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Neurology. Peds

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40 papers 0 to 25 followers
https://www.readbyqxmd.com/read/27872179/anti-n-methyl-d-aspartate-nmda-receptor-encephalitis-a-survey-of-treatment-progress-and-prospects-from-pediatric-neurologists
#1
Ilana Kahn, Guy Helman, Adeline Vanderver, Elizabeth Wells
Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis has been shown to be a treatable form of autoimmune encephalitis, but there remains no standardized approach to immunotherapy. We designed an anonymous survey sent to members of the Child Neurology Society to identify the current practices among child neurologists. A total of 151 pediatric neurologists responded to the survey. With these responses we were able to highlight areas of practice uniformity, including first-line treatment with intravenous immunoglobulin and intravenous methylprednisone and initiation of disease-modifying therapy with rituximab alone...
November 21, 2016: Journal of Child Neurology
https://www.readbyqxmd.com/read/27880878/neurocysticercosis-infection-and-disease-a-review
#2
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...
November 20, 2016: Acta Tropica
https://www.readbyqxmd.com/read/27862206/malformations-of-cortical-development
#3
REVIEW
Rahul S Desikan, A James Barkovich
Malformations of cortical development (MCDs) compose a diverse range of disorders that are common causes of neurodevelopmental delay and epilepsy. With improved imaging and genetic methodologies, the underlying molecular and pathobiological characteristics of several MCDs have been recently elucidated. In this review, we discuss genetic and molecular alterations that disrupt normal cortical development, with emphasis on recent discoveries, and provide detailed radiological features of the most common and important MCDs...
November 11, 2016: Annals of Neurology
https://www.readbyqxmd.com/read/27853394/long-term-impact-after-fulminant-guillain-barr%C3%A3-syndrome-case-report-and-literature-review
#4
Alain Rougé, Jérémie Lemarié, Sébastien Gibot, Pierre Edouard Bollaert
A 47-year-old man was admitted to the intensive care unit a few hours after presenting to emergency department with acute diplopia and dysphonia. Swallowing disorders and respiratory muscular weakness quickly required invasive ventilation. On day 3, the patient was in a "brain-death"-like state with deep coma and absent brainstem reflexes. Electroencephalogram ruled out brain death diagnosis as a paradoxical sleep trace was recorded. Cerebrospinal fluid analysis, electrophysiologic studies, and a recent history of diarrhea led to the diagnosis of Campylobacter jejuni-related fulminant Guillain-Barré syndrome (GBS) mimicking brain death...
2016: International Medical Case Reports Journal
https://www.readbyqxmd.com/read/27789461/hypopituitarism-in-children-with-cerebral-palsy
#5
REVIEW
Suma Uday, Nick Shaw, Ruth Krone, Jeremy Kirk
Poor growth and delayed puberty in children with cerebral palsy is frequently felt to be related to malnutrition. Although growth hormone deficiency is commonly described in these children, multiple pituitary hormone deficiency (MPHD) has not been previously reported. We present a series of four children with cerebral palsy who were born before 29 weeks gestation who were referred to the regional endocrinology service, three for delayed puberty and one for short stature, in whom investigations identified MPHD...
October 27, 2016: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/27843091/cause-of-death-in-children-with-mitochondrial-diseases
#6
Soyong Eom, Ha Neul Lee, Sunho Lee, Hoon-Chul Kang, Joon Soo Lee, Heung Dong Kim, Young-Mock Lee
BACKGROUND: We investigated the clinical characteristics that represent risk factors for death in pediatric patients with mitochondrial diseases. METHODS: The medical records of mitochondrial disease pediatric patients attended between 2006 and 2015 (n = 221) were reviewed for clinical characteristics, diagnosis, hospitalization, follow-up, survival, and cause of death. RESULTS: The global mortality rate in the cohort was 14% (average age at death, six years)...
October 13, 2016: Pediatric Neurology
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
#7
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/27647645/insomnia-parasomnias-and-narcolepsy-in-children-clinical-features-diagnosis-and-management
#8
REVIEW
Kiran Maski, Judith A Owens
Sleep problems are frequently encountered as presenting complaints in child neurology clinical practice. They can affect the functioning and quality of life of children, particularly those with primary neurological and neurodevelopmental disorders, since coexisting sleep problems can add substantially to neurocognitive and behavioural comorbidities. Additionally, symptoms of some sleep disorders such as parasomnias and narcolepsy can be confused with those of other neurological disorders (eg, epilepsy), posing diagnostic challenges for paediatric neurologists...
