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Neurology

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76 papers 25 to 100 followers
https://www.readbyqxmd.com/read/28214165/agenesis-of-the-corpus-callosum-and-aicardi-syndrome-a%C3%A2-neuroimaging-and-clinical-comparison
#1
T Govil-Dalela, A Kumar, R Agarwal, H T Chugani
BACKGROUND: Agenesis of the corpus callosum can occur in individuals with epilepsy, either in isolation or as part of various neurological conditions, such as Aicardi syndrome. In this study, we evaluated the clinical and neuroradiological differences between children with nonsyndromic agenesis of the corpus callosum and those with Aicardi syndrome. METHODS: We evaluated 31 children with epilepsy and agenesis of the corpus callosum (11 males, 20 females), 14 of whom had Aicardi syndrome (all females)...
March 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28029552/epilepsia-partialis-continua-a-review
#2
REVIEW
Rūta Mameniškienė, Peter Wolf
Epilepsia partialis contina (EPC) in a narrow definition is a variant of simple focal motor status epilepticus in which frequent repetitive muscle jerks, usually arrhythmic, continue over prolonged periods of time. In a broader definition (used in this review) it also includes non-motor manifestations otherwise known as aura continua. EPC may occur as a single episode, repetitive episodes, it may be chronic progressive or non-progressive. It appears as an unusual manifestation of epilepsy in which more typical paroxysmal events are partly or entirely replaced by the sustained repetition of seizure fragments in rapid succession...
January 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/27572861/pediatric-transverse-myelitis
#3
Michael Absoud, Benjamin M Greenberg, Ming Lim, Tim Lotze, Terrence Thomas, Kumaran Deiva
Pediatric acute transverse myelitis (ATM) is an immune-mediated CNS disorder and contributes to 20% of children experiencing a first acquired demyelinating syndrome (ADS). ATM must be differentiated from other presentations of myelopathy and may be the first presentation of relapsing ADS such as neuromyelitis optica (NMO) or multiple sclerosis (MS). The tenets of the diagnostic criteria for ATM established by the Transverse Myelitis Consortium Working Group can generally be applied in children; however, a clear sensory level may not be evident in some...
August 30, 2016: Neurology
https://www.readbyqxmd.com/read/27017016/migrainous-aura-visual-snow-and-alice-in-wonderland-syndrome-in-childhood
#4
REVIEW
Reena Gogia Rastogi, Juliana VanderPluym, Kara Stuart Lewis
Migraine is a condition that is common in the pediatric and adolescent population. Among children with migraine, visual aura can consist of either negative or positive features or both. Reports of sensory auras can also be elicited with a careful history. The understanding of the types of aura, as well as their relation to the more typical features of migraine, are discussed. The similar phenomena of visual snow and Alice in Wonderland syndrome in children are also described in detail.
February 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27017015/abdominal-migraine
#5
REVIEW
Paul Winner
Abdominal migraine is one of the episodic syndromes that may be associated with migraine, which often has its initial presentation in childhood. Recognition of these 2 conditions in the pediatric population is paramount to establish an appropriate diagnosis, treatment, and prevention. The aim of this article is to review the most recent International Classification of Headache Disorders-3 beta for abdominal migraine, and discuss the differential diagnosis and current suggested management.
February 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28181921/feed-induced-dystonias-in-children-with-severe-central-nervous-system-disorders
#6
Santosh Mordekar, Manjula Velayudhan, David I Campbell
Dystonias can arise from any painful stimuli in neurologically disabled children. Classically, feed induced dystonias from mediastinal pain due to severe gastroesophageal reflux disease are described as Sandifer's spasm. We report a case series of 12 severely neurologically impaired children with enteral feed induced dystonias. Intestinal dysmotility was demonstrated in several. Improvements are seen with jejunal feeds or gut rest with Total Parenteral Nutrition. Use of parenteral nutrition in children with severe neurodisability requires thorough discussion with patient groups and commissioners to give clinicians guidelines to standardise care...
February 8, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/21956720/evidence-report-genetic-and-metabolic-testing-on-children-with-global-developmental-delay-report-of-the-quality-standards-subcommittee-of-the-american-academy-of-neurology-and-the-practice-committee-of-the-child-neurology-society
#7
REVIEW
D J Michelson, M I Shevell, E H Sherr, J B Moeschler, A L Gropman, S Ashwal
OBJECTIVE: To systematically review the evidence concerning the diagnostic yield of genetic and metabolic evaluation of children with global developmental delay or intellectual disability (GDD/ID). METHODS: Relevant literature was reviewed, abstracted, and classified according to the 4-tiered American Academy of Neurology classification of evidence scheme. RESULTS AND CONCLUSIONS: In patients with GDD/ID, microarray testing is diagnostic on average in 7...
