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Ketogenic diet in the treatment of refractory epilepsy
S44 one, full regress model- backward elimination step, final model and stability validation. ME, MSE, SME, WRES and their 95% CI were induced to evaluate the accuracy and precision. Individual parameters were calculated using 1−2 concentrations by Bayesian estimation, levels predicted to conduct individualized therapy. Results: 229 concentrations were collected at different times after dosing during steady-state. Data from 168 patients with normal renal/hepatic functions were analysed. One-compartment model with first-order absorption and elimination best described the data. CBZ final PPK: • Cl/F (l/kg/h) = 0.103 × (0.676^(Wt/28.62)) × EXP(ETACl), • V/F (l/kg) = 2.68 × (1.8^(Dose/16.66)) × EXP(ETAV). CBZE final PPK: • Cl/F (l/kg/h) = 0.369 × (1 − 0.281×VPA) × (0.855^(Wt/28.62)) × EXP(ETACl), • V/F (l/kg) = 11.7 × (1.46^(Dose/16.66)) × EXP(ETAV). Prediction of CBZ and CBZE concentrations in 9 new patients were satisfactory for clinical application, based on the comparison of PRED, IPRE and observed values. Conclusions: PPK models of CBZ and CBZE for Chinese children were successfully established. Individual PK parameters can be rapidly obtained. This new way is very useful to optimize CBZ therapy for clinical sparse data in children.
066EpiTP Ketogenic diet in the treatment of refractory epilepsy 1 M. Koln´ıkova´ 1 , P. Sykora , V. Klcov ˇ a´ 2 , V. Bzduch ´ ´ 2, 2 3 1 a´ . Clinic of Paediatric Neurology, K. Fabr´ıciova´ , D. Behulov ´ Children’s Teaching Hospital, Bratislava, Slovakia, 2 Paediatric Clinic, Children’s Teaching Hospital, Bratislava, Slovakia, 3 Department of Clinical Biochemistry Paediatric Theaching Hospital, Bratislava, Slovakia
The ketogenic diet is accepted as a potent antiepileptic treatment method for intractable epilepsy. We report our results with the ketogenic diet (KD) in the treatment of children with refractory epilepsy. We treated 11 patients (10 boys and 1 girl) with the KD from April 2004 to December 2007. All patients suffered refractory epilepsy and were treated with two and more antiepileptic drugs. Mean age was 10.9 (the youngest patient 4 months, the oldest 27 years). Ten patients started the treatment with classic KD with lipid to nonlipid ratio of 4:1. One patient received diet with ratio 3:1. In time one patient changed the ratio 4.4:1 and in other patients ratio was reduced. Types of seizures were: tonic clonic, tonic, myoclonic, myoclonic astatic, complex partial evolving to secondarily generalized seizures, complex partial seizures and atypical absence. In cohort is one patient with GLUT 1 deficiency, one patient with methylenetetrahydrofolate reductase (MTHFR) deficiency, four with congenital malformations, two with Lennox Gastaut syndrome and three with cerebral palsy. Summary: Seven of the 11 children are on the diet more than 12 months. Three ended diet up to three months for the bad interactivity. One patient was lost from the evidence. One patient with leukodystrophy died. Two patients were seizure free, two had a 50−90% reduction of seizures and two had a more than 90% reduction of seizures. Complications in our cohort were: dehydration, gastrointestinal disturbances (constipations), hypertriglyceridemia, hypercholesterolemia, hyperuricemia, infectious diseases, asymptomatic hypoglycemia, metabolic acidosis, renal stone, weight loss and lethargy. The ketogenic diet can be used as a therapeutic alternative in patients with refractory epilepsy.
