title: [Metabolic disorders in epilepsy of early childhood].
abstract: Metabolic disorders are discussed which are associated with the pathophysiological mechanisms of the origin of convulsions. Homeostasis impairment, e. g. hyponatremia, hypo- and hyperkalemia, hypocalcemia is mentioned, as well as vitamin deficiencies, such as pyridoxin deficiency, and the problem of phenylketonuria is discussed in connection with aminoacid disorders. Possible connections between aminoacid disorders and BNS, occurring in 8.1% of 1,688 children treated for epilepsy at the neurological department of the Brno Faculty Children's Hospital, are further discussed. Results of screening for amino-aciduria (according to Berry's method) were negative in 3000 healthy infants, whereas careful investigation resulted in pathologic aminoaciduria in 17 out of 20 children with BNS. Also results of hormonal treatment in children with this sort of convulsions are reported. It is concluded that early therapy, even though incapable of influencing neurological abnormities, suppresses convulsions and may lead to the disappearance of hypsarythmia from the EEG curve. A benign influence upon mental development was observed in a small group of children in whom therapy had been initiated very early. It is emphasized that this, by no means indifferent, type of therapy should only be performed in a well equipped and managed pediatric department.
mesh_terms: D000592:Amino Acid Metabolism, Inborn Errors / Q000150:complications; D000596:Amino Acids / Q000652:urine; D001361:Avitaminosis / Q000150:complications; D001786:Blood Glucose / Q000378:metabolism; D001928:Brain Diseases, Metabolic / Q000209:etiology*; D002675:Child, Preschool; D004569:Electroencephalography; D004827:Epilepsy / Q000209:etiology*; D006801:Humans; D007223:Infant; D008607:Intellectual Disability / Q000209:etiology; D010661:Phenylketonurias / Q000150:complications; D014883:Water-Electrolyte Imbalance / Q000150:complications
chemical_list: D000596:Amino Acids; D001786:Blood Glucose
vernacular_title: Metabolische Störungen bei der Epilepsie des frühen Kindesalters.