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Neurology. Peds

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101 papers 25 to 100 followers
By M KKhan Pediatric Intensivist
https://www.readbyqxmd.com/read/28972277/high-sensitivity-and-specificity-in-proposed-clinical-diagnostic-criteria-for-anti-n-methyl-d-aspartate-receptor-encephalitis
#1
Alvin C C Ho, Shekeeb S Mohammad, Sekhar C Pillai, Esther Tantsis, Hannah Jones, Reena Ho, Ming Lim, Yael Hacohen, Angela Vincent, Russell C Dale
AIM: To determine the validity of the proposed clinical diagnostic criteria for anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis in paediatric patients. METHOD: The diagnostic criteria for anti-NMDAR encephalitis proposed by Graus et al. (2016) use clinical features and conventional investigations to facilitate early immunotherapy before antibody status is available. The criteria are satisfied if patients develop four out of six symptom groups within 3 months, together with at least one abnormal investigation (electroencephalography/cerebrospinal fluid) and reasonable exclusion of other disorders...
October 3, 2017: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/28968363/guillain-barr%C3%A3-syndrome
#2
Peter D Donofrio
PURPOSE OF REVIEW: This article reviews the current state of Guillain-Barré syndrome (GBS), including its clinical presentation, evaluation, pathophysiology, and treatment. RECENT FINDINGS: GBS is an acute/subacute-onset polyradiculoneuropathy typically presenting with sensory symptoms and weakness over several days, often leading to quadriparesis. Approximately 70% of patients report a recent preceding upper or lower respiratory tract infection or gastrointestinal illness...
October 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/28967695/intravenous-immunoglobulin-for-the-treatment-of-childhood-encephalitis
#3
REVIEW
Mildred A Iro, Natalie G Martin, Michael Absoud, Andrew J Pollard
BACKGROUND: Encephalitis is a syndrome of neurological dysfunction due to inflammation of the brain parenchyma, caused by an infection or an exaggerated host immune response, or both. Attenuation of brain inflammation through modulation of the immune response could improve patient outcomes. Biological agents such as immunoglobulin that have both anti-inflammatory and immunomodulatory properties may therefore be useful as adjunctive therapies for people with encephalitis. OBJECTIVES: To assess the efficacy and safety of intravenous immunoglobulin (IVIG) as add-on treatment for children with encephalitis...
October 2, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28638987/leukodystrophies-a-proposed-classification-system-based-on-pathological-changes-and-pathogenetic-mechanisms
#4
REVIEW
Marjo S van der Knaap, Marianna Bugiani
Leukodystrophies are genetically determined disorders characterized by the selective involvement of the central nervous system white matter. Onset may be at any age, from prenatal life to senescence. Many leukodystrophies are degenerative in nature, but some only impair white matter function. The clinical course is mostly progressive, but may also be static or even improving with time. Progressive leukodystrophies are often fatal, and no curative treatment is known. The last decade has witnessed a tremendous increase in the number of defined leukodystrophies also owing to a diagnostic approach combining magnetic resonance imaging pattern recognition and next generation sequencing...
June 21, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28833051/the-end-of-spasticity
#5
EDITORIAL
Bernard Dan
No abstract text is available yet for this article.
September 2017: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/28870420/value-of-protein-concentration-in-cerebrospinal-fluid-in-paediatric-patients-with-guillain-barre-syndrome
#6
Martha Esther Vidrio-Becerra, Jaime Valle-Leal, María Enriqueta Loaiza-Sarabia, Lucia Alvarez-Bastidas, Jesus Ignacio Lachica-Valle, Cruz Mónica López-Morales
INTRODUCTION AND OBJECTIVE: The albumin-cytologic dissociation in cerebrospinal fluid (CSF) supports the diagnosis of Guillain-Barre syndrome (GBS) but does not support the prognosis, so the aim of this study is to determine the usefulness of protein numbers in the CSF to predict progression in paediatric patients. PATIENTS AND METHODS: A diagnostic test was performed in paediatric patients with GBS, analysing sociodemographic, clinical and protein variables in CSF as well as electromyography...
