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Purpose: To identify factors correlating with poorer quality of life (QoL) in children and adolescents with epilepsy and regarding QoL and depression of their caregivers in Germany., Method: A cross-sectional multicenter study on QoL and depression was performed in two representative German states (Hessen and Schleswig-Holstein). Variance analysis, linear regression, and bivariate correlation were used to identify correlating factors for poorer QoL and symptoms of depression., Results: Data from 489 children and adolescents (mean age 10.4 ± 4.2 years, range 0.5-17.8; 54.0% male) and their caregivers were collected. We identified missing seizure freedom (p = 0.046), concomitant diseases (p = 0.007), hospitalization (p = 0.049), recent status epilepticus (p = 0.035), living in a nursing home or with foster parents (p = 0.049), and relevant degree of disability (p = 0.007) to correlate with poorer QoL in children and adolescents with epilepsy. Poorer QoL of caregivers was associated with longer disease duration (p = 0.004), non-idiopathic (mainly structural-metabolic) epilepsy (p = 0.003), ongoing seizures (p = 0.003), concomitant diseases (p = 0.003), relevant disability (p = 0.003), or status epilepticus (p = 0.003) as well as with unemployment of the primary caretaker (p = 0.010). Symptoms of depression of caregivers were associated with non-idiopathic epilepsy (p = 0.003), concomitant diseases (p = 0.003), missing seizure freedom (p = 0.007), status epilepticus (p = 0.004), or a relevant disability (p = 0.004) of their ward. A poorer QoL value of the children and adolescents correlated with a poorer QoL value of the caregivers (p < 0.001)., Conclusions: Epilepsy shows a considerable impact on QoL and symptoms of depression. Early and effective therapy should focus on reduction of seizure frequency and the probability for developing status epilepticus. Furthermore, comprehensive care should pay attention at comorbidities, consequences of disability and dependency on others., (Copyright © 2019 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Objective: To provide first data on the cost of epilepsy and cost-driving factors in children, adolescents, and their caregivers in Germany., Methods: A population-based, cross-sectional sample of consecutive children and adolescents with epilepsy was evaluated in the states of Hessen and Schleswig-Holstein (total of 8.796 million inhabitants) in all health care sectors in 2011. Data on socioeconomic status, course of epilepsy, and direct and indirect costs were recorded using patient questionnaires., Results: We collected data from 489 children and adolescents (mean age ± SD 10.4 ± 4.2 years, range 0.5-17.8 years; 264 [54.0%] male) who were treated by neuropediatricians (n = 253; 51.7%), at centers for social pediatrics ("Sozialpaediatrische Zentren," n = 110, 22.5%) and epilepsy centers (n = 126; 25.8%). Total direct costs summed up to €1,619 ± €4,375 per participant and 3-month period. Direct medical costs were due mainly to hospitalization (47.8%, €774 ± €3,595 per 3 months), anticonvulsants (13.2%, €213 ± €363), and ancillary treatment (9.1%, €147 ± €344). The total indirect costs amounted to €1,231 ± €2,830 in mothers and to €83 ± €593 in fathers; 17.4% (n = 85) of mothers and 0.6% (n = 3) of fathers reduced their working hours or quit work because of their child's epilepsy. Independent cost-driving factors were younger age, symptomatic cause, and polytherapy with anticonvulsants. Older age, active epilepsy, symptomatic cause, and polytherapy were independent predictors of higher antiepileptic drug (AED) costs, whereas younger age, longer epilepsy duration, symptomatic cause, disability, and parental depression were independent predictors for higher indirect costs., Significance: Treatment of children and adolescents with epilepsy is associated with high direct costs due to frequent inpatient admissions and high indirect costs due to productivity losses in mothers. Direct costs are age-dependent and higher in patients with symptomatic epilepsy and polytherapy. Indirect costs are higher in the presence of a child's disability and parental depression., (Wiley Periodicals, Inc. © 2015 International League Against Epilepsy.)

Purpose: Topiramate (TPM) inhibits carbonic anhydrase, with metabolic acidosis as a possible side effect, although this has been reported in only two adult cases. We investigated the acid-base metabolism in infants and toddlers treated with TPM., Methods: Nine infants and toddlers aged 5 months to 2.3 years (median, 6 months) were treated with TPM at maximal doses of 8.2-26 mg/kg/day (median, 11 mg/kg/day). The maximal TPM dose was achieved after 8-35 days (median, 17 days). TPM was given in addition to other antiepileptic drugs (AEDs) in five cases and as a sole AED in four patients with refractory epilepsy resistant to multiple AEDs. The diagnoses were infantile spasms (n = 5), epilepsia partialis continua (n = 1), infantile epileptic encephalopathy (n = 1), and Lennox-Gastaut syndrome (n = 2)., Results: The blood gases were normal before treatment with TPM in all nine children. Metabolic acidosis developed in eight children after 8-26 days (median, 14 days) of TPM treatment with a minimum of serum bicarbonate between 15 to 18 mM (median, 17 mM), a minimal base excess between -6.2 and -11.2 mM (median, -7.9 mM), and pH between 7.22 and 7.40 (median, 7.35). Four of nine children showed clinical signs of hyperventilation and received oral sodium bicarbonate (1-2 mmol/kg), while TPM was still effective., Conclusions: Because metabolic acidosis developed in eight of the nine infants and toddlers taking TPM, we would suggest that the acid-base metabolism be monitored in young children who receive TPM.

