[Benign familial neonatal seizures].
Benign familial neonatal seizures are a rare cause of newborn seizures which can be easily detected by the family history because of their autosomal dominant inheritance. Based on 20 patients documented in the literature and one patient admitted to our clinic, the clinical picture can be characterized as follows: Only newborns at term are affected. Seizure types include multifocal-clonic, focal-clonic and subtle, beginning mostly on the 3rd day after birth (range: 1-8 day), the frequency can reach 40 per day. Interictally the patients are neurologically normal. As a rule, clinical, laboratory and neuroradiological investigations show no relevant deviations. The seizures usually disappear within 8 months. Nearly all children were treated with phenobarbital. After discontinuation of therapy, the seizures relapsed transiently in a few patients. Referring to about 140 patients described in the literature, the following conclusions can be drawn with regard to prognosis: Generally, mental development is normal, however, 4% of the patients later showed mental retardation. About 10% of the children developed epilepsy during childhood or adulthood. Two children died in the neonatal period. The fifth day fits must be separated from benign familial neonatal seizures, these are not genetically determined.
Siemes
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Rittberg-Kinderklinik, Berlin.
D002493:Central Nervous System Diseases / Q000175:diagnosis; D002869:Chromosome Aberrations; D025063:Chromosome Disorders; D003937:Diagnosis, Differential; D004827:Epilepsy / Q000209:etiology; D005799:Genes, Dominant; D006801:Humans; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000209:etiology; D012640:Seizures / Q000150:complications / Q000175:diagnosis / Q000235:genetics*
Benigne familiäre neonatale Krampfanfälle.