October 25, 2011: Neurology
https://www.readbyqxmd.com/read/24304390/status-dystonicus-a-practice-guide
#8
REVIEW
Nicholas M Allen, Jean-Pierre Lin, Tim Lynch, Mary D King
Status dystonicus is a rare, but life-threatening movement disorder emergency. Urgent assessment is required and management is tailored to patient characteristics and complications. The use of dystonia action plans and early recognition of worsening dystonia may potentially facilitate intervention or prevent progression to status dystonicus. However, for established status dystonicus, rapidly deployed temporizing measures and different depths of sedation in an intensive care unit or high dependency unit are the most immediate and effective modalities for abating life-threatening spasms, while dystonia-specific treatment takes effect...
February 2014: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/26492121/dyke-davidoff-masson-syndrome-an-unusual-cause-of-status-epilepticus
#9
Ifrah Zawar, Ashfa A Khan, Tipu Sultan, Ahsan W Rathore
The Dyke-Davidoff-Masson Syndrome (DDMS) results from an insult to the growing brain in utero or early infancy, which lead to loss of neurons compromising the growth of the brain. Clinical presentation includes seizures, hemiparesis, facial asymmetry, and learning disability. Radiological findings include cerebral atrophy on one side. Here, we present a case with status epilepticus who had underlying DDMS. It is a rare syndrome and uncommon cause for status epilepticus. Infections of CNS, hypoxic ischemic encephalopathy, intracranial bleed, trauma, congenital vascular malformations are the common causes of this syndrome...
October 2015: Neurosciences: the Official Journal of the Pan Arab Union of Neurological Sciences
https://www.readbyqxmd.com/read/27017022/complementary-and-integrative-approaches-for-pediatric-headache
#10
REVIEW
Sita Kedia
In this article, the use of complementary and integrative medicine for the management of pediatric headache is reviewed. Despite limited numbers of studies for pediatric headaches, children and families seek these services. Integrative medicine focuses on treating the whole person, integrating conventional medicine with mind-body-spirit methods. Nutriceuticals include dietary supplements in the form of vitamins (vitamin D), minerals (magnesium), coenzyme Q, butterbur, and melatonin. Acupuncture, stimulation, physical therapy and Transcutaneous Electrical Nerve Stimulations (TENS) or Transcranial Magnetic Stimulation (TMS) may also be useful in selected patients...
February 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28095027/severe-and-extensive-traumatic-axonal-injury-following-minor-and-indirect-head-trauma
#11
Sung Ho Jang, Han Do Lee
OBJECTIVES: This study reports on a patient with mild traumatic brain injury (TBI) who showed severe and extensive traumatic axonal injury (TAI) of various neural tracts following minor and indirect head trauma, which was demonstrated by diffusion tensor tractography (DTT). CASE DESCRIPTION: A 26-year-old female patient suffered from indirect head trauma resulting from flexion-hyperextension injury after being hit from behind by a slowly moving car. At the time of head trauma, she felt tingling sensation on her four extremities; however, she did not experience loss of consciousness...
2017: Brain Injury: [BI]
https://www.readbyqxmd.com/read/23858412/lipoid-proteinosis-with-bilateral-amygdalae-calcifications-headache-and-cognitive-impairments
#12
David Arkadir, Israela Lerer, Laurent Klapholz, Michael Halpert, J P Newman, J Moshe Gomori, Alexander Lossos
No abstract text is available yet for this article.
July 16, 2013: Neurology
https://www.readbyqxmd.com/read/23439709/teaching-neuroimages-lipoid-proteinosis-urbach-wiethe-disease-typical-findings-in-this-rare-genodermatosis
#13
Marcelo Bianco Quirici, Antonio J da Rocha
No abstract text is available yet for this article.
February 26, 2013: Neurology
https://www.readbyqxmd.com/read/26564090/lipoid-proteinosis
#14
REVIEW
John A Mcgrath
Lipoid proteinosis is a rare autosomal recessive disorder caused by mutations in ECM1, encoding extracellular matrix protein 1, a glycoprotein expressed in many organs and which has important protein-protein interactions in tissue homeostasis. Although the disease usually presents clinically with warty infiltration of the skin and mucous membranes and a hoarse voice, neuropsychological and neuropsychiatric abnormalities are often prominent features. There may be bean- or comma-shaped intracranial calcifications, often selectively affecting the amygdala...