Posters, Epilepsy Treatment (EpiT) 067EpiTP Case report: Resistant epilepsy with severe dystony treated with AEDs and ketogenic diet (KD) in a 4-years-old girl with PDH deficiency T. Bregant, N. Zupancic. Department of Pediatric Neurology, Pediatrics clinics Ljubljana, Slovenia Background: We present a child with PDH deficiency on strict KD who has resistant epilepsy and a year-long episode of severe dystony. Movement disorder is questioned since dystonia, oculogyric crisis and autonomic symptoms are observed. Case report: We present a 4-years-old girl with PDH deficiency, diagnosed upon the muscle biopsy. At the age of 2 months she had right-sided clonic seizures. She was put on AEDs. Her developmental milestones were delayedDQ 85. KD was introduced at 1 year. She became seizurefree, development was observed. She started to walk at 2 years of age, she never spoke. The seizures were controlled until the age of 3, when she started to fall because of the dystony in her L leg. EEG showed slower rhythm without hypersinhronities. She started to have up to 60 dystonic and tonic seizures per day. The contact was lost. She received several doses of Fosphenytoin. KD was continued. Now, there is no contact, she is not ambulant, both hands are dystonic. Oculogyric crisis are observed. When awake, the seizures start with a cry, dystonic legs, eyes turned up and right, stridor with hyperventilation, usually ending with right sided TCS. When asleep, she holds the breath, and then has symmetrically brief clonic, ending with tonic, seizures. She has episodes of sweating and shivering. Neurodegenerative diseases screening showed lower Arylsulphatase A and b-Galactosidase activities and higher chitotriosidase activity. MRI showed unspecific, patchy hyperintensive lesion in deep white matter with normal basal ganglia and thalami. Conclusion: Unusual presentation of PDH deficiency raised suspicion of movement disorder. The dystony and seizures were attributed to the combination of AEDs and KD with the underlying disease. The effectiveness of ketogenic diet in our patient was questioned. 068EpiTP Valproate associated coagulopathies: factor VII deficiency may also be seen ¨ E. Arhan, B. Unal, A. Guven, G. Kose. Dı¸skapı Children’s ¨ ¨ Hospital, Turkey Valproic acid (VPA) is established an effective antiepileptic. VPA related hemostatic side effects and coagulopathies are most of the time disregarded. The objective of our study is to prospectively evaluate the influence of VPA on bleeding and coagulation system. Material and Method: The study group comprised 24 patients with epilepsy, aged 3.5 years to 14 years treated in the Pediatric Neurology of the Dı¸skapı Children’s Hospital. Valproate was started at a dose of 10 mg/kg/day in two doses and one week later the dose was raised to 20 mg/kg/day in two doses. Platelet count, fibrinogen level, prothrombin time, activated partial thromboplastin time, protein C, protein S, antithrombin-III, factor VII, factor VIII, von Willebrand factor antigen, ristocetin cofactor, homocystein, lipoprotein a levels, liver enzymes, total protein, albumin, fat metabolism test (high density lipoprotein [HDL], low density protein [LDL], cholesterol, tryglyceride) were recorded before the therapy and at 7, 30, 120 days after VPA initiation. Results: During VPA treatment, a significant reduction in platelet count was observed. At the fourth month of the therapy, there was a significant reduction in factor VII levels. No significant difference was seen in vWF concentrations and Ristocetin co-factor. Protein C concentrations showed a
066EpiTP Ketogenic diet in the treatment of refractory epilepsy 1 M. Koln´ıkova´ 1 , P. Sykora , V. Klcov ˇ a´ 2 , V. Bzduch ´ ´ 2, 2 3 1 a´ . Clinic of Paediatric Neurology, K. Fabr´ıciova´ , D. Behulov ´ Children’s Teaching Hospital, Bratislava, Slovakia, 2 Paediatric Clinic, Children’s Teaching Hospital, Bratislava, Slovakia, 3 Department of Clinical Biochemistry Paediatric Theaching Hospital, Bratislava, Slovakia
The ketogenic diet is accepted as a potent antiepileptic treatment method for intractable epilepsy. We report our results with the ketogenic diet (KD) in the treatment of children with refractory epilepsy. We treated 11 patients (10 boys and 1 girl) with the KD from April 2004 to December 2007. All patients suffered refractory epilepsy and were treated with two and more antiepileptic drugs. Mean age was 10.9 (the youngest patient 4 months, the oldest 27 years). Ten patients started the treatment with classic KD with lipid to nonlipid ratio of 4:1. One patient received diet with ratio 3:1. In time one patient changed the ratio 4.4:1 and in other patients ratio was reduced. Types of seizures were: tonic clonic, tonic, myoclonic, myoclonic astatic, complex partial evolving to secondarily generalized seizures, complex partial seizures and atypical absence. In cohort is one patient with GLUT 1 deficiency, one patient with methylenetetrahydrofolate reductase (MTHFR) deficiency, four with congenital malformations, two with Lennox Gastaut syndrome and three with cerebral palsy. Summary: Seven of the 11 children are on the diet more than 12 months. Three ended diet up to three months for the bad interactivity. One patient was lost from the evidence. One patient with leukodystrophy died. Two patients were seizure free, two had a 50−90% reduction of seizures and two had a more than 90% reduction of seizures. Complications in our cohort were: dehydration, gastrointestinal disturbances (constipations), hypertriglyceridemia, hypercholesterolemia, hyperuricemia, infectious diseases, asymptomatic hypoglycemia, metabolic acidosis, renal stone, weight loss and lethargy. The ketogenic diet can be used as a therapeutic alternative in patients with refractory epilepsy.
