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ABSTRACT: Die kognitive Entwicklung von Kindern nach einem Fieberkrampf wird insbesondere von deren Eltern aus Angst vor negativen Auswirkungen
auf die mentalen Bereiche mit großer Sorge gesehen. Populationsbasierte und erste klinikbasierte Studien können belegen, dass
diese Angst unberechtigt ist. Kinder nach Fieberkrämpfen besuchen ebenso häufig ein Gymnasium wie alle anderen Kinder. Eine
Ausnahme bilden die Kinder mit einem vorbestehenden neurologischen Defizit im Bezug auf ihre weitere kognitive Entwicklung.
Es wird eine Übersicht über relevante Studien zu dieser Frage gegeben.
The cognitive development of children after a febrile seizure is regarded with great anxiety by parents due to the fear of
negative effects on the mental development of their children. Population-based and clinical-based studies have substantiated
that these fears have no foundation. Children having experienced febrile seizures attend German high school (“Gymnasium”)
as often as all other children. Children with pre-existing neurological deficits are an exception with respect to their further
cognitive development. A summary on the relevant studies concerning this issue is presented.
Zeitschrift für Epileptologie 04/2012; 22(4):239-243.
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ABSTRACT: Epileptische Anfälle beginnen sehr häufig im Kindesalter; ihre Zeichen können gerade in dieser Altersklasse sehr variabel
sein. Die ebenso oft auftretenden altersabhängigen nichtepileptischen paroxysmalen Bewegungsstörungen werden mitunter mit
epileptischen Anfällen verwechselt. In dieser Übersicht wird das gesamte Spektrum der nichtepileptischen altersabhängigen
Bewegungsstörungen beschrieben. Diese zu erkennen ist wichtig, da die Therapie vollkommen anders gestaltet wird. Antiepileptische
Medikamente sind hier, mit Ausnahme bei der kinesiogenen Choreoathetose, kontraindiziert.
Epileptic seizures very often begin in childhood, and particularly in this age group their expressions may be quite variable.
They are frequently confused with age-related, non-epileptic paroxysmal movements, which are just as common. The complete
spectrum of non-epileptic movements in children is described in this article. It is important to distinguish these from epileptic
seizures, because therapeutic measures are completely different for non-epileptic paroxysmal movements. Antiepileptic drugs
are not necessary except for kinesiogenic choreoathetosis.
Zeitschrift für Epileptologie 04/2012; 22(3):126-134.
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ABSTRACT: Prospective memory (PM) describes the ability to fulfill previously planned intentions and is highly dependent on executive functions. Previous studies have shown deficits in executive functions in patients with juvenile myoclonic epilepsy (JME) and in their unaffected siblings. JME has a strong genetic predisposition and it is hypothesized that cognitive deficits are also genetically determined. The present study aimed at investigating potential differences in PM between patients with JME, their siblings, and healthy controls.
Nineteen patients with JME, 21 siblings, and 21 healthy controls were examined with a complex PM paradigm allowing us to evaluate the different phases of PM (i.e., intention formation, intention retention, intention initiation, intention execution).
Patients with JME and siblings showed specific deficits during intention formation and intention execution of PM. Patients with JME were more impaired than both siblings and healthy controls. Correlation analysis revealed an influence of planning on prospective memory abilities in patients with JME.
The results of this study support the hypothesis of frontal dysfunctions being part of the epileptic syndrome and therefore genetically determined. As in this study patients with JME are more severely cognitively impaired than their siblings, additional influencing factors, such as side effects of anticonvulsants or cognitive effects of subclinical epileptic discharges, might contribute to patients' performance.
Neurology 11/2010; 75(24):2161-7. · 8.31 Impact Factor
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ABSTRACT: This 12-week open label study explored cognitive and seizure outcomes of 53 children treated with topiramate (TPM). The digit symbol test and verbal learning memory test were administered at baseline and study endpoint. Topiramate was started either in monotherapy or add-on therapy. Overall, 57% of children experienced a >/=50% seizure reduction, 36% became seizure free and cognitive testing revealed no significant changes during TPM therapy. Due to the heterogeneity of the study population, post hoc analyses were added to compare patients in initial or conversion to TPM monotherapy as well as patients who continued add-on therapy. Verbal learning memory test parameters showed neither significant differences within any subgroup comparing baseline with endpoint nor significant differences between described subgroups except for one finding. The digit symbol test revealed no differences between each subgroup between baseline and endpoint. Comparing pre-post differences, TPM monotherapy was associated with better cognitive outcomes than treatment in add-on therapy. These results have to be interpreted with caution given the short study duration and the heterogeneity of the study population. Despite these limitations, our overall results suggest that treatment with topiramate is associated with improved seizure control without significant changes in cognitive functions at the low doses tested
Neuropediatrics 06/2010; 41(3). · 0.94 Impact Factor
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B A Neubauer,
S Waldegger,
J Heinzinger,
A Hahn, G Kurlemann,
B Fiedler,
F Eberhard,
H Muhle,
U Stephani,
S Garkisch,
O Eeg-Olofsson,
U Müller,
T Sander
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ABSTRACT: To explore the involvement of M-type potassium channels KCNQ2, Q3, and Q5 in the pathogenesis of common idiopathic epilepsies.