2015: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/26600871/progressive-multifocal-leukoencephalopathy-current-treatment-options-and-future-perspectives
#15
REVIEW
Dejan Pavlovic, Andriani C Patera, Fredrik Nyberg, Marianne Gerber, Maggie Liu
Progressive multifocal leukoencephalopathy (PML) is a rare but debilitating and frequently fatal viral disease of the central nervous system, primarily affecting individuals with chronically and severely suppressed immune systems. The disease was relatively obscure until the outbreak of HIV/AIDS, when it presented as one of the more frequent opportunistic infections in this immune deficiency syndrome. It attracted additional attention from the medical and scientific community following the discovery of significant PML risk associated with natalizumab, a monoclonal antibody used for treatment of relapsing-remitting multiple sclerosis...
November 2015: Therapeutic Advances in Neurological Disorders
https://www.readbyqxmd.com/read/28076964/trigeminal-neuralgia-diagnosis-and-treatment
#16
Stine Maarbjerg, Giulia Di Stefano, Lars Bendtsen, Giorgio Cruccu
Introduction Trigeminal neuralgia (TN) is characterized by touch-evoked unilateral brief shock-like paroxysmal pain in one or more divisions of the trigeminal nerve. In addition to the paroxysmal pain, some patients also have continuous pain. TN is divided into classical TN (CTN) and secondary TN (STN). Etiology and pathophysiology Demyelination of primary sensory trigeminal afferents in the root entry zone is the predominant pathophysiological mechanism. Most likely, demyelination paves the way for generation of ectopic impulses and ephaptic crosstalk...
January 1, 2017: Cephalalgia: An International Journal of Headache
https://www.readbyqxmd.com/read/27396529/notalgia-paresthetica-and-multiple-endocrine-neoplasia-syndrome-2a-a-case-report
#17
Francisco Alcántara, Marta Feito, Fátima Albizuri, María Beato, Raúl De Lucas
Notalgia paresthetica is characterized by a hyperpigmented macular pruritic skin lesion most commonly localized unilaterally in the middle and upper back region. This condition has been reported in association with multiple endocrine neoplasia syndrome type 2A (MEN 2A) in several families; it rarely affects children and it may serve as an early marker of MEN 2A. We report a 9-year-old girl diagnosed with MEN 2A and notalgia paresthetica.
September 2016: Pediatric Dermatology
https://www.readbyqxmd.com/read/28095967/intravenous-immunoglobulin-therapy-in-pediatric-narcolepsy-a-nonrandomized-open-label-controlled-longitudinal-observational-study
#18
Michel Lecendreux, Johanna Berthier, Jennifer Corny, Olivier Bourdon, Claire Dossier, Christophe Delclaux
STUDY OBJECTIVES: Previous case reports of intravenous immunoglobulins (IVIg) in pediatric narcolepsy have shown contradictory results. METHODS: This was a nonrandomized, open-label, controlled, longitudinal observational study of IVIg use in pediatric narcolepsy with retrospective data collection from medical files obtained from a single pediatric national reference center for the treatment of narcolepsy in France. Of 56 consecutively referred patients with narcolepsy, 24 received IVIg (3 infusions administered at 1-mo intervals) in addition to standard care (psychostimulants and/or anticataplectic agents), and 32 continued on standard care alone (controls)...
January 11, 2017: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
https://www.readbyqxmd.com/read/28073790/clemizole-and-modulators-of-serotonin-signalling-suppress-seizures-in-dravet-syndrome
#19
Aliesha Griffin, Kyla R Hamling, Kelly Knupp, SoonGweon Hong, Luke P Lee, Scott C Baraban
Dravet syndrome is a catastrophic childhood epilepsy with early-onset seizures, delayed language and motor development, sleep disturbances, anxiety-like behaviour, severe cognitive deficit and an increased risk of fatality. It is primarily caused by de novo mutations of the SCN1A gene encoding a neuronal voltage-activated sodium channel. Zebrafish with a mutation in the SCN1A homologue recapitulate spontaneous seizure activity and mimic the convulsive behavioural movements observed in Dravet syndrome. Here, we show that phenotypic screening of drug libraries in zebrafish scn1 mutants rapidly and successfully identifies new therapeutics...
January 10, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28045676/therapeutic-potential-of-flavonoids-in-spinal-cord-injury
#20
Peng Zhang, Christian Hölscher, Xun Ma
Spinal cord injury (SCI) is a catastrophic event that can profoundly affect a patient's life, with far-reaching social and economic effects. A consequential sequence of SCI is the significant neurological or psychological deficit, which obviously contributes to the overall burden of this condition. To date, there is no effective treatment for SCI. Therefore, developing novel therapeutic strategies for SCI is highly prioritized. Flavonoids, one of the most numerous and ubiquitous groups of plant metabolites, are the active ingredients of traditional Chinese medicine such as Scutellaria baicalensis Georgi (Huang Qin) or Ginkgo biloba (Ying Xin)...
January 1, 2017: Reviews in the Neurosciences
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