Posters, Epilepsy Treatment (EpiT) 067EpiTP Case report: Resistant epilepsy with severe dystony treated with AEDs and ketogenic diet (KD) in a 4-years-old girl with PDH deficiency T. Bregant, N. Zupancic. Department of Pediatric Neurology, Pediatrics clinics Ljubljana, Slovenia Background: We present a child with PDH deficiency on strict KD who has resistant epilepsy and a year-long episode of severe dystony. Movement disorder is questioned since dystonia, oculogyric crisis and autonomic symptoms are observed. Case report: We present a 4-years-old girl with PDH deficiency, diagnosed upon the muscle biopsy. At the age of 2 months she had right-sided clonic seizures. She was put on AEDs. Her developmental milestones were delayedDQ 85. KD was introduced at 1 year. She became seizurefree, development was observed. She started to walk at 2 years of age, she never spoke. The seizures were controlled until the age of 3, when she started to fall because of the dystony in her L leg. EEG showed slower rhythm without hypersinhronities. She started to have up to 60 dystonic and tonic seizures per day. The contact was lost. She received several doses of Fosphenytoin. KD was continued. Now, there is no contact, she is not ambulant, both hands are dystonic. Oculogyric crisis are observed. When awake, the seizures start with a cry, dystonic legs, eyes turned up and right, stridor with hyperventilation, usually ending with right sided TCS. When asleep, she holds the breath, and then has symmetrically brief clonic, ending with tonic, seizures. She has episodes of sweating and shivering. Neurodegenerative diseases screening showed lower Arylsulphatase A and b-Galactosidase activities and higher chitotriosidase activity. MRI showed unspecific, patchy hyperintensive lesion in deep white matter with normal basal ganglia and thalami. Conclusion: Unusual presentation of PDH deficiency raised suspicion of movement disorder. The dystony and seizures were attributed to the combination of AEDs and KD with the underlying disease. The effectiveness of ketogenic diet in our patient was questioned. 068EpiTP Valproate associated coagulopathies: factor VII deficiency may also be seen ¨ E. Arhan, B. Unal, A. Guven, G. Kose. Dı¸skapı Children’s ¨ ¨ Hospital, Turkey Valproic acid (VPA) is established an effective antiepileptic. VPA related hemostatic side effects and coagulopathies are most of the time disregarded. The objective of our study is to prospectively evaluate the influence of VPA on bleeding and coagulation system. Material and Method: The study group comprised 24 patients with epilepsy, aged 3.5 years to 14 years treated in the Pediatric Neurology of the Dı¸skapı Children’s Hospital. Valproate was started at a dose of 10 mg/kg/day in two doses and one week later the dose was raised to 20 mg/kg/day in two doses. Platelet count, fibrinogen level, prothrombin time, activated partial thromboplastin time, protein C, protein S, antithrombin-III, factor VII, factor VIII, von Willebrand factor antigen, ristocetin cofactor, homocystein, lipoprotein a levels, liver enzymes, total protein, albumin, fat metabolism test (high density lipoprotein [HDL], low density protein [LDL], cholesterol, tryglyceride) were recorded before the therapy and at 7, 30, 120 days after VPA initiation. Results: During VPA treatment, a significant reduction in platelet count was observed. At the fourth month of the therapy, there was a significant reduction in factor VII levels. No significant difference was seen in vWF concentrations and Ristocetin co-factor. Protein C concentrations showed a