Ketogenic diet in the treatment of refractory continuous spikes and waves during slow sleep.

PURPOSE: To evaluate the effect of the ketogenic diet on electroclinical characteristics and cognitive function in children with continuous spikes and waves during slow sleep (CSWS). METHODS: Five children (four boys, one girl) aged between 8 and 13 years with CSWS refractory to conventional antiepileptic drugs (AEDs), including levetiracetam, and steroids were included. The prospective electroclinical assessment was performed prior to the ketogenic diet and once every 6 months post initiation during the 2-year period. All children underwent neuropsychological testing prior to the ketogenic diet and four of the children again 12 months after the diet's introduction. In case 4 the testing has been performed after 7 months and the diet was withdrawn after 9 months because of the lack of efficacy and the parent's wishes. In two patients the cognitive functions were also evaluated after 24 months since the diet's initiation. During the period on the ketogenic diet the concomitant AED treatment was unchanged. RESULTS: Electrographic evaluation after 24 months on the ketogenic diet showed CSWS resolution in one patient, mild decrease of the spike-wave index in one, and lack of response in three patients. The ketogenic diet did not influence the neuropsychological outcome, and intelligence quotient (IQ) scores remained low at the end of the follow-up period. However, in two patients an improvement in attention and behavior was demonstrated. DISCUSSION: This is the first study evaluating the efficacy of the ketogenic diet in children with CSWS. Five presented cases were refractory to AEDs and steroids. Only one case responded with complete CSWS disappearance; in one the effect of the ketogenic diet was partial and intermittent, whereas in three patients no response has been observed. These results show that the ketogenic diet did not appear to influence the neuropsychological outcome; however, the absence of a control group makes it impossible to conclude with certainty.

[Valproate treatment during pregnancy: description of four cases with foetal valproate syndrome]. / Behandling med valproat under graviditet: beskrivelse af fire cases med føtalt valproatsyndrom.

INTRODUCTION: Treatment with valproate is associated with an increased risk of teratogenicity compared to other antiepileptic drugs and can cause a complex of serious symptoms usually referred to as "foetal valproate symdrome" which is characterised by major and minor malformations in association with developmental delay. This paper aims to give attention to the syndrome through four case descriptions. Furthermore, possible risk factors and the use of the mutation 677C-T as a risk marker are discussed. MATERIAL AND METHODS: Nine developmentally retarded children from a parent group, born of mothers who were treated with valproate during pregnancy, were neuropediatrically and neuropsychologically examined in a non-acute setting. The mothers were screened for the 677C-T mutation. RESULTS: Four of seven examined children fulfilled the criteria for foetal valproate syndrome. Only one of the four mothers was heterozygote for the 677C-T mutation (CT, n = 1/4) and none of the mothers were homozygote (TT, n = 0/4) CONCLUSION: The foetal valproate syndrome is a complex of symptoms which is probably underdiagnosed and should be considered in the diagnostic evaluation program for children with developmental delay who are born of mothers with epilepsy. The 677C-T mutation does not seem to be a useful genetic marker of this syndrome.

[Comorbidity in children with epilepsy. I: Behaviour problems, ADHD and intelligence]. / Komorbiditet hos børn med epilepsi. I: Adfaerdsforstyrrelse, attention deficit and hyperkinetic disorder og intelligens.

A search of the literature of studies on comorbidity in children with idiopathic and cryptogenic epilepsy revealed very few prospective longitudinal studies. The tendency was that children with epilepsy had a greater prevalence of comorbidity than healthy children, but more prospective longitudinal studies are needed to show the exact prevalence, grade and character. Behaviour problems seem to occur before the children have seizures, i.e. the behaviour problem seems to exist before the onset of epileptic seizures. There was no sound evidence for the prevalence of comorbidity in the form of ADHD. Children with epilepsy seem to have normal IQ.

[Comorbidity in children with epilepsy. II: Aetiology]. / Komorbiditet hos børn med epilepsi. II: Aetiologi.

A search of the literature of studies on comorbidity in children with idiopathic and cryptogenic epilepsy and its aetiology revealed few prospective longitudinal studies of evidence class II. There were too few studies on aetiology to determine the causes with certainty, but psychosocial factors seem vital, thus emphasizing the importance of information and support to the child and its family, including siblings. The cognitive and behavioural effect of antiepileptic medicine (other than phenobarbital) seems modest when AED is used monotherapeutically. Likewise, there only seems to be a modest connection between comorbidity and epilepsy-related variables such as age of onset, seizure types, EEG-changes and control of seizures.

[Epilepsy surgery of Danish children 1996-2002]. / Epilepsikirurgisk behandling af danske børn 1996-2002.

INTRODUCTION: Epilepsy surgery is done in order to obtain seizure freedom or to reduce the number of epileptic seizures in selected patients not responding to medical treatment. Few Danish children have had this treatment, most probably because of some restrictions imposed by The Danish National Health Board. We present the results of the first 22 Danish children who have had epilepsy surgery. MATERIAL AND METHODS: All children up to the age of 15 operated from January 1st 1996 to May 1st 2002 are included. According to the guidelines of The Danish National Health Board, 17 children have been operated abroad because the epileptic focus was extratemporal or intracranial EEG-monitoring was necessary. Follow-up varied from five years and six months to one month. RESULTS: At the time of the operation, all children had a long-lasting medical intractable epilepsy. 18 children had daily seizures and 15 were mentally retarded. Multi-lobar resection was performed in ten, hemispherectomy in three and lobar resection in eight children. Total seizure-free outcome was seen in 12 children, whereas three only had simple partial seizures (totally 68 per cent). The remaining children had at least a 50 per cent seizure reduction. On an average, the cognitive function was unchanged one year after the surgery. The majority of the parents stated that the behaviour of the child had improved on one or more specific issues. One mother stated that to a certain degree she regretted the operation. In four children, an unexpected complication was registered (slight hemiparesis and a hemianopsia). DISCUSSION: The results documented that epilepsy surgery is effective for selected children in spite of long-lasting severe epilepsy. In order to alleviate the consequences of severe epilepsy it is, however, important to refer the patients early. There seems to be a need for more and early referrals for childhood epilepsy surgery in Denmark.