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Neurology. Peds

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72 papers 25 to 100 followers
By M KKhan Pediatric Intensivist
https://www.readbyqxmd.com/read/28284393/epilepsy-in-inborn-errors-of-metabolism-with-therapeutic-options
#1
Jaume Campistol
Inborn errors of metabolism (IEM) are rare conditions that represent more than 1000 diseases, with a global prevalence of approximately 1:2000 individuals. Approximately, 40%-60% of IEM may present with epilepsy as one of the main neurologic signs. Epilepsy in IEM may appear at any age (fetal, newborn, infant, adolescent, or even adult). Different pathophysiological mechanisms may be responsible for the clinical phenotype, such as disturbances in energy metabolism (mitochondrial and fatty oxidation disorders, GLUT-1, and cerebral creatine deficiency), accumulation of complex molecules (lysosomal storage disorders), toxic mechanisms (organic acidurias and urea cycle disorders), or impairment of neurotransmission...
November 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28276062/ilae-classification-of-the-epilepsies-position-paper-of-the-ilae-commission-for-classification-and-terminology
#2
Ingrid E Scheffer, Samuel Berkovic, Giuseppe Capovilla, Mary B Connolly, Jacqueline French, Laura Guilhoto, Edouard Hirsch, Satish Jain, Gary W Mathern, Solomon L Moshé, Douglas R Nordli, Emilio Perucca, Torbjörn Tomson, Samuel Wiebe, Yue-Hua Zhang, Sameer M Zuberi
The International League Against Epilepsy (ILAE) Classification of the Epilepsies has been updated to reflect our gain in understanding of the epilepsies and their underlying mechanisms following the major scientific advances that have taken place since the last ratified classification in 1989. As a critical tool for the practicing clinician, epilepsy classification must be relevant and dynamic to changes in thinking, yet robust and translatable to all areas of the globe. Its primary purpose is for diagnosis of patients, but it is also critical for epilepsy research, development of antiepileptic therapies, and communication around the world...
April 2017: Epilepsia
https://www.readbyqxmd.com/read/28276064/instruction-manual-for-the-ilae-2017-operational-classification-of-seizure-types
#3
Robert S Fisher, J Helen Cross, Carol D'Souza, Jacqueline A French, Sheryl R Haut, Norimichi Higurashi, Edouard Hirsch, Floor E Jansen, Lieven Lagae, Solomon L Moshé, Jukka Peltola, Eliane Roulet Perez, Ingrid E Scheffer, Andreas Schulze-Bonhage, Ernest Somerville, Michael Sperling, Elza Márcia Yacubian, Sameer M Zuberi
This companion paper to the introduction of the International League Against Epilepsy (ILAE) 2017 classification of seizure types provides guidance on how to employ the classification. Illustration of the classification is enacted by tables, a glossary of relevant terms, mapping of old to new terms, suggested abbreviations, and examples. Basic and extended versions of the classification are available, depending on the desired degree of detail. Key signs and symptoms of seizures (semiology) are used as a basis for categories of seizures that are focal or generalized from onset or with unknown onset...
April 2017: Epilepsia
https://www.readbyqxmd.com/read/28128038/management-of-anti-n-methyl-d-aspartate-nmda-receptor-encephalitis-in-children
#4
Madhu Nagappa, S Bindu Parayil, Anita Mahadevan, Sanjib Sinha, Pavagada S Mathuranath, Arun B Taly
No abstract text is available yet for this article.
April 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28183838/autoimmune-encephalitis-pathophysiology-and-imaging-review-of-an-overlooked-diagnosis
#5
REVIEW
B P Kelley, S C Patel, H L Marin, J J Corrigan, P D Mitsias, B Griffith
Autoimmune encephalitis is a relatively new category of immune-mediated disease involving the central nervous system that demonstrates a widely variable spectrum of clinical presentations, ranging from the relatively mild or insidious onset of cognitive impairment to more complex forms of encephalopathy with refractory seizure. Due to its diverse clinical features, which can mimic a variety of other pathologic processes, autoimmune encephalitis presents a diagnostic challenge to clinicians. Imaging findings in patients with these disorders can also be quite variable, but recognizing characteristic findings within limbic structures suggestive of autoimmune encephalitis can be a key step in alerting clinicians to the potential diagnosis and ensuring a prompt and appropriate clinical work-up...
February 9, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28096104/recent-developments-in-the-surgical-management-of-paediatric-epilepsy
#6
REVIEW
Vijay M Ravindra, Matthew T Sweney, Robert J Bollo
Among the 1% of children affected by epilepsy, failure of pharmacological therapy and early age of seizure onset can lead to worse long-term cognitive outcomes, mental health disorders and impaired functional status. Surgical management often improves functional and cognitive outcomes in children with medically refractory epilepsy, especially when seizure remission is achieved. However, surgery remains underused in children with drug-resistant epilepsy, creating a large treatment gap. Several recent innovations have led to considerable improvement in surgical technique, including the recent development of minimally invasive diagnostic and therapeutic techniques such as stereotactic EEG, transcranial magnetic stimulation, MRI-guided laser ablation, as well as novel paradigms of neurostimulation...
January 17, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28232464/question-2-should-thrombolytic-agents-be-used-in-children-presenting-with-acute-ischaemic-stroke
#7
Dhinesh Baskaran, Nahin Hussain
No abstract text is available yet for this article.
April 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28224617/clinical-trials-in-childhood-stroke-from-consensus-to-reality
#8
Mitchell S V Elkind
No abstract text is available yet for this article.
February 22, 2017: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/28188465/dystonia-opportunities-to-gain-insights-into-underlying-pathophysiological-mechanisms
#9
K J Peall, N P Robertson
No abstract text is available yet for this article.
