Abstract:
:The objective of this study is to determine the long-term outcome of children with intractable epilepsy who have diffuse cortical hypometabolism on 2-deoxy-2-((18)F)fluoro-D-glucose positron emission tomography (FDG-PET) scans. Seventeen children with intractable epilepsy showing bilateral, diffuse cortical hypometabolism on FDG-PET were followed up through telephone interview from 1 year 4 months to 11 years 4 months (mean: 5 years 7 months ± 2 years 1 month) after their PET scans. One child succumbed to Sanfilippo disease at age 20 years. Only 2 children were seizure free. Fifty percent had walking difficulties, 56.25% were not toilet trained, all had speech difficulties, 43.75% had behavioral problems, 37.5% had poor eye contact, 75% had socialization difficulties, and 87.5% attended special schools. Three children were found to have genetic causes, including a 4-MB deletion of the mitochondrial genome, MECP2 duplication, and Lafora disease. In conclusion, the long-term outcome in this patient population is poor, and they tend to suffer from genetic/neurodegenerative diseases.
journal_name
J Child Neuroljournal_title
Journal of child neurologyauthors
Shandal V,Veenstra AL,Behen M,Sundaram S,Chugani Hdoi
10.1177/0883073811416363subject
Has Abstractpub_date
2012-01-01 00:00:00pages
39-45issue
1eissn
0883-0738issn
1708-8283pii
0883073811416363journal_volume
27pub_type
杂志文章abstract:BACKGROUND:We developed and validated a Spanish seizure screen for children based on a previously validated English seizure screen that could be administered by a trained research assistant in a 2-step process, approximating the clinical diagnostic process of a pediatric epilepsy specialist. This questionnaire was desi...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073820978001
更新日期:2020-12-18 00:00:00
abstract::Abnormal zinc metabolism in a 6-year-old male patient with Landau-Kleffner syndrome (acquired aphasia and convulsive disorder) is the subject of our report. We describe a significant decrease of erythrocyte and plasma zinc levels in the patient as compared with normal. Red blood cell zinc content is normally 38.5 +/- ...
journal_title:Journal of child neurology
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abstract:OBJECTIVE:Intravenous immunoglobulin and plasma exchange are proven treatments for Guillain-Barré syndrome. Despite these treatments, the prognosis for severe Guillain-Barré syndrome is still not satisfactory. This article seeks for a logical timing for plasma exchange-intravenous immunoglobulin synergy, which may impr...
journal_title:Journal of child neurology
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更新日期:2019-04-01 00:00:00
abstract:BACKGROUND:Rufinamide, a triazole derivative, is a new-generation antiseizure medication with a novel mechanism of action. We evaluated the efficacy and safety of rufinamide treatment in children with epilepsy younger than 4 years at our center. METHODS:In this retrospective study, we included children younger than 4 ...
journal_title:Journal of child neurology
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doi:10.1177/0883073820967159
更新日期:2021-03-01 00:00:00
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journal_title:Journal of child neurology
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doi:10.1177/7010.2006.00227
更新日期:2006-12-01 00:00:00
abstract::The purpose of this study was to assess the cognitive development of 27 children with nonhemorrhagic neonatal stroke (occurring within the first 28 days of life). The cognitive evaluation consisted of the Bayley Scales of Infant Development, administered at 12 and/or 24 months poststroke. Compared with the normative s...
journal_title:Journal of child neurology
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doi:10.1177/0883073807305784
更新日期:2007-09-01 00:00:00
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journal_title:Journal of child neurology
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abstract::Elevation and blur of the optic disc margin with hyperemia and flame hemorrhages are classic features of papilledema that may not be present with mild elevations of the cerebral spinal fluid pressure. In children, the disc can be dramatically elevated with indistinct margins in pseudopapilledema. Children with equivoc...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073807304192
更新日期:2007-07-01 00:00:00
abstract::Seizures occur in 25% to 40% of children with supratentorial tumors and are the presenting complaint in 10% to 15%. However, when divided by age, only 2% of children with seizures as the presenting complaint of brain tumors were less than 1 year of age. Three children, ranging in age from 20 days to 7 months and seen ...
journal_title:Journal of child neurology
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更新日期:1987-07-01 00:00:00
abstract::Attention-deficit hyperactivity disorder (ADHD) is described as the most common neurobehavioral condition of childhood. We raise the concern that ADHD is not a disease per se but rather a group of symptoms representing a final common behavioral pathway for a gamut of emotional, psychological, and/or learning problems....
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journal_title:Journal of child neurology
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doi:10.1177/0883073807304014
更新日期:2007-06-01 00:00:00
abstract::Mercury poisoning is a rare but fatal toxicologic emergency. Neurologic manifestations involving the central nervous system are seen usually with chronic mercury intoxication. The most commonly seen complaints are headache, tremor, impaired cognitive skills, weakness, muscle atrophy, and paresthesia. Here, we present ...
journal_title:Journal of child neurology
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doi:10.1177/0883073814538503
更新日期:2015-05-01 00:00:00
abstract::Nonconvulsive status epilepticus is a prolonged and continuous state of increased unawareness without overt motor seizures linked with repetitive generalized epileptic discharges. In children, it may occur de novo but more commonly may complicate a preexisting epileptic disorder. We report on a 2-year-old female who p...