Sequence analysis of the KCNQ2, Q3, and Q5 coding regions was performed in a screening sample consisting of 58 nuclear families with rolandic epilepsy. Subsequently, an association study was conducted for all discovered variants in a case-control sample comprising 459 German patients with idiopathic generalized epilepsy (IGE) and 462 population controls.
An in-frame deletion of codon 116 in KCNQ2 (p.Lys116del) and a missense mutation in KCNQ3 (p.Glu299Lys) were detected in two index cases exhibiting rolandic epilepsy and benign neonatal convulsions. Both mutations resulted in reduced potassium current amplitude in Xenopus oocytes. Mutation analysis of families with rolandic epilepsy without neonatal seizures discovered three novel missense variations (KCNQ2 p.Ile592Met, KCNQ3 p.Ala381Val, KCNQ3 p.Pro574Ser). The KCNQ2 p.Ile592Met variant displayed a significant reduction of potassium current amplitude in Xenopus oocytes and was present only once in 552 controls. Both missense variants identified in KCNQ3 (p.Ala381Val and p.Pro574Ser) were present in all affected family members and did not occur in controls, but did not show obvious functional abnormalities. The KCNQ3 missense variant p.Pro574Ser was also detected in 8 of 455 IGE patients but not in 454 controls (p = 0.008). In KCNQ2, a silent single nucleotide polymorphism (rs1801545) was found overrepresented in both epilepsy samples (IGE, p = 0.004).
Sequence variations of the KCNQ2 and KCNQ3 genes may contribute to the etiology of common idiopathic epilepsy syndromes.
Neurology 08/2008; 71(3):177-83. · 8.31 Impact Factor
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ABSTRACT: The authors conducted a double-blind, placebo-controlled, crossover study to investigate the efficacy of oral zolmitriptan in the treatment of migraine in children and adolescents. Patients (n = 32) received placebo, zolmitriptan 2.5 mg, and ibuprofen 200 to 400 mg to treat three consecutive migraine attacks. Pain relief rates after 2 hours were 28% for placebo, 62% for zolmitriptan, and 69% for ibuprofen (p < 0.05). Both drugs are well tolerated with only mild side effects.
Neurology 08/2006; 67(3):497-9. · 8.31 Impact Factor
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K Ebach,
H Joos,
H Doose,
U Stephani, G Kurlemann,
B Fiedler,
A Hahn,
E Hauser,
K Hundt,
H Holthausen,
U Müller,
B A Neubauer
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ABSTRACT: Severe myoclonic epilepsy in infancy (SMEI), severe idiopathic generalized epilepsy of infancy (SIGEI) with generalized tonic clonic seizures (GTCS), and myoclonic astatic epilepsy (MAE) may show semiological overlaps. In GEFS+ families, all three phenotypes were found associated with mutations in the SCN1A gene. We analyzed the SCN1A gene in 20 patients with non-familial myoclonic astatic epilepsy -- including 12 probands of the original cohort used by Doose et al. in 1970 to delineate MAE. In addition, 18 patients with sporadic SIGEI -- mostly without myoclonic-astatic seizures -- were analyzed. Novel SCN1A mutations were found in 3 individuals. A frame shift resulting in an early premature stop codon in a now 35-year-old woman with a borderline phenotype of MAE and SIGEI (L433fsX449) was identified. A splice site variant (IVS18 + 5 G --> C) and a missense mutation in the conserved pore region (40736 C --> A; R946 S) were detected each in a child with SIGEI. We conclude that, independent of precise syndromic delineation, myoclonic-astatic seizures are not predictive of SCN1A mutations in sporadic myoclonic epilepsies of infancy and early childhood.
Neuropediatrics 07/2005; 36(3):210-3. · 0.94 Impact Factor
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ABSTRACT: The aim of this study was to evaluate the differential uptake of O-(2-[18F]fluorethyl)-L-tyrosine (FET) in suspected primary brain tumours.