September 1, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28904568/pediatric-autoimmune-encephalitis
#7
REVIEW
Massimo Barbagallo, Giovanna Vitaliti, Piero Pavone, Catia Romano, Riccardo Lubrano, Raffaele Falsaperla
Autoimmune (antibody mediated) encephalitis (AE) is emerging as a more common cause of pediatric encephalopathy than previously thought. The autoimmune process may be triggered by an infection, vaccine, or occult neoplasm. In the latter case, onconeural autoantibodies are directed against intracellular neuronal antigens, but a recent heterogeneous group of encephalitic syndromes has been found not to have underlying tumor but is associated with autoantibodies to the neuronal surface or synaptic antigens. Neuropsychiatric symptoms are very common in autoimmune encephalopathy; as a result, affected children may be initially present to psychiatrists...
April 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28844551/safety-and-efficacy-of-high-dose-enteral-intravenous-and-transdermal-clonidine-for-the-acute-management-of-severe-intractable-childhood-dystonia-and-status-dystonicus-an-illustrative-case-series
#8
V Nakou, K Williamson, T Arichi, D E Lumsden, Steve Tomlin, M Kaminska, J-P Lin
OBJECTIVE: Acute dystonia in children is distressing, painful and can progress to life-threatening status dystonicus. Typical management involves benzodiazepines which can result in respiratory depression requiring PICU admission. Clonidine is less respiratory-depressant, and by facilitating sleep, switches dystonia off. It can also be administered via enteral, continuous intravenous infusion, and transdermal slow release routes. We describe the dose range and safety profile of clonidine management in a case-series of children with severe acute exacerbation of dystonia in a tertiary hospital setting...
November 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28667183/occipital-headaches-and-neuroimaging-in-children
#9
Joshua J Bear, Amy A Gelfand, Peter J Goadsby, Nancy Bass
OBJECTIVE: To investigate the common thinking, as reinforced by the International Classification of Headache Disorders, 3rd edition (beta), that occipital headaches in children are rare and suggestive of serious intracranial pathology. METHODS: We performed a retrospective chart review cohort study of all patients ≤18 years of age referred to a university child neurology clinic for headache in 2009. Patients were stratified by headache location: solely occipital, occipital plus other area(s) of head pain, or no occipital involvement...
August 1, 2017: Neurology
https://www.readbyqxmd.com/read/28798922/practical-approach-to-the-patient-with-acute-neuromuscular-weakness
#10
REVIEW
Rajeev Nayak
Acute neuromuscular paralysis (ANMP) is a clinical syndrome characterized by rapid onset muscle weakness progressing to maximum severity within several days to weeks (less than 4 wk). Bulbar and respiratory muscle weakness may or may not be present. It is a common neurological emergency which requires immediate and careful investigations to determine the etiology because accurate diagnosis has significant impact on therapy and prognosis. Respiratory failure caused by neuromuscular weakness is considered as more critical than lung disease because its development may be insidious or subtle until sudden decompensation leads to life threatening hypoxia...
July 16, 2017: World Journal of Clinical Cases
https://www.readbyqxmd.com/read/28738441/neuromuscular-disorders-in-children-and-adolescents
#11
EDITORIAL
Rudolf Korinthenberg
No abstract text is available yet for this article.
August 2017: Neuropediatrics
https://www.readbyqxmd.com/read/27920282/nmda-receptor-encephalitis-and-other-antibody-mediated-disorders-of-the-synapse-the-2016-cotzias-lecture
#12
REVIEW
Josep Dalmau
Investigations during the last 10 years have revealed a group of disorders mediated by antibodies against ion channels and synaptic receptors, which cause both neurologic and psychiatric symptoms. In this review, I discuss the process of discovery and immunologic triggers of these disorders, and use anti-NMDA receptor encephalitis to emphasize the importance of understanding the underlying physiopathologic mechanisms in those diseases. A better knowledge of these mechanisms reveals points of convergence with other disorders (e...
December 6, 2016: Neurology
https://www.readbyqxmd.com/read/28506503/extended-clinical-spectrum-of-anti-n-methyl-d-aspartate-receptor-encephalitis-in-children-a-case-series
#13
Ajay Goenka, Vivek Jain, Hiroki Nariai, Alfred Spiro, Mitchell Steinschneider
OBJECTIVE: There is a wide spectrum of clinical manifestations in children with anti-N-methyl-d-aspartate (NMDA) receptor antibody encephalitis from two different health care settings. METHODS: We describe our experience with 13 patients (median age, 7 years; range, 5 months to 19 years) presenting to tertiary referral centers in India and the United States. RESULTS: Initial manifestations were neurological (seizures or movement disorders) in eight patients, and psychiatric (e...
