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Abumansour I.S.,University of Manitoba | Wrogemann J.,University of Manitoba | Chudley A.E.,University of Manitoba | Chodirker B.N.,University of Manitoba | And 2 more authors.
Journal of Child Neurology | Year: 2014

Classical lissencephaly may be associated with cerebellar hypoplasia and when significant cerebellar abnormalities occur, defects in proteins encoded by TUBA1A, RELN, and very-low-density lipoprotein receptor (VLDLR) genes have been reported. We present a neonate with a severe neurologic phenotype associated with hypotonia, oropharyngeal incoordination that required a gastric tube for feeding, intractable epilepsy, and congenital cataracts. Her brain magnetic resonance imaging (MRI) showed classical lissencephaly, ventriculomegaly, absent corpus callosum, globular and vertical hippocampi, and severe cerebellar and brainstem hypoplasia. She died at 6 weeks of age. No specific molecular diagnosis was made. This likely represents a previously undescribed genetic lissencephaly syndrome. © The Author(s) 2013.


Khajeh L.,Rotterdam University | Cherian P.J.,Rotterdam University | Swarte R.M.,Section of Neonatology | Smit L.S.,Section of Pediatric Neurology | Lequin M.H.,Erasmus MC Sophia
Journal of Child Neurology | Year: 2014

Apneic neonatal seizures can present as apparent life-threatening events. We report a newborn with unexplained episodes of apnea associated with cyanosis and desaturation, starting on the first day postpartum. Biochemical tests were normal. Central nervous system infections as well as abnormalities of upper airways and cardiovascular system were excluded. Brain monitoring using amplitude-integrated electroencephalography (aEEG) was inconclusive. Continuous monitoring using video EEG revealed epileptic seizures originating from the left temporal region as the cause of the apneas. Magnetic resonance imaging (MRI) of the brain showed a developmental malformation of the left frontal and temporal lobes. The patient became seizure free after treatment with antiepileptic medication. This report illustrates that brain monitoring using amplitude-integrated EEG alone could miss focal neonatal seizures. When clinical suspicion of apneic seizures is high in infants with apparent life threatening events, multichannel polygraphic video-EEG monitoring is indicated. Prompt diagnosis and treatment can be life saving. © The Author(s) 2013.


Crepeau A.Z.,Barrow Neurological Institute | Kerrigan J.F.,Barrow Neurological Institute | Gerber P.,Neurodiagnostics Laboratory | Parikh G.,Barrow Neurological Institute | And 4 more authors.
Journal of Clinical Neurophysiology | Year: 2013

INTRODUCTION:: Nonconvulsive seizures and nonconvulsive status epilepticus commonly occur in patients with aneurysmal subarachnoid hemorrhages. When continuous EEG is used in patients in the neuro-intensive care unit, rhythmical and periodic patterns of uncertain significance are frequently encountered. It is unknown how these findings impact patient outcome. METHODS:: Patients were enrolled from a single tertiary care center with subarachnoid hemorrhages secondary to ruptured intracranial aneurysm, and either a witnessed seizure or significantly impaired mental status. Prospective clinical, laboratory, imaging, and short-term outcome data were collected. Continuous EEG monitoring was performed and scored according to American Clinical Neurophysiology Society (ACNS) Subcommittee on Research Terminology for Continuous EEG Monitoring. RESULTS:: Sixty-eight patients were enrolled. Fifty-four had a poor-grade subarachnoid hemorrhage upon admission. Fifty-one patients had rhythmical or periodic patterns: 33 with periodic discharges and 38 with rhythmic delta activity. Four patients had unequivocal electrographic seizures. Patients did poorly in the short term: 14 died and 42 were severely disabled at discharge. In hospital, mortality was 19.6% in patients with rhythmical or periodic patterns and 23.5% in patients without. Age, female gender, and endovascular treatment had a positive correlation with the occurrence of periodic discharges. However, there was no correlation between rhythmical and periodic patterns and outcome. DISCUSSION:: Using the ACNS Research Terminology, it is shown that rhythmical and periodic patterns are very common in critically ill patients with subarachnoid hemorrhage. However, the presence and the abundance of these patterns did not predict short-term outcome in this prospective, single-center observational study. We were unable to show that rhythmical and periodic EEG patterns are an independent predictor for outcome relative to other clinical features. Large multicenter studies will be required to determine if these patterns independently predict outcome and to demonstrate the impact of treatment interventions that are directed at rhythmical and periodic continuous EEG patterns. Copyright © 2013 by the American Clinical Neurophysiology Society.


Fernandez-Jaen A.,Pediatric Neurology Unit | Fernandez-Mayoralas D.M.,Pediatric Neurology Unit | Quinones Tapia D.,Neuroradiology Unit and Magnetic Resonance Unit | Calleja-Perez B.,Doctor Cirajas Healthcare Center | And 3 more authors.
Pediatric Neurology | Year: 2011

Fetal alcohol syndrome represents the classic and most severe manifestation of epigenetic changes induced by exposure to alcohol during pregnancy. Often these patients develop attention deficit hyperactivity disorder. We analyzed cortical thickness in 20 children and adolescents with fetal alcohol syndrome and attention deficit hyperactivity disorder (group 1), in 20 patients without fetal alcohol syndrome (group 2), and in 20 control cases. The first group revealed total cortical thickness significantly superior to those of the other two groups. In per-lobe analyses of cortical thickness, group 1 demonstrated greater cortical thickness in the frontal, occipital, and right temporal and left frontal lobes compared with the second group, and in both temporal lobes and the right frontal lobe compared with the control group. This study demonstrated greater cortical thickness in patients with attention deficit hyperactivity disorder and heavy prenatal exposure to alcohol, probably as an expression of immature or abnormal brain development. © 2011 Elsevier Ltd. All rights reserved.


Pakalnis A.,Ohio State University | Kring D.,Section of Pediatric Neurology
Journal of Child Neurology | Year: 2012

Obesity and headaches are common in children and adults. Adult studies suggest obesity is a risk factor for chronic daily headache and increased migraine frequency and severity. Pediatric studies have suggested a relationship between obesity, increasing headache frequency, and disability. The authors retrospectively evaluated 925 children from their Pediatric Headache Clinic between July 2004 and July 2008, assessing headache frequency, medication overuse, and body mass index compared to population-based norms. The pediatric headache group as a whole had a greater percentage of overweight than the general population. This was also true with the subgroup of patients with chronic tension-type headache, although the numbers were small. Data did not show increased incidence of overweight in children with medication overuse or chronic migraine. This contrasts with adult data, which have suggested a closer link between chronic migraine and obesity and have not supported a link with chronic tension-type headache. © 2012 The Author(s).

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