Li, Jing Wang, Huasong Zeng, Xiaofeng Zeng
Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical syndrome based on changes in clinical imaging, and it has been reported to mainly occur in adults. However, it has been recently discovered that RPLS is also prevalent in infant patients, particularly in those using glucocorticoids, immunosuppressant medications and cytotoxic drugs. The current study presents a 5-year-old male with a previous diagnosis of systemic-onset juvenile idiopathic arthritis (SoJIA) and macrophage-activation syndrome who developed posterior reversible encephalopathy syndrome during treatment with glucocorticoids, disease-modifying antirheumatic drugs and biological agent (etanercept) therapy...
January 2015: Biomedical Reports
https://www.readbyqxmd.com/read/27831913/acute-necrotising-encephalopathy-of-childhood-an-uncommon-cause-of-childhood-encephalopathy-with-recognisable-clinical-and-radiological-features-and-genetic-predisposition
#17
Nicholas Schindler, Gautam Ambegaonkar
No abstract text is available yet for this article.
October 8, 2016: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/27821570/clinical-reasoning-a-30-year-old-man-with-progressive-weakness-and-atrophy
#18
Colin Quinn, Steven A Moore, Tanya M Bardakjian, Chafic Karam
No abstract text is available yet for this article.
November 8, 2016: Neurology
https://www.readbyqxmd.com/read/27765754/fever-in-a-child-with-cerebrospinal-fluid-access-device-or-shunt-a-pragmatic-approach-to-management
#19
REVIEW
Betsy Cleave, Maria Cartmill, Shiu Shing Soo, Harish Vyas
Children with shunts commonly present with fever, and often the focus of infection will be unrelated to their shunt. However, as shunt infections may present with few or even no specific symptoms, evaluation of a child with a shunt presenting with fever should be careful and comprehensive to ensure shunt infections are not missed. Treatment of an infected shunt involves removal of the shunt followed by a long course of antibiotics; missing or partially treating shunt infections can result in significant morbidity and potentially even mortality...
October 20, 2016: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/27572869/pediatric-multiple-sclerosis-conventional-first-line-treatment-and-general-management
#20
Angelo Ghezzi, Maria Pia Amato, Naila Makhani, Teri Shreiner, Jutta Gärtner, Silvia Tenembaum
Many disease-modifying therapies are currently available for adults with relapsing-remitting multiple sclerosis (MS) but none of them has been tested in pediatric MS in randomized placebo-controlled trials. At present, as suggested by observational studies and experts' guidelines, interferon-β and glatiramer acetate continue to be the standard first-line treatments for pediatric MS. Observational studies and some controlled unblinded trials have shown a positive effect of these meditations in reducing relapse rate and delaying disease progression, with an acceptable safety profile...
August 30, 2016: Neurology
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