Abstract
The patients are two brothers aged 12 and 14 years respectively and their 8 years old half-sister. All three have the same father who is debil and whose family seems to be stigmatized by epilepsy and disorders of the nervous system.
All three patients show almost identical phenomena of typical seizure-activity in the EEG occurring upon closing of the eyes. The trigger-mechanism does not seem to be connected with photosensibility, because the seizure-activity occurs regardless of whether the patients are being examined in the light or in the darkness. The phenomenon occurs on voluntary as well as on involuntary closing of the eye-lids and also if the voluntary closing is forcefully prevented with the fingers of the examiner.
This seizure-activity consists of a generalized poly-spike-wave and spike-wave pattern with a frequency of 3,5/sec. and a duration for several seconds. The pattern is more rhytmic at the beginning but becomes irregular later on. The trigger-mechanism shows a tendency to exhaustion. Opening of the eyes discontinues the seizure-activity, but once initiated it persists for about 3—4 seconds even if it was triggered by a very short eye-closing.
At the same time and synchronously with the EEG-pattern one can observe a symmetric myoclonic twitching of the eye-lids and a deviation of the eye-balls upwards like Bell's phenomenon. In addition to the seizure-activity triggered by the closing of the eyes, there are in all three patients singular and occasional poly-spike-wave and spike-wave activities in duration of less then 1 second, which occur spontaneously also during the sleep.
The EEG with flash-stimulation shows photosensibility in one patient, while in the other two patients no photosensibility could be ascertained.
All three patients are mentally retarded. Laboratory and X-ray examinations in all three patients show normal findings.
The author surveys the literature and concludes that the trigger-mechanism responsible for this phenomen must be a disturbance in the structures of the brainstem.
To the author's knowledge this is the first observation of familiar occurrance of this phenomenon.
Zusammenfassung
Es werden 3 Fälle berichtet mit Anfallsaktivität bei Augenschluß. Es handelt sich um 2 Brüder im Alter von 12 und 14 Jahren und deren 8jährige Halbschwester.
Die Anfallsaktivität tritt sowohl in Helligkeit wie bei Dunkelheit auf und ist deutlich abhängig von der motorischen Aktivierung des Musculus orbicularis oculi. Synchron mit den EEG-Veränderungen werden Lidmyoclonien beobachtet sowie eine Aufwärtsbewegung der Bulbi im Sinne des BELLSchen Phänomens.
Keyword
Epilepsy - reflex epilepsy - seizure-activity