Abstract: Purpose: At present, neonatal seizures are usually treated with Phenobarbital (PB) despite the limited efficacy and the potential risk this treatment holds for the developing brain. We report here a prospective pilot feasibility study on the use of Levetiracetam as monotherapy in the treatment of neonatal seizures. Methods: Six newborns (body weight>2000g, gestational age>30 weeks) presenting with neonatal seizures were enrolled. Patients whose seizures were caused by electrolyte disturbances or hypoglycemia, or whose seizures did respond to pyridoxine were excluded. Patients previously treated with other antiepileptic drugs (AEDs), with the exception of single PB doses before and during titration, were excluded. LEV was administered orally, increasing the dose by 10mg/(kgday) over 3 days. Endpoint was the need of any additional AEDs (or PB) after day 3, or 3 months of LEV treatment. A decision regarding further treatment was made on an individual basis and follow-up was documented up to 8 months of age. Results: No severe adverse effects were observed. Mild sedation was reported in one infant. All six patients treated with oral LEV became seizure free within 6 days. Five patients remained seizure free after 3 months with ongoing LEV monotherapy. One infant developed pharmacoresistent epilepsy. Seizures relapsed later in the clinical course of two more patients, one of whom was no longer under LEV therapy. Discussion: Results from our small patient group indicate that LEV may be an alternative therapeutic option in neonatal seizures. [Copyright &y& Elsevier]

A retrospective study for a classification of prehypsarrhythmic elecroencephalographies (EEGs) was carried out to enable an early treatment option for West syndrome. Out of 39 infants with symptomatic West syndrome, 18 infants (age 3–14 months) with 61 serial non-REM sleep EEG records of the prehypsarrhythmic phase were identified. The prehypsarrhythmic phase encompassed 2 to 13 months (mean 4.5 months) after an initial insult. A classification system of three EEG types corresponding to the clinical states prior to West syndrome occurrence was developed. In addition, follow-up of all patients presenting with type 2 EEGs (n = 22) was evaluated. Three clinical states and corresponding EEG types were classified. Clinical state 1 (weeks to months, silent phase) presented with (multi-)focal epileptic discharges <50% of the non-REM EEG recording time (type 1 EEG). Clinical state 2 (several weeks, beginning mental deterioration) was accompanied by type 2 EEG with bihemispheric epileptic discharges >50% of the non-REM EEG recording time within abnormal background activity (imminent hypsarrhythmia). Clinical state 3 (mental deterioration) was characterized by hypsarryhthmia. Interrater reliability of seven blinded raters was good (median weighted κ 0.67). Out of 22 patients presenting with type 2 EEGs, two were lost for follow-up, and 16 developed West syndrome, whereas four were treated early with anti-epileptic drugs and remained stable. Infants with West syndrome could be reliably identified several weeks before the occurrence of hypsarrhythmia by a typical EEG pattern (type 2), thereby opening the way for early intervention studies. [ABSTRACT FROM AUTHOR]

Background Patients with LIS1 -associated classic lissencephaly typically present with severe psychomotor retardation and drug-resistant epilepsy within the first year. Aim To analyze the epileptogenic phenotype and response to antiepileptic therapy in LIS1 -associated classic lissencephaly. Method Retrospective evaluation of 22 patients (8 months–24 years) with genetically and radiologically confirmed LIS1 -associated classic lissencephaly in 16 study centers. Results All patients in our cohort developed drug-resistant epilepsy. In 82% onset of seizures was noted within the first six months of life, most frequently with infantile spasms. Later in infancy the epileptogentic phenotype became more variable and included different forms of focal seizures as well generalized as tonic–clonic seizures, with generalized tonic–clonic seizures being the predominant type. Lamotrigine and valproate were rated most successful with good or partial response rates in 88–100% of the patients. Both were evaluated significantly better than levetiracetam ( p < 0.05) and sulthiame ( p < 0.01) in the neuropediatric assessment and better than levetiracetam, sulthiame ( p < 0.05) and topiramate ( p < 0.01) in the family survey. Phenobarbital and vigabatrin achieved good or partial response in 62–83% of the patients. Conclusion Our findings suggest that patients with LIS1 -associated lissencephaly might benefit most from lamotrigine, valproate, vigabatrin or phenobarbital. [ABSTRACT FROM AUTHOR]