Clinico‐anatomical Study of a Case of Idiopathic Epilepsy with Impulsive Petit Mal (“Impulsiv‐Petit mal”) and Grand Mal on Awakening (“Aufwach‐Grand mal”)

Abstract

SUMMARY In contrast to the pathology of the so‐called temporal or psychomotor epilepsy which has been thoroughly investigated in recent times, there has been, until now, no corresponding research into the so‐called petit mal or centrencephalic epilepsy. We are therefore making a detailed examination of the clinico‐anatomical connections between a case of idiopathic epilepsy with impulsive petit mal (also called myoclonic petit mal with multi‐spikes‐waves) and grand mal on awakening. The case in question concerns a 28‐year old woman who ever since the age of 16 had suffered from convulsions of varying severity, occurring either singly or several at a time, in her shoulders, neck and arms, without however losing consciousness, frequently following lack of sleep and consumption of alcohol, and mainly after waking in the morning. During the whole course of the disease only 7 grand mal occurred which appeared mainly after waking and were characterized by opisthotonus during the seizure and extreme cyanosis. The cause of death was probably suicide. With reference to our pathological examination the following remarks can be made: 1. No evidence was discovered which could be diagnosed as responsible for causing the disease. 2. In view of the clinically benign course of the disease, the extent of the ictal damage was surprisingly great. We interpreted this as a consequence of the especially serious impulsive grand mal which took the form of paroxysmal herniations. 3. The sequence of ictal damage usually expected in idiopathic epilepsies according to Scholz varied somewhat in our case, in that the Ammon's horn was affected least of all. On the other hand, the most serious changes took place in the cerebellar cortex, the nucleus dentatus and, strangely enough, in the nucleus ruber. The predilection towards ictal damage in mesencephalic and cerebellar structures is completely consistent with the functional structures of the rubro‐neuronal system, which for clinical reasons have been diagnosed already as responsible for this type of petit mal. 4. The discovery of inhibited migration in the cerebellar cortex seemed significant to us in explaining the age dependency of impulsive petit mal, as well as a series of anomalies of constitution and growth characteristic of this manifestation of the disease. The question is raised as to how far disturbances of the development of this kind, which are too minor to be decisive for the aetiology, are an aid to lending profile to the characteristic features of the special clinical picture. RÉSUMÉ Au contraire de ce qui a été fait pour la pathologie de l'épilepsie dite temporale ou psycho‐motrice, qui a été étudiée à fond ces derniers temps, on n'a pas effectué jusqu'à présent de recherches analogues concernant ce qu'on appelle le petit mal ou épilepsie centrencéphalique. Pour cette raison, nous faisons un examen détaillé des rapports clinico‐anatomiques entre un cas d'épilepsie idiopathique avec petit mal impulsif (aussi appelé petit mal myoclonique avec polypointes‐ondes) et le...

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