Positron emission tomography (PET) was performed in 44 patients referred for the evaluation of a suspected brain tumour. Acquisition consisted of four 10-min frames starting upon i.v. injection of FET. Tumour uptake was calculated as the ratio of maximal tumour intensity to mean activity within a reference region (FETmax).
FET uptake above the cortical level was observed in 35/44 lesions. All histologically confirmed gliomas and many other lesions showed FET uptake to a variable extent. No uptake was observed in nine lesions (one inflammatory lesion, one dysembryoplastic neuroepithelial tumour, one mature teratoma, six lesions without histological confirmation). An analysis of uptake dynamics was done in the patients with increased FET uptake (22 gliomas, three lymphomas, three non-neoplastic lesions, three lesions with unknown histology and four other primaries). Upon classification of tumours into low (i.e. WHO I and II) and high grade (i.e. WHO III and IV), a significant difference in FETmax between the two categories was observed only in the first image frame (0-10 min p.i.), with FETmax=2.0 in low-grade and 3.2 in high-grade tumours (p<0.05); no significant differences were found in frame 4 (30-40 min p.i.), with FETmax=2.4 vs 2.7. Similar results were obtained when the analysis was applied only to astrocytic tumours (2.0 vs 3.1 in the first frame; 2.4 vs 2.6 in the fourth frame).
These initial results indicate that FET PET is a useful method to identify malignant brain lesions. It appears that high- and low-grade brain tumours exhibit a different uptake kinetics of FET. A kinetic analysis of FET PET may provide additional information in the differentiation of suspected brain lesions.
European journal of nuclear medicine and molecular imaging 04/2005; 32(4):422-9. · 4.99 Impact Factor
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ABSTRACT: To evaluate single photon emission computed tomography (SPECT) using the amino acid l-3-[123I]-alpha-methyl tyrosine (IMT) and contrast enhanced magnetic resonance imaging (MRI) as diagnostic tools in primary paediatric brain tumours in respect of non-invasive tumour grading. Patients, materials, methods: 45 children with primary brain tumours were retrospectively evaluated. IMT uptake was quantified as tumour/nontumour-ratio, a 4-value-scale was used to measure gadolinium enhancement on contrast enhanced MRI. Statistical analyses were performed to evaluate IMT uptake and gadolinium enhancement in low (WHO I/II) and high (WHO III/IV) grade tumours and to disclose a potential relationship of IMT uptake to disruption of blood brain barrier as measured in corresponding MRI scans.
IMT uptake above background level was observed in 35 of 45 patients. IMT uptake was slightly higher in high grade tumours but the difference failed to attain statistical significance. Grading of individual tumours was neither possible by IMT SPECT nor by gadolinium enhanced MRI.
IMT is accumulated in most brain tumours in children. Tumour grading was not possible using IMT or contrast enhancement as determined by MRI. Neither morphological nor functional imaging can replace histology in paediatric brain tumours.
Nuklearmedizin 02/2005; 44(4):131-6. · 1.28 Impact Factor
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Advances in neurology 02/2005; 95:217-26.
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Neurology 04/2004; 62(6):1020-1. · 8.31 Impact Factor
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ABSTRACT: Ischemic symptoms in children with Moyamoya syndrome are typically provoked by hyperventilation (HV) and are accompanied by the "re-build-up" phenomenon in EEG. The value of scintigraphic detection of HV-provoked perfusion deficits remains to be elucidated.
In seven children with Moyamoya syndrome regional cerebral blood flow was assessed by 99mTc-ethyl-cysteine-dimer (ECD) single photon emission computed tomography (SPECT) after HV and under baseline conditions to identify ischemia prone regions.
Regional marked hypoperfusion after HV was found in all patients. Predominant perfusion deficits were detected in the frontal lobes.
ECD SPECT is a potential tool for the preoperative evaluation of cerebral hemodynamics and for monitoring angiosurgical therapies in Moyamoya disease.
Nuklearmedizin 03/2002; 41(1):42-6. · 1.28 Impact Factor
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ABSTRACT: We report on a preterm infant born at 30+5/7 gestational weeks who developed severe cystic cerebral lesions after exposure to a car accident one day before delivery. The literature on car accidents during pregnancy is reviewed with specific focus on neonatal neurological outcome.
European Journal of Ultrasound 01/2002; 14(2-3):171-8.
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Chest 10/2001; 120(3):1038-40. · 5.25 Impact Factor
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ABSTRACT: Early infantile epileptic encephalopathy (EIEE) is a polyetiologic age-dependent neurological disorder. We present two patients with EIEE whose mothers experienced electric injury during pregnancy. After the accident one mother noticed decreased fetal movements. Neither other prenatal factors nor intrapartal damage or postnatally examined structural, metabolic or infectious causes which might have been responsible for the EIEE in these children could be found. The question of electric accident during pregnancy should be considered when documenting the history of children with Ohtahara syndrome.