March 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28190433/seizures-in-the-critically-ill
#10
REVIEW
J Ch'ang, J Claassen
Critically ill patients with seizures are either admitted to the intensive care unit because of uncontrolled seizures requiring aggressive treatment or are admitted for other reasons and develop seizures secondarily. These patients may have multiorgan failure and severe metabolic and electrolyte disarrangements, and may require complex medication regimens and interventions. Seizures can be seen as a result of an acute systemic illness, a primary neurologic pathology, or a medication side-effect and can present in a wide array of symptoms from convulsive activity, subtle twitching, to lethargy...
2017: Handbook of Clinical Neurology
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
#11
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/28181921/feed-induced-dystonias-in-children-with-severe-central-nervous-system-disorders
#12
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/28120042/recent-advances-in-epilepsy
#13
Mark Manford
This paper reviews advances in epilepsy in recent years with an emphasis on therapeutics and underlying mechanisms, including status epilepticus, drug and surgical treatments. Lessons from rarer epilepsies regarding the relationship between epilepsy type, mechanisms and choice of antiepileptic drugs (AED) are explored and data regarding AED use in pregnancy are reviewed. Concepts evolving towards a move from treating seizures to treating epilepsy are discussed, both in terms of the mechanisms of epileptogenesis, and in terms of epilepsy's broader comorbidity, especially depression...
January 24, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28134012/treatment-of-epileptic-encephalopathies
#14
Hiroki Nariai, Susan Duberstein, Shlomo Shinnar
Childhood epileptic encephalopathies are age-dependent disorders of the brain whose hallmarks include loss of neurologic function over time, abnormal electroencephalographic findings, and seizures. Ictal and interictal electrographic activity are conjointly thought to be at the root of the often devastating neuropsychological deterioration, which is specific to the maturing brain. The goals of treatment are not only to control seizures, but also to prevent or reverse neurologic loss of function. In general, time is of the essence in diagnosis, and experienced specialists should promptly design a treatment plan...
January 1, 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/27741987/status-epilepticus-what-s-new
#15
REVIEW
Danya Khoujah, Michael K Abraham
The emergent evaluation and treatment of generalized convulsive status epilepticus presents challenges for emergency physicians. This disease is one of the few in which minutes can mean the difference between life and significant morbidity and mortality. It is imperative to use parallel processing and have multiple treatment options planned in advance, in case the current treatment is not successful. There is also benefit to exploring, or initiating, treatment algorithms to standardize the care for these critically ill patients...
November 2016: Emergency Medicine Clinics of North America
https://www.readbyqxmd.com/read/26906964/a-clinical-approach-to-diagnosis-of-autoimmune-encephalitis
#16
REVIEW
Francesc Graus, Maarten J Titulaer, Ramani Balu, Susanne Benseler, Christian G Bien, Tania Cellucci, Irene Cortese, Russell C Dale, Jeffrey M Gelfand, Michael Geschwind, Carol A Glaser, Jerome Honnorat, Romana Höftberger, Takahiro Iizuka, Sarosh R Irani, Eric Lancaster, Frank Leypoldt, Harald Prüss, Alexander Rae-Grant, Markus Reindl, Myrna R Rosenfeld, Kevin Rostásy, Albert Saiz, Arun Venkatesan, Angela Vincent, Klaus-Peter Wandinger, Patrick Waters, Josep Dalmau
Encephalitis is a severe inflammatory disorder of the brain with many possible causes and a complex differential diagnosis. Advances in autoimmune encephalitis research in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to these disorders. However, existing criteria for autoimmune encephalitis are too reliant on antibody testing and response to immunotherapy, which might delay the diagnosis. We reviewed the literature and gathered the experience of a team of experts with the aims of developing a practical, syndrome-based diagnostic approach to autoimmune encephalitis and providing guidelines to navigate through the differential diagnosis...
April 2016: Lancet Neurology
https://www.readbyqxmd.com/read/26319044/autoimmune-encephalitis-in-the-icu-analysis-of-phenotypes-serologic-findings-and-outcomes
#17
Manoj K Mittal, Alejandro A Rabinstein, Sara E Hocker, Sean J Pittock, Eelco F M Wijdicks, Andrew McKeon
BACKGROUND: To report the clinical and laboratory characteristics, clinical courses, and outcomes of Mayo Clinic, Rochester, MN, ICU-managed autoimmune encephalitis patients (January 1st 2003-December 31st 2012). METHODS: Based on medical record review, twenty-five patients were assigned to Group 1 (had ≥1 of classic autoimmune encephalitis-specific IgGs, n = 13) or Group 2 (had ≥3 other characteristics supporting autoimmunity, n = 12). RESULTS: Median admission age was 47 years (range 22-88); 17 were women...
April 2016: Neurocritical Care
https://www.readbyqxmd.com/read/27017022/complementary-and-integrative-approaches-for-pediatric-headache
#18
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/27017017/complicated-migraines
#19
REVIEW
Alyssa E Blumenfeld, M Cristina Victorio, Frank R Berenson
Migraines are a common paroxysmal disorder that may present with a multitude of neurologic symptoms. Migraines have been re-categorized in the most recent edition of the International Classification of Headache Disorders. In this article, we review the literature on hemiplegic migraines, alternating hemiplegia of childhood, migraine with brainstem aura, retinal migraine, ophthalmoplegic migraine, Alice in Wonderland syndrome, and acute confusional migraine. We also discuss the principal clinical features, diagnostic criteria, and treatment options for these disorders...
February 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27017016/migrainous-aura-visual-snow-and-alice-in-wonderland-syndrome-in-childhood
#20
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
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