Affiliation: University Children's Hospital
- Glutamine supplementation in a child with inherited GS deficiency improves the clinical status and partially corrects the peripheral and central amino acid imbalanceJohannes Haberle
Division of Metabolism and Children s Research Center, University Children s Hospital Zurich, Zurich, Switzerland
Orphanet J Rare Dis 7:48. 2012..There was some clinical improvement but the patient had a long standing severe encephalopathy. Earlier supplementation with glutamine might have prevented some of the neuronal damage...
- Seizures and paroxysmal events: symptoms pointing to the diagnosis of pyridoxine-dependent epilepsy and pyridoxine phosphate oxidase deficiencyBernhard Schmitt
Department of Neurology, University Children s Hospital, Steinwiesstrasse 75, Zurich, Switzerland
Dev Med Child Neurol 52:e133-42. 2010..We report on seizures, paroxysmal events, and electroencephalogram (EEG) findings in four female infants with pyridoxine-dependent epilepsy (PDE) and in one female with pyridoxine phosphate oxidase deficiency (PNPO)...
- Effects of valproic acid on sleep in children with epilepsyBernhard Schmitt
Department of Pediatric Neurology, University Children s Hospital, Zurich, Switzerland
Epilepsia 50:1860-7. 2009..Parents frequently report increased sleep duration in their children during treatment with valproic acid (VPA). We assessed sleep duration and sleep behavior before and after tapering VPA in children treated for more than 6 months...
- Is ethosuximide a risk factor for generalised tonic-clonic seizures in absence epilepsy?B Schmitt
University Children s Hospital, Clinical Neurophysiology, Zurich, Switzerland
Neuropediatrics 38:83-7. 2007..002) and GTCS during treatment (p=0.02). GTCS are rare in patients with typical AE. Our data do not support the current opinion that ethosuximide is inefficacious on GTCS in AE...
- Neonatal seizures: eyes open or closed?Florian Bauder
University Children s Hospital, Zurich, Switzerland
Epilepsia 48:394-6. 2007..It has been shown that persistent eye closure during paroxysmal events in infants makes seizures unlikely. Our study aims to assess whether this is also true in neonates...
- Neonatal seizures with tonic clonic sequences and poor developmental outcomeBernhard Schmitt
University Children s Hospital, Steinwiesstrasse 75, CH 8032 Zurich, Switzerland
Epilepsy Res 65:161-8. 2005....
- Onychomadesis: a rare side-effect of valproic acid medication?Andrea Poretti
Paediatric Neurology, University Children s Hospital of Zurich, CH 8032Zurich, Switzerland
Pediatr Dermatol 26:749-50. 2009..We report on a child with onychomadesis as a possible side-effect of treatment with VPA. Normal nail growth was observed after stopping VPA. The pathomechanism of onychomadesis due to VPA treatment remains unclear...
- Impaired slow wave sleep downscaling in encephalopathy with status epilepticus during sleep (ESES)Bigna K Bölsterli
Division of Clinical Neurophysiology, University Children s Hospital Zurich, Zurich, Switzerland
Clin Neurophysiol 122:1779-87. 2011..The synaptic homeostasis hypothesis predicts that the strength of synapses is decreased during sleep. The slope of slow waves during NREM sleep best reflects this "downscaling"...
- Progressive encephalopathy with edema, hypsarrhythmia and optic atrophy (PEHO) syndrome in a Swiss childAndrea Klein
Department of Neurology, University Children s Hospital Zurich, Steinwiesstrasse 75, Zurich 8032, Switzerland
Eur J Paediatr Neurol 8:317-21. 2004..In patients with infantile spasms and severe developmental delay PEHO syndrome should be considered; it is not confined to Finnish heritage. Optic atrophy should be looked for and repeat MRI is indicated...
- Familial West syndrome and dystonia caused by an Aristaless related homeobox gene mutationGabriele Wohlrab
Department of Neuropaediatrics and Neurophysiology, University Children s Hospital, Steinwiesstrasse 75, 8032 Zurich, Switzerland
Eur J Pediatr 164:326-8. 2005
- Inflammatory response and neurodevelopmental outcome after open-heart surgery in childrenPeter Gessler
Division of Pediatric Intensive Care Medicine, University Children s Hospital of Zurich, Zurich, Switzerland
Pediatr Cardiol 30:301-5. 2009..The high frequency of postoperative neuromotor disabilities seems to be associated with the type of congenital heart defect but also with the procedure and possible complications of CPB...
- Effect of the NMDA-receptor antagonist dextromethorphan in infant rat pneumococcal meningitisJohann Sellner
Department of Neurology, Inselspital, Bern University Hospital, Switzerland
Curr Drug Metab 9:83-8. 2008....
- Long-term outcome of ten children with opsoclonus-myoclonus syndromeAndrea Klein
Department of Neurology, University Children s Hospital Zurich, Steinwiesstrasse 75, 8032, Zurich, Switzerland
Eur J Pediatr 166:359-63. 2007..Compared with previous reports, our patients were treated earlier. Larger studies and uniform treatment protocols are needed to demonstrate whether early and prolonged immunosupressant therapy has a favourable influence on outcome...
- Spindle activity in children during cardiac surgery and hypothermic cardiopulmonary bypassBernhard Schmitt
University Children s Hospital, University Hospital, University of Zurich, Switzerland
J Clin Neurophysiol 19:547-52. 2002..Although the temperature-dependent changes in this study confirm temperature coefficients of other EEG studies, the reasons for the clear age relationship and the "nature" of these spindles remain unknown...
- Pipecolic acid as a diagnostic marker of pyridoxine-dependent epilepsyB Plecko
Department of Pediatrics, University Hospital Graz, Graz, Austria
Neuropediatrics 36:200-5. 2005..It will in addition prevent patients with pyridoxine-dependent epilepsy from experiencing potentially dangerous pyridoxine-withdrawal, which until now has been necessary to prove the diagnosis...
- [Management of refractory status epilepticus from a neurologic and neuropediatric perspective]B Pohlmann-Eden
Epilepsie Zentrum Bethel, Evangelisches Krankenhaus Bielefeld, Bielefeld, Germany
Nervenarzt 78:871-82. 2007..Here we present the results of that discussion and state recommendations from a neurologic and neuropediatric perspective for current und future management of refractory status epilepticus...