Brain and Development 09/2001; 23(5):359-62. · 2.12 Impact Factor
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ABSTRACT: Despite a favourable prognosis, pilocytic astrocytomas may exhibit signs of malignancy on various neuroimaging modalities. This retrospective analysis was conducted to determine whether scintigraphic features of malignancy are also found on single-photon emission tomography (SPET) using L-3-[123I]iodo-alpha-methyl tyrosine (IMT) as a tracer. Twenty patients with pilocytic astrocytomas were retrospectively selected from a large series of patients referred for the evaluation of primary or recurrent brain tumours. IMT SPET was performed in 16 patients, positron emission tomography (PET) using 2-[18F]fluoro-2-deoxy-D-glucose (FDG) was available in 10 of the patients and SPET using technetium-99m tetrofosmin or thallium-201 had been performed in 11. Image analysis was performed using standard protocols to determine how many patients exceeded the respective thresholds of malignancy. Features of malignancy were found in 7/16 IMT SPET studies, in 7/10 FDG PET studies and in 7/11 of the residual SPET investigations. A significant correlation of tumour size and IMT uptake in primary pilocytic astrocytomas indicated partial volume effects to partly account for the differential uptake behaviour (n = 10, r = 0.87, P < 0.05). Differences in IMT uptake in primaries (1.7 +/- 0.6, n = 10) and in recurrent tumours (2.3 +/- 0.7, n = 6) did not attain statistical significance. IMT SPET results indicative of malignancy are regularly found in pilocytic astrocytomas, despite their good prognosis. No uptake may be detected in largely cystic or in small tumours.
European Journal of Nuclear Medicine 03/2001; 28(3):273-81.
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New England Journal of Medicine 12/2000; 343(21):1573. · 53.30 Impact Factor
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ABSTRACT: Positron emission tomography (PET) using 18F-radiolabeled deoxyglucose (18F-FDG) is a sensitive procedure for detection of epileptogenic foci. Although alterations in glucose consumption are not restricted to the area of seizure generation itself, the magnitude and extent of cerebral metabolic disturbances induced by epileptic discharges can be detected. Despite two decades of epilepsy research using 18F-FDG-PET, little is known about the metabolic changes during therapy of focal epilepsy. We report on a child with frontal epilepsy with severe glucose hypometabolism that was nearly completely normalized during drug therapy.
Interictal 18F-FDG-PET was performed at the onset of epilepsy and after optimized drug therapy in a 5-year-old boy with behavioral abnormalities and repetitive seizures of frontal origin with bifrontal interictal EEG slowing for 8 weeks. Both scans were anatomically matched; initial and intratherapeutic glucose metabolism were compared.
In accordance with the epileptogenic focus as identified by EEG and ictal/interictal perfusion single-photon emission tomography (SPECT), bifrontal hypometabolism was depicted by 18F-FDG-PET. Magnetic resonance imaging (MRI) was unremarkable. After dual-drug therapy (valproate, carbamazepine), the boy became seizure free, and his initial behavioral deficits disappeared. A control PET study after 3 months of therapy showed restored glucose consumption; the frontal EEG slowing was normalized.
This case demonstrates that reduction of glucose metabolism in epileptogenic foci may be a result of reversible neuronal dysfunction that correlates with the electroclinical follow-up.
Epilepsia 06/2000; 41(5):588-93. · 3.96 Impact Factor
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Neuropediatrics 03/1999; 30(1):50. · 0.94 Impact Factor
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ABSTRACT: Dissection of the carotid and vertebral arteries is a not so uncommon cause of stroke and has to be considered as a differential diagnosis especially in younger patients. Therapeutic and prognostic implications are different from those in extracranial atherosclerotic disease. Dissection results from hemorrhage into the vessel wall usually between the layers of the media. Digital subtraction angiography (DSA) depicts the resulting luminal compromise that may reveal some typical, but not specific, findings. The same is true for non-invasive angiographic techniques such as time-of-flight magnetic resonance angiography (MRA) and computed tomography angiography (CTA), which have shown accurate results compared with DSA. The main advantage of these techniques is the direct visualization of the vessel wall confirming the intramural hematoma. This is achieved best with MR imaging due to the high signal of blood degradation products on T1- and T2-weighted images. Therefore, MRI in combination with MRA is presently the method of choice for initial diagnosis and follow-up of craniocervical artery dissection (CCAD). In some questionable cases, CTA is a non-invasive alternative that is independent of flow phenomena.
European Radiology 02/1999; 9(7):1385-91. · 3.22 Impact Factor
