Our clinical experience with zonisamide in resistant generalized epilepsy syndromes.

Purpose: Zonisamide is licensed in the European Union for adjunctive therapy for partial epilepsy, but its efficacy in generalized epilepsy was less explored.

Methods: This prospective observational study included 47 patients (mean age 29 years, range 3-50) with different resistant generalized epilepsy syndromes: idiopathic generalized syndromes (IGE) 15 patients, (juvenile myoclonic epilepsy four, absence epilepsy four, myoclonic absence two, unclassified IGE five), progressive myoclonic epilepsy type 1 (PME1) four, severe myoclonic epilepsy of infancy (SMEI) three, borderline SMEI three, Lennox-Gastaut syndrome/secondary generalized epileptic encephalopties 23 patients. All patients were followed up for at least six months.

[Surgical treatment of resistant epilepsy, caused by hemispherical dysgenesis--case report].

A part of patients with the therapy resistant epilepsy can be cured by surgical interventions. The more concordant the presurgical evaluation data, the better prognosis the patient has postoperatively. In case of discordant examination data, multimodal evaluation or case-specific decision might be successful.

[Clinical and genetic diagnosis of Dravet syndrome: report of 20 cases].

Objective And Background: Severe myoclonic epilepsy in infancy (SMEI; Dravet's syndrome) is a malignant epilepsy syndrome characterized by prolonged febrile hemiconvulsions or generalized seizures starting in the first year of life. Later on myoclonic, atypical absence, and complex partial seizures appear. When one of these seizure forms is lacking the syndrome of borderline SMEI (SMEB) is defined.

[Devastating epileptic encephalopathy-pseudoencephalitis: the new type of catastrophe epilepsy in our department].

Purpose: Analysis of history of our five patients with intractable epilepsy whose illness have begun with prolonged status epilepticus (SE) and high-grade fever of unknown cause.

Methods: Retrospective study analysis of selected five intractable epileptic patients at a median age of 11.5 (8-14) years.

[Congenital cataracts facial dysmorphism neuropathy syndrome--first Hungarian case report].

The congenital cataracts facial dysmorphism neuropathy (CCFDN) syndrome (OMIM 604168) is a recently described autosomal recessive developmental disorder. It is almost completely restricted to an endogamous group of the European Vlax Roma population, called the Rudari. The CCFDN syndrome is a complex phenotype involving multiple systems, characterized by facial dysmorphism, congenital cataracts, microcorneae, delayed early motor and intellectual development, hypogonadotrop hypogonadism, hypomyelination of the peripheral nervous system, and serious complications related to general anaesthesia.

[Experience with levetiracetam in childhood epilepsy].

Objective: To evaluate the efficacy and tolerability of levetiracetam in children with drug resistant epilepsy from a retrospective study.

Methods: We report the result of a study of 85 pediatric patients (mean 10.5 years, range: 1-24) with refractory generalized and focal epilepsy, who received levetiracetam as add-on treatment.

Hippocampal sclerosis in severe myoclonic epilepsy in infancy: a retrospective MRI study.

Purpose: Severe myoclonic epilepsy in infancy (SMEI; Dravet's syndrome) is a malignant epilepsy syndrome characterized by early prolonged febrile convulsions (PFCs) with secondary psychomotor delay and a variety of therapy-resistant seizures. Although the initial symptoms are repeated PFCs, the MRI performed at the onset of disease shows no hippocampal structural abnormalities. We aimed to assess clinical and serial MRI data of patients with SMEI with a special attention to the temporomedial structures.

[Epilepsy surgery in childhood: theory and practice].

1-1.5% of the Hungarian child population has epilepsy, and around 20% of them produces seizures in spite of modern antiepileptic drugs. A part of the pharmaco-resistant children may benefit from surgical removal of the epileptogenic focus.

Comparative evaluation of concomitant structural and functional neuroimages in Rasmussen's encephalitis.

Background And Purpose: Rasmussen's encephalitis (RE) is a rare condition of unknown cause characterized by intractable seizures, progressive hemiparesis, mental impairment, and inflammatory histological findings in the cortex. The primary diagnosis is based on biopsy to confirm the typical clinical, electroencephalography, and brain imaging findings. The main objective of this study was to compare simultaneous structural and functional neuroimages in RE.

[The role of PET scan in the investigation of epileptic functional disorders].

Systematic research is needed to evaluate the exact role of pathological factors in the determination of the extension of the hypometabolic area in partial epileptic patients. Together with structural damage, previous seizures, deafferentation and inhibitory mechanisms may contribute to the functional disorders. Benzodiazepine receptor studies showed that the density and binding ability of these receptors decreased in the area of epileptic functional disorder.