October 2016: Lancet Neurology
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
#9
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/27789117/acute-ataxia-in-children-a-review-of-the-differential-diagnosis-and-evaluation-in-the-emergency-department
#10
REVIEW
Mauro Caffarelli, Amir A Kimia, Alcy R Torres
Acute ataxia in a pediatric patient poses a diagnostic dilemma for any physician. While the most common etiologies are benign, occasional individuals require urgent intervention. Children with stroke, toxic ingestion, infection, and neuro-inflammatory disorders frequently exhibit ataxia as an essential-if not the only-presenting feature. The available retrospective research utilize inconsistent definitions of acute ataxia, precluding the ability to pool data from these studies. No prospective data exist that report on patients presenting to the emergency department with ataxia...
December 2016: Pediatric Neurology
https://www.readbyqxmd.com/read/27803143/stroke-in-neonates-and-children
#11
Miya E Bernson-Leung, Michael J Rivkin
No abstract text is available yet for this article.
November 2016: Pediatrics in Review
https://www.readbyqxmd.com/read/27803144/pediatric-hydrocephalus-current-state-of-diagnosis-and-treatment
#12
Zachary Wright, Thomas W Larrew, Ramin Eskandari
No abstract text is available yet for this article.
November 2016: Pediatrics in Review
https://www.readbyqxmd.com/read/27720246/extra-central-nervous-system-target-for-assessment-and-treatment-in-refractory-anti-n-methyl-d-aspartate-receptor-encephalitis
#13
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...
September 22, 2016: Journal of Critical Care
https://www.readbyqxmd.com/read/27660176/guillain-barr%C3%A3-syndrome
#14
Eelco F M Wijdicks
No abstract text is available yet for this article.
October 2016: Neurocritical Care
https://www.readbyqxmd.com/read/26118918/seizures-in-sleep-clinical-spectrum-diagnostic-features-and-management
#15
REVIEW
Dawn Eliashiv, Alon Y Avidan
Sleep is disrupted in most patients hospitalized in the intensive care unit and the disturbances are even more profound in patients impacted by epilepsy. Nocturnal seizures must be differentiated from other common nocturnal events, such as delirium, parasomnias, and sedation. Many antiepileptic drugs produce undesirable side effects on sleep architecture that may further predispose patients to insomnia during the night and excessive sedation and hypersomnolence during the day. Failure to recognize, correctly diagnose, and adequately manage these disturbances may lead to more prolonged hospitalization, increased risk for nosocomial infections, poorer health-related qualify of life, and greater health care financial burden...
July 2015: Critical Care Clinics
https://www.readbyqxmd.com/read/27572869/pediatric-multiple-sclerosis-conventional-first-line-treatment-and-general-management
#16
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
https://www.readbyqxmd.com/read/27765754/fever-in-a-child-with-cerebrospinal-fluid-access-device-or-shunt-a-pragmatic-approach-to-management
#17
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/27758849/sensory-input-and-motor-responses-after-brain-death-diagnosis-a-word-of-caution
#18
Mohamed Y Rady, Joseph L Verheijde
No abstract text is available yet for this article.
October 6, 2016: Journal of Intensive Care Medicine
https://www.readbyqxmd.com/read/25664241/spectrum-of-complicated-migraine-in-children-a-common-profile-in-aid-to-clinical-diagnosis
#19
REVIEW
Surya N Gupta, Vikash S Gupta, Dawn M Fields
Complicated migraine encompasses several individual clinical syndromes of migraine. Such a syndrome in children frequently presents with various neurological symptoms in the Emergency Department. An acute presentation in the absence of headache presents a diagnostic challenge. A delay in diagnosis and treatment may have medicolegal implication. To date, there are no reports of a common clinical profile proposed in making a clinical diagnosis for the complicated migraine. In this clinical review, we propose and describe: (1) A common clinical profile in aid to clinical diagnosis for spectrum of complicated migraine; (2) How it can be used in differentiating complicated migraine from migraine without aura, migraine with aura, and seizure; (3) We discuss the status of complicated migraine in the International Headache Society classification 2013; and (4) In addition, a common treatment strategy for the spectrum of migraine has been described...
February 8, 2015: World Journal of Clinical Pediatrics
https://www.readbyqxmd.com/read/26060905/rasmussen-syndrome-and-other-inflammatory-epilepsies
#20
REVIEW
Sophia Varadkar, J Helen Cross
An underlying immune basis is emerging in an increasing number of epileptic and encephalopathic syndromes. The immunopathological mechanisms may be categorized into antibody-mediated, T-cell cytotoxicity, and microglia-induced degeneration. The immune basis in Rasmussen syndrome is thought to be T-cell mediated. Antibodies to extracellular and intracellular epitopes are implicated in limbic and other encephalitides, characterized by seizures, movement disorder, sleep disorder, obtundation, psychosis, mutism, and other psychiatric symptoms...
June 2015: Seminars in Neurology
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