journal_title:Journal of child neurology
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doi:10.1177/088307380101600310
更新日期:2001-03-01 00:00:00
abstract::Acute necrotizing encephalopathy is characterized by fever, seizures, acute encephalopathy, and rapid progression to coma. It is usually associated with viral illness and shows characteristic brain magnetic resonance imaging features, including symmetrical involvement of bilateral thalami, brain stem, white matter, an...
journal_title:Journal of child neurology
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doi:10.1177/0883073811435240
更新日期:2012-10-01 00:00:00
abstract::Computerized neurocognitive testing has become a growing practice across medical populations, but particularly within sports medicine and the management of sports-related concussion. Although traditional neuropsychological measures are solely administered and interpreted by neuropsychologists, computerized cognitive t...
journal_title:Journal of child neurology
pub_type: 杂志文章,评审
doi:10.1177/0883073814559645
更新日期:2016-01-01 00:00:00
abstract::It was recently postulated that because increased genetic load and increased parental age are both purportedly associated with the risk to develop an autism spectrum disorder, there must be a linkage between increasing genetic load and increasing parental age in autism spectrum disorder pathogenesis. The present study...
journal_title:Journal of child neurology
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doi:10.1177/0883073814541478
更新日期:2014-08-27 00:00:00
abstract::There are conflicting results concerning bone metabolism in children receiving antiepileptic medication, with data concentrating on neurologically impaired patients. We performed a multicenter cross-sectional study in otherwise healthy children who received monotherapy with valproic acid, oxcarbazepine, lamotrigine, s...
journal_title:Journal of child neurology
pub_type: 杂志文章,多中心研究
doi:10.1177/0883073812443005
更新日期:2013-02-01 00:00:00
abstract::There are scarce reports of childhood stroke from China. Our objective was to describe the clinical spectrum, risk factors, and imaging characteristics of childhood stroke in China. Using a hospital discharge database, children with stroke who were first admitted from 2002 to 2011 were retrospectively enrolled. We ide...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073814538667
更新日期:2015-03-01 00:00:00
abstract::Childhood disintegrative disorder, a rare, relentlessly progressive neurologic disorder, first described by Heller in 1908, remains a condition of great interest. It has long been debated whether it is a discrete disorder or simply a late-onset variant of childhood autism. We have studied 6 cases of childhood disinteg...
journal_title:Journal of child neurology
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doi:10.1177/0883073812472391
更新日期:2013-12-01 00:00:00
abstract::The clinical label attention deficit hyperactivity disorder (ADHD) suggests that this syndrome is a disorder of attention. However, the presumed attentional deficits have not been linked either to specific cognitive operations or to specific neural systems. To provide this link, theories of the cognitive anatomy of at...
journal_title:Journal of child neurology
pub_type: 杂志文章,评审
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更新日期:1991-01-01 00:00:00
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journal_title:Journal of child neurology
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journal_title:Journal of child neurology
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更新日期:1995-01-01 00:00:00
abstract::High-dose phenobarbital therapy is an effective treatment for refractory status epilepticus in children. The advantages of this therapy include milder adverse effects without limits for maximal phenobarbital levels or doses during the initial phase of treatment. However, little is known about the safety of continuing ...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073808331360
更新日期:2009-12-01 00:00:00
abstract::The outcomes of children with cryptogenic seizures most probably arising from the frontal lobe are difficult to predict. We retrospectively collected data on 865 pediatric patients with epilepsy. In 78 patients with cryptogenic frontal lobe epilepsy, the age at first seizure was inversely correlated with the outcome, ...
journal_title:Journal of child neurology
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更新日期:2014-12-01 00:00:00
abstract::Comprehensive Behavioral Intervention for Tics (CBIT) is an efficacious treatment with limited regional availability. As neurology and pediatric clinics are often the first point of therapeutic contact for individuals with tics, the present study assessed preliminary treatment response, acceptability, and feasibility ...
journal_title:Journal of child neurology
pub_type: 杂志文章,多中心研究
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更新日期:2016-03-01 00:00:00
abstract::CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is a progressive neurodegenerative condition, associated with mutations in the notch3 gene. Symptoms include migraine with aura, mood disorders, progressive cognitive decline, subcortical ischemic strokes, dementia, an...
journal_title:Journal of child neurology
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更新日期:2010-05-01 00:00:00
abstract::Tics, patterned movements distinct from stereotypies, myoclonus, and other hyperkinetic movements, are quite common in children, particularly among those with developmental and psychiatric disorders. Thus, tics can indicate the presence of atypical neurodevelopment or broader difficulties with cognition or mood. Tics ...
journal_title:Journal of child neurology
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更新日期:2006-08-01 00:00:00
abstract::Three patients with Leigh's syndrome developed respiratory failure following general anesthesia. Although all three had respiratory symptoms prior to the anesthetic, the diagnosis was not suspected at the time of the procedure in two of the children. We reviewed the case notes of 16 other patients with Leigh's syndrom...
journal_title:Journal of child neurology
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更新日期:1990-04-01 00:00:00
abstract::There are limited data on the pattern and prevalence of pediatric chronic neurologic conditions in the region. Therefore, the objective of this study was to establish the prevalence of these disorders in the Kingdom of Saudi Arabia. A multistage probability sampling design was used to select a random sample of Saudi h...
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