The impact of a ketogenic diet and liver dysfunction on serum very long-chain fatty acids levels.

Peroxisomes play an essential role in mammalian cellular metabolism, particularly in oxidation fatty acid pathways. Serum very long-chain fatty acids (VLCFA), the main biochemical diagnostic parameters for peroxisomal disorders, were examined in 25 neurological patients with epilepsy on a ketogenic diet and 27 patients with liver dysfunction. The data show that patients on a ketogenic diet have increased levels of C22:0 and C24:0, but not C26:0, and normal C24:0/C22:0 and C26:0/C22:0. Patients with liver insufficiency showed a slightly elevated level of C26:0, a normal level of C24:0 and a decreased level of C22:0; thus in 21/27 the ratio of C24:0/C22:0 was increased and 15/27 the ratio of C26:0/C22:0 was increased.

[Gabitril as an additive drug in therapy of intractable epileptic seizures in children]. / Gabitril w terapii dodanej w opornych napadach czesciowych zlozonych i wtórnie uogólnionych u dzieci. Doniesienie wstepne.

Tiagabine (Gabitril, Sanofi Synlhelabo) new antiepileptic drug was used in add-on therapy in 25 children with resistant partial complex and secondary generalized seizures. Treatment was carried out in children aged 4-17 years with low dose escalation from 5 to 45 mg/day, in three doses until good clinical effects were obtained. In 3 patients aged 4 years, in 11 children aged 5-12 years and in 11 children aged above 17 years Gabitril was used. Follow up period was 8-10 months. Frequency of epileptic seizures before implementation of Gabitril treatment, even during polytherapy with 2 or more antiepileptic drugs was several to hundred per day (status epilepticus was observed in 2 children with Rasmussen syndrome). During the observation 5 children became seizure free, in 11 patients reduction in seizures frequency above 50% was observed and in 9 children effects of treatment were not good enough. Gabitril was well tolerated, and any adverse events were observed in add-on therapy. Preliminary observation and good results of add-on therapy with Gabitril are positive. Drug is safe and generally well-tolerated with good effects at add-on therapy in 64% children with resistant partial complex and secondary generalized seizures.

[Rasmussen syndrome--indications for surgical treatment of refractory epilepsy: report of two cases]. / Zespól rasmussena--wskazanie do operacyjnego leczenia lekoopornej padaczki. Opis dwóch przypadków.

AIM: Presentation of two cases of children with Rasmussen's encephalitis, treated by functional hemispherectomy. Two boys aged 4 and 6, with a typical clinical course, typical findings in CT and MRI scans and characteristic pathologic changes in brain specimens. In both cases was performed functional hemispherectomy. RESULTS: Immediate cessation of seizures in the immediate postoperative period (Engel class I and II). Later, in a 4-months' follow-up period, a worthwhile improvement in psychomotor development and social functioning was noted. Up-to date opinions published in available literature, related to pathogenesis and treatment modalities of Rasmussen's encephalitis are presented. CONCLUSIONS: 1) Rasmussen's encephalitis is a definite nosologic entity, leading to drug-resistant epilepsy and a progressive psychomotor deterioration; 2) functional hemispherectomy is a viable alternative, which should be considered in the treatment of Rasmussen's encephalitis; 3) Functional; hemispherectomy, in spite of it's aggressiveness, is a relatively safe procedure; 4) in the follow-up time of 4 months, the results are promising.

[Callosotomy in the treatment of "catastrophic" drug-resistant epilepsy in children]. / Kallosotomia w leczeniu ciezkich postaci lekoopornej padaczki u dzieci.

UNLABELLED: The aim of this paper was to assess the preliminary results of callosotomy in children with "catastrophic" drug-resistant epilepsy. During the last 5 years, we performed callosotomy in 8 cases. Indications were: "catastrophic" drug-resistant epilepsy with frequent seizures, progressive psychomotor and intellectual deterioration, generalised structural and functional abnormalities in neuroimaging and neurophysiological studies. These children were of the mean age of 6 years, with the mean duration of illness 58 months. In the mean follow-up time of 27 months the following results were obtained: Engel class II--3 cases; class III--4 cases and class IV--1 case. Any intellectual improvement was noted in 2 cases only. CONCLUSIONS: (1) callosotomy effectively reduces the frequency and severity of seizures in children with drug-resistant epilepsy; (2) in spite of reduced seizures, in the majority of cases no significant psychomotor and/or intellectual improvement was achieved; (3) callosotomy did not produce any permanent morbidity.

[The comparison of ketamine with methohexital and thiopental in the intraoperative EEG in drug-resistant epilepsy]. / Porównanie ketaminy z methohexitalem i thiopentalem w aktywacji sródoperacyjnej elektrokortykografii, w lekoopornej padaczce.

UNLABELLED: The acute (ECoG) was examined in 291 patients with intractable epilepsy, without structural brain lesion--from 1971 to 1997. Temporal lobectomy was performed in 198 cases and extratemporal (frontal, parietal or occipital) in remaining 93 cases to achieve seizure control. Epileptic foci was activated during acute ECoG by i.v. administration of ketamine (154 cases) or short-acting barbiturates--methohexital (110 cases) and thiopental (27 cases). RESULTS: Ketamine significantly more often caused ECoG identified electrographic seizures than methohexital: p = 0, 00001 or thiopental, which in no cases resulted in seizures. Also electrographic seizures occurred more frequently after administration of ketamine in patients with the extratemporal seizure focus localisation in comparison with temporal focus (p < 0.05). Electrographic seizures provoked after administration of ketamine improved the localisation of the area to be resected, more often in extratemporal epileptic foci. CONCLUSIONS: The results of our investigations indicate that ketamine more effectively activated epileptic focus than short-acting barbiturates.