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40
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Text
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<a href="http://doi.org/10.1016/S0387-7604(03)00113-X" target="_blank" rel="noreferrer">http://doi.org/10.1016/S0387-7604(03)00113-X</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
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Unilateral closed-lip schizencephaly and epilepsy: a comparison with cases of unilateral polymicrogyria
Publisher
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Brain & Development
Date
A point or period of time associated with an event in the lifecycle of the resource
2004
Subject
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Child; Female; Humans; Male; Adult; Follow-Up Studies; Magnetic Resonance Imaging; adolescent; Preschool; infant; Q3 Literature Search; Tomography; X-Ray Computed; Electroencephalography; Epilepsy/pathology; Brain/abnormalities/pathology; Frontal Lobe/pathology; Gait Disorders; Neurologic/complications/pathology; Temporal Lobe/pathology
Creator
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Caraballo RH; Cersosimo RO; Fejerman N
Description
An account of the resource
We compared the electroclinical features and evolution of patients with two different types of abnormal cortical organization: unilateral closed-lip schizencephaly (SCHZ) and unilateral polymicrogyria (PMG). Between February 1990 and June 2002, 51 children with either unilateral PMG or closed-lip SCHZ were selected through neuroradiological analysis for investigation at our service. We evaluated the frequency of epilepsy, electroclinical features and evolution. The mean time of follow-up was 7 years (range 1-12 years). All patients underwent neurological examination, computed tomography scan and magnetic resonance imaging, serial electroencephalographic (EEG) recordings and neuropsychological assessment. Thirty-six of the 51 patients had unilateral PMG. All patients had hemiparesis with mild spasticity. Mental retardation was mild in 20 and moderate in 14. In two patients IQ was normal. Partial motor seizures were recorded in 28 patients, with secondary generalization in 20. The median age at onset of seizures was 2 years (range 4 months-7 years). Interictal EEGs showed unilateral spikes in all patients. In 21 patients epilepsy worsened between the ages of 4 and 8 (mean 5.6 years) with frequent atonic seizures, atypical absences, epileptic negative myoclonus and gait difficulties. EEGs showed continuous spike-wave activity or bilateral high-frequency spike discharges during slow-wave sleep. Frequent relapses of atonic and myoclonic seizures were seen in nine patients. At present, 16 patients are seizure-free. Fifteen patients with unilateral SCHZ were included in the study. Focal motor seizures were registered in seven cases, in three of them with secondary generalization. The median age at onset of epilepsy was 2.5 years (range 1-4 years). Interictal EEGs showed unilateral spikes in these seven cases. All patients except one presented mild spastic hemiparesis. Mental retardation was mild in ten children, moderate in two and IQ was normal in three. Although the underlying mechanisms leading to PMG and SCHZ are probably similar, the electroclinical phenomenon of secondary bilateral synchrony with frequent negative myoclonus was not present in our cases with unilateral closed-lip SCHZ.
2004
Identifier
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<a href="http://doi.org/10.1016/S0387-7604(03)00113-X" target="_blank" rel="noreferrer">10.1016/S0387-7604(03)00113-X</a>
Rights
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Type
The nature or genre of the resource
Journal Article
2004
Adolescent
Adult
Backlog
Brain & Development
Brain/abnormalities/pathology
Caraballo RH
Cersosimo RO
Child
Electroencephalography
Epilepsy/pathology
Fejerman N
Female
Follow-up Studies
Frontal Lobe/pathology
Gait Disorders
Humans
Infant
Journal Article
Magnetic Resonance Imaging
Male
Neurologic/complications/pathology
Preschool
Q3 Scoping Review Results
Temporal Lobe/pathology
Tomography
X-Ray Computed
-
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
Citation List Month
Backlog
URL Address
<a href="http://doi.org/10.1055/s-2007-973503" target="_blank" rel="noreferrer">http://doi.org/10.1055/s-2007-973503</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
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Clinical and neuroradiological follow-up in mucopolysaccharidosis type III (Sanfilippo syndrome)
Publisher
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Neuropediatrics
Date
A point or period of time associated with an event in the lifecycle of the resource
1999
Subject
The topic of the resource
Child; Female; Humans; Male; Disease Progression; Magnetic Resonance Imaging; Preschool; infant; Q3 Literature Search; Atrophy; Brain/abnormalities/pathology; Mental Retardation/etiology; Mucopolysaccharidosis III/pathology/physiopathology; Psychomotor Disorders/diagnosis/etiology
Creator
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Barone R; Nigro F; Triulzi F; Musumeci S; Fiumara A; Pavone L
Description
An account of the resource
Mucopolysaccharidosis type III (Sanfilippo syndrome) is an autosomal recessive disorder characterised by progressive nervous system involvement with mental retardation, behavioural problems and seizures. Three patients, of 20 months to 12 years of age, were followed up for 3 years both clinically and by using brain magnetic resonance imaging (MRI). Our results suggest that in MPS III patients MRI findings, including atrophy and abnormal or delayed myelination, may precede the onset of overt neurological symptoms. The increasing neurological morbidity is accompanied by different degrees of progressive atrophic changes, mainly affecting the cerebral cortex and the corpus callosum. However, it appears that, across subjects, the rate of MRI changes is unrelated to the severity of the clinical phenotype. On this basis it could be argued that in MPS III the worsening of the neurological symptoms might not necessarily reflect only the progressive cerebral abnormalities detectable by MRI.
1999
Identifier
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<a href="http://doi.org/10.1055/s-2007-973503" target="_blank" rel="noreferrer">10.1055/s-2007-973503</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Type
The nature or genre of the resource
Journal Article
1999
Atrophy
Backlog
Barone R
Brain/abnormalities/pathology
Child
Disease Progression
Female
Fiumara A
Humans
Infant
Journal Article
Magnetic Resonance Imaging
Male
Mental Retardation/etiology
Mucopolysaccharidosis III/pathology/physiopathology
Musumeci S
Neuropediatrics
Nigro F
Pavone L
Preschool
Psychomotor Disorders/diagnosis/etiology
Q3 Scoping Review Results
Triulzi F