SUMMARY The object of the study presented here was to obtain information about the genetics of absence epilepsies from the family histories of the patients and EEG examination of their siblings. There were 239 patients with absence epilepsy, and EEGs were recorded of 242 siblings of 109 patients, and of 685 control children. In 30% of the families, at least one relative had a history of seizures. The highest incidence, 7 % and 6 %, was in siblings and parents. The incidence of seizures was higher in the families of female than of male probands. The EEGs of 242 siblings showed theta rhythms, spike‐wave patterns or photosensitivity in 28 %. Of the siblings 1–8 yr old, 19 % had theta rhythms with maximum incidence, 27 %, in children 3–4 yr old. This was significantly more frequent than in control children. Spike‐waves were elicited by photic stimulation in 12% of the siblings, the maximum in children 11–12 yr old, 27%. Spike‐waves at rest and during hyperventilation were found in up to 20% of the siblings, depending on age. The maximum incidence in controls did not exceed 3 %. Occipital delta rhythms showed a different age distribution in siblings and controls with a maximum at 9–10 yr in siblings and at 3–4 yr in controls. The overall incidence of occipital delta rhythms was about the same in siblings and controls. The relations of the various EEG traits with each other were analyzed. Theta rhythms, spike‐waves evoked by photic stimulation, and spike‐waves at rest and during hyperventilation occurred in the siblings independently of each other. Occipital delta activity and photosensitivity were inversely related in that siblings of patients with delta rhythms were less often photosensitive than siblings of patients without delta activity. In accordance with former studies occipital delta rhythms are interpreted as a symptom of a partially genetically determined inhibitory cerebral function. The relations of EEG findings in siblings and clinical data of the probands were examined. The only positive relation was that the siblings of patients with myoclonicastatic seizures had a statistically significant higher incidence of photosensitivity. Taken as a whole, the results suggest that epilepsies with spike‐wave absences are not inherited by an autosomal dominant gene, but that several genetic factors are responsible, some mutually independent and some either reinforcing or inhibiting the others. RÉSUMÉ Le but de cette étude était ďobtenir des informations sur la génétique des absences épileptiques, à partir des histoires familiales des patients et de ľEEG de leurs frères et soeurs. II y avait 239 patients avec des absences épileptiques et ľon a pratiqué un EEG chez 242 frères et soeurs de 109 patients. Dans 30% des families, un des membres de celle‐ci présentait une histoire de crises. Le pourcentage le plus élevé, 7% et 6%, se trouve chez les frères et soeurs et leurs parents. Ľincidence ďune histoire familiale positive était plus élevée pour les propositus de sexe féminin que pour ceux de sexe masculin. Les EEG des 2...
SUMMARY A clinical trial with dipropylacetate (DPA), a new anti‐epileptic drug (Dépakine®, Ergenyl®) is reported in 116 patients. Primarily generalized epilepsies ‐ petit mal of different types and centrencephalic grand mal ‐ were mainly benefited. Less favourable results were obtained in epilepsy of focal origin. The side effects were usually minimal, consisting of gastric disturbances. Dipropylacetate increased the effect of other anti‐epileptics, especially barbiturates, primidone and benzodiazepines. Some patients were more alert and their behaviour improved on DPA. RÉSUMÉ On rapporte ľétude clinique de ľeffet du dipropylacétate (DPA) (Dépakine®, Ergenyl®), une nouvelle drogue anti‐épileptique chez 116 sujets. Ce fut surtout bénéfique dans les épilepsies généralisées primaires‐petit mal de différents types et grand mal centrencéphalique. On obtient de moins bons résultats dans ľépilepsie ďorigine focale. Les effets secondaires étaient généralement minimes, consistant en des troubles gastriques. Le dipropylacétate augmentait ľeffet des autres drogues anti‐épileptiques, particulièrement des barbituriques, du primidone et des benzodiazépines. Quelques patients avaient une meilleure conduite et étaient plus réveillés avec le DPA. ZUSAMMENFASSUNG Das Antikonvulsivum Dipropylacetat (Dépakine®, Ergenyl®) wurde bei 116 Patienten erprobt. Die bevorzugte Indikation sind primär generalisierte Epilepsien (Petit mal verschiedenen Typs und sogenanntes zentrenzephales Grand mal). Bei Epilepsien fokaler Genese sind die Erfolge weniger gut. Myoklonische Syndrome bei degenera‐tiven Hirnerkrankungen können günstig beeinflusst werden. Die Nebenwirkungen des Präparates sind insgesamt gering. Dipropylacetat verstarkt die Wirkung anderer Antiepileptica, insbesondere der Barbiturate, des Primidone und der Benzodiazepine. In machen Fällen entfaltet das Präparat eine günstige Wirkung auf das psychische Verhalten der Patienten. RESUMEN Estudio clinico en 116 pacientes con dipropilacetato (DPA), nueva droga antiepiléptica (Depakine®, Ergenyl®). Se han benificiado principalmente de los efectos de esta medicateón las epilepsias generalizadas primarias (diferentes formas de petit mal y gran mal centroencefálico). Se han obtenido resultados menos favorables en la epilepsia de origin focal. La intolerancia ha sido generalmente mínima consistiendo en transtornos gastricos. El dipropilacetato aumenta el efecto de otros antiepilépticos especialmente de los barbitúricos, la Primidona y las benzodiacepinaz. En algunos pacientes ha mejorado su conducta y su vigilancia con DPA.
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