July 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28599358/understanding-the-autonomic-nervous-system-in-cerebral-palsy
#14
EDITORIAL
Bernard Dan
No abstract text is available yet for this article.
July 2017: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/28389587/l-dopa-in-dystonia-a-modern-perspective
#15
REVIEW
Roderick P P W M Maas, Tessa Wassenberg, Jean-Pierre Lin, Bart P C van de Warrenburg, Michèl A A P Willemsen
"Every child exhibiting dystonia merits an l-dopa trial, lest the potentially treatable condition of dopa-responsive dystonia (DRD) is missed" has been a commonly cited and highly conserved adage in movement disorders literature stemming from the 1980s. We here provide a historical perspective on this statement, discuss the current diagnostic and therapeutic applications of l-dopa in everyday neurologic practice, contrast these with its approved indications, and finish with our view on both a diagnostic and therapeutic trial in children and adults with dystonia...
May 9, 2017: Neurology
https://www.readbyqxmd.com/read/27940011/pediatric-intracranial-hypertension
#16
REVIEW
Shawn C Aylward, Rachel E Reem
Primary (idiopathic) intracranial hypertension has been considered to be a rare entity, but with no precise estimates of the pediatric incidence in the United States. There have been attempts to revise the criteria over the years and adapt the adult criteria for use in pediatrics. The clinical presentation varies with age, and symptoms tending to be less obvious in younger individuals. In the prepubertal population, incidentally discovered optic disc edema is relatively common. By far the most consistent symptom is headache; other symptoms include nausea, vomiting tinnitus, and diplopia...
January 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/27857121/guillain-barr%C3%A3-syndrome-a-century-of-progress
#17
REVIEW
John A Goodfellow, Hugh J Willison
In 1916, Guillain, Barré and Strohl reported on two cases of acute flaccid paralysis with high cerebrospinal fluid protein levels and normal cell counts - novel findings that identified the disease we now know as Guillain-Barré syndrome (GBS). 100 years on, we have made great progress with the clinical and pathological characterization of GBS. Early clinicopathological and animal studies indicated that GBS was an immune-mediated demyelinating disorder, and that severe GBS could result in secondary axonal injury; the current treatments of plasma exchange and intravenous immunoglobulin, which were developed in the 1980s, are based on this premise...
December 2016: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/27894444/unusual-structural-autonomic-disorders-presenting-in-pediatrics-disorders-associated-with-hypoventilation-and-autonomic-neuropathies
#18
REVIEW
Gisela Chelimsky, Thomas Chelimsky
Structural autonomic disorders (producing structural damage to the autonomic nervous system or autonomic centers) are far less common than functional autonomic disorders (reflected in abnormal function of a fundamentally normal autonomic nervous system) in children and teenagers. This article focuses on this uncommon first group in the pediatric clinic. These disorders are grouped into 2 main categories: those characterized by hypoventilation and those that feature an autonomic neuropathy.
February 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/27894447/differentiating-familial-neuropathies-from-guillain-barr%C3%A3-syndrome
#19
REVIEW
Brett J Bordini, Priya Monrad
Differentiating Guillain-Barré syndrome (GBS) from inherited neuropathies and other acquired peripheral neuropathies requires understanding the atypical presentations of GBS and its variant forms, as well as historical and physical features suggestive of inherited neuropathies. GBS is typically characterized by the acute onset of ascending flaccid paralysis, areflexia, and dysesthesia secondary to peripheral nerve fiber demyelination. The disorder usually arises following a benign gastrointestinal or respiratory illness, is monophasic, reaches a nadir with several weeks, and responds to immunomodulatory therapy...
February 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/27894452/immune-mediated-diseases-of-the-central-nervous-system-a-specificity-focused-diagnostic-paradigm
#20
REVIEW
Dominic O Co, Brett J Bordini, Arthur B Meyers, Christopher Inglese
Immune-mediated diseases of the central nervous system show wide variability both symptomatically and with respect to underlying pathophysiology. Recognizing aberrant immunologic activity as the cause of neurologic dysfunction requires establishing as precise a neuroanatomic and functional phenotype as possible, and a diagnostic and therapeutic strategy that stabilizes the patient, excludes broad categories of disease via rapidly available diagnostic assays, and maintains a broad differential diagnosis that includes immune-mediated conditions...
February 2017: Pediatric Clinics of North America
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